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Sample records for idiopathic interstitial pneumonia

  1. Pathology of Idiopathic Interstitial Pneumonias

    PubMed Central

    Hashisako, Mikiko; Fukuoka, Junya

    2015-01-01

    The updated classification of idiopathic interstitial pneumonias (IIPs) in 2013 by American Thoracic Society/European Respiratory Society included several important revisions to the categories described in the 2002 classification. In the updated classification, lymphoid interstitial pneumonia (LIP) was moved from major to rare IIPs, pleuroparenchymal fibroelastosis (PPFE) was newly included in the rare IIPs, acute fibrinous and organizing pneumonia (AFOP) and interstitial pneumonias with a bronchiolocentric distribution are recognized as rare histologic patterns, and unclassifiable IIP (UCIP) was classified as an IIP. However, recent reports indicate the areas of concern that may require further evaluation. Here, we describe the histopathologic features of the updated IIPs and their rare histologic patterns and also point out some of the issues to be considered in this context. PMID:26949346

  2. [Histopathological classification of idiopathic interstitial pneumonias].

    PubMed

    Povýsil, Ctibor

    2010-01-01

    The classification scheme of interstitial lung diseases has undergone numerous revisions. The criteria for distinguishing seven distinct subtypes of idiopathic interstitial pneumonias are now well defined by consensus in the recently published ATS/ERS classification of these lung diseases. In our present review the histological patterns of the different types are described and the differential diagnosis of idiopathic interstitial pneumonias is discussed. Surgical lung biopsy remains the gold standard for the diagnosis of interstitial pneumonias, and sampling from at least 2 sites is recommended. Video-assisted thoracoscopic surgical biopsy is the preferred method for obtaining lung tissue as this procedure offers a similar yield as an open thoracotomy The most common histological subtype of chronic interstitial lung disease is the usual interstitial pneumonia [UIP] which makes up 47-71% of cases. The key histologic features include patchy subpleural and paraseptal distribution of remodeling lung architecture with dense fibrosis, frequent honeycombing, and large fibroblastic foci. Temporal and spatial heterogeneity are the hallmarks. Nonspecific interstitial pneumonia [NSIP] occurs primarily in middle-aged women who have never smoked, with more than 5-years survival rate in 80% of patients. The major feature of NSIP is a uniform interstitial thickening of alveolar septa by a fibrosing or cellular process. The cardinal histological feature in respiratory bronchiolitis and desquamative pneumonia is an excess of intraalveolar histiocytes. In both patterns, there is variable interstitial fibrosis and chronic inflammation, and a strong association with a history of smoking. Organizing pneumonia (idiopathic bronchiolitis obliterans-organizing pneumonia [BOOP]) is not strictly an interstitial process, because the alveoli and bronchioles are filled by intraluminal polyps of fibroblastic tissue and the expansion of the interstitium is mild. Lymphocytic interstitial pneumonia

  3. Acute and subacute idiopathic interstitial pneumonias.

    PubMed

    Taniguchi, Hiroyuki; Kondoh, Yasuhiro

    2016-07-01

    Idiopathic interstitial pneumonias (IIPs) may have an acute or subacute presentation, or acute exacerbation may occur in a previously subclinical or unrecognized chronic IIP. Acute or subacute IIPs include acute interstitial pneumonia (AIP), cryptogenic organizing pneumonia (COP), nonspecific interstitial pneumonia (NSIP), acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) and AE-NSIP. Interstitial lung diseases (ILDs) including connective tissue disease (CTD) associated ILD, hypersensitivity pneumonitis, acute eosinophilic pneumonia, drug-induced lung disease and diffuse alveolar haemorrhage need to be differentiated from acute and subacute IIPs. Despite the severe lack of randomized controlled trials for the treatment of acute and subacute IIPs, the mainstream treatment remains corticosteroid therapy. Other potential therapies reported in the literature include corticosteroids and immunosuppression, antibiotics, anticoagulants, neutrophil elastase inhibitor, autoantibody-targeted treatment, antifibrotics and hemoperfusion therapy. With regard to mechanical ventilation, patients in recent studies with acute and subacute IIPs have shown better survival than those in previous studies. Therefore, a careful value-laden decision about the indications for endotracheal intubation should be made for each patient. Noninvasive ventilation may be beneficial to reduce ventilator associated pneumonia. PMID:27123874

  4. Acute and subacute idiopathic interstitial pneumonias.

    PubMed

    Taniguchi, Hiroyuki; Kondoh, Yasuhiro

    2016-07-01

    Idiopathic interstitial pneumonias (IIPs) may have an acute or subacute presentation, or acute exacerbation may occur in a previously subclinical or unrecognized chronic IIP. Acute or subacute IIPs include acute interstitial pneumonia (AIP), cryptogenic organizing pneumonia (COP), nonspecific interstitial pneumonia (NSIP), acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) and AE-NSIP. Interstitial lung diseases (ILDs) including connective tissue disease (CTD) associated ILD, hypersensitivity pneumonitis, acute eosinophilic pneumonia, drug-induced lung disease and diffuse alveolar haemorrhage need to be differentiated from acute and subacute IIPs. Despite the severe lack of randomized controlled trials for the treatment of acute and subacute IIPs, the mainstream treatment remains corticosteroid therapy. Other potential therapies reported in the literature include corticosteroids and immunosuppression, antibiotics, anticoagulants, neutrophil elastase inhibitor, autoantibody-targeted treatment, antifibrotics and hemoperfusion therapy. With regard to mechanical ventilation, patients in recent studies with acute and subacute IIPs have shown better survival than those in previous studies. Therefore, a careful value-laden decision about the indications for endotracheal intubation should be made for each patient. Noninvasive ventilation may be beneficial to reduce ventilator associated pneumonia.

  5. Idiopathic non-specific interstitial pneumonia.

    PubMed

    Belloli, Elizabeth A; Beckford, Rosemarie; Hadley, Ryan; Flaherty, Kevin R

    2016-02-01

    Non-specific interstitial pneumonia (NSIP) is an interstitial lung disease that may be idiopathic or secondary to connective tissue disease, toxins or numerous other causes. Idiopathic NSIP is a rare diagnosis and requires exclusion of these other possible causes. Patients typically present in mid-adulthood with dyspnoea, cough and often constitutional symptoms including fever and fatigue. The disease has a female predominance, and more than 50% of patients have never smoked. Physical exam features mild hypoxaemia and inspiratory rales. Pulmonary function tests demonstrate restriction and a low diffusing capacity for carbon monoxide. High-resolution computed tomography abnormalities include predominantly lower lobe subpleural reticular changes, traction bronchiectasis and ground-glass opacities; honeycombing is rarely seen. An evaluation of the underlying pathology is necessary for a firm diagnosis. Histologically, alveolar and interstitial mononuclear cell inflammation and fibrosis are seen in a temporally uniform pattern with preserved underlying alveolar architecture. NSIP must be differentiated from other parenchymal lung diseases including idiopathic pulmonary fibrosis and hypersensitivity pneumonitis. A thorough exposure history and assessment for underlying connective tissue diseases are highly important, as positive findings in these categories would likely denote a case of secondary NSIP. A multi-disciplinary discussion that includes pulmonologist(s), radiologist(s) and pathologist(s) assists in reaching a consensus diagnosis and improves diagnostic accuracy. Treatment of idiopathic NSIP, although not well proven, is generally instituted in the form of immunosuppression. Prognosis is favourable compared with idiopathic pulmonary fibrosis, although the diagnosis still carries an attributable mortality. Herein we will summarize the clinical characteristics and management of idiopathic NSIP. PMID:26564810

  6. Facts and controversies in the classification of idiopathic interstitial pneumonias.

    PubMed

    Poletti, V; Kitaichi, M

    2000-10-01

    Idiopathic interstitial pneumonias are defined from the pathological point of view as non granulomatous intralobular inflammatory and fibrotic processes involving the alveolar walls. More than thirty years ago Liebow and Carrington pioneered the notion that morphological characteristics could be used with benefit in separating the different entities found in this group, which present with typical, but not pathognomonic clinical features. In the mid-1980s some entities, including giant cell interstitial pneumonia (GIP) and lymphocytic interstitial pneumonia (LIP), were removed from this group and considered as peculiar forms. In the early 90s the concept of cellular or nonspecific interstitial pneumonia was reconsidered, leading to an in depth revision of various types of interstitial pneumonia of unknown etiology. The histological pattern observed in patients with idiopathic pulmonary fibrosis is now referred to as usual interstitial pneumonia (UIP). Other entities that have been revised during the last ten years are desquamative interstitial pneumonia/alveolar macrophage pneumonia (DIP/AMP), respiratory bronchiolitis-interstitial lung disease (RB-ILD), acute interstitial pneumonia (AIP), cryptogenic organizing pneumonia (COP) and nonspecific interstitial pneumonia (NSIP). This paper provides a detailed description of pulmonary disorders which have been included in the new classification systems of idiopathic interstitial pneumonias. In the second part of the paper we will discuss several doubts and controversies that this new classification schemes leave unresolved.

  7. Smoking-related idiopathic interstitial pneumonia.

    PubMed

    Flaherty, Kevin R; Fell, Charlene; Aubry, Marie-Christine; Brown, Kevin; Colby, Thomas; Costabel, Ulrich; Franks, Teri J; Gross, Barry H; Hansell, David M; Kazerooni, Ella; Kim, Dong Soon; King, Talmadge E; Kitachi, Masanori; Lynch, David; Myers, Jeff; Nagai, Sonoko; Nicholson, Andrew G; Poletti, Venerino; Raghu, Ganesh; Selman, Moises; Toews, Galen; Travis, William; Wells, Athol U; Vassallo, Robert; Martinez, Fernando J

    2014-09-01

    Cigarette smoking is a key factor in the development of numerous pulmonary diseases. An international group of clinicians, radiologists and pathologists evaluated patients with previously identified idiopathic interstitial pneumonia (IIP) to determine unique features of cigarette smoking. Phase 1 (derivation group) identified smoking-related features in patients with a history of smoking (n=41). Phase 2 (validation group) determined if these features correctly predicted the smoking status of IIP patients (n=100) to participants blinded to smoking history. Finally, the investigators sought to determine if a new smoking-related interstitial lung disease phenotype could be defined. Phase 1 suggested that preserved forced vital capacity with disproportionately reduced diffusing capacity of the lung for carbon monoxide, and various radiographic and histopathological findings were smoking-related features. In phase 2, the kappa coefficient among clinicians was 0.16 (95% CI 0.11-0.21), among the pathologists 0.36 (95% CI 0.32-0.40) and among the radiologists 0.43 (95% CI 0.35-0.52) for smoking-related features. Eight of the 100 cases were felt to represent a potential smoking-related interstitial lung disease. Smoking-related features of interstitial lung disease were identified in a minority of smokers and were not specific for smoking. This study is limited by its retrospective design, the potential for recall bias in smoking history and lack of information on second-hand smoke exposure. Further research is needed to understand the relationship between smoking and interstitial lung disease. PMID:25063244

  8. Lymphatic fluctuation in the parenchymal remodeling stage of acute interstitial pneumonia, organizing pneumonia, nonspecific interstitial pneumonia and idiopathic pulmonary fibrosis.

    PubMed

    Parra, E R; Araujo, C A L; Lombardi, J G; Ab'Saber, A M; Carvalho, C R R; Kairalla, R A; Capelozzi, V L

    2012-05-01

    Because the superficial lymphatics in the lungs are distributed in the subpleural, interlobular and peribroncovascular interstitium, lymphatic impairment may occur in the lungs of patients with idiopathic interstitial pneumonias (IIPs) and increase their severity. We investigated the distribution of lymphatics in different remodeling stages of IIPs by immunohistochemistry using the D2-40 antibody. Pulmonary tissue was obtained from 69 patients with acute interstitial pneumonia/diffuse alveolar damage (AIP/DAD, N = 24), cryptogenic organizing pneumonia/organizing pneumonia (COP/OP, N = 6), nonspecific interstitial pneumonia (NSIP/NSIP, N = 20), and idiopathic pulmonary fibrosis/usual interstitial pneumonia (IPF/UIP, N = 19). D2-40+ lymphatic in the lesions was quantitatively determined and associated with remodeling stage score. We observed an increase in the D2-40+ percent from DAD (6.66 ± 1.11) to UIP (23.45 ± 5.24, P = 0.008) with the advanced process of remodeling stage of the lesions. Kaplan-Meier survival curves showed a better survival for patients with higher lymphatic D2-40+ expression than 9.3%. Lymphatic impairment occurs in the lungs of IIPs and its severity increases according to remodeling stage. The results suggest that disruption of the superficial lymphatics may impair alveolar clearance, delay organ repair and cause severe disease progress mainly in patients with AIP/DAD. Therefore, lymphatic distribution may serve as a surrogate marker for the identification of patients at greatest risk for death due to IIPs.

  9. Idiopathic interstitial pneumonia following bone marrow transplantation: the relationship with total body irradiation

    SciTech Connect

    Keane, T.J.; Van Dyk, J.; Rider, W.D.

    1981-10-01

    Interstitial pneumonia is a frequent and often fatal complication of allogenic bone marrow transplantation. Thirty to 40 percent of such cases are of unknown etiology and have been labelled as cases of idiopathic interstitial pneumonia. Idiopathic cases are more commonly associated with the use of total body irradiation; their occurrence appears to be independent of immunosupression or graft versus host disease. Evidence is presented from the literature suggesting that the development of idiopathic interstitial pneumonia is related to the absolute absorbed dose of radiation to lung. The similarity of idiopathic pneumonia to radiation pneumonitis seen in a different clinical setting is described.

  10. Acute interstitial pneumonia and acute exacerbations of idiopathic pulmonary fibrosis.

    PubMed

    Swigris, Jeffrey J; Brown, Kevin K

    2006-12-01

    Acute interstitial pneumonia (AIP) and acute exacerbations of idiopathic pulmonary fibrosis (AEIPF) are similar respiratory disorders characterized by the rapid development of progressive dyspnea and cough. Both frequently lead to respiratory failure and death. Pathologically, each is characterized by the presence of a diffuse alveolar damage (DAD) pattern; in AIP, DAD is the sole pattern, whereas in AEIPF DAD is superimposed upon a background usual interstitial pneumonia. They differ in that patients with AEIPF have preexisting idiopathic pulmonary fibrosis, whereas patients with AIP have no predisposing disorders to account for their disease. Because both presentations overlap with multiple other causes of acute lung injury, a comprehensive evaluation is necessary to rule out disorders such as overwhelming infection or congestive heart failure. Although a confident diagnosis can be achieved without it, a surgical lung biopsy is necessary to provide a definitive diagnosis. Despite minimal evidence, glucocorticoids are frequently begun once microbiological evaluation confirms the absence of infection. Despite therapy, the case fatality rate ranges up to 70% for both, with most patients dying in the first 2 weeks. Survivors of the acute event can recover to their previous baseline; however, most AIP survivors will stabilize with some functional impairment, whereas in those with AEIPF, progressive fibrosis with functional deterioration is the rule.

  11. Severe pulmonary hypertension in idiopathic nonspecific interstitial pneumonia

    PubMed Central

    Hallowell, Robert W.; Reed, Robert M.; Fraig, Mostafa; Horton, Maureen R.; Girgis, Reda E.

    2012-01-01

    Pulmonary hypertension (PH) is a common complication of interstitial lung disease (ILD), particularly in idiopathic pulmonary fibrosis (IPF) and ILD associated with connective tissue disease, where the underlying pathology is often a nonspecific interstitial pneumonia (NSIP) pattern. The degree of PH in ILD is typically mild to moderate and radiographic changes of ILD are usually prominent. We describe four patients with idiopathic NSIP and severe PH (mPAP > 40 mmHg). The average mean pulmonary artery pressure was 51±7 mmHg and pulmonary vascular resistance was 13±4 Wood's units. Pulmonary function was characterized by mild restriction (total lung capacity 63–94% predicted) and profound reductions in DLCO (19–53% predicted). Computed tomographic imaging revealed minimal to moderate interstitial thickening without honeycombing. In two of the cases, an initial clinical diagnosis of idiopathic pulmonary arterial hypertension was made. Both were treated with intravenous epoprostenol, which was associated with worsening of hypoxemia. All four patients died or underwent lung transplant within 4 years of PH diagnonsis. Lung pathology in all four demonstrated fibrotic NSIP with medial thickening of the small and medium pulmonary arteries, and proliferative intimal lesions that stained negative for endothelial markers (CD31 and CD34) and positive for smooth muscle actin. There were no plexiform lesions. Severe pulmonary hypertension can therefore occur in idiopathic NSIP, even in the absence of advanced radiographic changes. Clinicians should suspect underlying ILD as the basis for PH when DLCO is severely reduced or gas exchange deteriorates with pulmonary vasodilator therapy. PMID:22558525

  12. Idiopathic interstitial pneumonias with connective tissue diseases features: A review.

    PubMed

    Cottin, Vincent

    2016-02-01

    A systematic approach is recommended to search for clinical and biological features of connective tissue disease (CTD) in any patient with interstitial lung disease (ILD). In the diagnostic approach to ILD, a diagnosis of CTD should be considered particularly in women and subjects younger than 50 years, and in those with an imaging and/or pathological pattern of non-specific interstitial pneumonia. However, the diagnosis of CTD may be difficult when ILD is the presenting or the dominant manifestation of CTD. A proportion of patients with ILD present symptoms that belong to the spectrum of CTD and/or biological autoimmune features, but do not fulfil diagnostic criteria for a given CTD. Some imaging and histopathological patterns may also suggest the presence of an underlying CTD. Although studies published to date used heterogeneous definitions and terminology for this condition, evidence is accumulating that even limited CTD features are relevant regarding symptoms, imaging features, pathological pattern and possibly evolution to overt CTD, whereas the impact on prognosis needs confirmation. Conversely, autoantibodies alone do not seem to impact the prognosis or management in patients with otherwise typical idiopathic pulmonary fibrosis and no extra-pulmonary manifestation. A collective international multidisciplinary effort has proposed a uniform definition and criteria for 'interstitial pneumonia with autoimmune features', a condition characterized by limited CTD features occurring in the setting of ILD, with the aim of fostering future clinical studies. Referral of ILD patients suspect to have CTD to a rheumatologist and possibly multidisciplinary discussion may contribute to a better management.

  13. Bosentan in Pulmonary Hypertension Associated with Fibrotic Idiopathic Interstitial Pneumonia

    PubMed Central

    Corte, Tamera J.; Keir, Gregory J.; Dimopoulos, Konstantinos; Howard, Luke; Corris, Paul A.; Parfitt, Lisa; Foley, Claire; Yanez-Lopez, Monica; Babalis, Daphne; Marino, Philip; Maher, Toby M.; Renzoni, Elizabeth A.; Spencer, Lisa; Elliot, Charlie A.; Birring, Surinder S.; O’Reilly, Katherine; Gatzoulis, Michael A.; Wells, Athol U.

    2014-01-01

    Rationale: Pulmonary hypertension (PH) associated with fibrotic idiopathic interstitial pneumonia (IIP; idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia) confers important additional morbidity and mortality. Objectives: To evaluate the safety and clinical efficacy of the dual endothelin-1 receptor antagonist bosentan in this patient group. Methods: In a randomized, double-blind, placebo-controlled study, 60 patients with fibrotic IIP and right heart catheter confirmed PH were randomized 2:1 to bosentan (n = 40) or placebo (n = 20). The primary study endpoint was a fall from baseline pulmonary vascular resistance index (PVRi) of 20% or more over 16 weeks. Measurements and Main Results: Sixty patients (42 men; mean age, 66.6 ± 9.2 yr), with a mean pulmonary artery pressure of 36.0 (± 8.9) mm Hg, PVRi 13.0 (± 6.7) Wood Units/m2 and reduced cardiac index of 2.21 (± 0.5) L/min/m2 were recruited to the study. Accounting for deaths and withdrawals, paired right heart catheter data were available for analysis in 39 patients (bosentan = 25, placebo = 14). No difference in the primary outcome was detected, with seven (28.0%) patients receiving bosentan, and four (28.6%) receiving placebo achieving a reduction in PVRi of greater than or equal to 20% (P = 0.97) at 16 weeks. There was no change in functional capacity or symptoms between the two groups at 16 weeks, nor any difference in rates of serious adverse events or deaths (three deaths in each group). Conclusions: This study shows no difference in invasive pulmonary hemodynamics, functional capacity, or symptoms between the bosentan and placebo groups over 16 weeks. Our data do not support the use of the dual endothelin-1 receptor antagonist, bosentan, in patients with PH and fibrotic IIP. Clinical trial registered with www.clinicaltrials.gov (NCT 00637065). PMID:24937643

  14. Rare idiopathic interstitial pneumonias: LIP and PPFE and rare histologic patterns of interstitial pneumonias: AFOP and BPIP.

    PubMed

    Kokosi, Maria A; Nicholson, Andrew G; Hansell, David M; Wells, Athol U

    2016-05-01

    In the 2013 reclassification of the idiopathic interstitial pneumonias (IIPs), two rare IIPs (idiopathic lymphoid interstitial pneumonia (LIP), idiopathic pleuroparenchymal fibroelastosis (IPPFE)) and two rare histologic patterns (acute fibrinous and organizing pneumonia (AFOP), bronchiolocentric pattern of interstitial pneumonia (BPIP)) are described. All these entities are rare with small series published to date, mostly containing primary and secondary forms of disease. LIP is histologically characterized by diffuse polyclonal lymphoid cell infiltrate surrounding the airways and expanding the interstitium. Thin-walled cysts and diffuse ground glass are considered the typical radiologic features. The clinical course is highly variable with corticosteroid responsiveness evident in approximately half of cases. IPPFE is defined histologically by coexisting upper lobe pleural and intra-alveolar fibrosis with elastosis. Dense subpleural irregular fibrosis and consolidation are the cardinal radiologic features. A history of recurrent lower respiratory tract infection is frequent. Responses to immunomodulation have not been reported and the rate of progression appears to be highly variable. AFOP is a rare histologic pattern lying within the spectrum of acute/subacute lung injury, characterized by organizing pneumonia and intra-alveolar fibrin deposition without hyaline membranes. BPIP is characterized histologically by fibrosis and/or inflammation confined to the alveolar interstitium around bronchovascular bundles, overlapping with peribronchial metaplasia and fibrosis in some series. Currently, AFOP and BPIP are both best viewed as histological entities rather than true clinical disorders, in the absence of characteristic associated imaging patterns and clinical features. PMID:26627191

  15. Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias

    PubMed Central

    Hoeper, Marius M.; Behr, Juergen; Held, Matthias; Grunig, Ekkehard; Vizza, C. Dario; Vonk-Noordegraaf, Anton; Lange, Tobias J.; Claussen, Martin; Grohé, Christian; Klose, Hans; Olsson, Karen M.; Zelniker, Thomas; Neurohr, Claus; Distler, Oliver; Wirtz, Hubert; Opitz, Christian; Huscher, Doerte; Pittrow, David; Gibbs, J. Simon R.

    2015-01-01

    Background Pulmonary hypertension (PH) is a common finding in patients with chronic fibrosing idiopathic interstitial pneumonias (IIP). Little is known about the response to pulmonary vasodilator therapy in this patient population. COMPERA is an international registry that prospectively captures data from patients with various forms of PH receiving pulmonary vasodilator therapies. Methods We retrieved data from COMPERA to compare patient characteristics, treatment patterns, response to therapy and survival in newly diagnosed patients with idiopathic pulmonary arterial hypertension (IPAH) and PH associated with IIP (PH-IIP). Results Compared to patients with IPAH (n = 798), patients with PH-IIP (n = 151) were older and predominantly males. Patients with PH-IIP were treated predominantly with phosphodiesterase-5 inhibitors (88% at entry, 87% after 1 year). From baseline to the first follow-up visit, the median improvement in 6MWD was 30 m in patients with IPAH and 24.5 m in patients with PH-IIP (p = 0.457 for the difference between both groups). Improvements in NYHA functional class were observed in 22.4% and 29.5% of these patients, respectively (p = 0.179 for the difference between both groups). Survival rates were significantly worse in PH-IIP than in IPAH (3-year survival 34.0 versus 68.6%; p<0.001). Total lung capacity, NYHA class IV, and mixed-venous oxygen saturation were independent predictors of survival in patients with PH-IIP. Conclusions Patients with PH-IIP have a dismal prognosis. Our results suggest that pulmonary vasodilator therapy may be associated with short-term functional improvement in some of these patients but it is unclear whether this treatment affects survival. Trial Registration clinicaltrials.gov NCT01347216 PMID:26630396

  16. Connective tissue disease-associated interstitial pneumonia and idiopathic interstitial pneumonia: similarity and difference.

    PubMed

    Bryson, Thomas; Sundaram, Baskaran; Khanna, Dinesh; Kazerooni, Ella A

    2014-02-01

    Interstitial lung diseases (ILDs) are increasingly recognized in patients with systemic diseases. Patients with early ILD changes may be asymptomatic. Features of ILD overlap among systemic diseases and with idiopathic variety. High-resolution computed tomography plays a central role in diagnosing ILDs. Imaging features are often nonspecific. Therapy- and complication-related lung changes would pose difficulty in diagnosing and classifying an ILD. Biology and prognosis of secondary ILDs may differ between different disease-related ILDs and idiopathic variety. Combination of clinical features, serological tests, pulmonary and extrapulmonary imaging findings, and pathology findings may help to diagnose ILDs. PMID:24480141

  17. Prognostic implications of physiologic and radiographic changes in idiopathic interstitial pneumonia.

    PubMed

    Flaherty, Kevin R; Mumford, Jeanette A; Murray, Susan; Kazerooni, Ella A; Gross, Barry H; Colby, Thomas V; Travis, William D; Flint, Andrew; Toews, Galen B; Lynch, Joseph P; Martinez, Fernando J

    2003-09-01

    Idiopathic interstitial pneumonias are a diverse group of lung diseases with varied prognoses. We hypothesized that changes in physiologic and radiographic parameters would predict survival. We retrospectively examined 80 patients with usual interstitial pneumonia and 29 patients with nonspecific interstitial pneumonia. Baseline characteristics were examined together with 6-month change in forced vital capacity, diffusing capacity for carbon monoxide, and ground glass infiltrate and fibrosis on high resolution computed tomography. Patients with usual interstitial pneumonia were more likely to have a statistically significant or marginally significant decline in lung volume, diffusing capacity for carbon monoxide, and an increase in ground glass infiltrates (p < or = 0.08) compared with patients with nonspecific interstitial pneumonia. For patients with usual interstitial pneumonia, change in forced vital capacity was the best physiologic predictor of mortality (p = 0.05). In a multivariate Cox proportional hazards model controlling for histopathologic diagnosis, gender, smoking history, baseline forced vital capacity, and 6-month change in forced vital capacity, a decrease in forced vital capacity remained an independent risk factor for mortality (decrease > 10%; hazard ratio 2.47; 95% confidence interval 1.29, 4.73; p = 0.006). We conclude that a 6-month change in forced vital capacity gives additional prognostic information to baseline features for patients with idiopathic interstitial pneumonia.

  18. Clinical Features of Idiopathic Interstitial Pneumonia with Systemic Sclerosis-Related Autoantibody in Comparison with Interstitial Pneumonia with Systemic Sclerosis

    PubMed Central

    Yamakawa, Hideaki; Hagiwara, Eri; Kitamura, Hideya; Yamanaka, Yumie; Ikeda, Satoshi; Sekine, Akimasa; Baba, Tomohisa; Iso, Shinichiro; Okudela, Koji; Iwasawa, Tae; Takemura, Tamiko; Kuwano, Kazuyoshi; Ogura, Takashi

    2016-01-01

    Background Patients with idiopathic interstitial pneumonias sometimes have a few features of connective tissue disease (CTD) and yet do not fulfil the diagnostic criteria for any specific CTD. Objective This study was conducted to elucidate the characteristics, prognosis, and disease behavior in patients with interstitial lung disease (ILD) associated with systemic sclerosis (SSc)-related autoantibodies. Methods We retrospectively analyzed medical records of 72 ILD patients: 40 patients with SSc (SSc-ILD) and 32 patients with SSc-related autoantibody-positive ILD but not with CTD (ScAb-ILD), indicating lung-dominant CTD with SSc-related autoantibody. Results Patients with SSc-ILD were predominantly females and non-smokers, and most had nonspecific interstitial pneumonia confirmed by high-resolution computed tomography (HRCT) and pathological analysis. However, about half of the patients with ScAb-ILD were male and current or ex-smokers. On HRCT analysis, honeycombing was more predominant in patients with ScAb-ILD than with SSc-ILD. Pathological analysis showed the severity of vascular intimal or medial thickening in the SSc-ILD patients to be significantly higher than that in the ScAb-ILD patients. Survival curves showed that the patients with ScAb-ILD had a significantly poorer outcome than those with SSc-ILD. Conclusion Data from this study suggest that lung-dominant CTD with SSc-related autoantibody is a different disease entity from SSc-ILD. PMID:27564852

  19. Acute interstitial pneumonia.

    PubMed

    Bouros, D; Nicholson, A C; Polychronopoulos, V; du Bois, R M

    2000-02-01

    The term "acute interstitial pneumonia" (AIP) describes an idiopathic clinicopathological condition, characterized clinically by an interstitial lung disease causing rapid onset of respiratory failure, which is distinguishable from the other more chronic forms of interstitial pneumonia. It is synonymous with Hamman-Rich syndrome, occurring in patients without pre-existing lung disease. The histopathological findings are those of diffuse alveolar damage. AIP radiologically and physiologically resembles acute respiratory distress syndrome (ARDS) and is considered to represent the small subset of patients with idiopathic ARDS. It is frequently confused with other clinical entities characterized by rapidly progressive interstitial pneumonia, especially secondary acute interstitial pneumonia, acute exacerbations and accelerated forms of cryptogenic fibrosing alveolitis . Furthermore, many authors use the above terms, both erroneously and interchangeably. It has a grave prognosis with >70% mortality in 3 months, despite mechanical ventilation. This review aims to clarify the relative clinical and pathological issues and terminology.

  20. American Thoracic Society-European Respiratory Society Classification of the Idiopathic Interstitial Pneumonias: Advances in Knowledge since 2002.

    PubMed

    Sverzellati, Nicola; Lynch, David A; Hansell, David M; Johkoh, Takeshi; King, Talmadge E; Travis, William D

    2015-01-01

    In the updated American Thoracic Society-European Respiratory Society classification of the idiopathic interstitial pneumonias (IIPs), the major entities have been preserved and grouped into (a) "chronic fibrosing IIPs" (idiopathic pulmonary fibrosis and idiopathic nonspecific interstitial pneumonia), (b) "smoking-related IIPs" (respiratory bronchiolitis-associated interstitial lung disease and desquamative interstitial pneumonia), (c) "acute or subacute IIPs" (cryptogenic organizing pneumonia and acute interstitial pneumonia), and (d) "rare IIPs" (lymphoid interstitial pneumonia and idiopathic pleuroparenchymal fibroelastosis). Furthermore, it has been acknowledged that a final diagnosis is not always achievable, and the category "unclassifiable IIP" has been proposed. The diagnostic interpretation of the IIPs is often challenging because other diseases with a known etiology (most notably, connective tissue disease and hypersensitivity pneumonitis) may show similar morphologic patterns. Indeed, more emphasis has been given to the integration of clinical, computed tomographic (CT), and pathologic findings for multidisciplinary diagnosis. Typical CT-based morphologic patterns are associated with the IIPs, and radiologists play an important role in diagnosis and characterization. Optimal CT quality and a systematic approach are both pivotal for evaluation of IIP. Interobserver variation for the various patterns encountered in the IIPs is an issue. It is important for radiologists to understand the longitudinal behavior of IIPs at serial CT examinations, especially for providing a framework for cases that are unclassifiable or in which a histologic diagnosis cannot be obtained.

  1. [Perioperative lung injury: acute exacerbation of idiopathic pulmonary fibrosis and acute interstitial pneumonia after pulmonary resection].

    PubMed

    Hoshikawa, Yasushi; Kondo, Takashi

    2004-12-01

    The mortality rate after surgical resection for lung cancer has been reported to range between 1% and 3%, with 30% caused by acute exacerbation of idiopathic pulmonary fibrosis (IPF) or acute interstitial pneumonia (AIP). Approximately 20% of patients with IPF have lung cancer, while 2% to 4% of lung cancer patients have IPF. The incidence of postoperative acute exacerbation of IPF is about 20%. Some investigations in Japan revealed that 10% to 17% of lung cancer patients undergoing lung resection, who have not been diagnosed with IPF preoperatively, have localized-usual interstitial pneumonia (Lo-UIP) lesions. Approximately 20% of patients with Lo-UIP show postoperative acute exacerbation, while about 0.5% of those without Lo-UIP develop AIP after surgery. There is no confirmed treatment or prophylaxis. Most patients who develop postoperative acute exacerbation or AIP are treated with methylpredonisolone (1,000 mg/day x 3 days), but the mortality rate is 50% or greater. We emphasize that more efforts should be made to develop strategies to prevent postoperative acute exacerbation of IPF and AIP.

  2. Identification of Clinical Phenotypes in Idiopathic Interstitial Pneumonia with Pulmonary Emphysema.

    PubMed

    Sato, Suguru; Tanino, Yoshinori; Misa, Kenichi; Fukuhara, Naoko; Nikaido, Takefumi; Uematsu, Manabu; Fukuhara, Atsuro; Wang, Xintao; Ishida, Takashi; Munakata, Mitsuru

    2016-01-01

    Objective Since the term "combined pulmonary fibrosis and emphysema" (CPFE) was first proposed, the co-existence of pulmonary fibrosis and pulmonary emphysema (PE) has drawn considerable attention. However, conflicting results on the clinical characteristics of patients with both pulmonary fibrosis and PE have been published because of the lack of an exact definition of CPFE. The goal of this study was thus to clarify the clinical characteristics and phenotypes of idiopathic interstitial pneumonia (IIP) with PE. Methods We retrospectively analyzed IIP patients who had been admitted to our hospital. Their chest high-resolution computed tomography images were classified into two groups according to the presence of PE. We then performed a cluster analysis to identify the phenotypes of IIP patients with PE. Results Forty-four (53.7%) out of 82 patients had at least mild emphysema in their bilateral lungs. The cluster analysis separated the IIP patients with PE into three clusters. The overall survival rate of one cluster that consisted of mainly idiopathic pulmonary fibrosis (IPF) patients was significantly worse than those of the other clusters. Conclusion Three different phenotypes can be identified in IIP patients with PE, and IPF with PE is a distinct clinical phenotype with a poor prognosis.

  3. Identification of Clinical Phenotypes in Idiopathic Interstitial Pneumonia with Pulmonary Emphysema.

    PubMed

    Sato, Suguru; Tanino, Yoshinori; Misa, Kenichi; Fukuhara, Naoko; Nikaido, Takefumi; Uematsu, Manabu; Fukuhara, Atsuro; Wang, Xintao; Ishida, Takashi; Munakata, Mitsuru

    2016-01-01

    Objective Since the term "combined pulmonary fibrosis and emphysema" (CPFE) was first proposed, the co-existence of pulmonary fibrosis and pulmonary emphysema (PE) has drawn considerable attention. However, conflicting results on the clinical characteristics of patients with both pulmonary fibrosis and PE have been published because of the lack of an exact definition of CPFE. The goal of this study was thus to clarify the clinical characteristics and phenotypes of idiopathic interstitial pneumonia (IIP) with PE. Methods We retrospectively analyzed IIP patients who had been admitted to our hospital. Their chest high-resolution computed tomography images were classified into two groups according to the presence of PE. We then performed a cluster analysis to identify the phenotypes of IIP patients with PE. Results Forty-four (53.7%) out of 82 patients had at least mild emphysema in their bilateral lungs. The cluster analysis separated the IIP patients with PE into three clusters. The overall survival rate of one cluster that consisted of mainly idiopathic pulmonary fibrosis (IPF) patients was significantly worse than those of the other clusters. Conclusion Three different phenotypes can be identified in IIP patients with PE, and IPF with PE is a distinct clinical phenotype with a poor prognosis. PMID:27301501

  4. Exercise related ventilation dynamics and clinical correlates in patients with fibrotic idiopathic interstitial pneumonias.

    PubMed

    De Martino, Marina; Cobuccio, Raffaele; Bruzzese, Dario; Rea, Gaetano; Meoli, Ilernando; Stefanelli, Francesco; Canora, Angelo; Capaccio, Annalisa; Sanduzzi, Alessandro; Matarese, Alessandro; Bocchino, Marialuisa

    2016-01-01

    Assessment of exercise performance is a key component in the management of interstitial lung diseases, as its limitation may occur very early. Aim of the present study was to assess ventilation dynamics in combination with pulse-oximetry changes in 54 clinically stable patients affected by idiopathic pulmonary fibrosis or idiopathic fibrotic nonspecific interstitial pneumonia. Testing was successfully performed with the Spiropalm 6-MWT Hand-held spirometer by the majority of cases (94%). End test oxygen saturation (SpO2) values <88% were common in most of patients (76%), with a mean distance walked of 403 meters. Ventilation significantly increased due to the contribution of the tidal volume and the respiratory frequency (RF). This finding was associated with a decrease of the end of test respiratory reserve (RR), that was <20% in 9 cases (17.6%). Lung function was inversely related to the end of test RF, while a positive correlation occurred with the end of test RR and the estimated maximal voluntary ventilation (MVV). RR was also a predictive factor of declining forced vital capacity and lung diffusion capacity for carbon monoxide (DLCO) over a 6-month period. Further factors of DLCO impairment were low SpO2 and MVV. Comparison with the cardio-pulmonary exercise test (CPET) showed that the 6-MWT end of test RR was inversely related to the CPET-derived peak RF and VE/VCO2 suggesting RR as pivotal in exercise limitation assessment. Our results open challenging perspectives in an unexplored field. Future research will include management of latent respiratory failure and monitoring of disease progression and therapy response. PMID:27537719

  5. Lung diffusing capacity for nitric oxide as a marker of fibrotic changes in idiopathic interstitial pneumonias.

    PubMed

    Barisione, Giovanni; Brusasco, Claudia; Garlaschi, Alessandro; Baroffio, Michele; Brusasco, Vito

    2016-05-01

    Lung diffusing capacity for carbon monoxide (DLCO) is decreased in both usual interstitial pneumonia-idiopathic pulmonary fibrosis (UIP-IPF) and nonspecific interstitial pneumonia (NSIP), but is moderately related to computed tomography (CT)-determined fibrotic changes. This may be due to the relative insensitivity of DLCO to changes in alveolar membrane diffusive conductance (DMCO). The purpose of this study was to determine whether measurement of lung diffusing capacity for nitric oxide (DLNO) better reflects fibrotic changes than DLCO DLNO-DLCO were measured simultaneously in 30 patients with UIP-IPF and 30 with NSIP. Eighty-one matched healthy subjects served as a control group. The amount of pulmonary fibrosis was estimated by CT volumetric analysis of visually bounded areas showing reticular opacities and honeycombing. DMCO and pulmonary capillary volume (VC) were calculated. DLNO was below the lower limit of normal in all patients irrespective of extent and nature of disease, whereas DLCO was within the normal range in a nonnegligible number of patients. Both DLNO and DLCO were significantly correlated with visual assessment of fibrosis but DLNO more closely than DLCO DMCO was also below the lower limit of normal in all UIP-IPF and NSIP patients and significantly correlated with fibrosis extent in both diseases, whereas VC was weakly correlated with fibrosis in UIP-IPF and uncorrelated in NSIP, with normal values in half of patients. In conclusion, measurement of DLNO may provide a more sensitive evaluation of fibrotic changes than DLCO in either UIP-IPF or NSIP, because it better reflects DMCO.

  6. Idiopathic interstitial pneumonias and emphysema: detection and classification using a texture-discriminative approach

    NASA Astrophysics Data System (ADS)

    Fetita, C.; Chang-Chien, K. C.; Brillet, P. Y.; Pr"teux, F.; Chang, R. F.

    2012-03-01

    Our study aims at developing a computer-aided diagnosis (CAD) system for fully automatic detection and classification of pathological lung parenchyma patterns in idiopathic interstitial pneumonias (IIP) and emphysema using multi-detector computed tomography (MDCT). The proposed CAD system is based on three-dimensional (3-D) mathematical morphology, texture and fuzzy logic analysis, and can be divided into four stages: (1) a multi-resolution decomposition scheme based on a 3-D morphological filter was exploited to discriminate the lung region patterns at different analysis scales. (2) An additional spatial lung partitioning based on the lung tissue texture was introduced to reinforce the spatial separation between patterns extracted at the same resolution level in the decomposition pyramid. Then, (3) a hierarchic tree structure was exploited to describe the relationship between patterns at different resolution levels, and for each pattern, six fuzzy membership functions were established for assigning a probability of association with a normal tissue or a pathological target. Finally, (4) a decision step exploiting the fuzzy-logic assignments selects the target class of each lung pattern among the following categories: normal (N), emphysema (EM), fibrosis/honeycombing (FHC), and ground glass (GDG). According to a preliminary evaluation on an extended database, the proposed method can overcome the drawbacks of a previously developed approach and achieve higher sensitivity and specificity.

  7. Understanding Idiopathic Interstitial Pneumonia: A Gene-Based Review of Stressed Lungs

    PubMed Central

    van Moorsel, Coline H. M.; Hoffman, Thijs W.; van Batenburg, Aernoud A.; Klay, Dymph; van der Vis, Joanne J.; Grutters, Jan C.

    2015-01-01

    Pulmonary fibrosis is the main cause of severe morbidity and mortality in idiopathic interstitial pneumonias (IIP). In the past years, there has been major progress in the discovery of genetic factors that contribute to disease. Genes with highly penetrant mutations or strongly predisposing common risk alleles have been identified in familial and sporadic IIP. This review summarizes genes harbouring causative rare mutations and replicated common predisposing alleles. To date, rare mutations in nine different genes and five risk alleles fulfil this criterion. Mutated genes represent three genes involved in surfactant homeostasis and six genes involved in telomere maintenance. We summarize gene function, gene expressing cells, and pathological consequences of genetic alterations associated with disease. Consequences of the genetic alteration include dysfunctional surfactant processing, ER stress, immune dysregulation, and maintenance of telomere length. Biological evidence shows that these processes point towards a central role for alveolar epithelial type II cell dysfunction. However, tabulation also shows that function and consequence of most common risk alleles are not known. Most importantly, the predisposition of the MUC5B risk allele to disease is not understood. We propose a mechanism whereby MUC5B decreases surface tension lowering capacity of alveolar surfactant at areas with maximal mechanical stress. PMID:26539479

  8. Lymphocytic Interstitial Pneumonia.

    PubMed

    Panchabhai, Tanmay S; Farver, Carol; Highland, Kristin B

    2016-09-01

    Lymphocytic interstitial pneumonia (LIP) is a rare lung disease on the spectrum of benign pulmonary lymphoproliferative disorders. LIP is frequently associated with connective tissue diseases or infections. Idiopathic LIP is rare; every attempt must be made to diagnose underlying conditions when LIP is diagnosed. Computed tomography of the chest in patients with LIP may reveal ground-glass opacities, centrilobular and subpleural nodules, and randomly distributed thin-walled cysts. Demonstrating polyclonality with immunohistochemistry is the key to differentiating LIP from lymphoma. The 5-year mortality remains between 33% and 50% and is likely to vary based on the underlying disease process. PMID:27514593

  9. Ultrasound in Rheumatologic Interstitial Lung Disease: A Case Report of Nonspecific Interstitial Pneumonia in Rheumatoid Arthritis.

    PubMed

    Laria, A; Lurati, A; Scarpellini, M

    2015-01-01

    According to the American Thoracic Society (ATS)/European Respiratory Society consensus classification, idiopathic interstitial pneumonias (IIPs) include several clinic-radiologic-pathologic entities: idiopathic pulmonary fibrosis (IPF), usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis-associated ILD, desquamative interstitial pneumonia, and lymphoid interstitial pneumonia. Ultrasound Lung Comets (ULCs) are an echographic chest-sonography hallmark of pulmonary interstitial fibrosis. We describe the ultrasound (US) findings in the follow-up of a NSIP's case in rheumatoid arthritis (RA). PMID:26240772

  10. Evidence of type II pneumocyte apoptosis in the pathogenesis of idiopathic pulmonary fibrosis (IFP)/usual interstitial pneumonia (UIP)

    PubMed Central

    Barbas-Filho, J; Ferreira, M; Sesso, A; Kairalla, R; Carvalho, C; Capelozzi, V

    2001-01-01

    Background/Aims—The pathogenesis of idiopathic pulmonary fibrosis (IPF)/usual interstitial pneumonia (UIP), a chronic and incurable human respiratory disease, is not well established. This study was designed to investigate whether the apoptosis of type II pneumocytes could be the precipitating factor in the pathogenesis of IPF. Methods—Nineteen specimens obtained by retrospective review of the medical and pathological records of 55 patients with IPF, four normal subjects, and 10 disease control lungs were analysed. The selected specimens had normal alveoli with intervening patchy scarring of the lung parenchyma, fulfilling the pathological criteria for UIP. To identify individual cells undergoing apoptosis in the normal alveoli, electron microscopy and in situ end labelling of fragmented DNA were performed on paraffin wax embedded sections using digoxigenin-11-dUTP and the enzyme terminal deoxynucleotidyl transferase. Results—Apoptosis was detected in the normal alveoli of 17 of the 19 patients with IPF/UIP and was absent in the controls. Electron microscopy demonstrated apoptotic changes in type II pneumocytes. These results indicate that apoptotic type II pneumocyte death occurs in normal alveoli of IPF/UIP and could be the principal cause of several events that account for the histological, clinical, and functional alterations seen in IPF/UIP. Conclusions—In conclusion, numerous type II pneumocytes from the normal alveoli of most patients with IPF/UIP actively undergo programmed cell death. This finding may shed new light on the pathogenesis of this disease, with implications mainly for the treatment of affected patients. Key Words: apoptosis • type II pneumocytes • idiopathic pulmonary fibrosis • pathogenesis PMID:11215282

  11. Idiopathic Interstitial Pneumonias

    MedlinePlus

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  12. Feedlot Acute Interstitial Pneumonia.

    PubMed

    Woolums, Amelia R

    2015-11-01

    Acute interstitial pneumonia (AIP) of feedlot cattle is a sporadically occurring respiratory condition that is often fatal. Affected cattle have a sudden onset of labored breathing. There is no confirmed effective treatment of feedlot AIP; however, administration of antibiotics effective against common bacterial respiratory pathogens and nonsteroidal anti-inflammatory drugs, especially aspirin, has been recommended. Protective strategies are not well defined, but efforts to limit dust exposure and heat stress; to ensure consistent formulation, mixing, and delivery of feed; and to identify and treat infectious respiratory disease in a timely manner may decrease rates of feedlot AIP.

  13. Usual interstitial pneumonia in rheumatoid arthritis-associated interstitial lung disease.

    PubMed

    Kim, E J; Elicker, B M; Maldonado, F; Webb, W R; Ryu, J H; Van Uden, J H; Lee, J S; King, T E; Collard, H R

    2010-06-01

    Interstitial lung disease is a common manifestation of rheumatoid arthritis; however, little is known about factors that influence its prognosis. The aim of the present study was to determine whether or not the usual interstitial pneumonia pattern found on high-resolution computed tomography (HRCT) is of prognostic significance in rheumatoid arthritis-associated interstitial lung disease (RA-ILD). Patients with RA-ILD were identified retrospectively (n = 82). The relationship of a definite usual interstitial pneumonia pattern on HRCT to survival was determined and compared to that in a cohort of patients with radiologically diagnosed idiopathic pulmonary fibrosis (n = 51). A definite usual interstitial pneumonia pattern was seen in 20 (24%) out of 82 patients with RA-ILD. These patients showed worse survival than those without this pattern (median survival 3.2 versus 6.6 yrs), and a similar survival to those with idiopathic pulmonary fibrosis. On multivariate analysis, a definite usual interstitial pneumonia pattern on HRCT was associated with worse survival (hazard ratio of 2.3). Analysis of specific HRCT features demonstrated that traction bronchiectasis and honeycomb fibrosis were associated with worse survival (hazard ratio of 2.6 and 2.1, respectively). Female sex (hazard ratio of 0.30) and a higher baseline diffusing capacity of the lung for carbon monoxide (hazard ratio of 0.96) were associated with better survival. A definite usual interstitial pneumonia pattern on HRCT has important prognostic implications in RA-ILD.

  14. Rheumatoid Arthritis-Associated Interstitial Lung Disease and Idiopathic Pulmonary Fibrosis: Shared Mechanistic and Phenotypic Traits Suggest Overlapping Disease Mechanisms.

    PubMed

    Paulin, Francisco; Doyle, Tracy J; Fletcher, Elaine A; Ascherman, Dana P; Rosas, Ivan O

    2015-01-01

    The prevalence of clinically evident interstitial lung disease in patients with rheumatoid arthritis is approximately 10%. An additional 33% of undiagnosed patients have interstitial lung abnormalities that can be detected with high-resolution computed tomography. Rheumatoid arthritis-interstitial lung disease patients have three times the risk of death compared to those with rheumatoid arthritis occurring in the absence of interstitial lung disease, and the mortality related to interstitial lung disease is rising. Rheumatoid arthritis-interstitial lung disease is most commonly classified as the usual interstitial pneumonia pattern, overlapping mechanistically and phenotypically with idiopathic pulmonary fibrosis, but can occur in a non-usual interstitial pneumonia pattern, mainly nonspecific interstitial pneumonia. Based on this, we propose two possible pathways to explain the coexistence of rheumatoid arthritis and interstitial lung disease: (i) Rheumatoid arthritis-interstitial lung disease with a non-usual interstitial pneumonia pattern may come about when an immune response against citrullinated peptides taking place in another site (e.g. the joints) subsequently affects the lungs; (ii) Rheumatoid arthritis-interstitial lung disease with a usual interstitial pneumonia pattern may represent a disease process in which idiopathic pulmonary fibrosis-like pathology triggers an immune response against citrullinated proteins that promotes articular disease indicative of rheumatoid arthritis. More studies focused on elucidating the basic mechanisms leading to different sub-phenotypes of rheumatoid arthritis-interstitial lung disease and the overlap with idiopathic pulmonary fibrosis are necessary to improve our understanding of the disease process and to define new therapeutic targets.

  15. The correlation of symptoms, pulmonary function tests and exercise testing with high-resolution computed tomography in patients with idiopathic interstitial pneumonia in a tertiary care hospital in South India

    PubMed Central

    Isaac, Barney Thomas Jesudason; Thangakunam, Balamugesh; Cherian, Rekha A; Christopher, Devasahayam Jesudas

    2015-01-01

    Context: For the follow-up of patients with idiopathic interstitial pneumonias (IIP), it is unclear which parameters of pulmonary function tests (PFT) and exercise testing would correlate best with high-resolution computed tomography (HRCT).. Aim: To find out the correlation of symptom scores, PFTs and exercise testing with HRCT scoring in patients diagnosed as idiopathic interstitial pneumonia. Settings and Design: Cross-sectional study done in pulmonary medicine outpatients department of a tertiary care hospital in South India. Materials and Methods: Consecutive patients who were diagnosed as IIP by a standard algorithm were included into the study. Cough and dyspnea were graded for severity and duration. Pulmonary function tests and exercise testing parameters were noted. HRCT was scored based on an alveolar score, an interstitial score and a total score. The HRCT was correlated with each of the clinical and physiologic parameters. Pearson's/Spearman's correlation coefficient was used for the correlation of symptoms and parameters of ABG, PFT and 6MWT with the HRCT scores. Results: A total of 94 patients were included in the study. Cough and dyspnea severity (r = 0.336 and 0.299), FVC (r = −0.48), TLC (r = −0.439) and DLCO and distance saturation product (DSP) (r = −0.368) and lowest saturation (r = −0.324) had significant correlation with total HRCT score. Among these, DLCO, particularly DLCO corrected % of predicted, correlated best with HRCT score (r = −0.721).. Conclusion: Symptoms, PFT and exercise testing had good correlation with HRCT. DLCO corrected % of predicted correlated best with HRCT. PMID:26664164

  16. A review of interstitial pneumonia in cattle.

    PubMed

    Kerr, L A; Linnabary, R D

    1989-06-01

    Interstitial pneumonias comprise a significant proportion of cattle respiratory diseases. Known by different names, such as acute bovine pulmonary emphysema and edema (ABPE), fog fever, atypical interstitial pneumonia (AIP) and cow asthma, the condition seems to occur predominantly in late summer or fall. However, depending on the etiology, cases have occurred throughout the year. Interstitial pneumonia often begins with acute respiratory distress in animals that were clinically normal 12 hr earlier. Animals are observed breathing very rapid and shallow with their mouths open. If disturbed, death may occur rapidly from hypoxia. Causes of interstitial pneumonia are quite varied ranging from parasitic, viral and bacterial to toxic. Toxic agents constitute the most economically important cause of this condition in cattle. The primary toxin is the amino acid L-tryptophan in lush pasture grasses, a compound which is converted to 3-methylindole by rumen microorganisms. Other leading toxic causes of interstitial pneumonia are perilla mint and moldy sweet potatoes. Although treatments are mainly symptomatic and ineffective, preventive measures will reduce the occurrence of interstitial pneumonia. Prevention consists of denying animals exposure to know pneumotoxic agents, eliminating certain rumen microflora that break down the toxic compounds to reactive metabolites, and supplying ample good forage so that cattle will not as likely consume toxic plants.

  17. Update on cryptogenic organising pneumonia (idiopathic bronchiolitis obliterans organising pneumonia).

    PubMed

    Cordier, Jean-François

    2002-11-23

    Organising pneumonia, defined by intraalveolar buds of connective tissue, may be a disorder secondary to a determined cause (infectious agents, drugs) or occurring in a specific context (as the connective tissue disorders). It may also be a cryptogenic interstitial pneumonia with characteristic clinical and imaging features and especially an excellent response to corticosteroids.

  18. Nonspecific Interstitial Pneumonia: What Is the Optimal Approach to Management?

    PubMed

    Tomassetti, Sara; Ryu, Jay H; Piciucchi, Sara; Chilosi, Marco; Poletti, Venerino

    2016-06-01

    We reviewed current aspects of the clinical and pathogenic profile of nonspecific interstitial pneumonia (NSIP), to better elucidate the complex issue of management and treatment options for NSIP patients. Recent findings suggest that idiopathic NSIP is a complex clinical entity with a disease spectrum that includes at least three different phenotypes: NSIP associated with autoimmune features, emphysema, and familial interstitial lung disease. This distinction, based mainly on clinical findings, may be of critical importance when it comes to making a decision on patients' management. This hypothesis warrants further studies. Currently, two major radiologic-pathologic different profiles have been well established. First, the "inflammatory type" characterized by prominent lymphocytic inflammation both on biopsy and bronchoalveolar lavage (BAL), and high-resolution computed tomography (HRCT) with mixed NSIP/organizing pneumonia pattern that tends to have a better response to corticosteroid and immunosuppressive treatment. Second, the "highly fibrotic" subgroup that shows prominent reticular changes and traction bronchiectasis by HRCT, high fibrotic background on biopsy, and no lymphocytosis on BAL. The latter fibrotic NSIP is the subgroup with less potential to respond to immunosuppressive treatment and a marginal risk to evolve into "full-blown idiopathic pulmonary fibrosis." The management of patients with fibrotic, progressive, and immunosuppressive treatment, refractory NSIP remains uncertain, and further studies are needed to address the role of antifibrotic drug in this settings. Oxygen therapy, pulmonary rehabilitation, and lung transplantation are of importance in the current management of severe, progressive, and refractory NSIP patients.

  19. Nonspecific Interstitial Pneumonia: What Is the Optimal Approach to Management?

    PubMed

    Tomassetti, Sara; Ryu, Jay H; Piciucchi, Sara; Chilosi, Marco; Poletti, Venerino

    2016-06-01

    We reviewed current aspects of the clinical and pathogenic profile of nonspecific interstitial pneumonia (NSIP), to better elucidate the complex issue of management and treatment options for NSIP patients. Recent findings suggest that idiopathic NSIP is a complex clinical entity with a disease spectrum that includes at least three different phenotypes: NSIP associated with autoimmune features, emphysema, and familial interstitial lung disease. This distinction, based mainly on clinical findings, may be of critical importance when it comes to making a decision on patients' management. This hypothesis warrants further studies. Currently, two major radiologic-pathologic different profiles have been well established. First, the "inflammatory type" characterized by prominent lymphocytic inflammation both on biopsy and bronchoalveolar lavage (BAL), and high-resolution computed tomography (HRCT) with mixed NSIP/organizing pneumonia pattern that tends to have a better response to corticosteroid and immunosuppressive treatment. Second, the "highly fibrotic" subgroup that shows prominent reticular changes and traction bronchiectasis by HRCT, high fibrotic background on biopsy, and no lymphocytosis on BAL. The latter fibrotic NSIP is the subgroup with less potential to respond to immunosuppressive treatment and a marginal risk to evolve into "full-blown idiopathic pulmonary fibrosis." The management of patients with fibrotic, progressive, and immunosuppressive treatment, refractory NSIP remains uncertain, and further studies are needed to address the role of antifibrotic drug in this settings. Oxygen therapy, pulmonary rehabilitation, and lung transplantation are of importance in the current management of severe, progressive, and refractory NSIP patients. PMID:27231862

  20. Genetic Analysis of Sporadic and Familial Interstitial Pneumonia

    PubMed Central

    Schwartz, David A.

    2008-01-01

    Although much progress has been made in understanding the biology and clinical course of interstitial pneumonia, the etiology of this disease remains elusive. Epidemiologic studies have consistently identified cigarette smoke as an important exposure; however, most smokers do not develop interstitial pneumonia and many individuals with interstitial pneumonia do not smoke cigarettes. Moreover, interstitial pneumonias have been reported to cluster in families. Thus, a more thorough understanding of the genetic etiology of interstitial pneumonia may prove critically important in defining the biology and clinical course of this complex human disease. PMID:18403331

  1. Interstitial Lung Disease in Idiopathic Inflammatory Myopathy

    PubMed Central

    Saketkoo, Lesley Ann; Ascherman, Dana P.; Cottin, Vincent; Christopher-Stine, Lisa; Danoff, Sonye K.; Oddis, Chester V.

    2011-01-01

    The lung is one of the most common extra-muscular targets in idiopathic inflammatory myopathies (IIM) and interstitial lung disease (ILD) is a prevalent and often devastating manifestation of IIM. IIM-associated ILD (IIM-ILD) contributes to nearly 80% of the mortality in IIM with a reported prevalence of 65% of newly diagnosed IIM cases. Although ILD frequently accompanies clinical and laboratory findings of myositis, overt signs of muscle disease may be absent in the setting of significant lung disease. Understanding the varied scope of presentation of these diseases is essential to providing optimal patient care. This review will provide an in depth examination of ILD in IIM both from a rheumatologic and pulmonary perspective and will discuss the scope of disease, presenting features, genetic associations, pathogenesis, diagnosis, radiographic and histopathologic findings, along with biomarker assessment and a rationale for therapeutic intervention. PMID:21941374

  2. Histopathologic variability in usual and nonspecific interstitial pneumonias.

    PubMed

    Flaherty, K R; Travis, W D; Colby, T V; Toews, G B; Kazerooni, E A; Gross, B H; Jain, A; Strawderman, R L; Flint, A; Lynch, J P; Martinez, F J

    2001-11-01

    Findings of surgical lung biopsy (SLB) are important in categorizing patients with idiopathic interstitial pneumonia (IIP). We investigated whether histologic variability would be evident in SLB specimens from multiple lobes in patients with IIP. SLBs from 168 patients, 109 of whom had multiple lobes biopsied, were reviewed by three pathologists. A diagnosis was assigned to each lobe. A different diagnosis was found between lobes in 26% of the patients. Patients with usual interstitial pneumonia (UIP) in all lobes were categorized as concordant for UIP (n = 51) and those with UIP in at least one lobe were categorized as discordant for UIP (n = 28). Patients with nonspecific interstitial pneumonia (NSIP) in all lobes were categorized as having fibrotic (n = 25) or cellular NSIP (n = 5). No consistent distribution of lobar histology was noted. Patients concordant for UIP were older (63 +/- 9 [mean +/- SD] yr; p < 0.05 as compared with all other groups) than those discordant for UIP (57 +/- 12 yr) or with fibrotic NSIP (56 +/- 11 yr) or cellular NSIP (50 +/- 9 yr). Semiquantitative high-resolution computed tomography demonstrated a varied profusion of fibrosis (p < 0.05 for all group comparisons), with more fibrosis in concordant UIP (2.13 +/- 0.62) than in discordant UIP (1.42 +/- 0.73), fibrotic NSIP (0.83 +/- 0.58), or cellular NSIP (0.44 +/- 0.42). Survival was better for patients with NSIP than for those in both UIP groups (p < 0.001), although survival in the two UIP groups was comparable (p = 0.16). Lobar histologic variability is frequent in patients with IIP, patients with a histologic pattern of UIP in any lobe should be classified as having UIP.

  3. Asbestosis and environmental causes of usual interstitial pneumonia

    PubMed Central

    Gulati, Mridu; Redlich, Carrie A.

    2015-01-01

    Purpose of review Recent epidemiologic investigations suggest that occupational and environmental exposures contribute to the overall burden of idiopathic pulmonary fibrosis (IPF). This article explores the epidemiologic and clinical challenges to establishing exposure associations, the current literature regarding exposure disease relationships and the diagnostic work-up of IPF and asbestosis patients. Recent findings IPF patients demonstrate a histopathologic pattern of usual interstitial pneumonia. In the absence of a known cause or association, a usual interstitial pneumonia pattern leads to an IPF diagnosis, which is a progressive and often terminal fibrotic lung disease. It has long been recognized that asbestos exposure can cause pathologic and radiographic changes indistinguishable from IPF. Several epidemiologic studies, primarily case control in design, have found that a number of other exposures that can increase risk of developing IPF include cigarette smoke, wood dust, metal dust, sand/silica and agricultural exposures. Lung mineralogic analyses have provided additional support to causal associations. Genetic variation may explain differences in disease susceptibility among the population. Summary An accumulating body of literature suggests that occupational and environmental exposure can contribute to the development of IPF. The impact of exposure on the pathogenesis and clinical course of disease requires further study. PMID:25621562

  4. Usual interstitial pneumonia end-stage features from explants with radiologic and pathological correlations.

    PubMed

    Rabeyrin, Maud; Thivolet, Françoise; Ferretti, Gilbert R; Chalabreysse, Lara; Jankowski, Adrien; Cottin, Vincent; Pison, Christophe; Cordier, Jean-François; Lantuejoul, Sylvie

    2015-08-01

    Idiopathic pulmonary fibrosis (IPF) is the most frequent and severe idiopathic interstitial pneumonia, with typical high-resolution computed tomography (HRCT) features and histologic pattern of usual interstitial pneumonia (UIP); its main differential diagnosis is fibrotic nonspecific interstitial pneumonia (F-NSIP). Usual interstitial pneumonia was mainly described from lung biopsies, and little is known on explants. Twenty-two UIP/IPF explants were analyzed histologically and compared with previous open lung biopsies (OLBs; n = 11) and HRCT (n = 19), when available. Temporospatial heterogeneity and subpleural and paraseptal fibrosis were similarly found in UIP/IPF explants and OLB (91%-95%). Fibroblastic foci were found in 82% of OLBs and 100% of explants, with a higher mean score in explants (P = .023). Honeycombing was present in 64% of OLBs and 95% of explants, with a higher mean score in explants (P = .005). Almost 60% of UIP/IPF explants showed NSIP areas and 41% peribronchiolar fibrosis; inflammation, bronchiolar metaplasia, and vascular changes were more frequent in UIP/IPF explants; and Desquamative Interstitial Pneumonia (DIP)-like areas were not common (18%-27%). Numerous large airspace enlargements with fibrosis were frequent in UIP/IPF explants (59%). On HRCT, honeycombing was observed in 95% of the cases and ground-glass opacities in 53%, correlating with NSIP areas or acute exacerbation at histology. Six patients had combined IPF and emphysema. Lesions were more severe in UIP/IPF explants, reflecting the worsening of the disease. Usual interstitial pneumonia/IPF explants more frequently presented with confounding lesions such as NSIP areas, peribronchiolar fibrosis, and airspace enlargements with fibrosis sometimes associated with emphysema. PMID:26025258

  5. Acute interstitial pneumonia: report of a series.

    PubMed

    Bonaccorsi, A; Cancellieri, A; Chilosi, M; Trisolini, R; Boaron, M; Crimi, N; Poletti, V

    2003-01-01

    Four cases of acute interstitial pneumonia (AIP) are described with special emphasis on clinical background, lung imaging and bronchoalveolar lavage findings. A retrospective chart review of four patients with histologically-proven AIP, diagnosed between 1998 and 2000, was carried out. Clinical data, bronchoalveolar lavage (BAL) findings, high-resolution computed tomography (HRCT) and histological features were analysed. Three patients died and only one is in follow-up. HRCT showed areas of ground glass attenuation and alveolar consolidation in all patients. Histology, documented by open lung biopsy or autopsy specimens, was consistent with the organising form of diffuse alveolar damage pattern. BAL findings were characteristic, with a huge neutrophilia associated with scattered atypical type II pneumocytes collected in clusters with extracellular amorphous material (fragments of hyaline membranes) observed in two out of three cases. In this paper, four cases of acute interstitial pneumonia are reported in detail. The poor prognosis associated with this entity has been confirmed and the possible diagnostic role of the bronchoalveolar lavage is emphasised.

  6. Auscultation of Velcro Crackles is Associated With Usual Interstitial Pneumonia

    PubMed Central

    Sellarés, Jacobo; Hernández-González, Fernanda; Lucena, Carmen Mª; Paradela, Marina; Brito-Zerón, Pilar; Prieto-González, Sergio; Benegas, Mariana; Cuerpo, Sandra; Espinosa, Gerard; Ramírez, José; Sánchez, Marcelo; Xaubet, Antoni

    2016-01-01

    Abstract Auscultation of Velcro crackles has been proposed as a key finding in physical lung examination in patients with interstitial lung diseases (ILDs), especially in idiopathic pulmonary fibrosis (IPF). However, no studies have been carried out to assess the association of Velcro crackles with other clinical variables. We evaluated a cohort of 132 patients, prospectively and consecutively included in our ILD diagnostic program at a tertiary referral center. All patients were auscultated during the physical examination. The patients were divided into 2 groups: “presence” or “nonpresence” of bilateral Velcro crackles. Of all patients assessed, 83 (63%) presented Velcro crackles in the respiratory auscultation. Patients with Velcro crackles usually had more frequently cough and dyspnea at the moment of diagnosis. Forced vital capacity (P = 0.002) and lung diffusion capacity for carbon monoxide (P = 0.04) was lower in these patients. The ILD-GAP index was higher in the group with Velcro crackles (P = 0.01). All patients with usual interstitial pneumonia (UIP) in high-resolution computed tomography and all patients with final IPF diagnosis presented Velcro crackles. In multivariate analysis, the presence of Velcro crackles was independently associated with an UIP pattern. In patients suspected of having ILD, the auscultation of Velcro crackles was associated with UIP, a possibility which must be taken into consideration in early ILD detection in primary care. PMID:26844464

  7. The alphabet soup revisited: the chronic interstitial pneumonias in the 1990s.

    PubMed

    McAdams, H P; Rosado-de-Christenson, M L; Wehunt, W D; Fishback, N F

    1996-09-01

    Liebow classified the idiopathic interstitial pneumonias as usual (UIP), desquamative (DIP), bronchiolitis obliterans (BIP), lymphoid (LIP), and giant cell (GIP) interstitial pneumonias. This classification was modified to exclude LIP and GIP. UIP, the most common type, is characterized by synchronous foci of inflammation, collagen deposition, and fibrosis with interspersed normal lung. It usually affects men 40-60 years old and manifests radiologically with bilateral, basilar irregular opacities and volume loss. In most cases, a confident diagnosis can be made at high-resolution computed tomography because of characteristic subpleural irregular linear opacities, ground-glass opacities, honeycombing, and traction bronchiectasis. DIP affects younger patients and is characterized by diffuse intraalveolar macrophage aggregation. Typical radiologic features include bilateral, basilar ground-glass opacities and preserved lung volumes. BIP, renamed bronchiolitis obliterans with organizing pneumonia, affects middle-aged patients and manifests with multifocal plugs of immature fibroblasts in the air spaces. Typical radiologic features include bilateral consolidations and normal lung volumes. Recently described entities include acute (AIP) and nonspecific (NIP) interstitial pneumonias and respiratory bronchiolitis with interstitial lung disease (RB-ILD). AIP is a rapidly progressive, often fatal, illness characterized by diffuse alveolar damage and manifests with clinical and radiologic features of adult respiratory distress syndrome. NIP is a heterogeneous group of fibrosing disorders that cannot be otherwise classified. RB-ILD is a disease of smokers with a good prognosis.

  8. [Acute interstitial pneumonia: diagnostic approach and management].

    PubMed

    Feuillet, S; Tazi, A

    2011-06-01

    Acute interstitial pneumonia (AIP) encompasses a spectrum of pulmonary disorders characterized by involvement of the lung interstitium and distal airways (bronchioles and alveoli). The onset of respiratory symptoms is acute, most often within two weeks. Most AIP take place de novo, but sometimes represent an acute exacerbation of chronic lung disease. The clinical presentation of AIP comprises rapidly progressive dyspnoea, associated sometimes with cough, fever, myalgia and asthenia. Chest radiography shows diffuse pulmonary opacities. The associated hypoxemia may be severe enough to cause acute respiratory failure. Underlying aetiologies are numerous and variable, particularly in relation to the underlying immune status of the host. Various histopathological entities may be responsible for AIP although diffuse alveolar damage is the predominant pattern. The diagnostic approach to a patient presenting with AIP is to try to determine the most likely underlying histopathological pattern and to search for a precise aetiology. It relies mainly on a meticulous clinical evaluation and accurate biological investigation, essentially guided by the results of bronchoalveolar lavage performed in an area identified by abnormalities on high resolution computed tomography of the lungs. Initial therapeutic management includes symptomatic measures, broad-spectrum antibiotic treatment adapted to the clinical context, frequently combined with systemic corticosteroid therapy.

  9. Desquamative alveolar disease (desquamative interstitial pneumonia): case report 1

    PubMed Central

    Cruz, Edgardo; Rodriguez, Jaime; Lisboa, Carmen; Ferretti, Ricardo

    1969-01-01

    Desquamative interstitial pneumonia is a disease characterized by massive alveolar cell proliferation and desquamation with sparse interstitial involvement. The reported case shows an unusually widespread radiographic reticulo-nodular image and abundant alveolar cells in the sputum. Functional studies reveal the expected diffusion defect with practically normal mechanical properties of the lung, in contrast with interstitial fibrosing lung diseases. On the basis of the pathological findings, especially the behaviour of alveolar cells, the individuality of this disease is discussed. We think that it is different from other diseases classed as varieties of a single disease or as different entities under the names of primary interstitial fibrosis or chronic fibrosing alveolitis. Images PMID:5822250

  10. Acute interstitial pneumonia (AIP): relationship to Hamman-Rich syndrome, diffuse alveolar damage (DAD), and acute respiratory distress syndrome (ARDS).

    PubMed

    Mukhopadhyay, Sanjay; Parambil, Joseph G

    2012-10-01

    Acute interstitial pneumonia (AIP) is a term used for an idiopathic form of acute lung injury characterized clinically by acute respiratory failure with bilateral lung infiltrates and histologically by diffuse alveolar damage (DAD), a combination of findings previously known as the Hamman-Rich syndrome. This review aims to clarify the diagnostic criteria of AIP, its relationship with DAD and acute respiratory distress syndrome (ARDS), key etiologies that need to be excluded before making the diagnosis, and the salient clinical features. Cases that meet clinical and pathologic criteria for AIP overlap substantially with those that fulfill clinical criteria for ARDS. The main differences between AIP and ARDS are that AIP requires a histologic diagnosis of DAD and exclusion of known etiologies. AIP should also be distinguished from "acute exacerbation of IPF," a condition in which acute lung injury (usually DAD) supervenes on underlying usual interstitial pneumonia (UIP)/idiopathic pulmonary fibrosis (IPF).

  11. Pulmonary interstitial emphysema complicating pneumonia in an unventilated term infant.

    PubMed

    Lee, Hyun Seung; Im, Soo Ah

    2010-09-01

    A case of pulmonary interstitial emphysema with pneumothorax and pneumomediastinum complicating pneumonia in a 6-week-old infant is reported. The patient had no history of resuscitation, bag and mask ventilation, nasal continuous positive airway pressure or mechanical ventilation. PMID:20799076

  12. Case-based discussion: Lymphocytic interstitial pneumonia a rare presentation in an immunocompetent adult male

    PubMed Central

    Chitnis, Ajay; Vyas, Pradeep Kumar; Chaudhary, Priyanka; Ghatavat, Gaurav

    2015-01-01

    Lymphocytic interstitial pneumonia (LIP) is a rare form of interstitial lung disease usually associated with other systemic diseases; however, idiopathic cases are being reported. As per recent ATS/ERS 2013 guidelines, diagnostic criteria of clinical, radiological and histopathological for LIP is same as 2002 except some cystic changes on HRCT chest. Many cases diagnosed in the past as LIP now turn out to be NSIP; therefore as per new ATS/ERS classification whenever anybody report a case of LIP, NSIP should always be kept in mind as differential diagnosis. Here we present a case of LIP in an immunocompetent adult male presented with history of persistent dry cough and breathlessness on exertion, confirmed on HRCT chest and histopathologically, treated successfully with steroids. PMID:26628770

  13. Immune complexes, gallium lung scans, and bronchoalveolar lavage in idiopathic interstitial pneumonitis-fibrosis

    SciTech Connect

    Gelb, A.F.; Dreisen, R.B.; Epstein, J.D.; Silverthorne, J.D.; Bickel, Y.; Fields, M.; Border, W.A.; Taylor, C.R.

    1983-08-01

    We obtained results of lung immune complexes (LIC), circulating immune complexes (CIC), 48-hour gallium lung scans (scans), bronchoalveolar lavage (BAL), and pulmonary function tests in 20 patients with idiopathic interstitial pneumonitis-fibrosis. Sixteen patients had predominantly interstitial (13 cases UIP) and/or intraalveolar (3 cases DIP) cellular disease (group 1). Prior to corticosteroid therapy in group 1, scans were positive in 75 percent, CIC were elevated in 86 percent, LIC were present in 64 percent, and BAL was abnormal in 90 percent. Duration of follow-up after treatment was 3.5 +/- 1.0 year. In group 1 after treatment with corticosteroids in 13 patients and corticosteroids and penicillamine (three patients) and plasmapheresis (one patient), only four patients remain stable or improved. After corticosteroid therapy, elevated CIC returned to normal values despite progressive patient deterioration. In three patients, lung immune complexes were still detected after circulating immune complexes had returned to normal after corticosteroid therapy. In group 2 were four patients with fibrotic disease; scans and CIC were uniformly negative, LIC were weakly present in only one patient, and BAL was abnormal in all. Despite corticosteroid therapy, all have died or deteriorated. These results suggest that positive gallium lung scans, BAL, circulating immune complexes, and to a lesser extent, lung immune complexes are associated with the cellular phase of interstitial pneumonia, but do not reliably identify a corticosteroid-responsive group.

  14. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features.

    PubMed

    Fischer, Aryeh; Antoniou, Katerina M; Brown, Kevin K; Cadranel, Jacques; Corte, Tamera J; du Bois, Roland M; Lee, Joyce S; Leslie, Kevin O; Lynch, David A; Matteson, Eric L; Mosca, Marta; Noth, Imre; Richeldi, Luca; Strek, Mary E; Swigris, Jeffrey J; Wells, Athol U; West, Sterling G; Collard, Harold R; Cottin, Vincent

    2015-10-01

    Many patients with an idiopathic interstitial pneumonia (IIP) have clinical features that suggest an underlying autoimmune process but do not meet established criteria for a connective tissue disease (CTD). Researchers have proposed differing criteria and terms to describe these patients, and lack of consensus over nomenclature and classification limits the ability to conduct prospective studies of a uniform cohort.The "European Respiratory Society/American Thoracic Society Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease" was formed to create consensus regarding the nomenclature and classification criteria for patients with IIP and features of autoimmunity.The task force proposes the term "interstitial pneumonia with autoimmune features" (IPAF) and offers classification criteria organised around the presence of a combination of features from three domains: a clinical domain consisting of specific extra-thoracic features, a serologic domain consisting of specific autoantibodies, and a morphologic domain consisting of specific chest imaging, histopathologic or pulmonary physiologic features.A designation of IPAF should be used to identify individuals with IIP and features suggestive of, but not definitive for, a CTD. With IPAF, a sound platform has been provided from which to launch the requisite future research investigations of a more uniform cohort.

  15. Cytomegalovirus pneumonia in a patient with interstitial pneumonia and Nocardia asiatica presenting as cavitary lung lesions.

    PubMed

    Saraya, Takeshi; Ohkuma, Kosuke; Kikuchi, Ken; Tamura, Masaki; Honda, Kojiro; Yamada, Atsuko; Araki, Koji; Ishii, Haruyuki; Makino, Hiroshi; Takei, Hidefumi; Karita, Shin; Fujiwara, Masachika; Takizawa, Hajime; Goto, Hajime

    2013-01-01

    A 66-year-old man who suffered from an acute exacerbation of interstitial pneumonia developed a cavitary lesion after taking immunosuppressive drugs. He was diagnosed with cytomegalovirus (CMV) pneumonia. CMV was not thought to be the underlying cause of the cavitary lung lesions, as only six cases have been described thus far. However, this case clearly demonstrates that the development of cavitary lung lesions can be caused by CMV. Following CMV pneumonia, cavitary lesions again occurred in the patient's lungs that were thought to be the first case of cavitary lesions caused by Nocardia asiatica infection. PMID:23448771

  16. Intractable desquamative interstitial pneumonia in a tattooed man.

    PubMed

    Arai, Toru; Inoue, Yoshikazu; Hayashi, Seiji; Akira, Masanori; Yamamoto, Satoru; Travis, William D; Sakatani, Mitsunori

    2006-01-01

    A 20-year-old man with a 15 pack-year history of cigarette smoking had a tattoo outlined on his back with blue pigment. He noticed a dry cough and shortness of breath on exertion when the pigment of other colors was added at the age of 27. He visited our hospital two years later because of severe dyspnea. He was diagnosed with desquamative interstitial pneumonia by surgical lung biopsy. Steroid therapy with cessation of smoking was partially effective, however his disease worsened again and he died three and a half years after the diagnosis because of respiratory failure. PMID:17043377

  17. Diagnosis and classification of idiopathic pulmonary fibrosis.

    PubMed

    Kekevian, Alana; Gershwin, M Eric; Chang, Christopher

    2014-01-01

    Idiopathic pulmonary fibrosis is a difficult disease to diagnose. Idiopathic pulmonary fibrosis is a member of a class of diseases known as idiopathic interstitial pneumonias. Other members include nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and lymphocytic interstitial pneumonia. Usual interstitial pneumonia (UIP) is the pathological equivalent of idiopathic pulmonary fibrosis. Prior to 2011, the diagnosis was based on major and minor criteria, but because these criteria were not evidence based, the criteria were modified by consensus from the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society, and the Latin American Thoracic Association. These new criteria now include satisfying three core requirements, including exclusion of other possible cause of interstitial lung disease, specific findings of usual interstitial pneumonia on high resolution computed tomography, and a combination of "possible UIP" findings on high resolution computed tomography and UIP findings on lung biopsy. Idiopathic pulmonary fibrosis is a severe, progressive disease with limited treatment options, and exacerbations are associated with a high degree of morbidity and mortality.

  18. Acute interstitial pneumonia: radiographic and CT findings in nine patients.

    PubMed

    Primack, S L; Hartman, T E; Ikezoe, J; Akira, M; Sakatani, M; Müller, N L

    1993-09-01

    The radiologic findings were reviewed in nine patients with biopsy- or autopsy-proved acute interstitial pneumonia (AIP). All patients had bilateral air-space opacification on radiographs and bilateral, symmetric areas of ground-glass attenuation on computed tomographic (CT) scans. The areas of ground-glass attenuation had a patchy distribution in six patients (67%) and were diffuse in three patients. Air-space consolidation was seen at CT in six patients (67%) and involved mainly the lower lung zones in three patients and upper lung zones in one patient and was diffuse in two patients. A predominantly subpleural distribution of the consolidation was present in two patients. Eight of the nine patients died within 3 months of presentation. The authors conclude that the radiographic and CT features of AIP are similar to those of adult respiratory distress syndrome and represent acute alveolar damage. AIP differs from the more chronic forms of interstitial pneumonia in clinical presentation and in pathologic and radiologic findings.

  19. Clinical features and outcome of acute exacerbation of interstitial pneumonia associated with connective tissue disease.

    PubMed

    Toyoda, Yuko; Hanibuchi, Masaki; Kishi, Jun; Kawano, Hiroshi; Morizumi, Shun; Sato, Seidai; Kondo, Mayo; Takikura, Terumi; Tezuka, Toshifumi; Goto, Hisatsugu; Nishioka, Yasuhiko

    2016-01-01

    Acute exacerbation (AE) of interstitial lung disease is reported to be developed in not only idiopathic pulmonary fibrosis but also connective tissue disease-associated interstitial pneumonia (CTD-IP). As the significance of AE of CTD-IP has not been so widely recognized, its clinical feature is not fully elucidated. In the present study, we investigated the incidence, clinical features and outcome of AE of CTD-IP. We retrospectively reviewed admitted cases in our department with medical record from 2011 to 2015. Among 155 patients with CTD-IP, 10 (6.5%) cases developed AE (6 rheumatoid arthritis, 2 polymyositis/dermatomyositis, 1 systemic lupus erythematosus, 1 Sjögren syndrome), and one died of AE within 30 days. Median survival time after the onset of AE was 169 days in all 10 patients. The treatment with immunosuppressant just before AE onset might improve the prognosis of AE. The median survival time after the onset of AE was significantly longer in patients showing good response to corticosteroid compared with those with poor response to corticosteroid (805 days and 45 days, respectively) (p <0.05), suggesting that there are some cases in CTD-IP, showing the good response to corticosteroid even when AE was complicated. J. Med. Invest. 63: 294-299, August, 2016. PMID:27644575

  20. Acute interstitial pneumonia in siblings: a case report.

    PubMed

    Kwon, Seung Yeon; Kim, Jong Min; Sohn, Myung Hyun; Kim, Dong Soo; Kim, Myung Joon; Cho, Sang-Ho

    2008-06-01

    Acute interstitial pneumonia (AIP) is a rapidly progressive condition of unknown cause that occurs in a previously healthy individual and produces the histologic findings of diffuse alveolar damage. Since the term AIP was first introduced in 1986, there have been very few case reports of AIP in children. Here we present a case of AIP in a 3-yr-old girl whose other two siblings showed similar radiologic findings. The patient was confirmed to have AIP from autopsy showing histological findings of diffuse alveolar damage and proliferation of fibroblasts. Her 3-yr-old brother was also clinically and radiologically highly suspected as having AIP, and the other asymptomatic 8-yr-old sister was radiologically suspected as having AIP.

  1. [DIP (desquamative interstitial pneumonia): as a tobacco-associated disease -- case report].

    PubMed

    Sousa, Vitor; Carvalho, Lina

    2004-01-01

    DIP (desquamative interstitial pneumonia) is an interstitial lung disease with diffuse and uniform accumulation of alveolar macrophages. There is a strong association with tobacco since 90% of the patients are smokers. The interstitial lung diseases related to tobacco are diverse and include tumours, emphysema, chronic bronchitis, RBILD (Respiratory Bronchilites associated Interstitial Lung Disease), DIP and Langerhans Cell Histiocitosis. The authors present a case of DIP. A brief theorycal revision and discussion of a case is made facing the association with tobacco.

  2. Nonspecific interstitial pneumonia overlaps organizing pneumonia in lung-dominant connective tissue disease.

    PubMed

    Li, Xue-Ren; Peng, Shou-Chun; Wei, Lu-Qing

    2015-01-01

    Here, we reported two cases of nonspecific interstitial pneumonia overlap organizing pneumonia (NSIP/OP) with lung-dominant connective tissue disease (LD-ILD). The first case is a patient with hands of chapped skin, right-sided pleuritic chest discomfort, weakness, positive ANA and antibodies to Ro/SS-A (+++) and Ro-52 (++). In the second case, there were Reynaud's disease, and nucleolus-ANA increased (1:800). Chest high resolution CT scan in both cases showed ground-glass opacifications, predominantly in basal and subpleural region and the pathologic manifestation were correlated with NSIP/OP, which were previously discovered in Sjogren syndrome, PM/DM and other rheumatic diseases. The two cases of NSIP/OP with LD-CTD we reported expand disease spectrum of NSIP/OP pathological types in ILD. However, it is necessary to process large-scale studies.

  3. [Idiopathic bronchiolitis obliterans organizing pneumonia complicated by transitory cystic lesion in the healing stage].

    PubMed

    Nakayama, T; Hachisuka, H; Akusawa, H; Chin, T; Kayama, Y; Nomura, Y; Horie, T

    1999-11-01

    A 21-year-old man was admitted to our hospital because chest X-ray films disclosed infiltrative shadows indicative of Mycoplasma pneumonia. He experienced fever and dry cough for 2 weeks. Chest X-ray findings showed ground-glass shadows in the lower fields of both lungs. The patient was not responsive to antibiotic therapy (PAPM/BP 1 g/day and CLDM 1,200 mg/day); dyspnea advanced rapidly and spikes of fever persisted. On hospital day 5, chest computed tomographic (CT) films disclosed interstitial shadows in all lower lung fields with dense infiltration. A transbronchial lung biopsy (TBLB) was performed on day 7, and TBLB specimens demonstrated infiltration of mononuclear cells in alveolar septa and organizing exudate in alveolar ducts with polypoid granulation tissue. Bronchoalveolar lavage fluid findings revealed an increase in the total cell count and the percentage of lymphocytes. The CD 4/CD 8 ratio was normal. The findings of other laboratory tests ruled out drug-induced lung disease, infectious disease, and collagen disease. Idiopathic bronchiolitis obliterans with organizing pneumonia (BOOP) was diagnosed. Corticosteroid therapy (methyl prednisolone: 500 mg/day) was started. After 2 weeks of treatment (prednisolone: 30 mg/day), the dyspnea and fever disappeared. Chest CT films showed that the interstitial shadows had largely resolved, but that a large cystic lesion had formed rapidly in the right lower lung field (right S 6). To the best of our knowledge, no cases of BOOP complicated by cystic lesions in the healing stage have been reported to date. We speculated that polypoid granulation in a bronchiole had given rise to a check-valve mechanism. After 2 months of treatment (prednisolone: 15 mg/day), the cystic lesion disappeared. We concluded that the bronchiolar lesion of polypoid granulation had resolved in response to therapy, thus facilitating air-way communication and the release of air from the cyst. PMID:18217316

  4. A case of acute interstitial pneumonia indistinguishable from bronchiolitis obliterans organizing pneumonia/cryptogenic organizing pneumonia: high-resolution CT findings and pathologic correlation.

    PubMed

    Ichikado, K; Johkoh, T; Ikezoe, J; Yoshida, S; Honda, O; Mihara, N; Nakamura, H; Tsujimura, T; Suga, M; Ando, M

    1998-01-01

    We report a case of histologically proved acute interstitial pneumonia (AIP) with subacute onset whose high-resolution CT (HRCT) findings were indistinguishable from those of bronchiolitis obliterans organizing pneumonia (BOOP)/cryptogenic organizing pneumonia (COP). The HRCT findings were air-space consolidation with air-bronchiologram associated with little ground-glass attenuation, and nodules. Some cases of AIP present HRCT findings indistinguishable from those of BOOP/COP.

  5. Pulmonary besnoitiasis in captive maras (Dolichotis patagonum) associated with interstitial pneumonia.

    PubMed

    Rico-Hernández, G; Juan-Sallés, C; Garner, M M; Barr, B C

    2004-07-01

    Six captive maras (Dolichotis patagonum) were found to have cysts consistent with Besnoitia in the lungs by light microscopy and electron microscopy. Three were juveniles that died with no premonitory signs during a 17-month period. The most prominent finding consisted of severe, subacute, diffuse interstitial pneumonia with syncytia and Besnoitia cysts. The severity of pneumonia correlated with the number of cysts and the presence of lytic cysts, often surrounded by chronic granulomatous inflammation. Disseminated infection was observed in one of these maras. The three other maras died of unrelated conditions and had a few Besnoitia cysts in their lungs with mild or no inflammation associated. This is the first report of besnoitiasis in maras and of its association with fatal interstitial pneumonia in any species. Although other agents may have caused the interstitial pneumonia described here, it is possible that zoites released from lytic cysts were involved in the development of this lesion. PMID:15232142

  6. [Autopsy case of pulmonary zygomycosis and pneumocystis pneumonia in a patient with interstitial pneumonia treated by corticosteroid therapy].

    PubMed

    Mukasa, Yosuke; Ichiyasu, Hidenori; Akaike, Kimitaka; Okamoto, Shinichiro; Komohara, Yoshihiro; Kohrogi, Hirotsugu

    2010-11-01

    We report a 75-year-old man with pneumoconiosis, interstitial pneumonia and diabetes mellitus, who had carcinoma of the buccal mucosa. After resection of the carcinoma, he was given corticosteroids for the deterioration of interstitial pneumonia, but 38 days after initiating steroid therapy, he was admitted to our hospital with severe hypoxemia and multiple cavitary lesions superimposed on ground-glass attenuation in both lung fields. The Aspergillus antigen was positive in his serum and examination of his bronchoalveolar lavage (BAL) fluid revealed mixed infections with filamentous fungus and Pneumocystis jirovecii. Pulmonary aspergillosis and pneumocystis pneumonia with an immunocompromised state was diagnosed, and voriconazole, sulfamethoxazole-trimethoprim and high-dose corticosteroids were given. At 20 days after these treatments he developed bloody sputum, and Cunninghamella bertholletiae was isolated from the BAL fluid obtained at admission. A diagnosis of pulmonary zygomycosis was finally established. Amphotericin B therapy was started, and the dose was increased thereafter. Despite intensive treatment he died 18 days later. Histological examination of lung tissue obtained at autopsy showed invasive growth of zygomycetes in the necrotic tissue and the cavity wall. To the best of our knowledge, this is the first report of concurrent Cunninghamella bertholletiae and Pneumocystis jirovecii infection during steroid therapy for interstitial pneumonia. PMID:21141065

  7. Recent Treatment of Interstitial Lung Disease with Idiopathic Inflammatory Myopathies

    PubMed Central

    Kawasumi, Hidenaga; Gono, Takahisa; Kawaguchi, Yasushi; Yamanaka, Hisashi

    2015-01-01

    Interstitial lung disease (ILD) is a prognostic factor for poor outcome in polymyositis (PM)/dermatomyositis (DM). The appropriate management of ILD is very important to improve the prognosis of patients with PM/DM. ILD activity and severity depend on the disease subtype. Therefore, clinicians should determine therapeutic strategies according to the disease subtype in each patient with PM/DM. Anti–melanoma differentiation-associated gene 5 antibody and hyperferritinemia predict the development and severity of rapidly progressive (RP) ILD, particularly in East Asian patients. Combination therapy with corticosteroids, intravenous cyclophosphamide pulse, and calcineurin inhibitors should be administered in RP-ILD. In contrast, patients with anti–aminoacyl-tRNA synthetase (ARS) show better responses to corticosteroids alone. However, ILDs with anti-ARS often display disease recurrence or become refractory to corticosteroid monotherapy. Recent studies have demonstrated that the administration of tacrolimus or rituximab in addition to corticosteroids may be considered in ILD patients with anti-ARS. Large-scale, multicenter randomized clinical trials should be conducted in the future to confirm that the aforementioned agents exhibit efficacy in ILD patients with PM/DM. The pathophysiology of ILD with PM/DM should also be elucidated in greater detail to develop effective therapeutic strategies for patients with ILD in PM/DM. PMID:26279636

  8. Giant-cell interstitial pneumonia and hard-metal pneumoconiosis. A clinicopathologic study of four cases and review of the literature

    SciTech Connect

    Ohori, N.P.; Sciurba, F.C.; Owens, G.R.; Hodgson, M.J.; Yousem, S.A.

    1989-07-01

    We report four cases of giant-cell interstitial pneumonia that occurred in association with exposure to hard metals. All patients presented with chronic interstitial lung disease and had open-lung biopsies that revealed marked interstitial fibrosis, cellular interstitial infiltrates, and prominent intraalveolar macrophages as well as giant cells displaying cellular cannibalism. We also review the literature to determine the sensitivity and specificity of giant-cell interstitial pneumonia for hard-metal pneumoconiosis. Although hard-metal pneumoconiosis may take the form of usual interstitial pneumonia, desquamative interstitial pneumonia, and giant-cell interstitial pneumonia, the finding of giant-cell interstitial pneumonia is almost pathognomonic of hard-metal disease and should provoke an investigation of occupational exposure. 25 references.

  9. [Interstitial lung diseases. The pattern is important].

    PubMed

    Fink, L

    2014-11-01

    Interstitial lung diseases (ILDs) comprise a number of rare entities with an estimated incidence of 10-25 per 100,000 inhabitants but the incidence greatly increases beyond the age of 65 years. The prognosis depends on the underlying cause. The fibrotic disorders show a set of radiological and histopathological patterns that are distinct but not entirely specific. In the absence of a clear clinical picture and consistent high resolution computed tomography (HRCT) findings, patients are advised to undergo surgical lung biopsies from two or three lung lobes (or transbronchial biopsies) to determine the histopathological pattern. The ILDs are differentiated into disorders of known causes (e.g. collagen vascular disease, drug-related), idiopathic interstitial pneumonia (IIP), granulomatous ILDs (e.g. sarcoidosis) and other forms of ILD (e.g. Langerhans' cell histiocytosis). The IIPs encompass idiopathic pulmonary fibrosis (IPF), non-specific interstitial pneumonia, desquamative interstitial pneumonia, respiratory bronchiolitis-interstitial lung disease, cryptogen organizing pneumonia, lymphocytic interstitial pneumonia and acute interstitial pneumonia. Additionally, a category of unclassified interstitial pneumonia exists. The pathologist has to recognize and address the histopathological pattern. In a multidisciplinary discussion the disorder is allocated to a clinicopathological entity and the histopathological pattern plays a major role in the classification of the entity. Recognition of the underlying pattern and the respective histopathological differential diagnoses is important as the therapy varies depending on the cause and ranges from elimination of the stimulus (if possible) to antifibrotic drug therapy up to preparation for lung transplantation.

  10. X-linked agammaglobulinemia combined with juvenile idiopathic arthritis and invasive Klebsiella pneumoniae polyarticular septic arthritis.

    PubMed

    Zhu, Zaihua; Kang, Yuli; Lin, Zhenlang; Huang, Yanjing; Lv, Huoyang; Li, Yasong

    2015-02-01

    X-linked agammaglobulinemia (XLA) is a primary immunodeficiency disease caused by mutations in the Bruton's tyrosine kinase (BTK) gene. XLA can also present in combination with juvenile idiopathic arthritis (JIA), the major chronic rheumatologic disease in children. We report herein the first known case of a juvenile patient diagnosed with XLA combined with JIA that later developed into invasive Klebsiella pneumoniae polyarticular septic polyarthritis. An additional comprehensive review of XLA combined with JIA and invasive K. pneumoniae septic arthritis is also presented. XLA was identified by the detection of BTK mutations while the diagnosis of JIA was established by clinical and laboratory assessments. Septic arthritis caused by invasive K. pneumoniae was confirmed by culturing of the synovia and gene detection of the isolates. Invasive K. pneumoniae infections can not only result in liver abscesses but also septic arthritis, although this is rare. XLA combined with JIA may contribute to invasive K. pneumoniae infection.

  11. Significance of Myositis Autoantibody in Patients with Idiopathic Interstitial Lung Disease

    PubMed Central

    Song, Ju Sun; Hwang, Jiwon; Cha, Hoon-Suk; Jeong, Byeong-Ho; Suh, Gee Young; Chung, Man Pyo

    2015-01-01

    Purpose Some patients with interstitial lung disease (ILD) related to connective tissue disease (CTD) have a delayed diagnosis of the underlying CTD when the ILD is categorized as idiopathic. In this study, we evaluated the frequency of myositis autoantibodies in patients diagnosed with idiopathic ILD and investigated the clinical significance stemming from the presence of the antibodies. Materials and Methods A total 32 patients diagnosed with idiopathic ILD were enrolled in this study. We analyzed a panel of 11 myositis autoantibody specificities in the patients using a line blot immunoassay. Then, we divided them into myositis autoantibody-positive and -negative groups and compared the clinical features and laboratory data between the two groups. Results Of the 32 idiopathic ILD patients, 12 patients had myositis autoantibodies encompassing 9 specificities, except for anti-Mi-2 and anti-PM-Scl 100 (12/32, 38%). Anti-synthetase autoantibodies including Jo-1, EJ, OJ, PL-7, and PL-12 were present in 7 patients (7/32, 22%). The group with myositis autoantibodies presented more frequently with the symptom of mechanic's hand and showed abnormal pulmonary function test results with low forced vital capacity, diffusing capacity for carbon monoxide, total lung capacity, and high lactate dehydrogenase values in blood when compared with the group without myositis antibodies. Conclusion We strongly suggest that patients undergo an evaluation of myositis autoantibodies, if they are diagnosed with idiopathic ILD in the presence of clinical characteristics including mechanic's hand, arthralgia, and autoantibodies which are insufficient to make a diagnosis of a specific CTD category. PMID:25837172

  12. Development of interstitial pneumonia in a patient with rheumatoid arthritis induced by isoniazid for tuberculosis chemoprophylaxis.

    PubMed

    Migita, Kiyoshi; Umeno, Tetsuya; Miyagawa, Kana; Izumi, Yasumori; Sasaki, Eisuke; Kakugawa, Tomoyuki; Ito, Masahiro; Kinoshita, Akitoshi; Miyashita, Taichiro

    2012-05-01

    Here, we report a 56-year-old patient with rheumatoid arthritis (RA) who had been treated with methotrexate and sulfuasalazine, but the disease activity remained high. Therefore, we planned TNF-blocker treatment for this patient. A tuberculin skin test was positive, we started anti-tuberculosis (TB) chemoprophylaxis with isoniazid (INH). However, liver dysfunction was appeared after 2 weeks from the start of INH. Therefore, we discontinued INH transiently and tried the desensitization of INH. However, interstitial pneumonia was developed 2 weeks after the re-start of INH, we decided to stop the INH prophylaxis. Interstitial pneumonia was improved by corticosteroid treatments. This case report shows that INH-induced IP can be occurred during the course of anti-TB chemoprophylaxis in patients with RA.

  13. Biomarkers and Autoantibodies of Interstitial Lung Disease with Idiopathic Inflammatory Myopathies

    PubMed Central

    Yoshifuji, Hajime

    2015-01-01

    Various autoantibodies are seen in idiopathic inflammatory myopathies. Among myositis-specific antibodies, anti-aminoacyl-tRNA synthetase and anti-melanoma differentiation-associated protein 5 (MDA5) antibodies are associated with interstitial lung disease (ILD). Anti-MDA5 antibodies are associated with dermatomyositis (DM) or clinically amyopathic DM complicated with rapidly progressive ILD. In anti-MDA5-positive patients, a random ground-glass attenuation pattern is a characteristic finding of ILD in chest high-resolution computed tomography. Conversely, anti-aminoacyl-tRNA synthetase antibodies are not associated with rapidly progressive ILD but with chronic ILD. DM or clinically amyopathic DM patients with anti-MDA5, and characteristic high-resolution computed tomography findings are highly likely to have devastating ILD and need aggressive treatment. PMID:27081322

  14. Sorafenib-induced acute interstitial pneumonia in patients with advanced hepatocellular carcinoma: report of three cases.

    PubMed

    Takeda, Haruhiko; Nishikawa, Hiroki; Iguchi, Eriko; Matsuda, Fumihiro; Kita, Ryuichi; Kimura, Toru; Osaki, Yukio

    2012-01-01

    Little is known about acute interstitial pneumonia (AIP) induced by sorafenib therapy in patients with advanced hepatocellular carcinoma (HCC). Here, we present three patients with advanced HCC who developed AIP during sorafenib therapy, with fatal complications in two cases. Case 1 was a 76-year-old man who developed dyspnea. Chest CT showed interstitial pneumonia. Sorafenib was discontinued immediately, and prednisolone was started. His pneumonia resolved. A drug-induced lymphocyte stimulation test for sorafenib was positive. Case 2 was a 75-year-old man and case 3 was a 77-year-old man, both of whom developed high-grade fever and hypoxemia during sorafenib therapy, and were diagnosed with AIP. In spite of high-dose steroid therapy, their respiratory failure worsened and both patients died. In all three cases, serum KL-6 or surfactant protein D concentrations were elevated, and blood and sputum cultures did not grow pathogens. All three patients were smokers with restrictive lung disease on preoperative respiratory function testing, but did not have respiratory symptoms before sorafenib therapy. The clinical features of these three cases suggest that male gender, older age, smoking history, and lung disease are associated with acute sorafenib-induced AIP in patients with advanced HCC.

  15. [Interstitial lung disease in rheumatoid arthritis].

    PubMed

    Froidevaux-Janin, Sylvie; Dudler, Jean; Nicod, Laurent P; Lazor, Romain

    2011-11-23

    Interstitial lung disease (ILD) is found in up to 30% of patients with rheumatoid arthritis (RA) and is clinically manifest in 5 to 10%, resulting in significant morbidity and mortality. The most frequent histopathological forms are usual interstitial pneumonia and nonspecific interstitial pneumonia. Another recently described presentation is combined pulmonary fibrosis and emphysema. Similarly to idiopathic pulmonary fibrosis, acute exacerbation of ILD may occur in RA and is associated with severe prognosis. Smoking is a known risk factor of RA and may also play a role in the pathogenesis of RA-associated ILD, in combination with genetic and immunologic mechanisms. Several treatments of RA may also lead to drug-induced ILD.

  16. Successful treatment of steroid-resistant methotrexate-induced interstitial pneumonia with peripherally administered ulinastatin.

    PubMed

    Yamazaki, Hayato; Nagasaka, Kenji

    2011-02-01

    A 76-year-old woman with rheumatoid arthritis who had been taking methotrexate (MTX) for 9 months was admitted because of acute respiratory failure. A chest radiograph revealed diffuse ground-glass attenuation. MTX-induced interstitial pneumonia (IP) was strongly suspected. Her respiratory failure worsened in spite of steroid pulse therapy. Intravenous administration of ulinastatin, however, dramatically improved her clinical condition. The second ulinastatin treatment was also effective. This case suggests that peripherally administered ulinastatin may be effective for steroid-resistant MTX-induced IP.

  17. [Primary Sjögren's syndrome with lymphocytic interstitial pneumonia and pulmonary multiple cystic lesions].

    PubMed

    Hayasaka, S; Fujino, N; Yoshinaga, T; Kiyama, T; Maemoto, H; Outsuka, Y

    1999-10-01

    We report a case of primary Sjögren's syndrome with lymphocytic interstitial pneumonia and multiple cystic lesions. The patient was a 64-year-old woman. Abnormal chest shadows were detected by x-ray and computed tomographic (CT) examinations. The patient had no family history of disease and had never smoked. She had complained of dryness in the eyes and mouth for about 10 years. Laboratory tests were positive for anti-nuclear antigen, anti-SS-A antigen, and anti-SS-B antigen. Sialography revealed marked destruction of the salivary glands, yielding a diagnosis of Sjögren's syndrome. Chest X-ray films and CT scans showed multiple cystic lesions in both lungs, measuring from a few mm to 3 cm in diameter, as well as fine centrilobular nodules. Slight anemia and hyper gamma globlinemia were also detected. Pulmonary function tests showed mild obstructive disturbance. Bronchoalveolar lavage analysis disclosed an elevated lymphocytic fraction (28.6%), but transbronchial lung biopsy provided no adequate specimens for diagnosis. Thoracoscopic lung biopsy specimens demonstrated marked infiltration of lymphocytes and histiocytes through the interstitium of alveolar walls and peri-bronchovascular sheath, with some lymphoid follicles. The overall appearance was compatible with lymphocytic interstitial pneumonia. The cysts themselves were nonspecific, and no cellular infiltration was noted in the cyst walls. Because of the predominantly peribronchial distribution of the lesions, we suspected that the cysts were formed by the check valve mechanism. However, no definitive evidence was obtained. PMID:10586590

  18. Association of bovine respiratory syncytial virus with atypical interstitial pneumonia in feedlot cattle.

    PubMed

    Collins, J K; Jensen, R; Smith, G H; Flack, D E; Kerschen, R; Bennett, B W; Jones, R L; Alexander, A F

    1988-07-01

    Thirty-three cattle with fatal respiratory tract disease were examined for gross and histologic lesions and for the presence of viral and bacterial agents in the lungs. Fifteen cattle had lesions characteristic of atypical interstitial pneumonia (AIP), and 18 had other respiratory tract diseases, including infectious bovine rhinotracheitis, shipping fever pneumonia, bronchopneumonia, pulmonary abscess, and edema of the trachea. Gross necropsy findings in the cattle with AIP were uncollapsed and emphysematous lungs; histopathologic findings included interstitial edema, thickening of alveolar walls, hyaline membrane formation, and hyperplasia of type-II pneumonocytes. The infective agents found in the lungs of the 33 cattle included bovine respiratory syncytial virus, bovine herpesvirus type 1, Pasteurella sp, mycoplasmas, and Corynebacterium pyogenes. Bovine respiratory syncytial virus was detected by use of immunofluorescence and immunoperoxidase on lung tissue sections; bovine herpesvirus type 1 was detected by these techniques and by isolation of the virus. Bovine respiratory syncytial virus was significantly (P = 0.01) associated with lesions of AIP (11 of 15), compared with those of other respiratory tract diseases (5 of 18).

  19. Modeling pulmonary fibrosis by abnormal expression of telomerase/apoptosis/collagen V in experimental usual interstitial pneumonia.

    PubMed

    Parra, E R; Pincelli, M S; Teodoro, W R; Velosa, A P P; Martins, V; Rangel, M P; Barbas-Filho, J V; Capelozzi, V L

    2014-07-01

    Limitations on tissue proliferation capacity determined by telomerase/apoptosis balance have been implicated in pathogenesis of idiopathic pulmonary fibrosis. In addition, collagen V shows promise as an inductor of apoptosis. We evaluated the quantitative relationship between the telomerase/apoptosis index, collagen V synthesis, and epithelial/fibroblast replication in mice exposed to butylated hydroxytoluene (BHT) at high oxygen concentration. Two groups of mice were analyzed: 20 mice received BHT, and 10 control mice received corn oil. Telomerase expression, apoptosis, collagen I, III, and V fibers, and hydroxyproline were evaluated by immunohistochemistry, in situ detection of apoptosis, electron microscopy, immunofluorescence, and histomorphometry. Electron microscopy confirmed the presence of increased alveolar epithelial cells type 1 (AEC1) in apoptosis. Immunostaining showed increased nuclear expression of telomerase in AEC type 2 (AEC2) between normal and chronic scarring areas of usual interstitial pneumonia (UIP). Control lungs and normal areas from UIP lungs showed weak green birefringence of type I and III collagens in the alveolar wall and type V collagen in the basement membrane of alveolar capillaries. The increase in collagen V was greater than collagens I and III in scarring areas of UIP. A significant direct association was found between collagen V and AEC2 apoptosis. We concluded that telomerase, collagen V fiber density, and apoptosis evaluation in experimental UIP offers the potential to control reepithelization of alveolar septa and fibroblast proliferation. Strategies aimed at preventing high rates of collagen V synthesis, or local responses to high rates of cell apoptosis, may have a significant impact in pulmonary fibrosis. PMID:24919172

  20. Increased prevalence of high anti-Cladosporium antibody titers in interstitial lung diseases.

    PubMed

    Watanuki, Zenta; Okada, Shinji; Chiba, Shigeki; Kamei, Katsuhiko; Suzuki, Yasuko; Yamada, Norihiro

    2012-01-01

    Interstitial lung diseases (ILDs) represent a large group of different diseases, with a large part comprising idiopathic interstitial pneumonias. Differentiating hypersensitivity pneumonitis (HP), especially its chronic form and other ILDs, is difficult because of similarities in radiological manifestation and clinical course, and the difficulty of identifying causative antigens. We recently experienced a patient with Cladosporium-induced chronic HP that developed in a household environment, but the cause had been misdiagnosed as idiopathic interstitial pneumonia for several years. This case highlighted the need for measures differentiating HP from idiopathic interstitial pneumonia. In this study, we examined fungal exposure in ILDs using an antibody titer in serum to identify possible fungus-related HP. We measured the antibody titer to Cladosporium spp. in 34 patients with various ILDs, 17 patients with bronchial asthma, and 21 control subjects using an immunofluorescence assay. ILDs included HP (5 patients), idiopathic interstitial pneumonias (21 patients), and ILDs with collagen vascular diseases (8 patients). Results showed a significantly higher tendency for high anti-Cladosporium antibody titers in ILD groups (12 patients out of 34 patients), compared to patients with bronchial asthma (0/17) or control subjects (0/21). This increase in antibody titers was observed not only in patients with HP, but also in those with idiopathic interstitial pneumonias and those exhibiting collagen vascular diseases with ILDs. This report highlights the pathogenic role of fungal antigens in various ILDs. In conclusion, fungi commonly observed in our living environment such as Cladosporium could be involved in the development of ILDs.

  1. Acute exacerbations of fibrotic interstitial lung disease.

    PubMed

    Churg, Andrew; Wright, Joanne L; Tazelaar, Henry D

    2011-03-01

    An acute exacerbation is the development of acute lung injury, usually resulting in acute respiratory distress syndrome, in a patient with a pre-existing fibrosing interstitial pneumonia. By definition, acute exacerbations are not caused by infection, heart failure, aspiration or drug reaction. Most patients with acute exacerbations have underlying usual interstitial pneumonia, either idiopathic or in association with a connective tissue disease, but the same process has been reported in patients with fibrotic non-specific interstitial pneumonia, fibrotic hypersensitivity pneumonitis, desquamative interstitial pneumonia and asbestosis. Occasionally an acute exacerbation is the initial manifestation of underlying interstitial lung disease. On biopsy, acute exacerbations appear as diffuse alveolar damage or bronchiolitis obliterans organizing pneumonia (BOOP) superimposed upon the fibrosing interstitial pneumonia. Biopsies may be extremely confusing, because the acute injury pattern can completely obscure the underlying disease; a useful clue is that diffuse alveolar damage and organizing pneumonia should not be associated with old dense fibrosis and peripheral honeycomb change. Consultation with radiology can also be extremely helpful, because the fibrosing disease may be evident on old or concurrent computed tomography scans. The aetiology of acute exacerbations is unknown, and the prognosis is poor; however, some patients survive with high-dose steroid therapy.

  2. High-flow nasal cannula oxygen therapy for acute exacerbation of interstitial pneumonia: A case series.

    PubMed

    Horio, Yukihiro; Takihara, Takahisa; Niimi, Kyoko; Komatsu, Masamichi; Sato, Masako; Tanaka, Jun; Takiguchi, Hiroto; Tomomatsu, Hiromi; Tomomatsu, Katsuyoshi; Hayama, Naoki; Oguma, Tsuyoshi; Aoki, Takuya; Urano, Tetsuya; Takagi, Atsushi; Asano, Koichiro

    2016-03-01

    We report 3 cases (all men, age: 69-81 years) of acute exacerbation of interstitial pneumonia (AEIP) that were successfully treated with a high-flow nasal cannula (HFNC), which delivers heated, humidified gas at a fraction of inspired oxygen (FIO2) up to 1.0 (100%). Oxygenation was insufficient under non-rebreathing face masks; however, the introduction of HFNC with an FIO2 of 0.7-1.0 (flow rate: 40 L/min) improved oxygenation and was well-tolerated until the partial pressure of oxygen in blood/FIO2 ratio increased (between 21 and 26 days). Thus, HFNC might be an effective and well-tolerated therapeutic addition to the management of AEIP. PMID:26879483

  3. Giant cell interstitial pneumonia in a 15-year-old boy.

    PubMed

    Kakugawa, Tomoyuki; Mukae, Hiroshi; Nagata, Towako; Ishii, Hiroshi; Kaida, Hideyuki; Hayashi, Tomayoshi; Suematsu, Takashi; Kadota, Jun-ichi; Kohno, Shigeru

    2002-11-01

    Giant cell interstitial pneumonia (GIP) is a very uncommon respiratory disease. We report a juvenile patient with GIP aged 15 years. Although he has a negative past history of direct exposure to hard metals, we could not exclude possible exposure in very small amounts through his parents. Microscopic examination of lung biopsy specimen obtained by video-assisted thoracoscopy revealed marked cellular interstitial infiltrates and prominent intraalveolar macrophages as well as giant cells showing cellular cannibalism. Analysis of the biopsied lung tissue for cobalt and tungsten was negative. Clinical symptoms, laboratory, and radiological findings improved markedly after treatment with corticosteroids. To our knowledge, only eleven cases of GIP have been reported in Japan. Although possible exposure to hard metals was identified in 9 of the 11 reported cases, there is no clear dose-dependent relationship with onset and prognosis. The average age at onset was 46.2+/-15.0 years. Our patient is the youngest case of GIP reported in the world. PMID:12487179

  4. [Case of acute interstitial pneumonia that responded to therapy but relapsed six months later].

    PubMed

    Isobe, Zen; Suga, Tatsuo; Hamaguchi, Shigeto; Yamaguchi, Shouzaburou; Hara, Kenichirou; Aoki, Fumiaki; Aoki, Nozomi; Aoyagi, Kana; Ueno, Manabu; Maeno, Toshitaka; Kasiwabara, Kenji; Kurabayashi, Masahiko; Kawabata, Yoshinori

    2007-10-01

    A 66-year-old man was admitted because of general fatigue. A chest computed tomography showed bilateral alveolar consolidation and ground glass opacities. Although we treated him with broad-spectrum antibiotics, his symptoms and chest image findings did not improve. Thoracoscopic lung biopsy (rS2, S9) was performed. The specimens showed obstructive type intraluminar organization and interstitial inflammatory thickening. Membranous organization was seen in a limited area. The etiology of the illness could not be identified. We diagnosed acute interstitial pneumonia (AIP) because the specimens showed diffuse alveolar damage pattern (DAD/P) and because of unknown etiology. The symptoms and chest image findings were improved on treatment with corticosteroid and cyclophosphamide. However, he was readmitted because of dyspnea 6 months later after the thoracoscopic lung biopsy. Chest computed tomography showed bilateral diffuse ground glass opacities and reticular opacities in both lower lobes. We employed mechanical ventilation, antibiotics, sivelestat sodium hydrate and steroid pulse therapy, but he died without any response to treatment. The findings of autopsy revealed DAD/P accompanied by a new lesion mainly composed of membranous organization and hyaline membrane. We believe this case is valuable when considering the variety of responses to treatment of AIP and prognosis.

  5. A fatal case of acute interstitial pneumonia (AIP) in a woman affected by glioblastoma.

    PubMed

    Balzarini, Laura; Mancini, Chiara; Marvisi, Maurizio

    2014-03-01

    This report presents the case of a 67-year-old woman affected by glioblastoma. After a few days of adjuvant therapy with temozolomide and prophylaxis with trimetrophin-sulfamethoxazolo to prevent Pneumocystis Jiroveci, she had progressive and rapid worsening of symptoms with weakness, dyspnea and orthopnea. She had peripheral edema and proximal hyposthenia of the lower limbs. Chest CT showed bilateral ground-glass opacities and laboratory exams revealed hypoxemia and hypocapnia, an initial reduction in platelet and white blood cells, and an elevation of LDH, AST, ALT, and active urinary sediment. Blood cultures, bronchoalveolar lavage (BAL) data and transbronchial biopsy showed no infections, and in particular no evidence of Pneumocystis Jiroveci pneumonia. Histological examination revealed a typical pattern of AIP. She was treated with broad-spectrum antibiotics and high-dose steroids. The symptoms worsened and respiratory failure required mechanical ventilation. The pneumonia was not responsive to medical or invasive care. She died after ten days of hospitalization. At present very little can be found in the literature about lung toxicity caused by temozolomide. This case can be added as a new report describing this risk. The combination therapy with temozolamide and trimetophin-sulfamethoxazolo could have a synergistic action inducing various forms of pulmonary toxicity. ESTABLISHED FACTS: Acute interstitial pneumonia is a common manifestation of lung toxicity caused by drugs. The clinical course is favorable with a good response to corticosteroids. NOVEL INSIGHT: This is the first fatal case of lung toxicity caused by Temozolomide. Clinicians must be aware that a combination therapy including trimetophin-sulfamethoxazolo could have a synergistic action in inducing pulmonary toxicity.

  6. [A case of recurrent colon cancer with angina pectoris and interstitial pneumonia during cetuximab therapy with death by carcinomatous lymphangiosis].

    PubMed

    Shibahara, Hiroaki; Kuze, Shingo; Kyokane, Takanori; Takamizawa, Junichi; Nakamura, Hayato; Morikawa, Syuji; Hayashi, Eijiro; Kinoshita, Mana; Baba, Satoshi

    2010-11-01

    The case was a man in his 60s with no past history of heart and lung. Chest tightness was felt during the first course of cetuximab therapy for recurrent colon cancer. He was diagnosed as having vasospastic angina, and administration of vasodilatation agents was done. After the therapy, no chest pain attack was seen. Chemotherapy was continued. After 3 courses, fever elevation, chest tightness and dyspnea were seen. Chest X-ray and CT revealed diffuse interstitial pneumonia in bilateral lung. Although steroid pulse therapy and intensive therapy with mandatory ventilation were performed, he died of respiratory failure. Pathological findings of autopsy revealed remarkable metastasis of cancer cells to the bilateral lungs accompanied chiefly with carcinomatous lymphangiosis. Furthermore, acute and subacute interstitial pneumonia with diffuse alveolar damage were seen in the background of the lungs. Cardiopulmonary disorder as well as skin disorder should be considered as possible adverse events of cetuximab therapy.

  7. A case of catastrophic antiphospholipid syndrome, which presented an acute interstitial pneumonia-like image on chest CT scan.

    PubMed

    Kameda, Tomohiro; Dobashi, Hiroaki; Susaki, Kentaro; Danjo, Junichi; Nakashima, Shusaku; Shimada, Hiromi; Izumikawa, Miharu; Takeuchi, Yohei; Mitsunaka, Hiroki; Bandoh, Shuji; Imataki, Osamu; Nose, Masato; Matsunaga, Takuya

    2015-01-01

    We report the case of catastrophic antiphospholipid syndrome (CAPS) complicated with mixed connective tissue disease (MCTD). A female patient was diagnosed with acute interstitial pneumonia (AIP) with MCTD by chest CT scan. Corticosteroid therapy was refractory for lung involvement, and she died due to acute respiratory failure. The autopsy revealed that AIP was compatible with lung involvement of CAPS. We therefore suggest that chest CT might reveal AIP-like findings in CAPS patients whose condition is complicated with pulmonary manifestations.

  8. Characterization of acute interstitial pneumonia in cattle in southern Alberta feedyards.

    PubMed Central

    Ayroud, M; Popp, J D; VanderKop, M A; Yost, G S; Haines, D M; Majak, W; Karren, D; Yanke, L J; McAllister, T A

    2000-01-01

    Field data were collected over 2 consecutive years to characterize acute interstitial pneumonia (AIP) in feedyard cattle. Thirty-eight cattle with clinical symptoms of AIP were examined following emergency slaughter; 31 (all heifers) were confirmed to have AIP on the basis of gross and histological lung pathology. The 7 without AIP, plus 17 asymptomatic penmates, were used as contemporary controls. Plasma concentrations of 3-methylindole (3MI) metabolites were higher (P < 0.001) in heifers afflicted with AIP than in the control animals, and concentrations of 3MI mercapturates in the urine were lower (P < 0.007) in affected heifers. Concentrations of 3MI adducts in lung tissue and in microsomal protein did not differ (P > 0.05) between the 2 groups, and 3MI was not detected in ruminal fluid from either group. Total ruminal bacterial numbers and populations of lactobacilli and protozoa were similar (P > 0.05) between the AIP-positive and unafflicted groups, but fewer (P < 0.05) cellulolytic bacteria were present in the positive group. Bovine respiratory syncytial virus antigen was not found in lung tissue from any of the heifers confirmed to have AIP. To our knowledge, this study is the first to implicate 3MI metabolites as having a role in feedyard AIP. Further research is required to determine the factors responsible for the elevation in 3MI adducts in plasma and urine of feedyard cattle afflicted with AIP. Images Figure 1. PMID:10907577

  9. Acute interstitial pneumonia in feedlot cattle: effects of feeding feather meal or vitamin E.

    PubMed

    Stanford, Kim; McAllister, Tim A; Ayroud, Mejid; Bray, Tammy M; Yost, Garold S

    2007-04-01

    We evaluated the effects of feeding 1.5% cysteine-rich feather meal or 550 IU of vitamin E for 40 d before slaughter on the rates of death and emergency slaughter due to acute interstitial pneumonia (AIP) in commercial feedlots. Blood and lung tissue were collected at slaughter from 83 animals clinically diagnosed with AIP, 40 asymptomatic penmates, and 40 heifers receiving either feather meal (20) or vitamin E (20); the left lung was subsampled for histologic examination. Blood and lung tissue were analyzed for thiol adducts of 3-methyleneindolenine (3ME) and reduced glutathione. Supplementation with feather meal or vitamin E had no effect on the rates of death and emergency slaughter attributable to AIP and did not influence the levels of 3ME or reduced glutathione in blood or lung tissue. Although supplementation with greater amounts of feather meal or vitamin E may have been necessary to significantly affect factors related to feedlot AIP, increased supplementation would be uneconomical for commercial feedlots, given the relatively low incidence of AIP.

  10. Characterization of acute interstitial pneumonia in cattle in southern Alberta feedyards.

    PubMed

    Ayroud, M; Popp, J D; VanderKop, M A; Yost, G S; Haines, D M; Majak, W; Karren, D; Yanke, L J; McAllister, T A

    2000-07-01

    Field data were collected over 2 consecutive years to characterize acute interstitial pneumonia (AIP) in feedyard cattle. Thirty-eight cattle with clinical symptoms of AIP were examined following emergency slaughter; 31 (all heifers) were confirmed to have AIP on the basis of gross and histological lung pathology. The 7 without AIP, plus 17 asymptomatic penmates, were used as contemporary controls. Plasma concentrations of 3-methylindole (3MI) metabolites were higher (P < 0.001) in heifers afflicted with AIP than in the control animals, and concentrations of 3MI mercapturates in the urine were lower (P < 0.007) in affected heifers. Concentrations of 3MI adducts in lung tissue and in microsomal protein did not differ (P > 0.05) between the 2 groups, and 3MI was not detected in ruminal fluid from either group. Total ruminal bacterial numbers and populations of lactobacilli and protozoa were similar (P > 0.05) between the AIP-positive and unafflicted groups, but fewer (P < 0.05) cellulolytic bacteria were present in the positive group. Bovine respiratory syncytial virus antigen was not found in lung tissue from any of the heifers confirmed to have AIP. To our knowledge, this study is the first to implicate 3MI metabolites as having a role in feedyard AIP. Further research is required to determine the factors responsible for the elevation in 3MI adducts in plasma and urine of feedyard cattle afflicted with AIP.

  11. Effect of dietary melengestrol acetate on the incidence of acute interstitial pneumonia in feedlot heifers.

    PubMed

    Stanford, Kim; McAllister, Tim A; Ayroud, Mejid; Bray, Tammy M; Yost, Garold S

    2006-07-01

    Over a 3-y period, 906,000 cattle were monitored in 23 feedlots in southern Alberta for symptoms of acute interstitial pneumonia (AIP). Plasma, urine, and lung tissue were collected at slaughter from 299 animals clinically diagnosed with AIP and from 156 healthy penmates and analyzed for 3-methylindole (3MI) derivatives and reduced glutathione concentration. From each animal, the left lung was subsampled for histologic examination. Concentrations of glutathione in lung tissue were reduced (P < 0.001) in animals showing clinical symptoms of AIP as compared with their asymptomatic penmates. Animals histologically confirmed as having AIP had higher levels of 3MI protein adducts in blood and lung tissue (P < 0.05) than did emergency-slaughtered animals without AIP. Within feedlots, where pens of heifers were fed either a standard dosage of melengestrol acetate (MGA) or none, the rate of death attributable to AIP was similar between treatment groups, but emergency slaughter after clinical diagnosis of AIP was done 3.2 times more often (P < 0.001) in the MGA-fed heifers than in the group not fed MGA. Use of MGA did not influence glutathione concentration. As growth performance of heifers given steroidal implants may not be improved by feeding MGA, the most cost-effective method of reducing the incidence of AIP-related emergency slaughter in feedlot heifers may be to eliminate MGA from the diet.

  12. Effect of dietary melengestrol acetate on the incidence of acute interstitial pneumonia in feedlot heifers

    PubMed Central

    McAllister, Tim A.; Ayroud, Mejid; Bray, Tammy M.; Yost, Garold S.

    2006-01-01

    Abstract Over a 3-y period, 906 000 cattle were monitored in 23 feedlots in southern Alberta for symptoms of acute interstitial pneumonia (AIP). Plasma, urine, and lung tissue were collected at slaughter from 299 animals clinically diagnosed with AIP and from 156 healthy penmates and analyzed for 3-methylindole (3MI) derivatives and reduced glutathione concentration. From each animal, the left lung was subsampled for histologic examination. Concentrations of glutathione in lung tissue were reduced (P < 0.001) in animals showing clinical symptoms of AIP as compared with their asymptomatic penmates. Animals histologically confirmed as having AIP had higher levels of 3MI protein adducts in blood and lung tissue (P < 0.05) than did emergency-slaughtered animals without AIP. Within feedlots, where pens of heifers were fed either a standard dosage of melengestrol acetate (MGA) or none, the rate of death attributable to AIP was similar between treatment groups, but emergency slaughter after clinical diagnosis of AIP was done 3.2 times more often (P < 0.001) in the MGA-fed heifers than in the group not fed MGA. Use of MGA did not influence glutathione concentration. As growth performance of heifers given steroidal implants may not be improved by feeding MGA, the most cost-effective method of reducing the incidence of AIP-related emergency slaughter in feedlot heifers may be to eliminate MGA from the diet. PMID:16850945

  13. [Atypical interstitial pneumonia (AIP) in calves and young cattle in Schleswig-Holstein in conjunction with an infection by the bovine respiratory syncytial virus (BRSV)].

    PubMed

    Appel, G; Heckert, H P

    1989-04-01

    It is reported on atypical interstitial pneumonia (AIP) in 16 mostly Holstein-Frisian calves and feeders from 13 different farms in Schleswig-Holstein in association with an infection by the respiratory syncytial virus (BRSV). All animals were submitted with identical clinical histories. Macroscopically the lung lesions were characterized by alveolar and interstitial edema and emphysema. Microscopically there was a diffuse interstitial pneumonia with formation of hyaline membranes and multinucleated giant cells. From the investigation material of the 16 animals BRSV was confirmed by direct immunofluorescence with monoclonal antibodies in 4 animals from 4 different farms.

  14. Interstitial lung disease - adults - discharge

    MedlinePlus

    Diffuse parenchymal lung disease - discharge; Alveolitis - discharge; Idiopathic pulmonary pneumonitis - discharge; IPP - discharge; Chronic interstitial lung - discharge; Chronic respiratory interstitial lung - ...

  15. Automated Classification of Usual Interstitial Pneumonia using Regional Volumetric Texture Analysis in High-Resolution CT

    PubMed Central

    Depeursinge, Adrien; Chin, Anne S.; Leung, Ann N.; Terrone, Donato; Bristow, Michael; Rosen, Glenn; Rubin, Daniel L.

    2014-01-01

    Objectives We propose a novel computational approach for the automated classification of classic versus atypical usual interstitial pneumonia (UIP). Materials and Methods 33 patients with UIP were enrolled in this study. They were classified as classic versus atypical UIP by a consensus of two thoracic radiologists with more than 15 years of experience using the American Thoracic Society evidence–based guidelines for CT diagnosis of UIP. Two cardiothoracic fellows with one year of subspecialty training provided independent readings. The system is based on regional characterization of the morphological tissue properties of lung using volumetric texture analysis of multiple detector CT images. A simple digital atlas with 36 lung subregions is used to locate texture properties, from which the responses of multi-directional Riesz wavelets are obtained. Machine learning is used to aggregate and to map the regional texture attributes to a simple score that can be used to stratify patients with UIP into classic and atypical subtypes. Results We compared the predictions based on regional volumetric texture analysis with the ground truth established by expert consensus. The area under the receiver operating characteristic curve of the proposed score was estimated to be 0.81 using a leave-one-patient-out cross-validation, with high specificity for classic UIP. The performance of our automated method was found to be similar to that of the two fellows and to the agreement between experienced chest radiologists reported in the literature. However, the errors of our method and the fellows occurred on different cases, which suggests that combining human and computerized evaluations may be synergistic. Conclusions Our results are encouraging and suggest that an automated system may be useful in routine clinical practice as a diagnostic aid for identifying patients with complex lung disease such as classic UIP, obviating the need for invasive surgical lung biopsy and its

  16. Giant cell interstitial pneumonia in a hard-metal worker. Cytologic, histologic and analytical electron microscopic investigation

    SciTech Connect

    Tabatowski, K.; Roggli, V.L.; Fulkerson, W.J.; Langley, R.L.; Benning, T.; Johnston, W.W.

    1988-03-01

    A case of biopsy-proven giant cell interstitial pneumonia in a patient with occupational exposure to hard-metal dust is reported. Bronchial washings performed several days prior to open-lung biopsy yielded an almost exclusive population of nonpigmented alveolar macrophages and pleomorphic, phagocytic multinucleated giant cells. Microorganisms, viral inclusions in the giant cells, epithelioid histiocytes and well-formed granulomas were not seen. This cytologic picture strongly suggests the presence of giant cell interstitial pneumonia in a patient with restrictive lung disease, particularly when exposure to hard-metal dust is known or suspected. A specific diagnosis early in the course of the disease may facilitate removal of the individual from the workplace and forestall the development of end-stage interstitial fibrosis. Additionally, the working environment may be modified to minimize inhalational exposure. Recognition of this entity by the cytopathologist may direct diagnostic efforts toward accurate histologic evaluation and the identification of particulates by microprobe analysis of either cellular or biopsy material.

  17. A case of non-specific interstitial pneumonia with recurrent gastric carcinoma and anti-Jo-1 antibody positive myositis.

    PubMed

    Ebisutani, Chikara; Ito, Isao; Kitaichi, Masanori; Tanabe, Naoya; Mishima, Michiaki; Kadowaki, Seizo

    2016-07-01

    We report the first case of non-specific interstitial pneumonia (NSIP) in a patient with cancer-associated myositis (CAM) that emerged along with the recurrence of the cancer. A 60-year-old woman, with a history of partial gastrectomy for gastric cancer 11 years ago, presented with exertional dyspnea with anti-Jo-1 antibody-positive myositis. Surgical lung biopsy showed NSIP with metastatic gastric cancer. Accordingly, her condition was diagnosed as CAM with cancer recurrence. In patients with a history of cancer, development of myositis may indicate cancer recurrence; therefore, careful observation would be necessary.

  18. Pneumonia

    MedlinePlus

    ... How Can I Help a Friend Who Cuts? Pneumonia KidsHealth > For Teens > Pneumonia Print A A A ... having to go to the hospital. What Is Pneumonia? Pneumonia (pronounced: noo-MOW-nyuh) is an infection ...

  19. A Histologically Distinctive Interstitial Pneumonia Induced by Overexpression of the Interleukin 6, Transforming Growth Factor β1, or Platelet-Derived Growth Factor B Gene

    NASA Astrophysics Data System (ADS)

    Yoshida, Mitsuhiro; Sakuma, Junko; Hayashi, Seiji; Abe, Kin'ya; Saito, Izumu; Harada, Shizuko; Sakatani, Mitsunoir; Yamamoto, Satoru; Matsumoto, Norinao; Kaneda, Yasufumi; Kishmoto, Tadamitsu

    1995-10-01

    Interstitial pneumonia is characterized by alveolitis with resulting fibrosis of the interstitium. To determine the relevance of humoral factors in the pathogenesis of interstitial pneumonia, we introduced expression vectors into Wistar rats via the trachea to locally overexpress humoral factors in the lungs. Human interleukin (IL) 6 and IL-6 receptor genes induced lymphocytic alveolitis without marked fibroblast proliferation. In contrast, overexpression of human transforming growth factor β1 or human platelet-derived growth factor B gene induced only mild or apparent cellular infiltration in the alveoli, respectively. However, both factors induced significant proliferation of fibroblasts and deposition of collagen fibrils. These histopathologic changes induced by the transforming growth factor β1 and platelet-derived growth factor B gene are partly akin to those changes seen in lung tissues from patients with pulmonary fibrosis and markedly contrast with the changes induced by overexpression of the IL-6 and IL-6 receptor genes that mimics lymphocytic interstitial pneumonia.

  20. Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity.

    PubMed

    Frankel, Stephen K; Cool, Carlyne D; Lynch, David A; Brown, Kevin K

    2004-12-01

    Between 1996 and 2001, we identified five cases of a unique idiopathic pleuroparenchymal lung disease characterized by a clinical presentation suggestive of a chronic idiopathic interstitial pneumonia, marked pleural and parenchymal radiographic involvement with an upper lobe predominance, and surgical lung biopsy findings that did not fit with any of the currently defined interstitial pneumonias. The pathologic findings included the following: (1) intense fibrosis of the visceral pleura; (2) prominent, homogenous, subpleural fibroelastosis; (3) sparing of the parenchyma distant from the pleura; (4) mild, patchy lymphoplasmacytic infiltrates; and (5) small numbers of fibroblastic foci present at the leading edge of the fibrosis. In this report, we characterize the clinical, radiographic, physiologic, and pathologic findings of this entity, which we term idiopathic pleuroparenchymal fibroelastosis. PMID:15596706

  1. [Rituximab therapy in the treatment of anti-neutrophil cytoplasmic antibody (ANCA) -positive interstitial pneumonia: case report].

    PubMed

    Miyaoka, Tokiko; Itabashi, Mitsuyo; Kumon, Saeko; Akiyama, Kenichi; Iwabuchi, Yuko; Kataoka, Hiroshi; Moriyama, Takahito; Takei, Takashi; Nitta, Kosaku

    2016-01-01

    We report a patient treated with rituximab for interstitial pneumonia (IP) associated with microscopic polyangiitis (MPA) and who was undergoing hemodialysis. A 59-year-old woman who had been treated with tacrolimus for 1 year for rheumatic arthritis was referred to the Department of Nephrology for fatigue, fever, weight loss, and rapidly developing renal dysfunction. On the first admission, severe renal dysfunction, proteinuria, hematuria, and an elevated titer of MPO-ANCA were observed, and the woman was diagnosed with rapidly progressive glomerulonephritis because of MPA. At that point, IP was found to be present but not active. Although steroid semipulse therapy following an initial prednisolone (PSL) administration of 40 mg/day, IVCY, and plasma exchange were administered, renal dysfunction did not recover, and the patient required maintenance hemodialysis. Upon discharge, a high titer of MPO-ANCA was continuously observed. Nine months after the initiation of hemodialysis, respiratory discomfort and desaturation developed. Interstitial shadow and ground glass opacity were seen on a CT scan, and the patient was diagnosed with exacerbation of interstitial pneumonia caused by MPA recurrence. At the second admission, acute findings identified by imaging techniques had improved. However, the high titer of MPO-ANCA continued in spite of the steroid semi-pulse therapy following PSL administration, and rituximab corresponding to 200 mg/weekly for 1 month was also administered. The dose of rituximab was decreased subsequently because the patient was judged to be compromised by the hemodialysis. At the same time, internal administration of sulfamethoxazole/trimethoprim was initiated. After the rituximab treatment, MPO-ANCA antibodies gradually decreased, and the respiratory condition improved. Five months after the rituximab treatment, respiratory dysfunction recurred. Based on the CT findings and a high level of β-D-glycan, the patient was diagnosed with ARDS due to

  2. Value of bronchoalveolar lavage in the management of severe acute pneumonia and interstitial pneumonitis in the immunocompromised child.

    PubMed Central

    de Blic, J; McKelvie, P; Le Bourgeois, M; Blanche, S; Benoist, M R; Scheinmann, P

    1987-01-01

    The diagnostic value of 73 bronchoalveolar lavages was assessed in 67 immunocompromised children (aged 3 months to 16 years) with pulmonary infiltrates. Thirty one children had primary and 19 secondary immune deficiency, 14 acquired immunodeficiency syndrome (AIDS), and three AIDS related complex. Bronchoalveolar lavage was performed during fibreoptic bronchoscopy, under local anaesthesia in all but two. One or more infective agents was found in eight of 11 patients with severe acute pneumonia and in 26 of 62 patients with interstitial pneumonitis. In interstitial pneumonitis, the most frequently encountered agents were Pneumocystis carinii (12), cytomegalovirus (8), and Aspergillus fumigatus (3). The yield was related to the severity of interstitial pneumonitis. The mean cellular count and cytological profile in lavage returns from patients with varying infective agents or underlying pathological conditions showed no significant difference, except in those children with AIDS and AIDS related complex who had appreciable lymphocytosis (mean percentage of lymphocytes 28 (SD 17]. In children with AIDS and chronic interstitial pneumonitis lymphocytosis without pneumocystis infection was observed in eight of nine bronchoalveolar lavage returns and was suggestive of pulmonary lymphoid hyperplasia. Finally, bronchoalveolar lavage produced a specific diagnosis from the microbiological or cytological findings in 44 instances (60%). Transient exacerbation of tachypnoea was observed in the most severely ill children but there was no case of respiratory decompensation attributable to the bronchoscopy. Bronchoalveolar lavage is a safe and rapid examination for the investigation of pulmonary infiltrates in immunocompromised children. It should be performed as a first line investigation and should reduce the use of open lung biopsy techniques. PMID:2827334

  3. Differentiating Smoking-Related Interstitial Fibrosis (SRIF) from Usual Interstitial Pneumonia (UIP) with Emphysema Using CT Features Based on Pathologically Proven Cases

    PubMed Central

    Chae, Kum Ju; Jin, Gong Yong; Jung, Hyun Nyeong; Kwon, Keun Sang; Choi, Hyemi; Lee, Yong Chul; Chung, Myoung Ja; Park, Ho Sung

    2016-01-01

    Objective To differentiate smoking-related interstitial fibrosis (SRIF) from usual interstitial pneumonia (UIP) with emphysema on CT in combined pulmonary fibrosis and emphysema (CPFE) patients. Materials and Methods This study was approved by the institutional review board and informed consent was waived. We included 65 patients who underwent lung biopsy under the suspicion of UIP pattern on HRCT, and after radiologic-pathologic correlation, they were divided into three groups: UIP without emphysema (n = 30), UIP with emphysema (n = 26), and SRIF (n = 9). The quantitative extent of emphysema in the entire lung was visually assessed and fibrotic patterns were qualitatively analyzed based on six characteristics (asymmetry, juxta-subpleural sparing, emphysema beside the honeycombing area, absence of ground grass attenuation/reticulation in honeycombing area, inhomogeneous honeycombing, and absence of honeycombing in the upper lobes). Kaplan-Meier analysis was used for survival analysis, and logistic regression with a receiver operating characteristic curve was used to predict the possibility of SRIF. Results In qualitative analysis of fibrotic patterns, SRIF tended to exhibit more than three of six fibrotic features, whereas UIP with emphysema demonstrated about two of these characteristics (p = 0.035). In addition, SRIF had a higher extent of emphysema than UIP with emphysema when they have same amount of fibrosis (p = 0.014). In patients with SRIF, 5-year survival rate was 85.7%, while it was 40.7% in UIP with emphysema patients (p = 0.035). Conclusion Fibrotic CT patterns and survival rate differed between SRIF and UIP with emphysema among CPFE patients, which explains the variable prognosis of CPFE. PMID:27611866

  4. Detectability of simulated interstitial pneumonia on chest radiographs: comparison between irradiation side sampling indirect flat-panel detector and computed radiography

    PubMed Central

    Yabuuchi, H; Kairada, A; Tanaka, N; Morishita, J; Akasaka, T; Matsuo, Y; Kamitani, T; Yamasaki, Y; Nagao, M; Sasaki, M

    2014-01-01

    Objective: To compare the detectability of simulated interstitial pneumonia on chest radiographs between an irradiation side sampling indirect flat-panel detector (ISS-FPD) and computed radiography (CR). Methods: Simulated interstitial pneumonia findings (ground-glass opacity, reticular opacity and honeycomb lung) were superimposed on an anthropomorphic chest phantom. Chest radiographs were acquired under three exposure levels (4.0, 3.2 and 2.0 mAs) with an ISS-FPD and with CR. 5 thoracic radiologists evaluated 72 images for the presence or absence of a lesion over each of 6 areas. A total of 1296 observations were analysed in a receiver–operating characteristic analysis. A jackknife method was used for the statistical analysis. Results: The areas under the curves (AUCs) for the detection of simulated honeycomb lung obtained with the ISS-FPD were significantly larger than those obtained with CR at all exposure conditions. For the detection of simulated ground-glass opacity and reticular opacity, there were no significant differences between the two systems. In addition, the AUCs for the detectability of simulated honeycomb lung obtained with the ISS-FPD at all exposure levels were significantly larger than those obtained with CR at 4 mAs. Conclusion: The ISS-FPD was superior to CR for the detection of simulated honeycomb lung. Provided that the chosen model is representative of interstitial pneumonia, the use of an ISS-FPD might reduce a patient's exposure dose during the detection of interstitial pneumonia. Advances in knowledge: The ISS-FPD has shown its advantage compared with CR in the detection of honeycombing, one sign of interstitial pneumonia. PMID:24874767

  5. Bovine atypical interstitial pneumonia associated with the ingestion of damaged sweet potatoes (Ipomoea batatas) in Northeastern Brazil.

    PubMed

    Medeiros, R M; Simões, S V; Tabosa, I M; Nóbrega, W D; Riet-Correa, F

    2001-08-01

    Atypical interstitial pneumonia in cattle associated with the ingestion of damaged sweet potatoes is reported in the State of Paraíba, Northeastern Brazil. The sweet potatoes were severely damaged by Myzus tersicae, and had an obvious fungal infection. Eighteen milking cows, 1 bull and 1 steer were fed approximately 400 kg of the sweet potatoes. Six days after consumption 13 cows were affected with labored abdominal breathing, extended and lowered head, coughing, expiratory grunt, salivation and protruded tongue. Six animals died and the others recovered in 4-7 d. At necropsy lungs were distended and did not collapse when the thorax was opened. Bullous emphysema and gelatinous exudates were observed in the interlobular, peribronchial and subpleural tissues. Microscopically, the lungs had severe edema and emphysema, congestion, and alveolar epithelial cell hyperplasia. The sweet potatoes were cultured but Fusarium spp was not isolated, probably because a zigomycete fungus covered the plates in 48 h of incubation.

  6. Sjögren Syndrome Complicated by Mucosa-Associated Lymphoid Tissue Lymphoma and Lymphocytic Interstitial Pneumonia.

    PubMed

    Ahmed, Fatma; Raslan, Osama; Muzaffar, Razi; Parkar, Nadeem; Marwaha, Nitin; Osman, Medhat M

    2015-01-01

    Sjögren syndrome (SS) is an autoimmune disease with exocrine glands dysfunction and multiorgan involvement. It is associated with increased risk of lymphoproliferative disorders, especially B-cell marginal zone lymphoma. While the role of F-18 Fluorodeoxyglucose position emission tomography/computed tomography (F-18 FDG PET/CT) for evaluation of lymphoma has been established, its use in patients with a chronic history of SS to evaluate for possible lymphoproliferative disorders or multiorgan involvement is limited. We present a case of chronic SS in which F-18 FDG PET/CT demonstrated FDG avid intraparotid and cervical lymph nodes pathologically proven to be mucosa-associated lymphoid tissue lymphoma. In addition, the patient had bibasilar cystic changes consistent with lymphocytic interstitial pneumonia.

  7. From lymphocytic interstitial pneumonia to MALT lymphoma of lung: a case report with a 5-year diagnostic dilemma.

    PubMed

    Wu, Wei; Zhou, Jing; Di, Li-Gai; Chen, Hui

    2015-01-01

    Lymphocytic interstitial pneumonia (LIP) and mucosa-associated lymphoid tissue (MALT) lymphoma of lung are all uncommon disorders of respiratory system. MALT lymphoma of lung is a distinct and unique subtype of marginal zone B-cell non-Hodgkin's lymphoma (NHL) characterized by malignant cells arising from extranodal sites. They are characteristic of exuberant lymphoid infiltration in pathological tissue. Therefore, in some cases, they are too similar in clinical manifestation, chest imaging and pathology to make differential diagnosis. Here, we report a 43-year-old woman who underwent a tough process for the final diagnosis. From this case, we could get a well understanding of difference between LIP and MALT lymphoma of lung. PMID:26464740

  8. Acute Onset Anti-Synthetase Syndrome With Pericardial Effusion and Non-Specific Interstitial Pneumonia

    PubMed Central

    Shah, Aditya; Patel, Samir R.

    2016-01-01

    Anti-synthetase syndrome (AS) is a clinical entity which is described classically by the triad of interstitial lung disease (ILD), inflammatory myositis and presence of aminoacyl-tRNA synthetase antibodies (ASA). We describe a rare presentation of this condition with regard to the uncharacteristically acute nature of presentation, acute decompensation in clinical condition, development of acute interstitial pneumonitis requiring rescue extracorporeal membrane oxygenation (ECMO) and accompaniment of significant pericardial effusion on presentation, followed by rapid improvement with initiation of steroids. PMID:27540445

  9. Lack of p47(phox) in Akita Diabetic Mice Is Associated with Interstitial Pneumonia, Fibrosis, and Oral Inflammation.

    PubMed

    Zamakhchari, Mai F; Sima, Corneliu; Sama, Kishore; Fine, Noah; Glogauer, Michael; Van Dyke, Thomas E; Gyurko, Robert

    2016-03-01

    Excess reactive oxygen species production is central to the development of diabetic complications. The contribution of leukocyte reactive oxygen species produced by the NADPH oxidase to altered inflammatory responses associated with uncontrolled hyperglycemia is poorly understood. To get insight into the role of phagocytic superoxide in the onset of diabetic complications, we used a model of periodontitis in mice with chronic hyperglycemia and lack of leukocyte p47(phox) (Akita/Ncf1) bred from C57BL/6-Ins2(Akita)/J (Akita) and neutrophil cytosolic factor 1 knockout (Ncf1) mice. Akita/Nfc1 mice showed progressive cachexia starting at early age and increased mortality by six months. Their lungs developed infiltrative interstitial lesions that obliterated air spaces as early as 12 weeks when fungal colonization of lungs also was observed. Neutrophils of Akita/Ncf1 mice had normal degranulation and phagocytic efficiency when compared with wild-type mice. Although Akita/Ncf1 mice had increased prevalence of oral infections and more severe periodontitis compared with wild-type mice, bone loss was only marginally higher compared with Akita and Ncf1 null mice. Altogether these results indicate that lack of leukocyte superoxide production in mice with chronic hyperglycemia results in interstitial pneumonia and increased susceptibility to infections. PMID:26747235

  10. Diffuse alveolar damage: a common phenomenon in progressive interstitial lung disorders.

    PubMed

    Kaarteenaho, Riitta; Kinnula, Vuokko L

    2011-01-01

    It has become obvious that several interstitial lung diseases, and even viral lung infections, can progress rapidly, and exhibit similar features in their lung morphology. The final histopathological feature, common in these lung disorders, is diffuse alveolar damage (DAD). The histopathology of DAD is considered to represent end stage phenomenon in acutely behaving interstitial pneumonias, such as acute interstitial pneumonia (AIP) and acute exacerbations of idiopathic pulmonary fibrosis (IPF). Acute worsening and DAD may occur also in patients with nonspecific interstitial pneumonias (NSIPs), and even in severe viral lung infections where there is DAD histopathology in the lung. A better understanding of the mechanisms underlying the DAD reaction is needed to clarify the treatment for these serious lung diseases. There is an urgent need for international efforts for studying DAD-associated lung diseases, since the prognosis of these patients has been and is still dismal.

  11. Pulmonary Disease Secondary to Reflux Mimicking Interstitial Pneumonia in Systemic Sclerosis: Case Report and Literature Review

    PubMed Central

    Montes, Ricardo Azêdo de Luca; Mazolli Veiga, Nathalia; Lanzieri, Pedro Gemal; Mocarzel, Luis Otávio Cardoso

    2016-01-01

    Systemic sclerosis is a complex disease due to the variety of clinical presentations, often superimposed on other conditions, related or not to the connective tissue. We report a 43-year-old Brazilian woman with limited systemic sclerosis and pulmonary symptoms secondary to gastroesophageal reflux disease, with a clinical presentation similar to a diffuse interstitial lung disease. Because of the frequency of interstitial lung injury due to systemic sclerosis, this was an important differential diagnosis, which could be excluded after optimized treatment of reflux disease, with clinical and radiological improvement. Clinical management of patients with collagen diseases requires clinician skills to identify the natural history and understand its nuances. This is a common situation in clinical practice, but with a few discussions in international literature. PMID:26885429

  12. [Interstitial pulmonary diseases].

    PubMed

    Brasch, F

    2006-03-01

    Interstitial pneumonia is a rare disease, posing a diagnostic challenge to pneumologists, pediatricians, radiologists and pathologists. Only by the combined efforts of the European Respiratory Society (ERS) and the American Thoracic Society (ATS) has has been possible to standardize the formerly different European and Northern American nomenclature of interstitial lung diseases (alveolitis versus interstitial pneumonia) in adults and to clearly and unambiguously define the diagnostic criteria. The ATS/ERS classification of 2002 comprises seven entities: usual interstitial pneumonia (UIP), non-specific interstitial pneumonia (NSIP), desquamative interstitial pneumonia (DIP), respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), cryptogenic organizing pneumonia (COP), lymphocyte interstitial pneumonia (LIP), and acute interstitial pneumonia (AIP). Using the ATS/ERS classification of interstitial pulmonary diseases in premature infants, infants and children is problematic, since UIP, RB-ILD and AIP do not occur at this age. Although infants with severe respiratory insufficiency may sometimes show morphological features similar to DIP or NSIP, this entity should rather be classified as chronic pneumonitis of infancy (CPI) because of differences in etiology, pathogenesis and prognostic outcome.

  13. Granulomatous interstitial pneumonia in a miniature swine associated with repeated intravenous injections of Tc-99m human serum albumin: concise communication

    SciTech Connect

    Whinnery, J.E.; Young, J.T.

    1980-03-01

    Albumin lung-scanning agents have a proven high degree of safety, with the only contraindication to their use being allergic hypersensitivity. We have used these agents to investigate the physiologic effects of high G/sub z/ acceleratory forces on pulmonary perfusion using the miniature swine. Multiple doses of human macroaggregated albumin and human-albumin microspheres were given to a miniature swine at various levels of centrifugal acceleration over a 6-wk period. The dosages given were the same per kilogram as those used for routine clinical human studies. The animal subsequently died from a severe granulomatous interstitial pneumonia. The granulomatous lesions suggest that the pathogenesis may have involved a cell-mediated delayed hypersensitivity. This interstitial pneumonia may represent the end point in a chronic hypersensitivity response to the human-albumin lung-scanning agents.

  14. Interstitial lung disease.

    PubMed

    Cottin, Vincent

    2013-03-01

    This article reviews the most important articles published in interstitial lung disease, as reviewed during the Clinical Year in Review session at the 2012 annual European Respiratory Society Congress in Vienna, Austria. Since the recent international guidelines for the management of idiopathic pulmonary fibrosis (IPF), important new evidence is available. The anti-fibrotic drug pirfenidone has been recently approved in Europe. Other pharmacological agents, especially nintedanib, are still being tested. The so-called triple combination therapy, anticoagulation therapy and endothelin receptor antagonists, especially ambrisentan, are either harmful or ineffective in IPF and are not recommended as treatment. Although the clinical course of IPF is highly variable, novel tools have been developed for individual prediction of prognosis. Acute exacerbations of IPF are associated with increased mortality and may occur with higher frequency in IPF patients with associated pulmonary hypertension. Interstitial lung disease associated with connective tissue disease has been definitely established to have a better long-term survival than IPF. A subset of patients present with symptoms and/or biological autoimmune features, but do not fulfil diagnostic criteria for a given autoimmune disease; this condition is associated with a higher prevalence of nonspecific interstitial pneumonia pattern, female sex and younger age, although survival relevance is unclear.

  15. Successful combination therapy with corticosteroids, biweekly intravenous pulse cyclophosphamide and cyclosporin A for acute interstitial pneumonia in patients with dermatomyositis : report of three cases.

    PubMed

    Suzuki, Akitake; Shoji, Norikazu; Kikuchi, Eigo; Uekubo, Kazuaki; Aoki, Naoko; Sonoda, Yui; Torigai, Hideyuki; Yamashita, Hiroyuki; Fujita, Kentaro; Okai, Takahiro

    2013-01-01

    We report three patients with dermatomyositis (DM) complicated with acute interstitial pneumonia (AIP). All of them complained of fever and acutely worsening dyspnea and were treated immediately by combination therapies with pulse therapy with methylprednisone (mPSL) followed by corticosteroids, biweekly intravenous pulse cyclophosphamide (IVCY) and cyclosporine A (CSA). They recovered rapidly soon after an initiation of this combination regimen. Early intervention with aggressive combination therapy is life-saving for the treatment of AIP in patients with DM.

  16. Interstitial pneumonia associated to peginterferon alpha-2a: A focus on lung function

    PubMed Central

    Cortés-Telles, Arturo

    2016-01-01

    Pulmonary toxicity related to the use of pegylated interferon alpha-2a during treatment of hepatitis C infections is rare; nonetheless, some cases with fatal outcomes have been reported. Evaluating patients’ pulmonary function is a key to diagnosis, follow-up and prognosis of several respiratory diseases, but case reports of respiratory manifestations related to the use of pegylated interferon alpha-2a have limited their findings to only baseline measurements. This paper examines the case of a 65-year-old woman with chronic hepatitis C virus infection who developed interstitial pneumonitis associated with pegylated interferon alpha-2a. Initial lung function evaluation revealed a marked reduction compared to an earlier assessment; the results were consistent with a moderate restricted pattern. Fortunately, over the ensuing 8 weeks of follow-up after discontinuing the drug, the patient recovered her lung function and experienced an overall improvement in her respiratory symptoms. PMID:27051119

  17. [Antisynthetase (anti PL-7 antibody) syndrome presenting as a skin rash and exacerbation of interstitial pneumonia during treatment for rheumatoid arthritis].

    PubMed

    Ishiguro, Takashi; Takayanagi, Noboru; Miyahara, Yousuke; Yanagisawa, Tsutomu; Sugita, Yutaka

    2010-03-01

    A 52-year-old man was admitted to our hospital for progressive dyspnea of 3 months duration and a skin rash of 4 months duration. Previously, he had been given a diagnosis of rheumatoid arthritis at age 40, then a diagnosis of interstitial pneumonia at age 46. Tacrolimus and prednisolone were begun at age 50, and 50 mg/week of etanercept was added 17 months before admission to our hospital. Due to renal dysfunction, tacrolimus was discontinued 9 months before admission. A skin rash developed 4 months before admission, and progressive dyspnea developed over the 3 months before admission. Tacrolimus was restarted at 1 mg/day and prednisolone was increased from 5 mg/day to 15 mg/day; however, neither the skin rash nor the dyspnea improved. After visiting a local physician, the patient was then referred to our institution. On presentation, skin changes such as erythema of the superior palpebrae and fingers were noted. His serum creatine phosphokinase level was elevated, but muscle strength was normal and no abnormal electromyographic and muscle biopsy findings were found. Anti-Jo-1 antibody was negative but anti PL-7 antibody was positive. The patient did not meet the diagnostic criteria of dermatomyositis/ polymyositis, so antisynthetase syndrome was diagnosed. Etanercept was discontinued and the prednisolone increased, which resulted in improvement of the interstitial pneumonia and skin rash. Antisynthetase syndrome should be considered as a differential diagnosis when skin rash and exacerbation of interstitial pneumonia are found during treatment for rheumatoid arthritis.

  18. Update on diagnosis and treatment of idiopathic pulmonary fibrosis

    PubMed Central

    Baddini-Martinez, José; Baldi, Bruno Guedes; da Costa, Cláudia Henrique; Jezler, Sérgio; Lima, Mariana Silva; Rufino, Rogério

    2015-01-01

    Idiopathic pulmonary fibrosis is a type of chronic fibrosing interstitial pneumonia, of unknown etiology, which is associated with a progressive decrease in pulmonary function and with high mortality rates. Interest in and knowledge of this disorder have grown substantially in recent years. In this review article, we broadly discuss distinct aspects related to the diagnosis and treatment of idiopathic pulmonary fibrosis. We list the current diagnostic criteria and describe the therapeutic approaches currently available, symptomatic treatments, the action of new drugs that are effective in slowing the decline in pulmonary function, and indications for lung transplantation. PMID:26578138

  19. Lymphangiogenesis and Lesion Heterogeneity in Interstitial Lung Diseases

    PubMed Central

    Yamashita, Masahiro

    2015-01-01

    The lymphatic system has several physiological roles, including fluid homeostasis and the activation of adaptive immunity by fluid drainage and cell transport. Lymphangiogenesis occurs in adult tissues during various pathologic conditions. In addition, lymphangiogenesis is closely linked to capillary angiogenesis, and the balanced interrelationship between capillary angiogenesis and lymphangiogenesis is essential for maintaining homeostasis in tissues. Recently, an increasing body of information regarding the biology of lymphatic endothelial cells has allowed us to immunohistochemically characterize lymphangiogenesis in several lung diseases. Particular interest has been given to the interstitial lung diseases. Idiopathic interstitial pneumonias (IIPs) are characterized by heterogeneity in pathologic changes and lesions, as typified by idiopathic pulmonary fibrosis/usual interstitial pneumonia. In IIPs, lymphangiogenesis is likely to have different types of localized functions within each disorder, corresponding to the heterogeneity of lesions in terms of inflammation and fibrosis. These functions include inhibitory absorption of interstitial fluid and small molecules and maturation of fibrosis by excessive interstitial fluid drainage, caused by an unbalanced relationship between capillary angiogenesis and lymphangiogenesis and trafficking of antigen-presenting cells and induction of fibrogenesis via CCL21 and CCR7 signals. Better understanding for regional functions of lymphangiogenesis might provide new treatment strategies tailored to lesion heterogeneity in these complicated diseases. PMID:26823655

  20. Pneumonia

    MedlinePlus

    ... en español Neumonía You're out in the rain, jumping around in puddles, and somebody yells, "Get ... you really catch it from playing in the rain? What Is Pneumonia? Pneumonia (say: noo-MOW-nyuh) ...

  1. Epidermal growth factor receptor tyrosine kinase inhibition up-regulates interleukin-6 in cancer cells and induces subsequent development of interstitial pneumonia.

    PubMed

    Ishiguro, Yukari; Ishiguro, Hitoshi; Miyamoto, Hiroshi

    2013-04-01

    Acute interstitial pneumonia is one of serious side effects of epidermal growth factor receptor (EGFR)-tyrosine kinase inhibitor (TKI) treatment, while it often has significant clinical benefit in cancer patients. Therefore, it is necessary to clarify underlying mechanisms for the development of the adverse effects by EGFR-TKI. In the present study, we attempted to determine how EGFR-TKI treatment in cancer cells induced interstitial pneumonia. The growth of tongue cancer HSC-3 and lung cancer A549 cell lines treated with EGFR-TKI was assessed by MTT assay. Cytokines and growth factors in conditioned medium (CM) obtained from EGFR-TKI-treated cancer cells were analyzed using cytokine membrane array and ELISA. Interleukin-6 (IL-6) promoter activity was measured by luciferase assay. We found that EGFR-TKI treatment significantly decreased the cell viability yet increased expression levels of IL-6 protein and mRNA, IL-6 secretion, and IL-6 transcriptional activity in these lines. In addition, using the co-culture model and IL-6 treatment was found to increase the expression of collagen and α-actin, which were markers for fibrosis, in lung fibroblast cells. These results suggest that up-regulated IL-6 plays an important role in the development of EGFR-TKI-induced interstitial fibroblastic proliferation. Therefore, blocking of IL-6 signaling could be beneficial to cancer patients undergoing EGFR-TKI treatment for reducing the risk of its unfavorable effects.

  2. Pneumonia

    MedlinePlus

    ... better than treating it. Vaccines are available to prevent pneumococcal pneumonia and the flu. Other preventive measures include washing your hands frequently and not smoking. NIH: National Heart, Lung, and Blood Institute

  3. Connective tissue disease related interstitial lung diseases and idiopathic pulmonary fibrosis: provisional core sets of domains and instruments for use in clinical trials

    PubMed Central

    Saketkoo, Lesley Ann; Mittoo, Shikha; Huscher, Dörte; Khanna, Dinesh; Dellaripa, Paul F; Distler, Oliver; Flaherty, Kevin R; Frankel, Sid; Oddis, Chester V; Denton, Christopher P; Fischer, Aryeh; Kowal-Bielecka, Otylia M; LeSage, Daphne; Merkel, Peter A; Phillips, Kristine; Pittrow, David; Swigris, Jeffrey; Antoniou, Katerina; Baughman, Robert P; Castelino, Flavia V; Christmann, Romy B; Christopher-Stine, Lisa; Collard, Harold R; Cottin, Vincent; Danoff, Sonye; Highland, Kristin B; Hummers, Laura; Shah, Ami A; Kim, Dong Soon; Lynch, David A; Miller, Frederick W; Proudman, Susanna M; Richeldi, Luca; Ryu, Jay H; Sandorfi, Nora; Sarver, Catherine; Wells, Athol U; Strand, Vibeke; Matteson, Eric L; Brown, Kevin K; Seibold, James R

    2014-01-01

    Rationale Clinical trial design in interstitial lung diseases (ILDs) has been hampered by lack of consensus on appropriate outcome measures for reliably assessing treatment response. In the setting of connective tissue diseases (CTDs), some measures of ILD disease activity and severity may be confounded by non-pulmonary comorbidities. Methods The Connective Tissue Disease associated Interstitial Lung Disease (CTD-ILD) working group of Outcome Measures in Rheumatology—a non-profit international organisation dedicated to consensus methodology in identification of outcome measures—conducted a series of investigations which included a Delphi process including >248 ILD medical experts as well as patient focus groups culminating in a nominal group panel of ILD experts and patients. The goal was to define and develop a consensus on the status of outcome measure candidates for use in randomised controlled trials in CTD-ILD and idiopathic pulmonary fibrosis (IPF). Results A core set comprising specific measures in the domains of lung physiology, lung imaging, survival, dyspnoea, cough and health-related quality of life is proposed as appropriate for consideration for use in a hypothetical 1-year multicentre clinical trial for either CTD-ILD or IPF. As many widely used instruments were found to lack full validation, an agenda for future research is proposed. Conclusion Identification of consensus preliminary domains and instruments to measure them was attained and is a major advance anticipated to facilitate multicentre RCTs in the field. PMID:24368713

  4. The association between red blood cell and platelet transfusion and subsequently developing idiopathic pneumonia syndrome after hematopoietic stem cell transplantation

    PubMed Central

    Vusse, Lisa K. Vande; Madtes, David K.; Guthrie, Katherine A.; Gernsheimer, Terry B.; Curtis, J. Randall; Watkins, Timothy R.

    2014-01-01

    BACKGROUND Blood transfusions are common during hematopoietic stem cell transplantation (HSCT) and may contribute to lung injury. STUDY DESIGN AND METHODS This study examined the associations between red blood cell (RBC) and platelet (PLT) transfusions and idiopathic pneumonia syndrome (IPS) among 914 individuals who underwent myeloablative allogeneic HSCT between 1997 and 2001. Patients received allogeneic blood transfusions at their physicians' discretion. RBCs, PLTs, and a composite of “other” transfusions were quantified as the sum of units received each 7-day period from 6 days before transplant until IPS onset, death, or Posttransplant Day 120. RBC and PLT transfusions were modeled as separate time-varying exposures in proportional hazards models adjusted for IPS risk factors (age, baseline disease, irradiation dose) and other transfusions. Timing of PLT transfusion relative to myeloid engraftment and PLT ABO blood group (match vs. mismatch) were included as potential interaction terms. RESULTS Patients received a median of 9 PLT and 10 RBC units. There were 77 IPS cases (8.4%). Each additional PLT unit transfused in the prior week was associated with 16% higher IPS risk (hazard ratio, 1.16; 95% confidence interval, 1.09–1.23; p < 0.001). Recent RBC and PLT transfusions were each significantly associated with greater risk of IPS when examined without the other; only PLT transfusions retained significance when both exposures were included in the model. The PLT association was not modified by engraftment or ABO mismatch. CONCLUSION PLT transfusions are associated with greater risk of IPS after myeloablative HSCT. RBCs may also contribute; however, these findings need confirmation. PMID:24033082

  5. Human Biomarker Discovery and Predictive Models for Disease Progression for Idiopathic Pneumonia Syndrome Following Allogeneic Stem Cell Transplantation*

    PubMed Central

    Schlatzer, Daniela M.; Dazard, Jean-Eudes; Ewing, Rob M.; Ilchenko, Serguei; Tomcheko, Sara E.; Eid, Saada; Ho, Vincent; Yanik, Greg; Chance, Mark R.; Cooke, Kenneth R.

    2012-01-01

    Allogeneic hematopoietic stem cell transplantation (SCT) is the only curative therapy for many malignant and nonmalignant conditions. Idiopathic pneumonia syndrome (IPS) is a frequently fatal complication that limits successful outcomes. Preclinical models suggest that IPS represents an immune mediated attack on the lung involving elements of both the adaptive and the innate immune system. However, the etiology of IPS in humans is less well understood. To explore the disease pathway and uncover potential biomarkers of disease, we performed two separate label-free, proteomics experiments defining the plasma protein profiles of allogeneic SCT patients with IPS. Samples obtained from SCT recipients without complications served as controls. The initial discovery study, intended to explore the disease pathway in humans, identified a set of 81 IPS-associated proteins. These data revealed similarities between the known IPS pathways in mice and the condition in humans, in particular in the acute phase response. In addition, pattern recognition pathways were judged to be significant as a function of development of IPS, and from this pathway we chose the lipopolysaccaharide-binding protein (LBP) protein as a candidate molecular diagnostic for IPS, and verified its increase as a function of disease using an ELISA assay. In a separately designed study, we identified protein-based classifiers that could predict, at day 0 of SCT, patients who: 1) progress to IPS and 2) respond to cytokine neutralization therapy. Using cross-validation strategies, we built highly predictive classifier models of both disease progression and therapeutic response. In sum, data generated in this report confirm previous clinical and experimental findings, provide new insights into the pathophysiology of IPS, identify potential molecular classifiers of the condition, and uncover a set of markers potentially of interest for patient stratification as a basis for individualized therapy. PMID:22337588

  6. Synoviolin inhibitor LS-102 reduces endoplasmic reticulum stress-induced collagen secretion in an in vitro model of stress-related interstitial pneumonia.

    PubMed

    Nakajima, Fukami; Aratani, Satoko; Fujita, Hidetoshi; Yagishita, Naoko; Ichinose, Shizuko; Makita, Koshi; Setoguchi, Yasuhiro; Nakajima, Toshihiro

    2015-01-01

    The deletion mutation of exon 4 in surfactant protein C (SP-C), a lung surfactant protein, has been identified in parent-child cases of familial interstitial pneumonia. It has been shown that this mutation induces endoplasmic reticulum (ER) stress. Synoviolin is an E3 ubiquitin ligase that is localized to the ER and is an important factor in the degradation of ER-related proteins. It has been demonstrated that synoviolin is involved in liver fibrosis. In the present study, we investigated the involvement of synoviolin in the pathogenesis of interstitial pneumonia caused by the exon 4 deletion in the SP-C gene. We transfected wild-type and exon 4-deleted SP-C genes into A549 human lung adenocarcinoma cells and measured the secretion of collagen, which is a representative extracellular matrix protein involved in fibrosis. Secreted collagen levels were increased in the culture medium in SP-C mutants compared to the wild-type cells. Furthermore, the transcription of mRNAs coding for factors associated with fibrosis was increased. Subsequently, to assess the involvement of synoviolin, we constructed plasmids with a luciferase gene under the control of the synoviolin promoter. The A549 cells were transfected with the construct along with the exon 4-deleted SP-C plasmid for use in the luciferase assay. We found a 1.6-fold increase in luciferase activity in the cells carrying exon 4 deleted SP-C, as well as an increase in intrinsic synoviolin expression at the mRNA and protein levels. Collagen secretion was decreased by the addition of LS-102, a synoviolin inhibitor, to the A549 culture medium following transfection with wild-type and exon 4-deleted SP-C. These results demonstrate that synoviolin is involved in the onset of interstitial pneumonia induced by exon 4-deleted SP-C, which suggests that synoviolin inhibitors may be used in the treatment of the disease.

  7. Rheumatoid arthritis-associated interstitial lung disease

    PubMed Central

    Solomon, Joshua J; Brown, Kevin K

    2012-01-01

    Rheumatoid arthritis (RA) is a systemic inflammatory disorder affecting 1% of the US population. Patients can have extra-articular manifestations of their disease and the lungs are commonly involved. RA can affect any compartment of the respiratory system and high resolution computed tomography (HRCT) of the lung is abnormal in over half of these patients. Interstitial lung disease is a dreaded complication of RA. It is more prevalent in smokers, males, and those with high antibody titers. The pathogenesis is unknown but data suggest an environmental insult in the setting of a genetic predisposition. Smoking may play a role in the pathogenesis of disease through citrullination of protein in the lung leading to the development of autoimmunity. Patients usually present in middle age with cough and dyspnea. Pulmonary function testing most commonly shows reduced diffusion capacity for carbon monoxide and HRCT reveals a combination of reticulation and ground glass abnormalities. The most common pattern on HRCT and histopathology is usual interstitial pneumonia (UIP), with nonspecific interstitial pneumonia seen less frequently. There are no large-scale well-controlled treatment trials. In severe or progressive cases, treatment usually consists of corticosteroids with or without a cytotoxic agent for 6 months or longer. RA interstitial lung disease is progressive; over half of patients show radiographic progression within 2 years. Patients with a UIP pattern on biopsy have a survival similar to idiopathic pulmonary fibrosis.

  8. Lung tumors masquerading as desquamative interstitial pneumonia (DIP): report of 7 cases and review of the literature.

    PubMed

    Raparia, Kirtee; Ketterer, James; Dalurzo, Mercedes L; Chang, Yu-Hui; Colby, Thomas V; Leslie, Kevin O

    2014-07-01

    Malignant tumors in the lung (both primary and metastatic) rarely may be associated with markedly discohesive tumor cells, resulting in airspace filling reminiscent of "desquamative interstitial pneumonia" (DIP) on histopathology evaluation. A peculiar aspect of this growth pattern is the relatively bland appearance of the tumor cells, in many cases simulating benign alveolar macrophages at scanning magnification. We searched the Charles Carrington Memorial consultation files in the Department of Laboratory Medicine and Pathology at Mayo Clinic Arizona for instances of malignant tumors in lung simulating DIP, from 1992 to 2011. We identified 7 cases involving transbronchial biopsies, needle core samples, or resected lung specimens. Clinical, histopathologic, and immunohistochemical analyses of these 7 patients were performed, including detailed morphometric analysis of the individual tumor cells using calibrated measurement tools on digital images. We compared the results with those of a control group of 4 patients with benign DIP-macrophage reactions in smoking-related lung disease. The study group comprised 5 male and 2 female patients, 48 to 86 years in age (median: 67 y). The radiologic findings included lobar consolidation, localized ground-glass opacities, and 1 or more nodules. None of the patients had typical bilateral infiltrates of DIP. Microscopically, the lung parenchyma was dominated by the presence of prominent tumor cells filling alveolar spaces. Four patients had primary lung carcinoma (adenocarcinoma), whereas 3 had metastases from other sites, including a melanoma. Immunohistochemical staining studies were performed on 6 of 7 cases to establish the diagnosis. Nuclear diameter, cytoplasmic diameter, and nuclear/cytoplasmic (N/C) ratios in patient and control groups were compared using the Wilcoxon rank sum test. No significant difference in the diameters of nucleus and cytoplasm between cases and control groups (P=0.3447 and 0.7055, respectively

  9. Low absolute lymphocyte count and addition of rituximab confer high risk for interstitial pneumonia in patients with diffuse large B-cell lymphoma.

    PubMed

    Huang, Yu-Chung; Liu, Chia-Jen; Liu, Chun-Yu; Pai, Jih-Tung; Hong, Ying-Chung; Teng, Hao-Wei; Hsiao, Liang-Tsai; Chao, Ta-Chung; Gau, Jyh-Pyng; Liu, Jin-Hwang; Hsu, Hui-Chi; Chiou, Tzeon-Jye; Chen, Po-Min; Yu, Yuan-Bin; Tzeng, Cheng-Hwai

    2011-10-01

    Several small-scale studies have reported pulmonary toxicity among patients with diffuse large B-cell lymphoma (DLBCL) receiving rituximab-containing chemotherapy, though whether the use of rituximab predisposes to interstitial pneumonia (IP) remains unclear. This retrospective study was intended to identify the characteristics and risk factors of IP in patients with DLBCL. Between 2000 and 2009, 529 consecutive patients with DLBCL receiving first-line tri-weekly COP- or CHOP-based chemotherapy with or without rituximab were enrolled as subjects. IP was defined as diffuse pulmonary interstitial infiltrates found on computed tomography scans in conjunction with respiratory symptoms. IP was observed in 26 patients (4.9%), six of whom were confirmed with Pneumocystis jirovecii pneumonia. The median number of chemotherapy courses before IP was four cycles. Using multivariate analysis, absolute lymphocyte count less than 1×10(9)/l at diagnosis [odds ratio (OR) 2.75, p=0.014] and the addition of rituximab to chemotherapy (OR 4.56, p=0.003) were identified as independent risk factors for IP. In conclusion, the incidence of IP is increased in patients with DLBCL receiving rituximab-containing chemotherapy. Specific subgroups with lymphopenia at diagnosis may justify close scrutiny to detect pulmonary complications. PMID:21647583

  10. The MUC5B Variant Is Associated with Idiopathic Pulmonary Fibrosis but Not with Systemic Sclerosis Interstitial Lung Disease in the European Caucasian Population

    PubMed Central

    Borie, Raphael; Crestani, Bruno; Dieude, Philippe; Nunes, Hilario; Allanore, Yannick; Kannengiesser, Caroline; Airo, Paolo; Matucci-Cerinic, Marco; Wallaert, Benoit; Israel-Biet, Dominique; Cadranel, Jacques; Cottin, Vincent; Gazal, Steven; Peljto, Anna L.; Varga, John; Schwartz, David A.; Valeyre, Dominique; Grandchamp, Bernard

    2013-01-01

    A polymorphism on the MUC5B promoter (rs35705950) has been associated with idiopathic pulmonary fibrosis (IPF) but not with systemic sclerosis (SSc) with interstitial lung disease (ILD). We genotyped the MUC5B promoter in the first 142 patients of the French national prospective cohort of IPF, in 981 French patients with SSc (346 ILD), 598 Italian patients with SSc (207 ILD), 1383 French controls and 494 Italian controls. A meta-analysis was performed including all American data available. The T risk allele was present in 41.9% of the IPF patients, 10.8% of the controls (P = 2×10–44), OR 6.3 [4.6–8.7] for heterozygous patients and OR 21.7 [10.4–45.3] for homozygous patients. Prevalence of the T allele was not modified according to age, gender, smoking in IPF patients. However, none of the black patients with IPF presented the T allele. The prevalence of the T risk allele was similar between French (10%) and Italian (12%) cohorts of SSc whatever the presence of an ILD (11.1% and 13.5%, respectively). Meta-analysis confirmed the similarity between French, Italian and American cohorts of IPF or SSc-ILD. This study confirms 1) an association between the T allele risk and IPF, 2) an absence of association with SSc-ILD, suggesting different pathophysiology. PMID:23940607

  11. Immunosuppressive agents and interstitial lung disease: what are the risks?

    PubMed

    Meyer, Keith C

    2014-06-01

    Idiopathic pulmonary fibrosis is unlikely to respond to immunosuppressive therapies, and patients with idiopathic pulmonary fibrosis may be harmed by such therapy. In contrast, some forms of interstitial lung disease can respond well to treatment with immunosuppressive drug therapies. Such agents can, however, be associated with significant risk of adverse effects such as infection, diabetes, osteoporosis, myopathy, bone marrow suppression, hepatitis, urinary tract injury, and drug-induced pneumonitis. Treating clinicians must be aware of potential adverse reactions to any immunosuppressive drug that they prescribe for their patients, and they should implement appropriate pre-therapy screening (e.g., tuberculosis, hepatitis, renal insufficiency) and monitoring that is recommended to avoid/minimize risk during the treatment period. Some disorders (e.g., cellular non-specific interstitial pneumonia, organizing pneumonia, or sarcoidosis) may respond very well to immunosuppressive therapies including corticosteroids as monotherapy, and the use of steroid-sparing agents can minimize corticosteroid side effects and may enhance treatment efficacy for disorders such as sarcoidosis or connective tissue disease-associated forms of interstitial lung disease.

  12. Different microcirculatory and interstitial matrix patterns in idiopathic dilated cardiomyopathy and Chagas' disease: a three dimensional confocal microscopy study

    PubMed Central

    Higuchi, M; Fukasawa, S; De Brito, T; Parzianello, L; Bellotti, G; Ramires, J

    1999-01-01

    OBJECTIVE—To analyse the morphological aspects of the extracellular matrix and microcirculation to clarify whether chronic Chagas' cardiopathy (CCC) is an accurate model to study the pathogenesis of idiopathic dilated cardiomyopathy (IDCM).
DESIGN—Thick histological myocardial sections were prepared to analyse collagen, and microcirculation was examined during confocal laser and light microscopy.
SETTING—The specimens were prepared at the pathology service of the Heart Institute of São Paulo, Brazil.
PATIENTS—Nine control hearts, eight IDCM hearts, and 10 CCC hearts were studied after necropsy.
MAIN OUTCOME MEASURES—The number of collagen struts per 100× field, the area of fibrosis (%), and the diameters of arterioles and capillaries were measured in each heart to establish outcome.
RESULTS—A smaller number (mean (SD)) of collagen struts was seen in the hearts in the IDCM group (9.1 (4.1)) than in the control (22.4 (3.2)) (p < 0.05) or CCC (15.7 (7.4)) (p > 0.05) groups. Fibrosis was greater in the CCC hearts (13.8 (10.5)%) than in the IDCM hearts (5.9 (6.6)%) (p > 0.05). Major increases in arteriole (65.4 (9.9) µm) and capillary (9.9 (1.7) µm) diameters were seen in the CCC hearts but not in the IDCM hearts (arteriole diameter 40.3 (7.9) µm; capillary diameter 7.9 (1.3) µm).
CONCLUSIONS—Hearts demonstrating CCC and IDCM present different extracellular and microvessel alterations. This suggests that distinct pathogenic mechanisms are responsible for each condition and that CCC is not an effective model to study IDCM.


Keywords: microcirculation; Chagas' disease; dilated cardiomyopathy; extracellular matrix PMID:10455076

  13. Acute microbiologically negative hypoxic interstitial pneumonia on HAART: Immune Reconstitution Inflammatory Syndrome unmasking Pneumocystis Jiroveci infection with an atypical presentation

    PubMed Central

    Sovaila, S; de Raigniac, A; Picard, C; Taulera, O; Lascoux-Combe, C; Sereni, D; Bourgarit, A

    2012-01-01

    Highly active antiretroviral therapy for AIDS sometimes engenders inflammatory manifestations resulting from an inappropriate and unbalanced immune-system restoration, called Immune Reconstitution inflammatory Syndrome, which, in turn, can unmask a subclinical infection/pathology. Despite our patient’s evident syndrome, the atypical clinical, microbiologic and radiologic feature of Pneumocystis pneumonia made its diagnosis difficult. PMID:22802889

  14. Idiopathic pulmonary fibrosis.

    PubMed

    Meltzer, Eric B; Noble, Paul W

    2008-01-01

    Idiopathic pulmonary fibrosis (IPF) is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation. IPF is a rare disease which affects approximately 5 million persons worldwide. The prevalence is estimated to be slightly greater in men (20.2/100,000) than in women (13.2/100,000). The mean age at presentation is 66 years. IPF initially manifests with symptoms of exercise-induced breathless and dry coughing. Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of IPF patients. Cor pulmonale develops in association with end-stage disease. In that case, classic signs of right heart failure may be present. Etiology remains incompletely understood. Some environmental factors may be associated with IPF (cigarette smoking, exposure to silica and livestock). IPF is recognized on high-resolution computed tomography by peripheral, subpleural lower lobe reticular opacities in association with subpleural honeycomb changes. IPF is associated with a pathological lesion known as usual interstitial pneumonia (UIP). The UIP pattern consists of normal lung alternating with patches of dense fibrosis, taking the form of collagen sheets. The diagnosis of IPF requires correlation of the clinical setting with radiographic images and a lung biopsy. In the absence of lung biopsy, the diagnosis of IPF can be made by defined clinical criteria that were published in guidelines endorsed by several professional societies. Differential diagnosis includes other idiopathic interstitial pneumonia, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis), forme fruste of autoimmune disorders, chronic hypersensitivity pneumonitis and other environmental (sometimes occupational) exposures. IPF is typically progressive and leads to significant disability. The median

  15. A case of drug-induced interstitial pneumonia potentially related to quetiapine (seroquel) therapy for behavioral and psychological symptoms.

    PubMed

    Kim, Se-Jin; Han, Sang-Don; Lee, Jung Yeon; Chon, Gyu Rak

    2014-10-01

    Quetiapine is regarded as an effective and safe treatment for delirium. An 82-year-old man presented with a 1-week history of violent behavior and dizziness accompanied by weakness on the left side of his body. He was diagnosed with acute cerebral cortical infarction and delirium associated with alcohol abuse. After quetiapine treatment, he complained of fever and coughed up sputum, whereas his aggressive behavior improved. His symptoms persisted despite empirical antibiotic treatment. All diagnostic tests for infectious causes were negative. High-resolution computed tomography revealed bilateral consolidations and ground-glass opacities with predominantly peribronchial and subpleural distributions. The primary differential diagnosis was drug-associated interstitial lung disease, and therefore, we discontinued quetiapine and began methylprednisolone treatment. His symptoms and radiologic findings significantly improved after receiving steroid therapy. We propose that clinicians need to be aware of the possibility that quetiapine is associated with lung injury.

  16. A case of chronic hepatitis C patient of myocardial ischemia accompanied with interstitial pneumonia induced by pegylated interferon alpha-2a

    PubMed Central

    Qu, Caihong; Zheng, Zhenda; Liu, Weimin; Jiao, Ju; Zhu, Jieming

    2014-01-01

    After 3 months of combination treatment using interferon α-2a and Ribavirin, a case of 59-year-old female patient with chronic viral hepatitis C demonstrated symptoms such as headache, dizziness accompanied by nausea, vomiting, dry cough, breathing difficulty, and shortness of breath. Dynamic electrocardiogram showed occasional atrial premature beats, paroxysmal tachycardia, and abnormal ST-T (T wave inversion and prolongation of the QT interval). Ambulatory blood pressure indicated that mean blood pressure was elevated than before. Myocardial radionuclide scan showed focal myocardial ischemia in left ventricular inferior wall. Pulmonary function tests showed that pulmonary diffusion function was decreased, indicating the possibility of interstitial pneumonia. The patient had no history of coronary heart disease or chest X-ray abnormalities before medication, but had hypertensive medical history for 8 years with good blood pressure control. After withdrawal of antiviral drugs, symptoms such as dry cough, breathing difficulty and T wave inversion were gradually relieved. This case indicated that myocardial ischemia and pulmonary lesions were associated with the application of pegylated interferon α-2a. PMID:25197402

  17. Early detection of colon cancer by increased serum level of Krebs von den Lungen-6 in a patient with dermatomyositis-associated interstitial pneumonia.

    PubMed

    Fukuhara, Naoko; Tanino, Yoshinori; Sato, Suguru; Fukuhara, Atsuro; Uematsu, Manabu; Nikaido, Takefumi; Misa, Kenichi; Sato, Yasuko; Saito, Junpei; Wang, Xintao; Munakata, Mitsuru

    2015-09-14

    Krebs von den Lungen-6 (KL-6) is a high-molecular-weight glycoprotein which is elevated in serum of patients with interstitial pneumonia (IP). Serum KL-6 level is clinically used for the diagnosis of IP as well as the evaluation of its disease activity. KL-6 is originally identified when exploring novel soluble antigens in patients with lung cancer, and is known to be elevated in patients with several malignant tumors. The risk of malignant tumors is high in IP patients with polymyositis and dermatomyositis (PM/DM), and follow-up of KL-6 levels may allow earlier detection of such tumors. However, to date, there are only a few reports showing the usefulness of following-up serum KL-6 levels for finding malignant tumors in IP patients with PM/DM. Here, we described the first patient in whom increased serum KL-6 led to the diagnosis of colon cancer during follow-up of DM-associated IP.

  18. [An Elderly Patient with Metastatic Breast Cancer Who Developed Severe Adverse Events such as Stomatitis and Interstitial Pneumonia after Everolimus plus Exemestane Treatment].

    PubMed

    Sakiyama, Kana; Yoshida, Takashi; Goto, Yoshinari; Kimura, Morihiko

    2016-06-01

    An 80-year-old woman was diagnosed with right breast cancer with clinical Stage IIIA 6 years previously. She underwent mastectomy and axillary lymph node dissection. The pathological diagnosis was invasive micropapillary carcinoma with lymph node involvement. Immunohistochemically, the tumor was positive for estrogen receptor and progesterone receptor, and negative for HER2. Postoperatively, the patient was treated with adjuvant chemotherapy consisting of cyclophosphamide, epirubicin, 5-fluorouracil, and paclitaxel, followed by endocrine therapy with letrozole. Four years after surgery, she experienced a recurrence of breast cancer in the thoracic wall, and was treated with exemestane, toremifene, and fulvestrant for 1 year and 5 months. However, she developed carcinomatous pleurisy and was treated with eribulin. This last treatment was ineffective. Subsequently, she received combination therapy with everolimus and exemestane. Although the pleural effusion reduced markedly after 5 weeks, stomatitis, diarrhea, melena, and interstitial pneumonia occurred as adverse events. The symptoms improved after drug discontinuation and steroid therapy. The combination therapy with everolimus and exemestane is a prospective therapy for hormone-resistant recurrent breast cancer, but the management of adverse events is very important. PMID:27306814

  19. Interstitial lung disease in connective tissue disease--mechanisms and management.

    PubMed

    Wells, Athol U; Denton, Christopher P

    2014-12-01

    Pulmonary complications are an important extra-articular feature of autoimmune rheumatic diseases and a major cause of mortality. The underlying pathogenesis probably involves multiple cellular compartments, including the epithelium, lung fibroblasts, and the innate and adaptive immune system. Heterogeneity in the extent and progression of lung fibrosis probably reflects differences in underlying pathogenic mechanisms. Growing understanding of the key pathogenic drivers of lung fibrosis might lead to the development of more effective targeted therapies to replicate the treatment advances in other aspects of these diseases. Interstitial lung disease (ILD) in connective tissue disease (CTD) is characterized using the classification of the idiopathic interstitial pneumonias. Systemic sclerosis is most frequently associated with ILD and, in most of these patients, ILD manifests as a histological pattern of nonspecific interstitial pneumonia. Conversely, in rheumatoid arthritis, the pattern of ILD is most often usual interstitial pneumonia. The key goals of clinical assessment of patients with both ILD and CTD are the detection of ILD and prognostic evaluation to determine which patients should be treated. Data from treatment trials in systemic sclerosis support the use of immunosuppressive therapy, with the treatment benefit largely relating to the prevention of progression of lung disease.

  20. Pulmonary Langerhans Histiocytosis: an uncommon cause of interstitial pneumonia in a patient with Sjögren syndrome.

    PubMed

    González García, Andrés; Callejas Rubio, José Luis; Ríos Fernández, Raquel; Ortego Centeno, Norberto

    2016-03-01

    Sjögren syndrome is a chronic, systemic, and autoimmune disorder that targets exocrine glands by remarkable B cell hyperactivity. Eventually, it is associated with extra-glandular clinical manifestations that affect essentially any organ system, including pulmonary involvement. Interstitial lung disease is one of the most serious pulmonary complications, and the early diagnosis is essential to initiate a prompt therapy. On the other hand, Sjögren syndrome could present concomitantly with several rheumatologic diseases such as systemic lupus erythematosus or rheumatoid arthritis. Pulmonary Langerhans Histiocytosis is a rare clonal proliferative disease characterized by pulmonary involvement by cells phenotypically similar to Langerhans cells. We describe the case of a nonsmoker 62-year-old woman with Sjögren syndrome who presented concomitantly a Pulmonary Langerhans Histiocytosis mimicking a pulmonary complication of its Sjögren. Fortunately, she had a well response to corticosteroids and azathioprine regimen. The aim of the paper is to emphasize the importance of the good differential diagnosis related to the pulmonary involvement. To the best of our knowledge, this is the first description of these two entities in the literature.

  1. Acute respiratory failure caused by organizing pneumonia secondary to antineoplastic therapy for non-Hodgkin's lymphoma

    PubMed Central

    Santana, Adriell Ramalho; Amorim, Fábio Ferreira; Soares, Paulo Henrique Alves; de Moura, Edmilson Bastos; Maia, Marcelo de Oliveira

    2012-01-01

    Interstitial lung diseases belong to a group of diseases that typically exhibit a subacute or chronic progression but that may cause acute respiratory failure. The male patient, who was 37 years of age and undergoing therapy for non-Hodgkin's lymphoma, was admitted with cough, fever, dyspnea and acute hypoxemic respiratory failure. Mechanical ventilation and antibiotic therapy were initiated but were associated with unfavorable progression. Thoracic computed tomography showed bilateral pulmonary "ground glass" opacities. Methylprednisolone pulse therapy was initiated with satisfactory response because the patient had used three drugs related to organizing pneumonia (cyclophosphamide, doxorubicin and rituximab), and the clinical and radiological symptoms were suggestive. Organizing pneumonia may be idiopathic or linked to collagen diseases, drugs and cancer and usually responds to corticosteroid therapy. The diagnosis was anatomopathological, but the patient's clinical condition precluded performing a lung biopsy. Organizing pneumonia should be a differential diagnosis in patients with apparent pneumonia and a progression that is unfavorable to antimicrobial treatment. PMID:23917942

  2. Pleuroparenchymal fibroelastosis: is it also an idiopathic entity?

    PubMed

    Portillo, Karina; Guasch Arriaga, Ignasi; Ruiz-Manzano, Juan

    2015-10-01

    Pleuroparenchymal fibroelastosis (PPFE) is a rare disease that has been recently included in the updated consensus on idiopathic interstitial pneumonias. It shares some clinical features with other chronic interstitial pneumonias (dyspnea, dry cough), and is radiologically characterized by pleural and subpleural parenchymal fibrosis and elastosis, mainly in the upper lobes. The main histological findings include pleural fibrosis and prominent subpleural and parenchymal fibroelastosis. Its characterization is based on the increasing number of cases reported in the literature, so several aspects of the etiology, pathogenesis and natural history are still unknown. Although some cases have been described as idiopathic, PPFE has been reported as a complication after bone marrow transplantation, lung transplantation and chemotherapy, especially with alkylating agents.Spontaneous or iatrogenic pneumothorax is a frequently reported complication of invasive diagnostic tests for identifying PPFE. The disease course is variable, ranging from slow progression to rapid clinical deterioration. No treatment has shown evidence of efficacy, and lung transplantation remains the only option for patients who fulfill the diagnostic criteria for this option. Recognizing and disseminating the specific features of PPFE is essential to raise the level of clinical suspicion for this entity, and to implement appropriate multidisciplinary diagnostic management.

  3. Pleuroparenchymal fibroelastosis: is it also an idiopathic entity?

    PubMed

    Portillo, Karina; Guasch Arriaga, Ignasi; Ruiz-Manzano, Juan

    2015-10-01

    Pleuroparenchymal fibroelastosis (PPFE) is a rare disease that has been recently included in the updated consensus on idiopathic interstitial pneumonias. It shares some clinical features with other chronic interstitial pneumonias (dyspnea, dry cough), and is radiologically characterized by pleural and subpleural parenchymal fibrosis and elastosis, mainly in the upper lobes. The main histological findings include pleural fibrosis and prominent subpleural and parenchymal fibroelastosis. Its characterization is based on the increasing number of cases reported in the literature, so several aspects of the etiology, pathogenesis and natural history are still unknown. Although some cases have been described as idiopathic, PPFE has been reported as a complication after bone marrow transplantation, lung transplantation and chemotherapy, especially with alkylating agents.Spontaneous or iatrogenic pneumothorax is a frequently reported complication of invasive diagnostic tests for identifying PPFE. The disease course is variable, ranging from slow progression to rapid clinical deterioration. No treatment has shown evidence of efficacy, and lung transplantation remains the only option for patients who fulfill the diagnostic criteria for this option. Recognizing and disseminating the specific features of PPFE is essential to raise the level of clinical suspicion for this entity, and to implement appropriate multidisciplinary diagnostic management. PMID:26099364

  4. Interstitial nephritis

    MedlinePlus

    Tubulointerstitial nephritis; Nephritis - interstitial; Acute interstitial (allergic) nephritis ... Interstitial nephritis may be temporary ( acute ), or it may be long-lasting ( chronic ) and get worse over ...

  5. Eosinophilic alveolitis in immunologic interstitial lung disorders.

    PubMed

    Olivieri, D; Pesci, A; Bertorelli, G

    1990-01-01

    To analyze the role of eosinophils in alveolitis due to immunological interstitial lung disorders, 568 bronchoalveolar lavage (BAL) from 537 patients affected by 13 types of interstitial lung disease involving immunologic mechanisms were considered. An arbitrary cut-off of 4% of eosinophils in BAL was assumed. In five (idiopathic pulmonary fibrosis (IPF), allergic bronchopulmonary aspergillosis (ABPA), amiodarone-induced pneumonitis (AIP), chronic eosinophilic pneumonia (CEP), Churg-Strauss syndrome (CSS)) out of the thirteen groups we took into consideration, the level of eosinophils was greater than 4%. In CEP and CSS in particular, the arbitrary cut-off of 4% was greatly exceeded (28.9% +/- 27.4, p less than 0.01 and 33.6% +/- 14.5, p less than 0.01, respectively). In the same two groups the increase of eosinophils in BAL was isolated with a direct correlation to the number of eosinophils in blood. By contrast, the increase of eosinophils in BAL of IPF, AIP and ABPA was of lesser extent (4.7% +/- 5.7 p less than 0.01, 5.0% +/- 3.0 p less than 0.01 and 6.1% +/- 10.4 p less than 0.01, respectively) and was accompanied by an increase of neutrophils in IPF, of lymphocytes in AIP and both in ABPA. These patterns are generally defined as "mixed alveolitis." On the basis of these data we conclude that the term "eosinophilic alveolitis" should be reserved for CEP and CSS.

  6. Chronic eosinophilic pneumonia due to radiographic contrast administration: an orphan disease?

    PubMed Central

    Hohenforst-Schmidt, Wolfgang; Riedel, Andreas; Zarogoulidis, Paul; Franke, Christian; Gschwendtner, Andreas; Huang, Haidong; Machairiotis, Nikolaos; Dramba, Vasiliki; Zarogoulidis, Konstantinos; Brachmann, Johnannes

    2012-01-01

    Pulmonary eosinophilia comprises a heterogeneous group of diseases that are defined by eosinophilia in pulmonary infiltrates or in tissue. Drugs can cause almost all histopathologic patterns of interstitial pneumonias, such as cellular and fibrotic nonspecific interstitial pneumonia, pulmonary infiltrates and eosinophilia, organizing pneumonia, lymphocytic interstitial pneumonia, desquamative interstitial pneumonia, a pulmonary granulomatosis-like reaction, and a usual interstitial pneumonia-like pattern. We present a very rare case of chronic eosinophilic pneumonia due to radiographic contrast infusion diagnosed with video-assisted thoracoscopy. The patient after 1 year is still under corticosteroid treatment with the disease stabilized. PMID:23251088

  7. Up-to-Date Information on Rheumatoid Arthritis-Associated Interstitial Lung Disease.

    PubMed

    Suda, Takafumi

    2015-01-01

    Pulmonary involvement is common in rheumatoid arthritis (RA) and affects all the components of the lung. Interstitial lung disease (ILD) is the most predominant pulmonary manifestation and has been identified as the main cause of morbidity and mortality in RA. Clinically significant RA-ILD occurs in approximately 10% of RA patients. Several risk factors, such as old age, male gender, and smoking, have been reported to date. Histologically, the proportion of the usual interstitial pneumonia (UIP) pattern is higher in RA-ILD than in ILD associated with other connective tissue diseases, and RA-ILD also shows nonspecific interstitial pneumonia and organizing pneumonia patterns. High-resolution computed tomography scans are highly predictive of the histological UIP pattern with a specificity of 96%-100%. Acute exacerbation, which is the acute deterioration of the respiratory status characterized by newly developed bilateral infiltrates with unknown etiologies, has been reported in RA-ILD. Although acute exacerbation of RA-ILD has high mortality, similar to that of idiopathic pulmonary fibrosis, its incidence is lower in RA-ILD than in idiopathic pulmonary fibrosis. A consensus treatment has not yet been established. Current therapeutic regimens typically include corticosteroids with or without cytotoxic agents. Recent large longitudinal studies reported that the prognosis of RA-ILD was poor with a median survival of 2.6-3.0 years. Furthermore, histological and/or radiological patterns, such as UIP or non-UIP, have significant prognostic implications. RA-ILD patients with histological or radiological UIP patterns have poorer prognoses than those with non-UIP patterns. This review assessed the characteristics of RA-ILD by overviewing recent studies in the field and focused on the clinical significance of histological and/or radiological patterns in RA-ILD.

  8. Up-to-Date Information on Rheumatoid Arthritis-Associated Interstitial Lung Disease

    PubMed Central

    Suda, Takafumi

    2015-01-01

    Pulmonary involvement is common in rheumatoid arthritis (RA) and affects all the components of the lung. Interstitial lung disease (ILD) is the most predominant pulmonary manifestation and has been identified as the main cause of morbidity and mortality in RA. Clinically significant RA-ILD occurs in approximately 10% of RA patients. Several risk factors, such as old age, male gender, and smoking, have been reported to date. Histologically, the proportion of the usual interstitial pneumonia (UIP) pattern is higher in RA-ILD than in ILD associated with other connective tissue diseases, and RA-ILD also shows nonspecific interstitial pneumonia and organizing pneumonia patterns. High-resolution computed tomography scans are highly predictive of the histological UIP pattern with a specificity of 96%–100%. Acute exacerbation, which is the acute deterioration of the respiratory status characterized by newly developed bilateral infiltrates with unknown etiologies, has been reported in RA-ILD. Although acute exacerbation of RA-ILD has high mortality, similar to that of idiopathic pulmonary fibrosis, its incidence is lower in RA-ILD than in idiopathic pulmonary fibrosis. A consensus treatment has not yet been established. Current therapeutic regimens typically include corticosteroids with or without cytotoxic agents. Recent large longitudinal studies reported that the prognosis of RA-ILD was poor with a median survival of 2.6–3.0 years. Furthermore, histological and/or radiological patterns, such as UIP or non-UIP, have significant prognostic implications. RA-ILD patients with histological or radiological UIP patterns have poorer prognoses than those with non-UIP patterns. This review assessed the characteristics of RA-ILD by overviewing recent studies in the field and focused on the clinical significance of histological and/or radiological patterns in RA-ILD. PMID:27279757

  9. Idiopathic pulmonary fibrosis and coronary artery disease.

    PubMed

    Cicchitto, Gaetano; Musella, Valentina; Acitorio, Maria; Capuano, Nicola; Fiorenzano, Giuseppe; Owen, Caroline A; Polverino, Mario; Polverino, Francesca

    2014-01-01

    Idiopathic pulmonary fibrosis (IPF) is defined as a chronic fibrosing interstitial disease of unknown cause, limited to the lungs, and associated with the histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP); it generally progresses into respiratory failure and death. Although progression of the disease is the most common cause of death, there are increasing reports of its association with other pathologies has been reported: e.g., IPF patients seem more susceptible to cardiovascular diseases. Therefore, other pathologies might also influence the natural course. In this paper, we describe a case of IPF and coronary artery disease (CAD). We emphasize the importance of cardiopulmonary exercise test (CPET) as a useful procedure to monitor disease progression in IPF patients. We also stress the importance of a careful analysis of variables measured for an accurate interpretation of the clinical picture and an improvement of the clinical management of patients. Moreover, we suggest that a careful assessment of CPET parameters may additionally help in the early detection of high cardiovascular ischemic risk.

  10. Macrophage polarization in interstitial lung diseases

    PubMed Central

    Mierzejewski, Michał; Osińska, Iwona; Domagała-Kulawik, Joanna

    2016-01-01

    The role of bronchoalveolar lavage fluid (BALf) examination in differential diagnosis of interstitial lung diseases (ILD) was established. Currently, functional polarization into M1 (pro-inflammatory) and M2 (anti-inflammatory) subpopulations is emphasized. The aim of our study was to compare the proportion of M1 and M2 in BALf of patients with different ILD. BALf samples were collected from 75 ILD patients: sarcoidosis (SA, 36), hypersensitivity pneumonitis (HP, 10), non-specific interstitial pneumonia (NSIP, 8), idiopathic pulmonary fibrosis (IPF, 6) and other ILD (15). Phenotyping was performed by immunocytochemistry with anti-CD40 and CD163 antibodies (for M1 and M2, respectively). For both, CD40 and CD163, three populations of cells have been specified: small cells with strong (+++), large cells with weak (+) and cells with no (–) reaction. Due to lack of statistically significant differences between patients with HP, NSIP and IPF, they were classified into a common group and compared to the group of patients with sarcoidosis. The median proportion of macrophage population was as follows: for CD40: 61%, 35%, 2% in patients with SA and 49%, 47%, 3% in patients with other ILD and for CD163: 55%, 35%, 5% in SA and 53%, 43%, 1% in ILD patients, respectively. We found a significantly higher proportion of M1 in SA when compared with other ILD. Our study showed no evidence of defined polarization of alveolar macrophages in different types of interstitial lung diseases. However, we emphasized the role of CD40 positive cells in sarcoidosis and the role of CD163 positive cells in fibrotic diffuse lung diseases. PMID:27536201

  11. Rheumatoid Arthritis (RA) associated interstitial lung disease (ILD).

    PubMed

    O'Dwyer, David N; Armstrong, Michelle E; Cooke, Gordon; Dodd, Jonathan D; Veale, Douglas J; Donnelly, Seamas C

    2013-10-01

    Rheumatoid Arthritis (RA) is the most common Connective Tissue Disease (CTD) and represents an increasing burden on global health resources. Interstitial lung disease (ILD) has been recognised as a complication of RA but its potential for mortality and morbidity has arguably been under appreciated for decades. New studies have underscored a significant lifetime risk of ILD development in RA. Contemporary work has identified an increased risk of mortality associated with the Usual Interstitial Pneumonia (UIP) pattern which shares similarity with the most devastating of the interstitial pulmonary diseases, namely Idiopathic Pulmonary Fibrosis (IPF). In this paper, we discuss recent studies highlighting the associated increase in mortality in RA-UIP. We explore associations between radiological and histopathological features of RA-ILD and the prognostic implications of same. We emphasise the need for translational research in this area given the growing burden of RA-ILD. We highlight the importance of the respiratory physician as a key stakeholder in the multidisciplinary management of this disorder. RA-ILD focused research offers the opportunity to identify early asymptomatic disease and define the natural history of this extra articular manifestation. This may provide a unique opportunity to define key regulatory fibrotic events driving progressive disease. We also discuss some of the more challenging and novel aspects of therapy for RA-ILD.

  12. Idiopathic pulmonary fibrosis in a Christmas Island nuclear test veteran

    PubMed Central

    Parfrey, H; Babar, J; Fiddler, CA; Chilvers, ER

    2010-01-01

    We describe the case of a 71-year-old man with idiopathic pulmonary fibrosis (usual interstitial pneumonia (UIP) pattern) diagnosed on clinical, radiological and lung function criteria, in accordance with the American Thoracic Society/European Respiratory Society consensus criteria (2000), who had been in close proximity to three atmospheric nuclear bomb blasts during military service in 1957. He does not have clubbing and clinically and radiologically his lung disease is stable. He also has bladder carcinoma and carotid arteriosclerosis, both recognised consequences of radiation injury. This is the first reported case of UIP in a nuclear test veteran. Awareness of this potential association is important given the current attempts of the British Nuclear Test Veterans Association to gain compensation for claimed injuries. PMID:22797205

  13. Eosinophil alveolitis in two patients with idiopathic pulmonary fibrosis.

    PubMed

    Brix, Ninna; Rasmussen, Finn; Poletti, Venerino; Bendstrup, Elisabeth

    2016-01-01

    Bronchoalveolar lavage fluid (BALF) in patients with idiopathic pulmonary fibrosis (IPF) is typically characterized by a neutrophil inflammatory pattern and to a lesser extent (<25%) a mild eosinophil alveolitis. We here present two patients with a definite usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography of the thorax (HRCT) which demonstrated unusually high eosinophil counts in the BALF (40% and 51%). Based on HRCT, lack of response to steroids and the disease course they were both diagnosed as IPF after a multidisciplinary team discussion. This report discusses the diagnostic and etiological considerations of a coexisting UIP pattern and an eosinophil alveolitis. We conclude that these cases illustrate that high level BALF eosinophilia (40-50%) may occur among patients with IPF. PMID:27625983

  14. Idiopathic pulmonary fibrosis in a Christmas Island nuclear test veteran.

    PubMed

    Parfrey, H; Babar, J; Fiddler, C A; Chilvers, E R

    2010-01-01

    We describe the case of a 71-year-old man with idiopathic pulmonary fibrosis (usual interstitial pneumonia (UIP) pattern) diagnosed on clinical, radiological and lung function criteria, in accordance with the American Thoracic Society/European Respiratory Society consensus criteria (2000), who had been in close proximity to three atmospheric nuclear bomb blasts during military service in 1957. He does not have clubbing and clinically and radiologically his lung disease is stable. He also has bladder carcinoma and carotid arteriosclerosis, both recognised consequences of radiation injury. This is the first reported case of UIP in a nuclear test veteran. Awareness of this potential association is important given the current attempts of the British Nuclear Test Veterans Association to gain compensation for claimed injuries.

  15. In situ molecular hybridization for detection of Aleutian mink disease parvovirus DNA by using strand-specific probes: identification of target cells for viral replication in cell cultures and in mink kits with virus-induced interstitial pneumonia.

    PubMed

    Alexandersen, S; Bloom, M E; Wolfinbarger, J; Race, R E

    1987-08-01

    Strand-specific hybridization probes were utilized in in situ molecular hybridization specifically to localize replicative form DNA of Aleutian mink disease parvovirus (ADV). Throughout in vitro infection, duplex replicative form DNA of ADV was located in the cell nuclei. Single-stranded virion DNA and capsid proteins were present in the nuclei early in infection, but were later translocated to the cytoplasm. In neonatal mink, ADV causes acute interstitial pneumonia, and replicative forms of viral DNA were found predominantly in alveolar type II cells of the lung. Viral DNA was also found in other organs, but strand-specific probes made it possible to show that most of this DNA represented virus sequestration. In addition, glomerular immune complexes containing intact virions were detected, suggesting that ADV virions may have a role in the genesis of ADV-induced glomerulonephritis.

  16. Thin-Section CT Characteristics and Longitudinal CT Follow-up of Chemotherapy Induced Interstitial Pneumonitis

    PubMed Central

    Lee, Han Na; Kim, Mi Young; Koo, Hyun Jung; Kim, Sung-Soo; Yoon, Dok Hyun; Lee, Jae Cheol; Song, Jin Woo

    2016-01-01

    Abstract To describe the computed tomography (CT) features of chemotherapy-induced interstitial pneumonitis (CIIP) with longitudinal follow-up. The study was approved by the local ethics committee. One hundred consecutive patients with CIIP between May 2005 and March 2015 were retrospectively enrolled. The initial CT was reviewed by 2 independent chest radiologists and categorized into 1 of 4 CT patterns in accordance with the 2013 guidelines for idiopathic interstitial pneumonia: nonspecific interstitial pneumonia (NSIP), organizing pneumonia (OP), hypersensitivity pneumonitis (HP) mimicking desquamative interstitial pneumonitis, and diffuse alveolar damage (DAD). We assessed semiquantitative analysis on a 5% scale to assess the extent of parenchymal abnormalities (emphysema, reticulation, ground-glass opacity, consolidation, honeycombing cyst) and their distribution on initial (n = 100), subsequent (n = 87), and second follow-up CT (n = 48). Interval changes in extent on follow-up CT were compared using paired t test. The clinic-radiologic factors were compared between Group 1 (NSIP and OP patterns) and Group 2 (HP and DAD patterns) using χ2 and independent t tests. The most common pattern of CIIP on the initial CT was HP (51%), followed by NSIP (23%), OP (20%), and DAD (6%). Diffuse ground-glass opacity was the most common pulmonary abnormality. The predominant distribution was bilateral (99%) and symmetric (82%), with no craniocaudal (60%) or axial (79%) dominance. Subsequent and second follow-up CTs showed decreased extent of total pulmonary abnormalities (P < 0.001, respectively). In comparison with Group 1 CIIP, Group 2 CIIP was more likely to be caused by molecularly targeted drugs (P = 0.030), appeared earlier (P = 0.034), and underwent more complete resolution (P < 0.001). Use of a CT pattern–recognition approach to CIIP is appropriate and practical in interpreting radiological findings. PMID:26765442

  17. [Smoking-related interstitial lung diseases].

    PubMed

    Marten, Katharina

    2007-03-01

    The most important smoking-related interstitial lung diseases (ILD) are respiratory bronchiolitis, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and Langerhans' cell histiocytosis. Although traditionally considered to be discrete entities, smoking-related ILDs often coexist, thus accounting for the sometimes complex patterns encountered on high-resolution computed tomography (HRCT). Further studies are needed to elucidate the causative role of smoking in the development of pulmonary fibrosis.

  18. [A case of idiopathic ARDS treated by sivelestat sodium and fluid management based on extravascular lung water index].

    PubMed

    Yoshida, Takeshi; Rinka, Hiroshi; Kaji, Arito

    2006-12-01

    A 65-year-old woman was given emergency admission with fever, cough and dyspnea. Chest computed tomography (CT) findings showed bilateral ground-glass opacity, consolidation, and this case were fulfilled the criteria for acute respiratory distress syndrome (ARDS). We clinically diagnosed the patient as suffering from idiopathic ARDS including acute interstitial pneumonia (AIP) based on the absence of any known causes of ARDS and systemic immunologic diseases. We started treatment with sivelestat sodium and strictly managed fluid balance under mechanical ventilation. We found this treatment quite effective because there were significant improvements in the extravascular lung water index (EVLWI) measured by the PiCCO system and neutrophile elastase value and in oxygenation and the chest radiograph. This is apparently the first case report in the literature that clearly shows the treatment with sivelestat sodium and strict fluid management ended in a favorable outcome, as reducing EVLWI measured by the PiCCO system in an idiopathic ARDS patient.

  19. Consensus document for the diagnosis and treatment of idiopathic pulmonary fibrosis: Joint Consensus of Sociedade Portuguesa de Pneumologia, Sociedade Portuguesa de Radiologia e Medicina Nuclear e Sociedade Portuguesa de Anatomia Patológica.

    PubMed

    Robalo Cordeiro, C; Campos, P; Carvalho, L; Campainha, S; Clemente, S; Figueiredo, L; Jesus, J M; Marques, A; Souto-Moura, C; Pinto Basto, R; Ribeiro, A; Serrado, M; Morais, A

    2016-01-01

    Idiopathic pulmonary fibrosis is a rare interstitial lung disease included in the Idiopathic Interstitial Pneumonias group. Although several potential risk factors have been described, it is a progressive fibrosing disease of unknown cause affecting mainly adults over 50 years and associated with a poor prognosis, reflected in a median survival of 2-3 years after diagnosis. The concept of a multidisciplinary working group for the diagnosis of idiopathic pulmonary fibrosis is based on the need to have experienced pulmonologists, radiologists and pathologists in the evaluation and correct treatment of the disease, and requires the use of all available data about individual patients, standardized (largely through High Resolution Computed Tomography and pathology when needed) as well as non-standardized data (laboratory, serology and biomarkers). This approach helps to increase diagnostic accuracy and is an internationally accepted recommendation. In regard to therapy, the situation has changed radically since the publication of the ATS/ERS/JRS/ALAT 2011 guidelines on the diagnosis and management of idiopathic pulmonary fibrosis where it was stressed that no proven therapy exists for this disease. Currently besides non-pharmacological treatment, therapy of complications and comorbidities and palliative care, nintedanib and pirfenidone, two compounds with pleiotropic mechanisms of action, are to date, the two drugs with confirmed efficacy in slowing functional decline and disease progression in idiopathic pulmonary fibrosis patients.

  20. Genetics of Interstitial Lung Disease: Vol de Nuit (Night Flight)

    PubMed Central

    Furukawa, Hiroshi; Oka, Shomi; Shimada, Kota; Tsuchiya, Naoyuki; Tohma, Shigeto

    2015-01-01

    Interstitial lung disease (ILD) is a chronic, progressive fibrotic lung disease with a dismal prognosis. ILD of unknown etiology is referred to as idiopathic interstitial pneumonia (IIP), which is sporadic in the majority of cases. ILD is frequently accompanied by rheumatoid arthritis (RA), systemic sclerosis (SSc), polymyositis/dermatomyositis (PM/DM), and other autoimmune diseases, and is referred to as collagen vascular disease-associated ILD (CVD-ILD). Susceptibility to ILD is influenced by genetic and environmental factors. Recent advances in radiographic imaging techniques such as high-resolution computed tomography (CT) scanning as well as high-throughput genomic analyses have provided insights into the genetics of ILD. These studies have repeatedly revealed an association between IIP (sporadic and familial) and a single nucleotide polymorphism (SNP) in the promoter region of the mucin 5B (MUC5B). HLA-DRB1*11 alleles have been reported to correlate with ILD in European patients with SSc, whereas in Japanese patients with RA, the HLA-DR2 serological group was identified. The aim of this review is to describe the genetic background of sporadic IIP, CVD-ILD, drug-induced-ILD (DI-ILD), pneumoconiosis, and hypersensitivity pneumonitis. The genetics of ILD is still in progress. However, this information will enhance the understanding of the pathogenesis of ILD and aid the identification of novel therapeutic targets for personalized medicine in future. PMID:26056507

  1. Undifferentiated connective tissue disease-associated interstitial lung disease: changes in lung function.

    PubMed

    Kinder, Brent W; Shariat, Cyrus; Collard, Harold R; Koth, Laura L; Wolters, Paul J; Golden, Jeffrey A; Panos, Ralph J; King, Talmadge E

    2010-04-01

    Undifferentiated connective tissue disease (UCTD) is a distinct clinical entity that may be accompanied by interstitial lung disease (ILD). The natural history of UCTD-ILD is unknown. We hypothesized that patients with UCTD-ILD would be more likely to have improvement in lung function than those with idiopathic pulmonary fibrosis (IPF) during longitudinal follow-up. We identified subjects enrolled in the UCSF ILD cohort study with a diagnosis of IPF or UCTD. The primary outcome compared the presence or absence of a > or = 5% increase in percent predicted forced vital capacity (FVC) in IPF and UCTD. Regression models were used to account for potential confounding variables. Ninety subjects were identified; 59 subjects (30 IPF, 29 UCTD) had longitudinal pulmonary function data for inclusion in the analysis. After accounting for baseline pulmonary function tests, treatment, and duration between studies, UCTD was associated with substantial improvement in FVC (odds ratio = 8.23, 95% confidence interval, 1.27-53.2; p = 0.03) during follow-up (median, 8 months) compared with IPF. Patients with UCTD-ILD are more likely to have improved pulmonary function during follow-up than those with IPF. These findings demonstrate the clinical importance of identifying UCTD in patients presenting with an "idiopathic" interstitial pneumonia.

  2. Thin-Section CT Characteristics and Longitudinal CT Follow-up of Chemotherapy Induced Interstitial Pneumonitis: A Retrospective Cohort Study.

    PubMed

    Lee, Han Na; Kim, Mi Young; Koo, Hyun Jung; Kim, Sung-Soo; Yoon, Dok Hyun; Lee, Jae Cheol; Song, Jin Woo

    2016-01-01

    To describe the computed tomography (CT) features of chemotherapy-induced interstitial pneumonitis (CIIP) with longitudinal follow-up.The study was approved by the local ethics committee. One hundred consecutive patients with CIIP between May 2005 and March 2015 were retrospectively enrolled. The initial CT was reviewed by 2 independent chest radiologists and categorized into 1 of 4 CT patterns in accordance with the 2013 guidelines for idiopathic interstitial pneumonia: nonspecific interstitial pneumonia (NSIP), organizing pneumonia (OP), hypersensitivity pneumonitis (HP) mimicking desquamative interstitial pneumonitis, and diffuse alveolar damage (DAD). We assessed semiquantitative analysis on a 5% scale to assess the extent of parenchymal abnormalities (emphysema, reticulation, ground-glass opacity, consolidation, honeycombing cyst) and their distribution on initial (n = 100), subsequent (n = 87), and second follow-up CT (n = 48). Interval changes in extent on follow-up CT were compared using paired t test. The clinic-radiologic factors were compared between Group 1 (NSIP and OP patterns) and Group 2 (HP and DAD patterns) using χ and independent t tests.The most common pattern of CIIP on the initial CT was HP (51%), followed by NSIP (23%), OP (20%), and DAD (6%). Diffuse ground-glass opacity was the most common pulmonary abnormality. The predominant distribution was bilateral (99%) and symmetric (82%), with no craniocaudal (60%) or axial (79%) dominance. Subsequent and second follow-up CTs showed decreased extent of total pulmonary abnormalities (P < 0.001, respectively). In comparison with Group 1 CIIP, Group 2 CIIP was more likely to be caused by molecularly targeted drugs (P = 0.030), appeared earlier (P = 0.034), and underwent more complete resolution (P < 0.001). Use of a CT pattern-recognition approach to CIIP is appropriate and practical in interpreting radiological findings. PMID:26765442

  3. Aspiration pneumonia

    MedlinePlus

    Anaerobic pneumonia; Aspiration of vomitus; Necrotizing pneumonia; Aspiration pneumonitis ... The type of bacteria that caused the pneumonia depends on: Your ... facility, for example) Whether you were recently hospitalized ...

  4. Mesalizine-Induced Acute Pancreatitis and Interstitial Pneumonitis in a Patient with Ulcerative Colitis.

    PubMed

    Chung, Min Jae; Lee, Jae Hee; Moon, Kyung Rye

    2015-12-01

    Ulcerative colitis (UC) is a chronic idiopathic inflammatory bowel disease. Mesalizine for the first-line therapy of UC has adverse effects include pancreatitis, pneumonia and pericarditis. UC complicated by two coexisting conditions, however, is very rare. Moreover, drug-related pulmonary toxicity is particularly rare. An 11-year-old male patient was hospitalized for recurring upper abdominal pain after meals with vomiting, hematochezia and exertional dyspnea developing at 2 weeks of mesalizine therapy for UC. The serum level of lipase was elevated. Chest X-ray and thorax computed tomography showed interstitial pneumonitis. Mesalizine was discontinued and steroid therapy was initiated. Five days after admission, symptoms were resolved and mesalizine was resumed after a drop in amylase and lipase level. Symptoms returned the following day, however, accompanied by increased the serum levels of amylase and lipase. Mesalizine was discontinued again and recurring symptoms rapidly improved.

  5. Macitentan for the treatment of idiopathic pulmonary fibrosis: the randomised controlled MUSIC trial.

    PubMed

    Raghu, Ganesh; Million-Rousseau, Rachel; Morganti, Adele; Perchenet, Loyc; Behr, Juergen

    2013-12-01

    Idiopathic pulmonary fibrosis is a progressive, fatal disease. This prospective, randomised, double-blind, multicentre, parallel-group, placebo-controlled phase II trial (NCT00903331) investigated the efficacy and safety of the endothelin receptor antagonist macitentan in idiopathic pulmonary fibrosis. Eligible subjects were adults with idiopathic pulmonary fibrosis of <3 years duration and a histological pattern of usual interstitial pneumonia on surgical lung biopsy. The primary objective was to demonstrate that macitentan (10 mg once daily) positively affected forced vital capacity versus placebo. Using a centralised system, 178 subjects were randomised (2:1) to macitentan (n=119) or placebo (n=59). The median change from baseline up to month 12 in forced vital capacity was -0.20 L in the macitentan arm and -0.20 L in the placebo arm. Overall, no differences between treatments were observed in pulmonary function tests or time to disease worsening or death. Median exposures to macitentan and placebo were 14.5 months and 15.0 months, respectively. Alanine and/or aspartate aminotransferase elevations over three times upper limit of normal arose in 3.4% of macitentan-treated subjects and 5.1% of placebo recipients. In conclusion, the primary objective was not met. Long-term exposure to macitentan was well tolerated with a similar, low incidence of elevated hepatic aminotransferases in each treatment group.

  6. Idiopathic Gastroparesis

    PubMed Central

    Parkman, Henry P.

    2015-01-01

    Gastroparesis is a chronic symptomatic disorder of the stomach characterized by delayed emptying without evidence of mechanical obstruction. The three main causes of gastroparesis are diabetic, postsurgical, and idiopathic. Idiopathic gastroparesis refers to gastroparesis of unknown cause, that is, not from diabetes, not from prior gastric surgery, and not related to other endocrine, neurologic, rheumatologic causes of gastroparesis. The gastroparesis should not be related to medications that can delay gastric emptying, such as narcotic analgesic or anticholinergic medications. There is overlap in the symptoms of idiopathic gastroparesis and functional dyspepsia. A substantial minority of patients with functional dyspepsia can have delayed gastric emptying, blurring the distinction between idiopathic gastroparesis and functional dyspepsia. Patients with idiopathic gastroparesis often have a constellation of symptoms including nausea, vomiting, early satiety, postprandial fullness, and upper abdominal pain. Although the presentation of idiopathic gastroparesis is relatively similar to diabetic gastroparesis, abdominal pain occurs more often in idiopathic gastroparesis, whereas nausea and vomiting are more severe in diabetic gastroparesis. Treatment may employ agents used for diabetic gastroparesis and functional dyspepsia, including dietary management, prokinetics agents, antiemetic agents, and symptom modulators. Current treatment options do not adequately address clinical need for idiopathic gastroparesis. PMID:25667023

  7. Interstitial keratitis

    MedlinePlus

    ... cornea. This condition is often caused by infections. Syphilis is the most common cause of interstitial keratitis, ... Tuberculosis In the United States, most cases of syphilis are recognized and treated before this eye condition ...

  8. Idiopathic chronic eosinophilic pneumonia. A clinical and follow-up study of 62 cases. The Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P).

    PubMed

    Marchand, E; Reynaud-Gaubert, M; Lauque, D; Durieu, J; Tonnel, A B; Cordier, J F

    1998-09-01

    Idiopathic chronic eosinophilic pneumonia (CEP) is a rare disorder of unknown cause with nonspecific respiratory and systemic symptoms but rather characteristic peripheral alveolar infiltrates on imaging, developing mainly in women and in atopic subjects. The disorder is highly responsive to oral corticosteroid therapy, but relapses are frequent on reducing or stopping treatment. The long-term course of the disease and data regarding outcome, particularly the need for prolonged oral corticosteroid therapy and the development of severe asthma, are somewhat contradictory. A multicentric retrospective study was conducted in an attempt to describe better the initial features and, above all, the later course of CEP in a large homogeneous series of 62 stringently selected patients of whom 46 were followed for more than 1 year. The prevalence of smokers was low (6.5%) and about half of our patients (51.6%) had a previous, and often prolonged, history of asthma. The clinical and roentgenographic features were in keeping with previous studies, but we found that computed tomography could disclose ground glass opacities not detected by X-ray, and that migratory infiltrates before treatment were more frequent (25.5%) than reported previously. The bronchoalveolar lavage cellular count always showed a striking eosinophilic pattern, thus allowing distinction between CEP and cryptogenic organizing pneumonia, both syndromes sharing many common clinical and imaging features. About two-thirds of the patients (68%) showed a ventilatory defect in pulmonary function tests, with about one-half of these presenting with an obstructive pattern, sometimes without previous asthma. Along with the submucosal eosinophilic infiltration noted in 2 patients without ventilatory defect, this is strong evidence to confirm that CEP is not only an alveolointerstitial but also an airway disease. The dramatic response to oral corticosteroid therapy was observed in all treated patients. Although only 1

  9. TNF-receptor inhibitor therapy for the treatment of children with idiopathic pneumonia syndrome. A joint Pediatric Blood and Marrow Transplant Consortium and Children's Oncology Group Study (ASCT0521).

    PubMed

    Yanik, Gregory A; Grupp, Stephan A; Pulsipher, Michael A; Levine, John E; Schultz, Kirk R; Wall, Donna A; Langholz, Bryan; Dvorak, Christopher C; Alangaden, Keith; Goyal, Rakesh K; White, Eric S; Collura, Jennifer M; Skeens, Micah A; Eid, Saada; Pierce, Elizabeth M; Cooke, Kenneth R

    2015-01-01

    Idiopathic pneumonia syndrome (IPS) is an acute, noninfectious lung disorder associated with high morbidity and mortality after hematopoietic cell transplantation. Previous studies have suggested a role for TNFα in the pathogenesis of IPS. We report a multicenter phase II trial investigating a soluble TNF-binding protein, etanercept (Enbrel, Amgen, Thousand Oaks, CA), for the treatment of pediatric patients with IPS. Eligible patients were < 18 years old, within 120 days after transplantation, and with radiographic evidence of a diffuse pneumonitis. All patients underwent a pretherapy broncho-alveolor lavage (BAL) to establish the diagnosis of IPS. Systemic corticosteroids (2.0 mg/kg/day) plus etanercept (.4 mg/kg twice weekly × 8 doses) were administered. Response was defined as survival and discontinuation of supplemental oxygen support by day 28 of study. Thirty-nine patients (median age, 11 years; range, 1 to 17) were enrolled, with 11 of 39 patients nonevaluable because of identification of pathogens from their pretherapy BAL. In the remaining 28 patients, the median fraction of inspired oxygen at study entry was 45%, with 17 of 28 requiring mechanical ventilation. Complete responses were seen in 20 (71%) patients, with a median time to response of 10 days (range, 1 to 24). Response rates were higher for patients not requiring mechanical ventilation at study entry (100% versus 53%, P = .01). Overall survival at 28 days and 1 year after therapy were 89% (95% confidence interval [CI], 70% to 96%) and 63% (95% CI, 42% to 79%), respectively. Plasma levels of proinflammatory cytokines were significantly increased at onset of therapy, subsequently decreasing in responding patients. The addition of etanercept to high-dose corticosteroids was associated with high response rates and survival in children with IPS.

  10. Idiopathic scoliosis.

    PubMed

    Yaman, Onur; Dalbayrak, Sedat

    2014-01-01

    Scoliosis refers to curves exceeding 10 degrees observed through posterioanterior direct radiography. In fact, the diagnosis for idiopathic scoliosis is accepted to exclude already available causes. The aim of this paper was to review the etiopathogenesis, classification systems and the treatment management of idiopathic scoliosis. A search in the National Library of Medicine (Pubmed) database using the key words 'idiopathic' and 'scoliosis' was performed. For the literature review, papers concerning the etiopathogenesis, classification and treatment were selected among these articles. A search in the National Library of Medicine (Pubmed) database using the key words 'idiopathic' and 'scoliosis' yielded 4518 articles published between 1947 and 2013. The main hypothesis put forward included genetic factors, hormonal factors, bone and connective tissue anomalies. King, Lenke, Coonrad and Peking Union Medical College (PUMC) classifications were the main classification systems for idiopathic scoliosis. Exercise, bracing and anterior, posterior or combined surgery when indicated are the choices for the treatment. Every idiopathic scoliosis case has to be managed to its own characteristics. It is the post-operative appearance that the surgeons are perhaps the least interested but the adolescent patients the most interested in. The aim of scoliosis surgery is to restore the spine without neurological deficit. PMID:25269032

  11. Idiopathic scoliosis.

    PubMed

    Yaman, Onur; Dalbayrak, Sedat

    2014-01-01

    Scoliosis refers to curves exceeding 10 degrees observed through posterioanterior direct radiography. In fact, the diagnosis for idiopathic scoliosis is accepted to exclude already available causes. The aim of this paper was to review the etiopathogenesis, classification systems and the treatment management of idiopathic scoliosis. A search in the National Library of Medicine (Pubmed) database using the key words 'idiopathic' and 'scoliosis' was performed. For the literature review, papers concerning the etiopathogenesis, classification and treatment were selected among these articles. A search in the National Library of Medicine (Pubmed) database using the key words 'idiopathic' and 'scoliosis' yielded 4518 articles published between 1947 and 2013. The main hypothesis put forward included genetic factors, hormonal factors, bone and connective tissue anomalies. King, Lenke, Coonrad and Peking Union Medical College (PUMC) classifications were the main classification systems for idiopathic scoliosis. Exercise, bracing and anterior, posterior or combined surgery when indicated are the choices for the treatment. Every idiopathic scoliosis case has to be managed to its own characteristics. It is the post-operative appearance that the surgeons are perhaps the least interested but the adolescent patients the most interested in. The aim of scoliosis surgery is to restore the spine without neurological deficit.

  12. Guidelines for the diagnosis and treatment of idiopathic pulmonary fibrosis. Sociedad Española de Neumología y Cirugía Torácica (SEPAR) Research Group on Diffuse Pulmonary Diseases.

    PubMed

    Xaubet, Antoni; Ancochea, Julio; Bollo, Elena; Fernández-Fabrellas, Estrella; Franquet, Tomás; Molina-Molina, Maria; Montero, Maria Angeles; Serrano-Mollar, Anna

    2013-08-01

    Idiopathic pulmonary fibrosis is defined as a chronic fibrosing interstitial pneumonia limited to the lung, of unknown cause, with poor prognosis and few treatment options. In recent years there has been an increase in their prevalence, probably due to the optimization of diagnostic methods and increased life expectancy. The ATS/ERS Consensus (2000) established the diagnostic criteria and recommendations for the assessment of the disease course and treatment. Later studies have helped to redefine diagnostic criteria and treatment options. In 2011, an international consensus was published, establishing diagnostic criteria and new treatment strategies. These guidelines have been updated with the newest aspects of diagnosis and treatment of idiopathic pulmonary fibrosis. A level of evidence has been identified for the most relevant questions, particularly with regard to treatment options.

  13. Guidelines for the diagnosis and treatment of idiopathic pulmonary fibrosis. Sociedad Española de Neumología y Cirugía Torácica (SEPAR) Research Group on Diffuse Pulmonary Diseases.

    PubMed

    Xaubet, Antoni; Ancochea, Julio; Bollo, Elena; Fernández-Fabrellas, Estrella; Franquet, Tomás; Molina-Molina, Maria; Montero, Maria Angeles; Serrano-Mollar, Anna

    2013-08-01

    Idiopathic pulmonary fibrosis is defined as a chronic fibrosing interstitial pneumonia limited to the lung, of unknown cause, with poor prognosis and few treatment options. In recent years there has been an increase in their prevalence, probably due to the optimization of diagnostic methods and increased life expectancy. The ATS/ERS Consensus (2000) established the diagnostic criteria and recommendations for the assessment of the disease course and treatment. Later studies have helped to redefine diagnostic criteria and treatment options. In 2011, an international consensus was published, establishing diagnostic criteria and new treatment strategies. These guidelines have been updated with the newest aspects of diagnosis and treatment of idiopathic pulmonary fibrosis. A level of evidence has been identified for the most relevant questions, particularly with regard to treatment options. PMID:23742884

  14. Mineral Oil Aspiration Related Juvenile Idiopathic Arthritis

    PubMed Central

    Nelson, Andrew D.; Fischer, Philip R.; Reed, Ann M.; Wylam, Mark E.

    2015-01-01

    We describe the development of rheumatoid factor-positive migratory polyarthritis in a 5-year-old male who had been administered bidaily oral mineral oil as a laxative since birth. Minor respiratory symptoms, radiographic and bronchoscopic findings were consistent with chronic lipoid pneumonia. We speculate that immune sensitization to mineral oil promoted the clinical syndrome of juvenile idiopathic arthritis. PMID:26171269

  15. [The Role of Bronchoalveolar Lavage in the Diagnosis of Idiopathic Pulmonary Fibrosis: An Investigation of the Relevance of the Protein Content].

    PubMed

    Schildge, J; Frank, J; Klar, B

    2016-07-01

    Although bronchoalveolar lavage (BAL) is often used in the diagnosis of interstitial lung diseases (ILDs), its importance in investigating, in particular, idiopathic pulmonary fibrosis (IPF) is controversial. The cell distributions in the BAL are taken into account in the clinical routine, non-cellular characteristics of the BAL play no role.Using mathematical modeling of data, the present work investigated the extent to which BAL features enable drawing conclusions about the underlying ILK or help exclude IPF. Included in the calculation are cellular findings of the BAL, in addition the protein and albumin content of the BAL, the nicotine history (pack years), and spirometry (FEV1, IVC).Using linear discriminant analysis and creating classification trees, the relevance of the characteristics of 806 patients with ILK was examined (183 IPF, 191 cryptogenic organizing pneumonia, 147 lung involvement in autoimmune disease, 97 respiratory bronchiolitis interstitial lung disease, 118 extrinsic allergic alveolitis, 41 lymphocytic interstitial pneumonia (LIP), 23 non-specific interstitial pneumonia (NSIP), 88 controls).There was a close positive relationship between protein levels and lymphocytes in the group as a whole. No such correlations were seen in IPF and NSIP. Albumin was closely correlated with the protein content in all groups.The lymphocytes are best suited to distinguish between different ILDs. Yet, a reliable calculation of the ILD is not possible on the basis of the investigated factors, the classification error ranged from 23.5 % (IPF) to 100 % (LIP, NSIP).Constellations that likely (> 99 %) speak against an IPF are lymphocytosis > 34 % or protein content > 347 mg/l. The same applies to the constellation: lymphocytes > 25 % together with protein > 250 mg/l.In ILD, BAL findings can narrow the diagnosis, but they are seldom diagnostic. BAL can make an important contribution to excluding of IPF. PMID:27218212

  16. Many radiologic facies of pneumococcal pneumonia

    SciTech Connect

    Kantor, H.G.

    1981-12-01

    In 1978, 89 patients were treated for (S. pneumoniae) pneumonia at New York Hospital-Cornell Medical Center. Only 40 cases met rather strict diagnostic criteria. Of these, 12 demonstrated the classical consolidative (air space) pattern usually ascribed to this disease. A bronchopneumonic (patch) pattern was demonstrated in an equal number of patients; interstitial (irregular linear) infiltrates were manifest in nine cases and a mixed interstitial and patchy presentation shown in seven cases. Absence of the consolidative pattern does not exclude pneumococcal pneumonia. Bacteriologic investigation is required to determine the proper diagnosis and course of therapy.

  17. Ultrastructural differences between diabetic and idiopathic gastroparesis

    PubMed Central

    Faussone-Pellegrini, Maria Simonetta; Grover, Madhusudan; Pasricha, Pankaj J; Bernard, Cheryl E; Lurken, Matthew S; Smyrk, Thomas C; Parkman, Henry P; Abell, Thomas L; Snape, William J; Hasler, William L; Ünalp-Arida, Aynur; Nguyen, Linda; Koch, Kenneth L; Calles, Jorges; Lee, Linda; Tonascia, James; Hamilton, Frank A; Farrugia, Gianrico

    2012-01-01

    Abstract The ultrastructural changes in diabetic and idiopathic gastroparesis are not well studied and it is not known whether there are different defects in the two disorders. As part of the Gastroparesis Clinical Research Consortium, full thickness gastric body biopsies from 20 diabetic and 20 idiopathic gastroparetics were studied by light microscopy. Abnormalities were found in many (83%) but not all patients. Among the common defects were loss of interstitial cells of Cajal (ICC) and neural abnormalities. No distinguishing features were seen between diabetic and idiopathic gastroparesis. Our aim was to provide a detailed description of the ultrastructural abnormalities, compare findings between diabetic and idiopathic gastroparesis and determine if patients with apparently normal immunohistological features have ultrastructural abnormalities. Tissues from 40 gastroparetic patients and 24 age- and sex-matched controls were examined by transmission electron microscopy (TEM). Interstitial cells of Cajal showing changes suggestive of injury, large and empty nerve endings, presence of lipofuscin and lamellar bodies in the smooth muscle cells were found in all patients. However, the ultrastructural changes in ICC and nerves differed between diabetic and idiopathic gastroparesis and were more severe in idiopathic gastroparesis. A thickened basal lamina around smooth muscle cells and nerves was characteristic of diabetic gastroparesis whereas idiopathic gastroparetics had fibrosis, especially around the nerves. In conclusion, in all the patients TEM showed abnormalities in ICC, nerves and smooth muscle consistent with the delay in gastric emptying. The significant differences found between diabetic and idiopathic gastroparesis offers insight into pathophysiology as well as into potential targeted therapies. PMID:21914127

  18. Ultrasound in the evaluation of interstitial pneumonia.

    PubMed

    Lo Giudice, V; Bruni, A; Corcioni, E; Corcioni, B

    2008-03-01

    Sommario PREMESSA: L'ecografia del torace soltanto di recente ha assunto una sua completa dignità di metodica diagnostica: esperti ecografisti, con esperienza pneumologica, hanno, infatti, dimostrato la possibilità di una applicazione degli ultrasuoni in ambito toraco-polmonare, soprattutto in situazioni critiche, quali quelle emergenti in urgenza, in terapia intensiva o in ambito pediatrico o in corso di gravidanze. SCOPO: Da queste premesse si origina il lavoro, che si propone di individuare, se esistenti, gli aspetti ultrasonografici delle polmoniti interstiziali a genesi infettiva, e il ruolo di supporto (alla radiologia) degli ultrasuoni anche in questo ambito. MATERIALI E METODI: Sono stati studiati 55 soggetti afferiti, nel quinqennio 2001–2006, alla UOS di Ecografia toracica (UOC Pneumologia I) dell'Ospedale M. Santo e dell'UOS di Ecografia Internistica (UOC di Medicina Generale) dell'Ospedale di Rogliano dell'AO di Cosenza, perché affetti da sospetta patologia infettiva respiratoria virale o simil-virale. Tali pazienti sono stati valutati con indagini clinico-funzionali e strumentali (anamnesi + es. obiettivo + esami ematochimici + prove di funzionalità respiratoria + broncoscopia + Rx e HRTC del torace) e con esame ecografico, in cieco, del torace. RISULTATI: Dalla valutazione comparativa tra dati clinico-laboratoristico-strumentali e dati ecografici è emerso che i 55 soggetti studiati sono risultati affetti da polmonite interstiziale. In tali soggetti la diagnosi è stata formulata con l'ausilio delle comuni tecniche di studio, ma anche l'esame US ha permesso la individuazione di segni considerati diagnostici (Lichtenstein) di patologia interstiziale. Dei soggetti esaminati, infatti, 31 (56,36%) hanno mostrato all'ecografia la presenza di artefatti a coda di cometa (>5 per lato) nelle regioni anteriore e laterale del polmone (dato patognomico di patologia interstiziale) e 24 (46,64%) di “quadri misti” (aree di addensamento ecografico associate a ring down). In 34 (61,82%) casi sono stati descritti associati aspetti di patologia pleurica. CONCLUSIONI: Attraverso l'osservazione di segni ultrasonografici e la correlazione di questi con quelli clinico-laboratoristico-strumentali di routine, gli autori valutano la possibilità di attribuire rilievo anche alla indagine US nella diagnosi di polmonite interstiziale ad eziologia infettiva e, senza voler sostituire gli US alle tradizionali e opportune tecniche di approccio e diagnosi, la propongono quale complementare tecnica metodologica di indagine.

  19. [Diagnosis and problems in therapy of interstitial lung disease associated with rheumatoid arthritis].

    PubMed

    Bestaev, D V; Karateev, D E; Nasonov, E L

    2013-01-01

    Rheumatoid arthritis (RA) is an inflammatory rheumatic disease of unknown etiology, which is characterized by symmetric, chronic, and erosive arthritis (synovitis) of the peripheral joints and systemic inflammatory involvement of the viscera. Lung pathology, including interstitial lung disease (ILD), is one of the common extra-articular manifestations in RA. ILD is considered to be present in almost 25% of the RA patients. To study a prognosis in RA patients with ILD was the objective of some investigations in the past decade, the majority of which concluded that the mean survival after the diagnosis was about 3 years. These indicators may reflect the predominance of usual interstitial pneumonia (UIP) in patients in specific trials as this type of lung disease is associated with a poorer prognosis. In addition, there are discrepant results on survival differences between RA patients with ILD and those with idiopathic ILD. However, the data were limited by a small number of cases in both medical centers and daily clinical practice. ILD is the only extra-articular manifestation of RA, the rate of which is increasing. ILD is considered to be a cause of death in nearly 6% of all the patients with RA. The pattern of ILD may be determined by high-resolution computed tomography and may be a major prognostic marker; the development of UIP is worst. The material is dedicated to the successes recently achieved in the diagnosis and therapy of RA-associated ILD. The state-of-the-art of investigations in this area is discussed.

  20. Assessment and management of connective tissue disease-associated interstitial lung disease.

    PubMed

    Fischer, Aryeh; Chartrand, Sandra

    2015-01-01

    The intersection of the connective tissue diseases (CTD) and the interstitial lung diseases (ILD) is complex. Although often considered as a single entity, "CTD-ILD" actually reflects a heterogeneous spectrum of diverse CTDs and a variety of patterns of interstitial pneumonia. The evaluation of patients with CTD that develop ILD, or the assessment for underlying CTD in those presenting with presumed "idiopathic" ILD can be challenging and these evaluations can be optimized by effective multidisciplinary collaboration. When a diagnosis of CTD-ILD is confirmed, careful and thorough assessments to determine extra- versus intra-thoracic disease activity, and degrees of impairment are needed. Pharmacologic intervention with immunosuppression is the mainstay of therapy for all forms of CTD-ILD, but should be reserved only for those that demonstrate clinically significant and/or progressive disease. The management of CTD-ILD is not yet evidence based and there is a desperate need for controlled trials across the spectrum of CTD-ILD. Non-pharmacologic management strategies and addressing comorbidities or aggravating factors should be part of a comprehensive treatment plan for individuals with CTD-ILD.

  1. Epidemiology of Idiopathic Pulmonary Fibrosis in Northern Italy

    PubMed Central

    Caminati, Antonella; Conti, Sara; Cesana, Giancarlo

    2016-01-01

    Background Idiopathic pulmonary fibrosis (IPF) is the most common and severe form of idiopathic interstitial pneumonia. Despite its clinical relevance, few studies have examined the epidemiology of IPF and temporal variation in disease incidence and prevalence. Aim of the study was to investigate the prevalence, incidence and trends of IPF in Lombardy, a region with nearly 10 million inhabitants, during 2005–2010. Methods For the identification of IPF patients, we used healthcare administrative databases of Lombardy Healthcare System and adopted three algorithms: generic, broad and narrow case definition (GCD, BCD, NCD). IPF cases were identified according to diagnoses reported in inpatient and outpatient claims occurred during 2000–2010. We estimated age- and sex-adjusted annual prevalence and incidence rates from 2005 to 2010, thus allowing for a 5-year washout period. Results The mean annual incidence rate was estimated at 2.3 and 5.3 per 100,000 person-years using NCD and GCD, respectively. IPF incidence was higher among males, and increased with age. Trend remained stable over the years. The estimated annual prevalence rate was 35.5, 22.4, and 12.6 per 100,000 person-years using GCD, BCD and NCD, respectively, and increased with age. Moreover, we observed a positive trend over the years. Using BCD and NCD, prevalence was higher among males. Conclusions The results of this study, which is one of the largest population-based survey ever conducted according to strict criteria, indicated that prevalence of IPF increased across the years while incidence remained stable, thus suggesting that survival with IPF has improved. PMID:26841042

  2. Interstitial nephritis.

    PubMed Central

    Dixon, A J; Winearls, C G; Dunnill, M S

    1981-01-01

    The clinical and pathological findings are reviewed in ten cases where renal biopsy showed abnormalities predominantly within the interstitium. In six the nephritis was considered to be drug-induced; in two the aetiology was slightly obscure but the most likely diagnosis was considered to be sarcoidosis. Of the remaining two cases one was chronic pyelonephritis and the other polyarteritis nodosa. The diagnosis and pathogenesis of the renal lesions are discussed and attention is drawn to the importance of distinguishing primary interstitial changes from those found in association with glomerular disease. Images PMID:7251904

  3. Idiopathic pulmonary fibrosis: current treatment options and critical appraisal of nintedanib

    PubMed Central

    Bonella, Francesco; Stowasser, Susanne; Wollin, Lutz

    2015-01-01

    Idiopathic pulmonary fibrosis (IPF) is the most common type of idiopathic interstitial pneumonia and is characterized by a poor prognosis, with an estimated 5-year survival of approximately 20%. Progressive and irreversible lung functional impairment leads to chronic respiratory insufficiency with a severely impaired quality of life. In the last 2 decades, novel treatments for IPF have been developed as a consequence of an increasing understanding of disease pathogenesis and pathobiology. In IPF, injured dysfunctional alveolar epithelial cells promote fibroblast recruitment and proliferation, resulting in scarring of the lung tissue. Recently, pirfenidone and nintedanib have been approved for the treatment of IPF, having shown efficacy to slow functional decline and disease progression. This article focuses on the pharmacologic characteristics and clinical evidence supporting the use of nintedanib, a potent small-molecule tyrosine kinase inhibitor, as therapy for IPF. After introducing the mechanism of action and pharmacokinetics, an overview of the safety and efficacy results from the most recent clinical trials of nintedanib in IPF is presented. PMID:26715838

  4. Idiopathic pulmonary fibrosis: an Australian perspective.

    PubMed

    Prasad, J; Holland, A E; Glaspole, I; Westall, G

    2016-06-01

    Idiopathic pulmonary fibrosis is a progressive interstitial lung disease of unknown aetiology with a dismal median survival of 3 years. Patients typically develop progressive dyspnoea and increasing exercise limitation. With a rising incidence and prevalence, an unpredictable disease course and limited treatment options, it is rapidly becoming an important public health concern. To date, lung transplantation has been the sole viable hope for treatment for those who qualify. However, the landscape of idiopathic pulmonary fibrosis management is changing, with the recent emergence of novel pharmacotherapy shown to have a favourable influence on the natural history of this disease. PMID:27257148

  5. Interstitial cystitis - resources

    MedlinePlus

    Resources - interstitial cystitis ... The following organizations are good resources for information on interstitial cystitis : Interstitial Cystitis Association -- www.ichelp.org National Kidney and Urologic Diseases Information Clearinghouse -- www.kidney.niddk. ...

  6. Mast cell chymase: an indispensable instrument in the pathological symphony of idiopathic pulmonary fibrosis?

    PubMed

    Kosanovic, Djuro; Dahal, Bhola Kumar; Wygrecka, Malgorzata; Reiss, Irwin; Günther, Andreas; Ghofrani, Hossein Ardeschir; Weissmann, Norbert; Grimminger, Friedrich; Seeger, Werner; Schermuly, Ralph Theo; Banat, Gamal-Andre

    2013-06-01

    Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and fatal lung disease with no known etiology and treatment options. The hallmarks of the histopathology, which is characteristic of usual interstitial pneumonia (UIP) pattern, include interstitial fibrosis, honeycomb changes and fibroblast foci that develop owing to fibroblast proliferation and excessive matrix deposition. Although the complete pathomechanism is not yet understood, several molecular culprits, including transforming growth factor (TGF)-β, Angiotensin (Ang) II, endothelin (ET)-1, matrix metalloproteinases (MMPs) and cytokines have been identified. IPF is increasingly believed to be an epithelial-driven disease; however, the literature does support an implication of altered immune response and inflammatory processes in the onset or progression of the disease. Mast cells (MCs) are multifunctional tissue resident cells involved in the inflammatory and immune response. An increasing body of evidence suggests a role of MCs and their mediator chymase in the pathology of IPF. With regard to the underlying mechanisms, it is conceivable that MC chymase may function via activation or processing of factors such as proteases, cytokines and growth factors. In this review, we will discuss how MC chymase is linked to and can potentially contribute to the development of IPF. Moreover, the findings from animal model studies will be discussed to highlight the chymase inhibitors as a promising strategy for the treatment of pulmonary fibrosis.

  7. Interstitial cystitis.

    PubMed

    Erickson, D R; Davies, M F

    1998-01-01

    Interstitial cystitis (IC) is a multifactorial syndrome with symptoms of pelvic or perineal pain, urinary frequency and urgency. The etiologies are unknown, but several theories have been proposed. Diagnosis is often delayed because most of the conventional evaluation is normal. Pelvic examination is normal except for bladder tenderness. Urodynamics are normal except for increased bladder sensitivity and low capacity. Urinalysis, urine culture and office cystoscopy are also normal. The diagnostic test is cystoscopy under anesthesia with bladder distension. Small submucosal hemorrhages (glomerulations) or ulcers appear after distension. Many empiric treatments have been proposed for IC. None is universally effective, and so treatments are tried sequentially until good symptom relief is achieved. Bladder distension gives excellent (but transient) relief in some patients, especially those with severe bladder inflammation (who also tend to be older). A variety of oral, intravesical and adjunctive treatments are also described.

  8. Pneumonia (image)

    MedlinePlus

    Pneumonia is an inflammation of the lungs caused by an infection. Many different organisms can cause it, including bacteria, viruses, and fungi. Pneumonia is a common illness that affects millions of ...

  9. A case of interstitial lung disease associated with clinically amyopathic dermatomyositis: radiologic-pathologic correlation.

    PubMed

    Okubo, Gosuke; Noma, Satoshi; Nishimoto, Yuko; Sada, Ryuichi; Kobashi, Yoichiro

    2013-01-01

    This case report describes a 64-year-old woman with interstitial lung disease associated with clinically amyopathic dermatomyositis. Chest computed tomography revealed consolidations along bronchovascular bundles in the periphery of the lower lungs. Interstitial lung disease developed acutely, and the patient died 3 months after the clinical diagnosis. An autopsy was performed, and a large section of the lung specimen was prepared. Various interstitial lesions including organizing pneumonia, cellular and fibrotic nonspecific interstitial pneumonia, and diffuse alveolar damage were seen in the large section. Correlating the large section and computed tomography images was useful for determining the distribution of diffuse alveolar damage.

  10. Cough in interstitial lung disease.

    PubMed

    Garner, Justin; George, Peter M; Renzoni, Elisabetta

    2015-12-01

    Cough in the context of interstitial lung disease (ILD) has not been the focus of many studies. However, chronic cough has a major impact on quality of life in a significant proportion of patients with ILD. For the purpose of this review, we have chosen to highlight some of the more frequently encountered diffuse lung diseases including idiopathic pulmonary fibrosis, sarcoidosis, hypersensitivity pneumonitis and systemic sclerosis associated ILD. Many of the underlying mechanisms remain speculative and further research is now required to elucidate the complex pathways involved in the pathogenesis of chronic cough in ILD. This will hopefully pave the way for the identification of new therapeutic agents to alleviate this distressing and often intractable symptom.

  11. Idiopathic Inflammatory Myopathies

    PubMed Central

    Barohn, Richard J.; Amato, Anthony

    2014-01-01

    The idiopathic inflammatory myopathies (IIM) consist of rare heterogenous autoimmune disorders that present with marked proximal and symmetric muscle weakness, except for distal and asymmetric weakness in inclusion body myositis (IBM). Besides frequent creatine kinase (CK) elevation, the electromyogram confirms the presence of an irritative myopathy. Extramuscular involvement affects a significant number of cases with interstitial lung disease (ILD), cutaneous in dermatomyositis (DM), systemic or joint manifestations and increased risk of malignancy especially in DM. Myositis specific autoantibodies influence phenotype of the IIM. Jo-1 antibodies are frequently associated with ILD and the newly described HMG-CoA reductase antibodies are characteristic of autoimmune necrotizing myopathy (NM). Muscle pathology ranges from inflammatory exudates of variable distribution, to intact muscle fiber invasion, necrosis, phagocytosis and in the case of IBM rimmed vacuoles and protein deposits. Despite many similarities, the IIM are a quite heterogeneous from the histopathological and pathogenetic standpoints in addition to some clinical and treatment-response difference. The field has witnessed significant advances in our understanding of pathophysiology and treatment of these rare disorders. In this review, we focus on DM, polymyositis (PM) and NM and examine current and promising therapies. The reader interested in more details on IBM is referred to the corresponding chapter in this issue. PMID:25037081

  12. Increase of nitrosative stress in patients with eosinophilic pneumonia

    PubMed Central

    2011-01-01

    Background Exhaled nitric oxide (NO) production is increased in asthma and reflects the degree of airway inflammation. The alveolar NO concentration (Calv) in interstitial pneumonia is reported to be increased. However, it remains unknown whether NO production is increased and nitrosative stress occurs in eosinophilic pneumonia (EP). We hypothesized that nitrosative stress markers including Calv, inducible type of NO synthase (iNOS), and 3-nitrotyrosine (3-NT), are upregulated in EP. Methods Exhaled NO including fractional exhaled NO (FENO) and Calv was measured in ten healthy subjects, 13 patients with idiopathic pulmonary fibrosis (IPF), and 13 patients with EP. iNOS expression and 3-NT formation were assessed by immunocytochemistory in BALf cells. The exhaled NO, lung function, and systemic inflammatory markers of the EP patients were investigated after corticosteroid treatment for 4 weeks. Results The Calv levels in the EP group (14.4 ± 2.0 ppb) were significantly higher than those in the healthy subjects (5.1 ± 0.6 ppb, p < 0.01) and the IPF groups (6.3 ± 0.6 ppb, p < 0.01) as well as the FENO and the corrected Calv levels (all p < 0.01). More iNOS and 3-NT positive cells were observed in the EP group compared to the healthy subject and IPF patient. The Calv levels had significant positive correlations with both iNOS (r = 0.858, p < 0.05) and 3-NT positive cells (r = 0.924, p < 0.01). Corticosteroid treatment significantly reduced both the FENO (p < 0.05) and the Calv levels (p < 0.01). The magnitude of reduction in the Calv levels had a significant positive correlation with the peripheral blood eosinophil counts (r = 0.802, p < 0.05). Conclusions These results suggested that excessive nitrosative stress occurred in EP and that Calv could be a marker of the disease activity. PMID:21679473

  13. Pneumatoceles and pneumothorax after Pneumocystis carinii pneumonia.

    PubMed

    Sauleda, J; Aran, X; Gea, J; Aguar, M C; Sanz, M; Broquetas, J M

    1993-01-01

    Pneumocystis carinii pneumonia (PCP) is common in patients with AIDS. The usual chest X-ray pattern is a diffuse interstitial pulmonary infiltrate. Nevertheless, unusual roentgenographic forms can appear. A patient with PCP that resulted in pneumatoceles and a further pneumothorax is described. PMID:8284529

  14. Interstitial lung disease

    MedlinePlus

    Diffuse parenchymal lung disease; Alveolitis; Idiopathic pulmonary pneumonitis (IPP) ... The lungs contain tiny air sacs (alveoli), which is where oxygen is absorbed. These air sacs expand with each ...

  15. Viral pneumonia

    MedlinePlus

    ... Names Pneumonia - viral; "Walking pneumonia" - viral Images Lungs Respiratory system References Lee FE, Treanor J. Viral infections. In: Mason RJ, VC Broaddus, Martin TR, et al, eds. Murray and Nadel’s Textbook of Respiratory Medicine . 5th ed. Philadelphia, PA: Saunders Elsevier; 2010: ...

  16. Viral pneumonia.

    PubMed

    Greenberg, S B

    1991-09-01

    Viral pneumonias are common in infants and young children but rare in adults. Respiratory syncytial virus (RSV) and para-influenza viruses are the most frequent viral pathogens in infants and children. Influenza virus types A and B account for over one half of viral pneumonias in adults. Immunocompromised hosts are susceptible to pneumonias caused by cytomegalovirus (CMV) and other herpesviruses, as well as rubeola and adenovirus. Diagnosis of viral pneumonia depends on appropriate viral cultures and acute and convalescent sera for specific antibodies. Superinfection with bacteria is common in adults. Anti-viral therapy is available for several respiratory viruses. Ribavirin, amantadine/rimantadine, interferon alpha, and acyclovir are antiviral drugs that may be of benefit in treatment and prophylaxis. Prevention of viral pneumonia will depend upon improved viral immunization practices.

  17. Pirfenidone treatment in idiopathic pulmonary fibrosis: nationwide Danish results

    PubMed Central

    Salih, Goran Nadir; Shaker, Saher Burhan; Madsen, Helle Dall; Bendstrup, Elisabeth

    2016-01-01

    Background Pirfenidone was approved by the European Medicines Agency and introduced in most European countries in 2011 for treatment of idiopathic pulmonary fibrosis (IPF). Objective To describe the national Danish experiences of pirfenidone treatment for IPF during 30 months with respect to target population, safety, adherence to the treatment and effect analysis in a well-characterised IPF population in a real-life setting. Methods Retrospective data collection from medical records of all patients in Denmark with IPF from 2011 to 2014. Data included baseline demographics, high-resolution computed tomography (HRCT), histopathology, forced vital capacity (FVC) and 6-min walk test (6MWT). Longitudinal data on FVC, walk test, adherence to the treatment and vital status were also collected. Results Pirfenidone treatment was initiated in 113 patients. Mean age was 69.6±8.1 years (±SD), and 71% were male. Definite IPF diagnosis required thoracoscopic lung biopsy in 45 patients (39.8%). The remaining 68 cases had a definite (64 patients) or possible usual interstitial pneumonia (four patients) pattern on HRCT. Patients were followed for 0.1–33.8 months (median 9.4 months). Fifty-one patients (45.2%) needed dose adjustment, 18 (16%) patients discontinued therapy and 13 patients (11.5%) died. The annual mean decline in FVC was 164 ml (SE 33.2). The decline in 6MWT was 18.2 m (SE 11.2). Nausea (44.2%), fatigue (38.9%) and skin reactions (32.7%) were frequent adverse events. Conclusion Patients with IPF treated with pirfenidone experienced tolerable adverse events. Patients were maintained on treatment due to a careful follow-up and dose adjustment programme. The annual decline in physiological parameters and mortality rate was comparable to previous randomised controlled trials. PMID:27616539

  18. Interstitial lung disease in the connective tissue diseases.

    PubMed

    Antin-Ozerkis, Danielle; Rubinowitz, Ami; Evans, Janine; Homer, Robert J; Matthay, Richard A

    2012-03-01

    The connective tissue diseases (CTDs) are inflammatory, immune-mediated disorders in which interstitial lung disease (ILD) is common and clinically important. Interstitial lung disease may be the first manifestation of a CTD in a previously healthy patient. CTD-associated ILD frequently presents with the gradual onset of cough and dyspnea, although rarely may present with fulminant respiratory failure. Infection and drug reaction should always be ruled out. A diagnosis of idiopathic ILD should never be made without a careful search for subtle evidence of underlying CTD. Treatment of CTD-ILD typically includes corticosteroids and immunosuppressive agents.

  19. Hydrocarbon pneumonia

    MedlinePlus

    Pneumonia - hydrocarbon ... Coughing Fever Shortness of breath Smell of a hydrocarbon product on the breath Stupor (decreased level of ... Most children who drink or inhale hydrocarbon products and develop ... hydrocarbons may lead to rapid respiratory failure and death.

  20. Eosinophilic Pneumonias

    PubMed Central

    Akuthota, Praveen

    2012-01-01

    Summary: This review starts with discussions of several infectious causes of eosinophilic pneumonia, which are almost exclusively parasitic in nature. Pulmonary infections due specifically to Ascaris, hookworms, Strongyloides, Paragonimus, filariasis, and Toxocara are considered in detail. The discussion then moves to noninfectious causes of eosinophilic pulmonary infiltration, including allergic sensitization to Aspergillus, acute and chronic eosinophilic pneumonias, Churg-Strauss syndrome, hypereosinophilic syndromes, and pulmonary eosinophilia due to exposure to specific medications or toxins. PMID:23034324

  1. An immunohistochemical study in a fatal case of acute interstitial pneumonitis (Hamman-Rich syndrome) in a 15-year-old boy presenting as sudden death.

    PubMed

    Turillazzi, Emanuela; Di Donato, Sabina; Neri, Margherita; Riezzo, Irene; Fineschi, Vittorio

    2007-11-15

    Acute interstitial pneumonitis (AIP), also known as Hamman-Rich syndrome, is a distinct type of idiopathic interstitial pneumonia affecting patients of both genders without pre-existing lung diseases. We describe the case of a fulminant form of AIP and discuss the pathophysiological mechanisms of AIP with reference to the histological pattern. A 15-year-previously-healthy male boy presented to the Hospital with a 6-day history of malaise, fever and cough. The clinical prodromes were followed by the acute onset of increasing shortness of breath rapidly progressing in acute respiratory failure. Chest X-ray demonstrated bilateral diffuse airspace opacification; the high resolution CT confirmed the presence of bilateral, symmetric diffuse ground-glass attenuation. The patient was admitted to the intensive care unit, but died after few hours. An autopsy was performed within 24h. The histological examination of lung specimens showed a pattern of diffuse alveolar damage. immunohistochemical, microbiological and toxicological tests were also carried out. The clinical presentation, the histological findings and the exclusion of infective, traumatic, toxic and metabolic causes of acute respiratory distress syndrome (ARDS) allowed us to conclude that the boy was affected by AIP. In conclusion, AIP is a diagnosis of exclusion. It has a mortality rate ranging about 50%, despite mechanical ventilation. In fatal cases of AIP diagnosis can be based on clinical presentation, radiological, histological and microbiological findings and can be further confirmed by immunohistochemical analysis.

  2. Autoantibodies in adult patients with idiopathic inflammatory myopathies in Buenos Aires.

    PubMed

    Gómez, Graciela N; Gargiulo, María De Los Ángeles; Pérez, Nicolás; Collado, María Victoria; Suárez, Lorena V; Khoury, Marina; Sarano, Judith F

    2016-01-01

    The idiopathic inflammatory myopathies(IIM) are a heterogeneous group of diseases of the skeletal muscle. On the basis of clinical, serologic and histological differences, they are classified in dermatomyositis (DM), polymyositis (PM), inclusion body myositis and immunomediated necrotizing myopathy. Autoantibodies directed against nuclear and cytoplasmic antigens are present with variable frequencies among studies. Myositis-specific antibodies (MSAs) are useful in IIM because they contribute to the diagnosis, help to identify different clinical subsets, and have prognostic value. This study aimed to explore the frequency of autoantibodies, especially MSAs, and their relationship with clinical features in adult patients with DM, PM and overlap syndrome. Medical records were reviewed. Myositis-associated antibodies (non-specific) and MSAs (anti Jo-1, PL-7, PL-12, Mi-2 and SRP) were measured using commercial kits. Twelve patients had MSAs, an overall frequency similar to those of international series, but PL-12 and Mi-2 were more frequent than Jo-1, which is the most frequently observed elsewhere. All five patients with Mi-2 had classical DM with a favorable response to treatment. Interstitial pneumonia (n: 4) and/or treatment-refractory disease (n: 3) were found in the presence of anti-PL-12, alone or associated with anti-SRP and/or Jo-1. In conclusion, the coexistence of AEM, a rare finding, was found in three patients. The presence of MSAs aided to the diagnosis of IIM, in particular in those patients without available or conclusive biopsy results.

  3. Autoantibodies in adult patients with idiopathic inflammatory myopathies in Buenos Aires.

    PubMed

    Gómez, Graciela N; Gargiulo, María De Los Ángeles; Pérez, Nicolás; Collado, María Victoria; Suárez, Lorena V; Khoury, Marina; Sarano, Judith F

    2016-01-01

    The idiopathic inflammatory myopathies(IIM) are a heterogeneous group of diseases of the skeletal muscle. On the basis of clinical, serologic and histological differences, they are classified in dermatomyositis (DM), polymyositis (PM), inclusion body myositis and immunomediated necrotizing myopathy. Autoantibodies directed against nuclear and cytoplasmic antigens are present with variable frequencies among studies. Myositis-specific antibodies (MSAs) are useful in IIM because they contribute to the diagnosis, help to identify different clinical subsets, and have prognostic value. This study aimed to explore the frequency of autoantibodies, especially MSAs, and their relationship with clinical features in adult patients with DM, PM and overlap syndrome. Medical records were reviewed. Myositis-associated antibodies (non-specific) and MSAs (anti Jo-1, PL-7, PL-12, Mi-2 and SRP) were measured using commercial kits. Twelve patients had MSAs, an overall frequency similar to those of international series, but PL-12 and Mi-2 were more frequent than Jo-1, which is the most frequently observed elsewhere. All five patients with Mi-2 had classical DM with a favorable response to treatment. Interstitial pneumonia (n: 4) and/or treatment-refractory disease (n: 3) were found in the presence of anti-PL-12, alone or associated with anti-SRP and/or Jo-1. In conclusion, the coexistence of AEM, a rare finding, was found in three patients. The presence of MSAs aided to the diagnosis of IIM, in particular in those patients without available or conclusive biopsy results. PMID:27295700

  4. Idiopathic Pulmonary Fibrosis: Diagnosis and Clinical Manifestations

    PubMed Central

    Nakamura, Yutaro; Suda, Takafumi

    2015-01-01

    Idiopathic pulmonary fibrosis (IPF) is a parenchymal lung disease characterized by progressive interstitial fibrosis. The clinical course of IPF can be unpredictable and may be punctuated by acute exacerbations. Although much progress is being made in unraveling the mechanisms underlying IPF, effective therapy for improving survival remains elusive. Longitudinal disease profiling, especially in terms of clinical manifestations in a large cohort of patients, should lead to proper management of the patients and development of new treatments for IPF. Appropriate multidisciplinary assessment in ongoing registries is required to achieve this. This review summarizes the current status of the diagnosis and clinical manifestations of IPF. PMID:27625576

  5. Idiopathic Pulmonary Fibrosis: Diagnosis and Clinical Manifestations

    PubMed Central

    Nakamura, Yutaro; Suda, Takafumi

    2015-01-01

    Idiopathic pulmonary fibrosis (IPF) is a parenchymal lung disease characterized by progressive interstitial fibrosis. The clinical course of IPF can be unpredictable and may be punctuated by acute exacerbations. Although much progress is being made in unraveling the mechanisms underlying IPF, effective therapy for improving survival remains elusive. Longitudinal disease profiling, especially in terms of clinical manifestations in a large cohort of patients, should lead to proper management of the patients and development of new treatments for IPF. Appropriate multidisciplinary assessment in ongoing registries is required to achieve this. This review summarizes the current status of the diagnosis and clinical manifestations of IPF.

  6. Genome Sequencing of Idiopathic Pulmonary Fibrosis in Conjunction with a Medical School Human Anatomy Course

    PubMed Central

    Kumar, Akash; Arakawa, Christopher; Baudin, Jacob; Bogaard, Andrew; Salesky, Rebecca; Zhou, Qian; Smith, Kelly; Clark, John I.; Shendure, Jay; Horwitz, Marshall S.

    2014-01-01

    Even in cases where there is no obvious family history of disease, genome sequencing may contribute to clinical diagnosis and management. Clinical application of the genome has not yet become routine, however, in part because physicians are still learning how best to utilize such information. As an educational research exercise performed in conjunction with our medical school human anatomy course, we explored the potential utility of determining the whole genome sequence of a patient who had died following a clinical diagnosis of idiopathic pulmonary fibrosis (IPF). Medical students performed dissection and whole genome sequencing of the cadaver. Gross and microscopic findings were more consistent with the fibrosing variant of nonspecific interstitial pneumonia (NSIP), as opposed to IPF per se. Variants in genes causing Mendelian disorders predisposing to IPF were not detected. However, whole genome sequencing identified several common variants associated with IPF, including a single nucleotide polymorphism (SNP), rs35705950, located in the promoter region of the gene encoding mucin glycoprotein MUC5B. The MUC5B promoter polymorphism was recently found to markedly elevate risk for IPF, though a particular association with NSIP has not been previously reported, nor has its contribution to disease risk previously been evaluated in the genome-wide context of all genetic variants. We did not identify additional predicted functional variants in a region of linkage disequilibrium (LD) adjacent to MUC5B, nor did we discover other likely risk-contributing variants elsewhere in the genome. Whole genome sequencing thus corroborates the association of rs35705950 with MUC5B dysregulation and interstitial lung disease. This novel exercise additionally served a unique mission in bridging clinical and basic science education. PMID:25192356

  7. How Is Pneumonia Treated?

    MedlinePlus

    ... page from the NHLBI on Twitter. How Is Pneumonia Treated? Treatment for pneumonia depends on the type ... can go back to their normal routines. Bacterial Pneumonia Bacterial pneumonia is treated with medicines called antibiotics. ...

  8. Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

    MedlinePlus

    ... Asked Questions Español Condiciones Chinese Conditions Idiopathic Intracranial Hypertension (Pseudotumor Cerebri) En Español Read in Chinese What is idiopathic intracranial hypertension? Idiopathic intracranial hypertension (IIH) is a disorder that ...

  9. Interstitial Lung Diseases

    MedlinePlus

    Interstitial lung disease is the name for a large group of diseases that inflame or scar the lungs. The inflammation and scarring make it hard to ... air is responsible for some types of interstitial lung diseases. Specific types include Black lung disease among ...

  10. Morphometry of interstitial fibrosis.

    PubMed

    De Heer, E; Sijpkens, Y W; Verkade, M; den Dulk, M; Langers, A; Schutrups, J; Bruijn, J A; van Es, L A

    2000-01-01

    Several clinical studies have confirmed that histomorphometric changes in the tubulointerstitial compartment contain the best correlating parameters to predict the development of progressive renal insufficiency. The process of interstitial fibrosis is accompanied by an influx of inflammatory cells, up-regulation of fibrogenic cytokines such as transforming growth factor-beta and basic fibroblast growth factor, transient down-modulation of their antagonists, generation and proliferation of myofibroblasts, and, finally, by accumulation of interstitial collagens and proteoglycans. A careful morphometric analysis of interstitial fibrosis requires sensitive parameters through which the severity can be quantified and by which the progression into renal insufficiency can be predicted. We have addressed these issues by morphometric analysis of both human biopsies and by refining existing experimental models in the rat. Morphometric analysis was performed using a Zeiss microscope equipped with a full colour 3CCD camera and KS-400 image analysis software from Zeiss-Kontron. For studies with human material, biopsies were examined from patients with various renal diseases including patients with chronic allotransplant dysfunction. The development of interstitial fibrosis was correlated with clinical parameters. In experimental models, we analysed the interstitial composition and eventual glomerular alterations in rats with bovine serum albumin (BSA)-induced protein overload nephropathy and with human IgG-induced chronic serum sickness nephritis. Finally, we adapted and refined the model of ureter obstruction-induced interstitial fibrosis in the rat. For this purpose, custom-made titanium clips (S&T, Neuhaus, Switzerland) were implanted around the ureter in the abdomen of rats to obstruct the ureter without causing necrosis. The clips were removed at various time points after obstruction of the ureter (1-14 days). The subsequent remodelling of the interstitium was studied

  11. Calf pneumonia.

    PubMed

    Bryson, D G

    1985-07-01

    Infectious calf pneumonia is a high-mortality pneumonia of housed dairy-type calves. Viruses are important etiologic agents and infection with bovine respiratory syncytial virus (RSV) and parainfluenza type 3 virus (PI-3 virus) may result in extensive, and sometimes fatal, lung damage. Respiratory viral infections are frequently followed by mycoplasmal and secondary bacterial invasion of the lower respiratory tract, which increases the extent and severity of lung damage. Bad housing, particularly bad ventilation, will increase the severity of pneumonia outbreaks. Although the source of respiratory viral infections is not always obvious, it is likely that a proportion of calves acquired infection from their dams early in life. The possibility of cross-infections from other domestic animals and from humans must also be considered. Diagnosis of respiratory virus infections necessitates submission of suitable respiratory tract specimens that are taken at an early stage in the outbreak together with paired sera. Various therapeutic and prophylactic regimens for the control of calf pneumonia are described. PMID:3907774

  12. Immunohistochemical and morphometric evaluation of COX-1 and COX-2 in the remodeled lung in idiopathic pulmonary fibrosis and systemic sclerosis* ,**

    PubMed Central

    Parra, Edwin Roger; Lin, Flavia; Martins, Vanessa; Rangel, Maristela Peres; Capelozzi, Vera Luiza

    2013-01-01

    OBJECTIVE: To study the expression of COX-1 and COX-2 in the remodeled lung in systemic sclerosis (SSc) and idiopathic pulmonary fibrosis (IPF) patients, correlating that expression with patient survival. METHODS: We examined open lung biopsy specimens from 24 SSc patients and 30 IPF patients, using normal lung tissue as a control. The histological patterns included fibrotic nonspecific interstitial pneumonia (NSIP) in SSc patients and usual interstitial pneumonia (UIP) in IPF patients. We used immunohistochemistry and histomorphometry to evaluate the expression of COX-1 and COX-2 in alveolar septa, vessels, and bronchioles. We then correlated that expression with pulmonary function test results and evaluated its impact on patient survival. RESULTS: The expression of COX-1 and COX-2 in alveolar septa was significantly higher in IPF-UIP and SSc-NSIP lung tissue than in the control tissue. No difference was found between IPF-UIP and SSc-NSIP tissue regarding COX-1 and COX-2 expression. Multivariate analysis based on the Cox regression model showed that the factors associated with a low risk of death were younger age, high DLCO/alveolar volume, IPF, and high COX-1 expression in alveolar septa, whereas those associated with a high risk of death were advanced age, low DLCO/alveolar volume, SSc (with NSIP), and low COX-1 expression in alveolar septa. CONCLUSIONS: Our findings suggest that strategies aimed at preventing low COX-1 synthesis will have a greater impact on SSc, whereas those aimed at preventing high COX-2 synthesis will have a greater impact on IPF. However, prospective randomized clinical trials are needed in order to confirm that. PMID:24473763

  13. [Nosocomial pneumonia].

    PubMed

    Díaz, Emili; Martín-Loeches, Ignacio; Vallés, Jordi

    2013-12-01

    The hospital acquired pneumonia (HAP) is one of the most common infections acquired among hospitalised patients. Within the HAP, the ventilator-associated pneumonia (VAP) is the most common nosocomial infection complication among patients with acute respiratory failure. The VAP and HAP are associated with increased mortality and increased hospital costs. The rise in HAP due to antibiotic-resistant bacteria also causes an increase in the incidence of inappropriate empirical antibiotic therapy, with an associated increased risk of hospital mortality. It is very important to know the most common organisms responsible for these infections in each hospital and each Intensive Care Unit, as well as their antimicrobial susceptibility patterns, in order to reduce the incidence of inappropriate antibiotic therapy and improve the prognosis of patients. Additionally, clinical strategies aimed at the prevention of HAP and VAP should be employed in hospital settings caring for patients at risk for these infections.

  14. [Nosocomial pneumonia].

    PubMed

    Díaz, Emili; Martín-Loeches, Ignacio; Vallés, Jordi

    2013-12-01

    The hospital acquired pneumonia (HAP) is one of the most common infections acquired among hospitalised patients. Within the HAP, the ventilator-associated pneumonia (VAP) is the most common nosocomial infection complication among patients with acute respiratory failure. The VAP and HAP are associated with increased mortality and increased hospital costs. The rise in HAP due to antibiotic-resistant bacteria also causes an increase in the incidence of inappropriate empirical antibiotic therapy, with an associated increased risk of hospital mortality. It is very important to know the most common organisms responsible for these infections in each hospital and each Intensive Care Unit, as well as their antimicrobial susceptibility patterns, in order to reduce the incidence of inappropriate antibiotic therapy and improve the prognosis of patients. Additionally, clinical strategies aimed at the prevention of HAP and VAP should be employed in hospital settings caring for patients at risk for these infections. PMID:23827827

  15. Cellular Changes in Diabetic and Idiopathic Gastroparesis

    PubMed Central

    2011-01-01

    Background Cellular changes associated with diabetic and idiopathic gastroparesis are not well described. Aim Describe histologic abnormalities in gastroparesis and compare findings in idiopathic versus diabetic gastroparesis. Methods Full thickness gastric body biopsies were obtained from 40 gastroparetics (20 diabetic) and matched controls. Sections were stained for H&E and trichrome, and immunolabeled with antibodies against PGP 9.5, nNOS, VIP, substance P and tyrosine hydroxylase to quantify nerves, S100β for glia, Kit for interstitial cells of Cajal (ICC), CD45 and CD68, for immune cells and smoothelin for smooth muscle cells. Tissue was also examined by transmission electron microscopy (TEM). Results Histological abnormalities were found in 83% of patients. Most common defects were loss of ICC with remaining ICC showing injury, an abnormal immune infiltrate containing macrophages, and decreased nerve fibers. On light microscopy, no significant differences were found between diabetic and idiopathic gastroparesis with the exception of nNOS expression which was decreased in more idiopathic gastroparetics (40%) compared to diabetic (20%) patients by visual grading. On electron microscopy, a markedly increased connective tissue stroma was present in both disorders. Conclusion This study suggests that on full thickness biopsies, cellular abnormalities are found in the majority of patients with gastroparesis. Most common findings were loss of Kit expression suggesting loss of ICC and an increase in CD45 and CD68 immunoreactivity. These findings suggest that examination of tissue can lead to valuable insights into the pathophysiology of these disorders and offers hope that new therapeutic targets can be found. PMID:21300066

  16. How Is Pneumonia Diagnosed?

    MedlinePlus

    ... page from the NHLBI on Twitter. How Is Pneumonia Diagnosed? Pneumonia can be hard to diagnose because it may ... than these other conditions. Your doctor will diagnose pneumonia based on your medical history, a physical exam, ...

  17. What Is Pneumonia?

    MedlinePlus

    ... page from the NHLBI on Twitter. What Is Pneumonia? Pneumonia (nu-MO-ne-ah) is an infection in ... such as bacteria, viruses, and fungi—can cause pneumonia. The infection inflames your lungs' air sacs, which ...

  18. Pneumonia (For Parents)

    MedlinePlus

    ... kids under 6 years old. Take your child's temperature at least once each morning and each evening, ... Respiratory System Croup Fever and Taking Your Child's Temperature Influenza (Flu) Walking Pneumonia Word! Pneumonia Pneumonia Hib ...

  19. IDENTIFICATION AND CHARACTERIZATION OF AN IDIOPATHIC PULMONARY FIBROSIS-LIKE CONDITION IN CATS

    EPA Science Inventory

    Interstitial lung diseases are a heterogeneous group of disorders due to a variety of causes. In veterinary medicine, those with a prominent fibrotic component of unknown etiology are often called idiopathic pulmonary fibrosis (IPF). In human medicine, this term is reserved for ...

  20. Idiopathic Pulmonary Fibrosis

    MedlinePlus

    ... the NHLBI on Twitter. What Is Idiopathic Pulmonary Fibrosis? Pulmonary fibrosis (PULL-mun-ary fi-BRO-sis) is a ... time. The formation of scar tissue is called fibrosis. As the lung tissue thickens, your lungs can' ...

  1. Review of idiopathic pancreatitis

    PubMed Central

    Lee, Jason Kihyuk; Enns, Robert

    2007-01-01

    Recent advances in understanding of pancreatitis and advances in technology have uncovered the veils of idiopathic pancreatitis to a point where a thorough history and judicious use of diagnostic techniques elucidate the cause in over 80% of cases. This review examines the multitude of etiologies of what were once labeled idiopathic pancreatitis and provides the current evidence on each. This review begins with a background review of the current epidemiology of idiopathic pancreatitis prior to discussion of various etiologies. Etiologies of medications, infections, toxins, autoimmune disorders, vascular causes, and anatomic and functional causes are explored in detail. We conclude with management of true idiopathic pancreatitis and a summary of the various etiologic agents. Throughout this review, areas of controversies are highlighted. PMID:18081217

  2. Juvenile idiopathic arthritis

    MedlinePlus

    Juvenile rheumatoid arthritis (JRA); Juvenile chronic polyarthritis; Still disease; Juvenile spondyloarthritis ... The cause of juvenile idiopathic arthritis (JIA) is not known. It is thought to be an autoimmune illness . This means the body attacks ...

  3. Successful alectinib treatment after crizotinib-induced interstitial lung disease.

    PubMed

    Fujiuchi, Satoru; Fujita, Yuka; Sasaki, Takaaki; Ohsaki, Yoshinobu

    2016-05-01

    A 70-year-old woman with lung adenocarcinoma, harbouring anaplastic lymphoma kinase gene rearrangement, was treated with crizotinib as third-line chemotherapy. After 2 months, crizotinib was discontinued because of the development of crizotinib-induced interstitial lung disease (ILD). Steroid treatment was then introduced and tapered off. Following complete resolution of the interstitial shadow, cytotoxic chemotherapy was initiated, and continued for over 2 years, until new intrapulmonary lesions developed. Although there was a risk of drug-induced interstitial pneumonia, alectinib was initiated as the fifth-line therapy, without steroid supplementation, as there was no alternative treatment. No recurrence of ILD was noted at 10 months. To our knowledge, this is the first report of successful alectinib treatment after the development of crizotinib-induced ILD without the use of prednisolone. PMID:27516885

  4. Interstitial pulmonary disorders in indium-processing workers.

    PubMed

    Chonan, T; Taguchi, O; Omae, K

    2007-02-01

    The production of indium-tin oxide has increased, owing to the increased manufacture of liquid-crystal panels. It has been reported that interstitial pneumonia occurred in two indium-processing workers; therefore, the present study aimed to evaluate whether interstitial pulmonary disorders were prevalent among indium workers. The study was carried out in 108 male workers in the indium plant where the two interstitial pneumonia patients mentioned above were employed, and included high-resolution computed tomography (HRCT) of the lungs, pulmonary function tests and analysis of serum sialylated carbohydrate antigen KL-6 and the serum indium concentration. Significant interstitial changes were observed in 23 indium workers on HRCT and serum KL-6 was abnormally high (>500 U x mL(-1)) in 40 workers. Workers with serum indium concentrations in the highest quartile had significantly longer exposure periods, greater HRCT changes, lower diffusing capacity of the lung for carbon monoxide and higher KL-6 levels compared with those in the lowest quartile. The serum indium concentration was positively correlated with the KL-6 level and with the degree of HRCT changes. In conclusion, the results of the present study indicated that serum KL-6 and high-resolution computed tomography abnormalities were prevalent among indium workers and that these abnormalities increased with the indium burden, suggesting that inhaled indium could be a potential cause of occupational lung disease.

  5. Healthcare-associated Pneumonia and Aspiration Pneumonia.

    PubMed

    Komiya, Kosaku; Ishii, Hiroshi; Kadota, Jun-Ichi

    2015-02-01

    Healthcare-associated pneumonia (HCAP) is a new concept of pneumonia proposed by the American Thoracic Society/Infectious Diseases Society of America in 2005. This category is located between community-acquired pneumonia and hospital-acquired pneumonia with respect to the characteristics of the causative pathogens and mortality, and primarily targets elderly patients in healthcare facilities. Aspiration among such patients is recognized to be a primary mechanism for the development of pneumonia, particularly since the HCAP guidelines were published. However, it is difficult to manage patients with aspiration pneumonia because the definition of the condition is unclear, and the treatment is associated with ethical aspects. This review focused on the definition, prevalence and role of aspiration pneumonia as a prognostic factor in published studies of HCAP and attempted to identify problems associated with the concept of aspiration pneumonia.

  6. Karyomegalic Interstitial Nephritis

    PubMed Central

    Isnard, Pierre; Rabant, Marion; Labaye, Jacques; Antignac, Corinne; Knebelmann, Bertrand; Zaidan, Mohamad

    2016-01-01

    Abstract Karyomegalic interstitial nephritis is a rare cause of hereditary chronic interstitial nephritis, described for the first time over 40 years ago. A 36-year-old woman, of Turkish origin, presented with chronic kidney disease and high blood pressure. She had a history of recurrent upper respiratory tract infections but no familial history of nephropathy. Physical examination was unremarkable. Laboratory tests showed serum creatinine at 2.3 mg/dL with an estimated glomerular filtration rate of 26 mL/min/1.73m2, and gamma-glutamyl transpeptidase and alkaline phosphatase at 3 and 1.5 times the upper normal limit. Urinalysis showed 0.8 g/day of nonselective proteinuria, microscopic hematuria, and aseptic leukocyturia. Immunological tests and tests for human immunodeficiency and hepatitis B and C viruses were negative. Complement level and serum proteins electrophoresis were normal. Analysis of the renal biopsy showed severe interstitial fibrosis and tubular atrophy. Numerous tubular cells had nuclear enlargement with irregular outlines, hyperchromatic aspect, and prominent nucleoli. These findings were highly suggestive of karyomegalic interstitial nephritis, which was further confirmed by exome sequencing of FAN1 gene showing an identified homozygous frameshift mutation due to a one-base-pair deletion in exon 12 (c.2616delA). The present case illustrates a rare but severe cause of hereditary interstitial nephritis, sometimes accompanied by subtle extrarenal manifestations. Identification of mutations in FAN1 gene underscores recent insights linking inadequate DNA repair and susceptibility to chronic kidney disease. PMID:27196444

  7. Sleep disordered breathing in interstitial lung disease: A review

    PubMed Central

    Troy, Lauren K; Corte, Tamera J

    2014-01-01

    Patients with interstitial lung disease commonly exhibit abnormal sleep architecture and increased sleep fragmentation on polysomnography. Fatigue is a frequent complaint, and it is likely that poor sleep quality is a significant contributor. A number of studies have shown that sleep disordered breathing is prevalent in this population, particularly in the idiopathic pulmonary fibrosis subgroup. The factors that predispose these patients to obstructive sleep apnoea are not well understood, however it is believed that reduced caudal traction on the upper airway can enhance collapsibility. Ventilatory control system instability may also be an important factor, particularly in those with increased chemo-responsiveness, and in hypoxic conditions. Transient, repetitive nocturnal oxygen desaturation is frequently observed in interstitial lung disease, both with and without associated obstructive apnoeas. There is increasing evidence that sleep-desaturation is associated with increased mortality, and may be important in the pathogenesis of pulmonary hypertension in this population. PMID:25516856

  8. Sleep disordered breathing in interstitial lung disease: A review.

    PubMed

    Troy, Lauren K; Corte, Tamera J

    2014-12-16

    Patients with interstitial lung disease commonly exhibit abnormal sleep architecture and increased sleep fragmentation on polysomnography. Fatigue is a frequent complaint, and it is likely that poor sleep quality is a significant contributor. A number of studies have shown that sleep disordered breathing is prevalent in this population, particularly in the idiopathic pulmonary fibrosis subgroup. The factors that predispose these patients to obstructive sleep apnoea are not well understood, however it is believed that reduced caudal traction on the upper airway can enhance collapsibility. Ventilatory control system instability may also be an important factor, particularly in those with increased chemo-responsiveness, and in hypoxic conditions. Transient, repetitive nocturnal oxygen desaturation is frequently observed in interstitial lung disease, both with and without associated obstructive apnoeas. There is increasing evidence that sleep-desaturation is associated with increased mortality, and may be important in the pathogenesis of pulmonary hypertension in this population. PMID:25516856

  9. A Survey of Radiation-Induced Bronchiolitis Obliterans Organizing Pneumonia Syndrome After Breast-Conserving Therapy in Japan

    SciTech Connect

    Ogo, Etsuyo Komaki, Ritsuko; Fujimoto, Kiminori; Uchida, Masafumi; Abe, Toshi; Nakamura, Katsumasa; Mitsumori, Michihide; Sekiguchi, Kenji; Kaneyasu, Yuko; Hayabuchi, Naofumi

    2008-05-01

    Purpose: We observed a rare and unique occurrence of radiation-induced pulmonary injury outside the tangential field for early breast cancer treatment. The findings appeared to be idiopathic and were called radiation-induced bronchiolitis obliterans organizing pneumonia (BOOP) syndrome. We surveyed major hospitals in Japan to review their findings of radiation-induced BOOP, in particular the clinical and pictorial characteristics of the entity. Methods and Materials: We reviewed surveys completed and returned by 20 institutions. The survey responses were based on a total of 37 cases of BOOP syndrome. We also reviewed X-ray and computed tomography scans provided by these institutions. We discussed the information derived from the questionnaire and analyzed patients' characteristics, methods used in the treatment of BOOP syndrome, and prognosis. Results: The incidence of the radiation-induced BOOP syndrome was about 1.8% (37 of 2,056). We did not find a relationship between the characteristics of patients and the occurrence of radiation-induced BOOP syndrome. The pulmonary findings were classified into four patterns on chest computed tomography scans. Progression of the pulmonary lesions observed on chest X-ray was classified into three patterns. Pneumonitis appeared within 6 months after radiotherapy was completed and disappeared within 6-12 months after its onset. At 5-year follow-up, 2 patients had died, 1 of breast cancer and the other of interstitial pneumonitis, which seemed to be idiopathic and unrelated to the radiation-induced BOOP syndrome. Conclusions: Although the incidence of BOOP syndrome and its associated prognosis are not significant, the patients' clinical condition must be carefully followed.

  10. [Interstitial lung diseases].

    PubMed

    Mazzoccoli, Gianluigi; Carughi, Stefano; De Cata, Angelo; Giuliani, Antonio; Masciale, Nunzia; La Viola, Marco; Puzzolante, Felice; Balzanelli, Mario

    2003-05-01

    Interstitial lung diseases (ILD) are an heterogeneous group of inflammatory diseases characterized by an anatomical distortion of peripheral airways and interstitium, determined by a first stage of alveolitis and a following stage of fibrosis. Natural history of several ILD is characterized by slow and progressive destruction of alveolar-capillary functional units, often with respiratory failure and death. For their smoldering evolution and not specificity of symptoms (exertional dyspnea and cough) ILD may remain not diagnosed and not treated for a long time.

  11. Idiopathic epilepsy in dogs.

    PubMed

    Thomas, W B

    2000-01-01

    Idiopathic epilepsy is a chronic condition characterized by recurrent seizures for which there is no identifiable cause. It is the most common neurologic disorder in the dog. This article discusses the diagnostic evaluation and rational treatment of dogs with recurrent seizures. Types of seizures, client education, choice of therapy, use of specific drugs, therapeutic monitoring, and nondrug treatments are reviewed.

  12. Idiopathic Inflammatory Myopathies

    PubMed Central

    Dimachkie, Mazen M.; Barohn, Richard J.

    2012-01-01

    The idiopathic inflammatory myopathies are a group of rare disorders including polymyositis (PM), dermatomyositis (DM), and autoimmune necrotizing myopathies (NMs). The idiopathic inflammatory myopathies share many similarities. They present acutely, subacutely, or chronically with marked proximal and symmetric muscle weakness, except for associated distal and asymmetric weakness in inclusion body myositis. The idiopathic inflammatory myopathies also share a variable degree of creatine kinase (CK) elevation and a nonspecifically abnormal electromyogram demonstrating an irritative myopathy. The muscle pathology demonstrates inflammatory exudates of variable distribution within the muscle fascicle. Despite these similarities, the idiopathic inflammatory myopathies are a heterogeneous group. The overlap syndrome (OS) refers to the association of PM, DM, or NM with connective tissue disease, such as scleroderma or systemic lupus erythematosus. In addition to elevated antinuclear antibodies (ANA), patients with OS may be weaker in the proximal arms than the legs mimicking the pattern seen in some muscular dystrophies. In this review, we focus on DM, PM, and NM and examine current and promising therapies. PMID:23117947

  13. Idiopathic inflammatory myopathies.

    PubMed

    Dimachkie, Mazen M; Barohn, Richard J

    2012-07-01

    The idiopathic inflammatory myopathies are a group of rare disorders including polymyositis (PM), dermatomyositis (DM), and autoimmune necrotizing myopathies (NMs). The idiopathic inflammatory myopathies share many similarities. They present acutely, subacutely, or chronically with marked proximal and symmetric muscle weakness, except for associated distal and asymmetric weakness in inclusion body myositis. The idiopathic inflammatory myopathies also share a variable degree of creatine kinase (CK) elevation and a nonspecifically abnormal electromyogram demonstrating an irritative myopathy. The muscle pathology demonstrates inflammatory exudates of variable distribution within the muscle fascicle. Despite these similarities, the idiopathic inflammatory myopathies are a heterogeneous group. The overlap syndrome (OS) refers to the association of PM, DM, or NM with connective tissue disease, such as scleroderma or systemic lupus erythematosus. In addition to elevated antinuclear antibodies (ANA), patients with OS may be weaker in the proximal arms than the legs mimicking the pattern seen in some muscular dystrophies. In this review, we focus on DM, PM, and NM and examine current and promising therapies.

  14. [Idiopathic renal arteriovenous fistula].

    PubMed

    Bennani, S; Ait Bolbarod, A; el Mrini, M; Kadiri, R; Benjelloun, S

    1996-06-01

    The authors report a case of idiopathic renal arteriovenous fistula. The diagnosis was established angiographically in a 24 year old man presenting gross hematuria. Embolization of the fistula was performed. Efficiency of this treatment was appreciated clinically and by duplex renal ultrasonography. The characteristics of renal arteriovenous fistulas are reviewed. PMID:8763700

  15. Diagnosis and classification of idiopathic inflammatory myopathies.

    PubMed

    Lundberg, I E; Miller, F W; Tjärnlund, A; Bottai, M

    2016-07-01

    The idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of diseases, collectively termed myositis, sharing symptoms of muscle weakness, fatigue and inflammation. Other organs are frequently involved, supporting the notion that these are systemic inflammatory diseases. The IIMs can be subgrouped into dermatomyositis, polymyositis and inclusion body myositis. The myositis-specific autoantibodies (MSAs) identify other and often more distinct clinical phenotypes, such as the antisynthetase syndrome with antisynthetase autoantibodies and frequent interstitial lung disease and anti-SRP and anti-HMGCR autoantibodies that identify necrotizing myopathy. The MSAs are important both to support myositis diagnosis and to identify subgroups with different patterns of extramuscular organ involvement such as interstitial lung disease. Another cornerstone in the diagnostic procedure is muscle biopsy to identify inflammation and to exclude noninflammatory myopathies. Treatment effect and prognosis vary by subgroup. To develop new and better therapies, validated classification criteria that identify distinct subgroups of myositis are critical. The lack of such criteria was the main rationale for the development of new classification criteria for IIMs, which are summarized in this review; the historical background regarding previous diagnostic and classification criteria is also reviewed. As the IIMs are rare diseases with a prevalence of 10 in 100 000 individuals, an international collaboration was essential, as was the interdisciplinary effort including experts in adult and paediatric rheumatology, neurology, dermatology and epidemiology. The new criteria have been developed based on data from more than 1500 patients from 47 centres worldwide and are based on clinically easily available variables. PMID:27320359

  16. The Multifaceted Aspects of Interstitial Lung Disease in Rheumatoid Arthritis

    PubMed Central

    Grosso, Vittorio; Scorletti, Eva; Crepaldi, Gloria; Caporali, Roberto

    2013-01-01

    Interstitial lung disease (ILD) is a relevant extra-articular manifestation of rheumatoid arthritis (RA) that may occur either in early stages or as a complication of long-standing disease. RA related ILD (RA-ILD) significantly influences the quoad vitam prognosis of these patients. Several histopathological patterns of RA-ILD have been described: usual interstitial pneumonia (UIP) is the most frequent one, followed by nonspecific interstitial pneumonia (NSIP); other patterns are less commonly observed. Several factors have been associated with an increased risk of developing RA-ILD. The genetic background plays a fundamental but not sufficient role; smoking is an independent predictor of ILD, and a correlation with the presence of rheumatoid factor and anti-cyclic citrullinated peptide antibodies has also been reported. Moreover, both exnovo occurrence and progression of ILD have been related to drug therapies that are commonly prescribed in RA, such as methotrexate, leflunomide, anti-TNF alpha agents, and rituximab. A greater understanding of the disease process is necessary in order to improve the therapeutic approach to ILD and RA itself and to reduce the burden of this severe extra-articular manifestation. PMID:24205507

  17. The multifaceted aspects of interstitial lung disease in rheumatoid arthritis.

    PubMed

    Cavagna, Lorenzo; Monti, Sara; Grosso, Vittorio; Boffini, Nicola; Scorletti, Eva; Crepaldi, Gloria; Caporali, Roberto

    2013-01-01

    Interstitial lung disease (ILD) is a relevant extra-articular manifestation of rheumatoid arthritis (RA) that may occur either in early stages or as a complication of long-standing disease. RA related ILD (RA-ILD) significantly influences the quoad vitam prognosis of these patients. Several histopathological patterns of RA-ILD have been described: usual interstitial pneumonia (UIP) is the most frequent one, followed by nonspecific interstitial pneumonia (NSIP); other patterns are less commonly observed. Several factors have been associated with an increased risk of developing RA-ILD. The genetic background plays a fundamental but not sufficient role; smoking is an independent predictor of ILD, and a correlation with the presence of rheumatoid factor and anti-cyclic citrullinated peptide antibodies has also been reported. Moreover, both exnovo occurrence and progression of ILD have been related to drug therapies that are commonly prescribed in RA, such as methotrexate, leflunomide, anti-TNF alpha agents, and rituximab. A greater understanding of the disease process is necessary in order to improve the therapeutic approach to ILD and RA itself and to reduce the burden of this severe extra-articular manifestation.

  18. Organizing pneumonia: chest HRCT findings*

    PubMed Central

    Faria, Igor Murad; Zanetti, Gláucia; Barreto, Miriam Menna; Rodrigues, Rosana Souza; Araujo-Neto, Cesar Augusto; Silva, Jorge Luiz Pereira e; Escuissato, Dante Luiz; Souza, Arthur Soares; Irion, Klaus Loureiro; Mançano, Alexandre Dias; Nobre, Luiz Felipe; Hochhegger, Bruno; Marchiori, Edson

    2015-01-01

    OBJECTIVE: To determine the frequency of HRCT findings and their distribution in the lung parenchyma of patients with organizing pneumonia. METHODS: This was a retrospective review of the HRCT scans of 36 adult patients (26 females and 10 males) with biopsy-proven organizing pneumonia. The patients were between 19 and 82 years of age (mean age, 56.2 years). The HRCT images were evaluated by two independent observers, discordant interpretations being resolved by consensus. RESULTS: The most common HRCT finding was that of ground-glass opacities, which were seen in 88.9% of the cases. The second most common finding was consolidation (in 83.3% of cases), followed by peribronchovascular opacities (in 52.8%), reticulation (in 38.9%), bronchiectasis (in 33.3%), interstitial nodules (in 27.8%), interlobular septal thickening (in 27.8%), perilobular pattern (in 22.2%), the reversed halo sign (in 16.7%), airspace nodules (in 11.1%), and the halo sign (in 8.3%). The lesions were predominantly bilateral, the middle and lower lung fields being the areas most commonly affected. CONCLUSIONS: Ground-glass opacities and consolidation were the most common findings, with a predominantly random distribution, although they were more common in the middle and lower thirds of the lungs. PMID:26176521

  19. Expression of WNT5A in Idiopathic Pulmonary Fibrosis and Its Control by TGF-β and WNT7B in Human Lung Fibroblasts.

    PubMed

    Newman, Donna R; Sills, W Shane; Hanrahan, Katherine; Ziegler, Amanda; Tidd, Kathleen McGinnis; Cook, Elizabeth; Sannes, Philip L

    2016-02-01

    The wingless (Wnt) family of signaling ligands contributes significantly to lung development and is highly expressed in patients with usual interstitial pneumonia (UIP). We sought to define the cellular distribution of Wnt5A in the lung tissue of patients with idiopathic pulmonary fibrosis (IPF) and the signaling ligands that control its expression in human lung fibroblasts and IPF myofibroblasts. Tissue sections from 40 patients diagnosed with IPF or UIP were probed for the immunolocalization of Wnt5A. Further, isolated lung fibroblasts from normal or IPF human lungs, adenovirally transduced for the overexpression or silencing of Wnt7B or treated with TGF-β1 or its inhibitor, were analyzed for Wnt5A protein expression. Wnt5A was expressed in IPF lungs by airway and alveolar epithelium, smooth muscle cells, endothelium, and myofibroblasts of fibroblastic foci and throughout the interstitium. Forced overexpression of Wnt7B with or without TGF-β1 treatment significantly increased Wnt5A protein expression in normal human smooth muscle cells and fibroblasts but not in IPF myofibroblasts where Wnt5A was already highly expressed. The results demonstrate a wide distribution of Wnt5A expression in cells of the IPF lung and reveal that it is significantly increased by Wnt7B and TGF-β1, which, in combination, could represent key signaling pathways that modulate the pathogenesis of IPF.

  20. FG-3019 anti-connective tissue growth factor monoclonal antibody: results of an open-label clinical trial in idiopathic pulmonary fibrosis.

    PubMed

    Raghu, Ganesh; Scholand, Mary Beth; de Andrade, João; Lancaster, Lisa; Mageto, Yolanda; Goldin, Jonathan; Brown, Kevin K; Flaherty, Kevin R; Wencel, Mark; Wanger, Jack; Neff, Thomas; Valone, Frank; Stauffer, John; Porter, Seth

    2016-05-01

    FG-3019 is a fully human monoclonal antibody that interferes with the action of connective tissue growth factor, a central mediator in the pathogenesis of fibrosis.This open-label phase 2 trial evaluated the safety and efficacy of two doses of FG-3019 administered by intravenous infusion every 3 weeks for 45 weeks in patients with idiopathic pulmonary fibrosis (IPF). Subjects had a diagnosis of IPF within the prior 5 years defined by either usual interstitial pneumonia (UIP) pattern on a recent high-resolution computed tomography (HRCT) scan, or a possible UIP pattern on HRCT scan and a recent surgical lung biopsy showing UIP pattern. Pulmonary function tests were performed every 12 weeks, and changes in the extent of pulmonary fibrosis were measured by quantitative HRCT scans performed at baseline and every 24 weeks.FG-3019 was safe and well-tolerated in IPF patients participating in the study. Changes in fibrosis were correlated with changes in pulmonary function.Further investigation of FG-3019 in IPF with a placebo-controlled clinical trial is warranted and is underway.

  1. Pneumonia - weakened immune system

    MedlinePlus

    If you have a weakened immune system, you may receive daily antibiotics to prevent some types of pneumonia. Ask your provider if you should receive the influenza (flu) and pneumococcal (pneumonia) vaccines. Practice ...

  2. Genetics Home Reference: adolescent idiopathic scoliosis

    MedlinePlus

    ... Understand Genetics Home Health Conditions adolescent idiopathic scoliosis adolescent idiopathic scoliosis Enable Javascript to view the expand/ ... boxes. Download PDF Open All Close All Description Adolescent idiopathic scoliosis is an abnormal curvature of the ...

  3. Interstitial duplication 19p

    SciTech Connect

    Stratton, R.F.; DuPont, B.R.; Moore, C.M.

    1995-07-17

    We report on a 9-month-old girl with an interstitial duplication of 19p, developmental delay, and multiple anomalies including bifrontal prominence, obtuse frontonasal angle, short columella, additional midline philtral pillar, midline ridge on the tongue, vertical midline ridge at the mental symphysis, and a complex congenital heart defect including severe branch pulmonary artery stenosis, secundum atrial septal defect (ASD), and several ventricular septal defects (VSDs). Use of fluorescent in situ hybridization (FISH) with chromosome 19- specific probes showed a direct duplication of bands 19p13.13 and 19p13.2. 6 refs., 1 fig.

  4. Pneumocystis Pneumonia (For Parents)

    MedlinePlus

    ... 5 Things to Know About Zika & Pregnancy Pneumocystis Pneumonia KidsHealth > For Parents > Pneumocystis Pneumonia Print A A A Text Size What's in ... article? About PCP Diagnosing PCP Treating PCP Pneumocystis pneumonia (PCP) is an infection caused by Pneumocystis jiroveci , ...

  5. [Organising Pneumonia - a review and results from Icelandic studies].

    PubMed

    Sveinsson, Olafur A; Isaksson, Helgi J; Gudmundsson, Gunnar

    2008-01-01

    Organising pneumonia (OP) is a relatively rare interstitial lung disease. It s definition is based on a characteristic histological pattern in the presence of certain clinical and radiological features. Organising pneumonia represents also what has been called Bronchiolitis Obliterans Organising Pneumonia (BOOP). Recently it has been recommended to call OP cryptogenic organising pneumonia (COP) when no definite cause or characteristic clinical context is found and secondary organising pneumonia (SOP) when causes can be identified such as infection or it occurs in a characteristic clinical context such as connective tissue disorder. The most common clinical symptoms are dyspnea, cough, fever and general malaise. It is common that symptoms have been present for some weeks before the diagnosis is made. Patients commonly have lowered PO2 and a mildly restrictive spirometry. Radiographic features are most often patchy bilateral airspace opacities but an interstitial pattern or focal opacities can also be seen. Most of patients respond well to steroids but relapses are quite common. The aim of this paper is to present an overview of the disease and the main results from studies on OP in Iceland. The mean annual incidence for OP in Iceland was 1.97/100,000 inhabitants. Annual incidence for COP was 1.10/100,000 and 0.87/100,000 for SOP. This is higher than in most other studies. In Iceland patients with OP had a higher standardized mortality ratio than the general population despite good clinical responses. No clinical symptoms could separate between SOP and COP.

  6. Idiopathic toe walking.

    PubMed

    Oetgen, Matthew E; Peden, Sean

    2012-05-01

    Toe walking is a bilateral gait abnormality in which a normal heel strike is absent and most weight bearing occurs through the forefoot. This abnormality may not be pathologic in patients aged <2 years, but it is a common reason for referral to an orthopaedic surgeon. Toe walking can be caused by several neurologic and developmental abnormalities and may be the first sign of a global developmental problem. Cases that lack a definitive etiology are categorized as idiopathic. A detailed history, with careful documentation of the developmental history, and a thorough physical examination are required in the child with a primary report of toe walking. Treatment is based on age and the severity of the abnormality. Management includes observation, stretching, casting, bracing, chemodenervation, and surgical lengthening of the gastrocnemius-soleus complex and/or Achilles tendon. An understanding of idiopathic toe walking as well as treatment options and their outcomes can help the physician individualize treatment to achieve optimal results.

  7. [Idiopathic hypercalciuria. Differential diagnosis].

    PubMed

    Cano, F; Rodríguez, E; Delucchi, M A; Wolff, E

    1990-01-01

    In 50 children with hematuria or urolithiasis and idiopathic hypercalciuria, and in 15 control children, urinary calcium/creatinine concentration rates were measured after fasting and after calcium loading. Patients were classified into two groups depending on the results of an orally administered calcium loading test. Children were considered to have absorptive hypercalciuria (42%) when they had low fasting urinary calcium/creatinine concentration ratio (less than 0.21), and a large increase of this index after calcium administration (greater than 0.28). Patients were labeled as renal hypercalciuria (32%) if they had high fasting urinary calcium/creatinine concentration ratio (greater than 0.21), and variable increases of it after calcium overload. A third group of children (26%), were not classifiable by means of this test. Our data support the contention that this simple ambulatory test is very useful in the diagnostic workup of idiopathic hypercalciuria. PMID:2087593

  8. Exogenous lipoid pneumonia (ELP): when radiologist makes the difference

    PubMed Central

    Rea, G; Perna, F; Calabrese, G; Molino, A; Valente, T; Vatrella, A

    2016-01-01

    Lipoid pneumonia is an uncommon disorder characterized by accumulation of lipid components into the interstitial and alveolar compartment. The usual classification distinguishes endogenous and exogenous and acute or chronic forms, related to the type of fats, the amount of damage and the time of exposure. We describe a case of exogenous lipoid pneumonia by inhalation of vaseline used for cleaning of the tracheostoma in a 63-year-old female, presenting as cough, worsening dyspnea in few weeks. The diagnosis was finally established with a re-evaluation of BAL with specific staining for lipids, revealing the presence of foamy macrophages lipids rich, according to HRCT findings. PMID:27326397

  9. [Idiopathic granulomatous mastitis].

    PubMed

    Hello, M; Néel, A; Graveleau, J; Masseau, A; Agard, C; Caillon, J; Hamidou, M

    2013-06-01

    Idiopathic granulomatous mastitis (IGM) is a rare localized granulomatosis of unknown aetiology that usually affects women of childbearing age. It often mimics breast carcinoma or abscess. Histopathologic evaluation and elimination of the others aetiologies of granuloma play a crucial role in the diagnosis. Its etiopathogeny remains poorly understood, but Corynebacteria might be involved. The disease course is usually protracted, with a significant impact on quality of life. The management of IGM remains controversial, but corticosteroids are usually the first-line treatment.

  10. Juvenile idiopathic arthritis.

    PubMed

    Gowdie, Peter J; Tse, Shirley M L

    2012-04-01

    Juvenile idiopathic arthritis (JIA) encompasses a complex group of disorders with arthritis as a common feature. This article provides the pediatrician with a review of the epidemiology, classification, clinical manifestations, and complications of JIA. It also provides an update on the current understanding of the cause of JIA and recent developments in management and a recent review of the long-term outcome in JIA.

  11. Subclinical Interstitial Lung Disease

    PubMed Central

    Doyle, Tracy J.; Hunninghake, Gary M.

    2012-01-01

    The widespread use of high-resolution computed tomography in clinical and research settings has increased the detection of interstitial lung abnormalities (ILA) in asymptomatic and undiagnosed individuals. We reported that in smokers, ILA were present in about 1 of every 12 high-resolution computed tomographic scans; however, the long-term significance of these subclinical changes remains unclear. Studies in families affected with pulmonary fibrosis, smokers with chronic obstructive pulmonary disease, and patients with inflammatory lung disease have shown that asymptomatic and undiagnosed individuals with ILA have reductions in lung volume, functional limitations, increased pulmonary symptoms, histopathologic changes, and molecular profiles similar to those observed in patients with clinically significant interstitial lung disease (ILD). These findings suggest that, in select at-risk populations, ILA may represent early stages of pulmonary fibrosis or subclinical ILD. The growing interest surrounding this topic is motivated by our poor understanding of the inciting events and natural history of ILD, coupled with a lack of effective therapies. In this perspective, we outline past and current research focused on validating radiologic, physiological, and molecular methods to detect subclinical ILD. We discuss the limitations of the available cross-sectional studies and the need for future longitudinal studies to determine the prognostic and therapeutic implications of subclinical ILD in populations at risk of developing clinically significant ILD. PMID:22366047

  12. Perceptions, experiences and needs of patients with idiopathic pulmonary fibrosis

    PubMed Central

    Duck, Annette; Spencer, Lisa G; Bailey, Simon; Leonard, Colm; Ormes, Jennifer; Caress, Ann-Louise

    2015-01-01

    Aims To understand the perceptions, needs and experiences of patients with Idiopathic Pulmonary Fibrosis. Background Idiopathic pulmonary fibrosis is a progressive interstitial lung disease, with a mean life expectancy similar to some forms of cancer of 2–4 years from diagnosis. Unlike the cancer literature, which is rich with studies exploring the needs of their disease group, few publications exist on patient needs with this severe fibrotic lung disease. Design A Qualitative study which took place between 2007–2012. Methods Seventeen patients with a multidisciplinary team confirmed diagnosis of Idiopathic Pulmonary Fibrosis, with moderate to advanced disease severity and six of their informal carers were interviewed. An interview topic guide was developed by the researchers and service user group. The interviews were audio-recorded, semi-structured and took place at a regional respiratory and lung transplant centre in North West England. Interviews were transcribed verbatim and data analysed using Framework Analysis. Findings Three main themes were identified: ‘Struggling to get a diagnosis’; ‘Loss of the life I previously had’; and ‘Living with Idiopathic Pulmonary Fibrosis’. Patients reported struggling to get a diagnosis and coping with a life-limiting, rapidly progressive illness with no good treatment and few support structures. Conclusions There is an urgent need for a better understanding of the difficulties faced by people with Idiopathic Pulmonary Fibrosis and their carers. This can be used to develop better supportive care in the United Kingdom and ultimately improve the quality of life of these patients. PMID:25533573

  13. A novel model of rheumatoid arthritis-associated interstitial lung disease in SKG mice.

    PubMed

    Keith, Rebecca C; Powers, Jennifer L; Redente, Elizabeth F; Sergew, Amen; Martin, Richard J; Gizinski, Alison; Holers, V Michael; Sakaguchi, Shimon; Riches, David W H

    2012-03-01

    Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) is associated with increased mortality in up to 10% of patients with rheumatoid arthritis. Lung exposure to cigarette smoke has been implicated in disease development. Little is known about the mechanisms underlying the development of RA-ILD, in part due to the lack of an appropriate mouse model. The objectives of this study were (i) to test the suitability of SKG mice as a model of cellular and fibrotic interstitial pneumonia in the setting of autoimmune arthritis, and (ii) to determine the role of lung injury in the development of arthritis in SKG mice. Lung tissues were evaluated in arthritic SKG mice by quantifying cell accumulation in bronchoalveolar lavage, static compliance, collagen levels, and infiltrating cell phenotypes by flow cytometry and histology. Lung injury was induced by exposure to cigarette smoke or bleomycin. Arthritic SKG mice developed a patchy cellular and fibrotic interstitial pneumonia associated with reduced static compliance, increased collagen levels, and accumulation of inflammatory cells. Infiltrating cells comprised CD4+ T cells, B cells, macrophages, and neutrophils. Chronic exposure to cigarette smoke or initiation of lung injury with bleomycin did not cause arthritis. The pattern of lung disease suggests that arthritic SKG mice represent an authentic model of nonspecific interstitial pneumonia in RA-ILD patients. The lack of arthritis development after cigarette smoke or lung injury suggests that a model where breaches in immunologic tolerance are induced by lung inflammation and injury alone may be overly simplistic.

  14. Pulmonary hypertension in chronic interstitial lung diseases.

    PubMed

    Caminati, Antonella; Cassandro, Roberto; Harari, Sergio

    2013-09-01

    Pulmonary hypertension (PH) is a common complication of interstitial lung diseases (ILDs), particularly in idiopathic pulmonary fibrosis and ILD associated with connective tissue disease. However, other lung diseases, such as combined pulmonary fibrosis and emphysema syndrome, pulmonary Langerhans cell histiocytosis, and lymphangioleiomyomatosis, may also include PH in their clinical manifestations. In all of these diseases, PH is associated with reduced exercise capacity and poor prognosis. The degree of PH in ILDs is typically mild-to-moderate. However, some of these patients may develop a disproportionate increase in PH that cannot be justified solely by hypoxia and parenchymal injury: this condition has been termed "out-of-proportion" PH. The pathogenesis of PH in these diseases is various, incompletely understood and may be multifactorial. The clinical suspicion (i.e. increased dyspnoea, low diffusion capacity) and echocardiographic assessment are the first steps towards proper diagnosis of PH; however, right heart catheterisation remains the current gold standard for diagnosis of PH. At present, no specific therapies have been approved for the treatment of PH in patients with ILDs. PMID:23997057

  15. Renal Medullary Interstitial Cells

    NASA Astrophysics Data System (ADS)

    Rao, Reena; Hao, Chuan-Ming; Breyer, Matthew D.

    2007-04-01

    Renal medullary interstitial cells (RMICs) are specialized fibroblast-like cells that reside in the renal medulla among the vasa recta, the thin limbs of Henle's loop, and medullary collecting ducts. These cells are characterized by abundant lipid droplets in the cytoplasm. The lipid droplets are composed of triglycerides, cholesterol esters and free long-chain fatty acids, including arachidonic acid. RMICs are also a major site of cyclooxygenase2 (COX-2) expression, and thus a major site of COX-2 derived prostanoid biosynthesis. RMICs are also a potential target of hormones such as angiotensin II and endothelin. The RMIC COX-2 expression and the abundance of lipid droplets change with salt and water intake. These properties of RMICs are consistent with an important role of these cells in modulating physiologic and pathologic processes of the kidney.

  16. Nonspecific interstitial pneumonitis: a common cause of pulmonary disease in the acquired immunodeficiency syndrome

    SciTech Connect

    Suffredini, A.F.; Ognibene, F.P.; Lack, E.E.; Simmons, J.T.; Brenner, M.; Gill, V.J.; Lane, H.C.; Fauci, A.S.; Parrillo, J.E.; Masur, H.

    1987-07-01

    During a 4.4-year period, nonspecific interstitial pneumonitis was seen in 41 of 110 (38%) patients with the acquired immunodeficiency syndrome and accounted for 32% (48/152) of all episodes of clinical pneumonitis. Diffuse alveolar damage was typically a feature of nonspecific interstitial pneumonitis, but neither lung biopsy nor bronchoalveolar lavage detected a pathogen. Of these 41 patients, 13 had no associated pulmonary tumor and had not been exposed to pulmonary toxins, whereas 28 patients had either concurrent pulmonary Kaposi sarcoma, previous experimental therapies, or a history of pneumocystis pneumonia or drug abuse. Of these 41, 23 had normal chest radiographs. The clinical features of patients with nonspecific interstitial pneumonitis were similar to those of patients with pneumocystis pneumonia, although histologic findings showed less severe alveolar damage in patients with nonspecific interstitial pneumonitis (p less than 0.001). Pathologic evaluation and clinical follow-up suggest that many clinical episodes of pneumonitis in patients with the acquired immunodeficiency syndrome are due to nonspecific interstitial pneumonitis of unknown cause.

  17. Investigation into the cause of mortality in 49 cases of idiopathic inflammatory myopathy: A single center study

    PubMed Central

    XIAO, YIZHI; ZUO, XIAOXIA; YOU, YUNHUI; LUO, HUI; DUAN, LIPING; ZHANG, WEIRU; LI, YISHA; XIE, YANLI; ZHOU, YAOU; NING, WANGBIN; LI, TONG; LIU, SIJIA; ZHU, HONGLIN; JIANG, YING; WU, SIYAO; ZHAO, HONGJUN

    2016-01-01

    Idiopathic inflammatory myopathy (IIM) is an autoimmune disease characterized by chronic muscle weakness and myositis with unknown etiology. IIM may affect the function of multiple organs and has a poor prognosis. In the present study, the causes of mortality in patients with IIM admitted to the Xiangya Hospital during the last 14 years were investigated. The investigation included an analysis of frequent causes of IIM, and of infections and associated complications. A cohort study was conducted on 676 patients with IIM that were admitted to Xiangya Hospital from January, 2001 to January, 2015. There were 49 patient mortalities (7.2% of the total cases), of which 34 mortalities were infection-associated and 15 were not infection-associated. The proportion of infection-associated IIM mortalities had increased since 2001. Of the 34 infection-associated mortalities, 31 cases (63.3%) were of fungal and bacterial infections, most frequently infecting the lungs and the blood. Klebsiella pneumoniae and Acinetobacter baumannii were the most commonly isolated pathogens, and co-infection with the two pathogens was observed in the majority of cases. In the IIM mortalities not associated with infection, there were 2 acute myocardial infarction cases, 2 acute interstitial lung disease cases, 4 malignancies and 1 case of each of the following: Arrhythmia, pneumothorax, ventilator weakness, pulmonary artery hypertension, gastrointestinal bleeding, liver failure and renal failure. Three mortalities were secondary to viral hepatitis in the present study. Pathogenic infection was the most frequent cause of mortality in patients with IIM. The remaining causes of mortality included secondary to heart failure, lung dysfunction and malignancy. Following the ubiquitous application of glucocorticoids and immunosuppressants, the proportion of infection-associated mortalities increased in patients with IIM. Thus, in addition to focusing on the primary disease, infection should receive

  18. Could prominent airway-centered fibroblast foci in lung biopsies predict underlying chronic microaspiration in idiopathic pulmonary fibrosis patients?

    PubMed

    Bois, Melanie C; Hu, Xiaowen; Ryu, Jay H; Yi, Eunhee S

    2016-07-01

    Chronic occult aspiration of small droplets (microaspiration) due to gastroesophageal reflux disease (GERD) and/or hiatal hernia is postulated to be a contributing factor in the pathogenesis of idiopathic pulmonary fibrosis (IPF). Usual interstitial pneumonia (UIP) is the histopathologic correlate of IPF. We hypothesized that chronic microaspiration may manifest as prominent airway-centered fibroblastic foci (FFs) in IPF. UIP cases diagnosed by wedge biopsies over a 6-year period (2006-2011) were identified and scored (1-3) for the prominence of airway-centered FFs by 2 authors blinded for clinical history. Relevant clinical information was obtained. Thirty-seven patients (22 men) were diagnosed with IPF by multidisciplinary approach. Thirteen cases (35.1%) demonstrated high airway-centered FF score (score 3). Twenty (54.1%) patients carried a clinical diagnosis of GERD, and 3 patients (8.1%) had hiatal hernia. High airway-centered FF score was significantly associated with hiatal hernia diagnosis (P=.037) but not with a diagnosis of GERD or the use of proton pump inhibitors/histamine-2 receptor antagonists. High airway-centered FF score was associated with airway-centered acute inflammation (P=.028) and peribronchiolar granulomas (P=.042). In summary, IPF cases with hiatal hernia were more likely to have a prominent airway-centered FF. Given the strong association between hiatal hernia and GERD and their risk for developing chronic microaspiration, the prominent airway-centered FF in UIP might predict the presence of chronic microaspiration, acknowledging that GERD and proton pump inhibitor/histamine-2 receptor antagonist use failed to demonstrate a significant association. Larger studies are warranted for further investigation. PMID:26980038

  19. Idiopathic gingival fibromatosis

    PubMed Central

    Dani, Nitin Hemchandra; Khanna, Dinkar Parveen; Bhatt, Vaibhavi Hitesh; Joshi, Chaitanya Pradeep

    2015-01-01

    Idiopathic gingival fibromatosis (IGF) is a rare hereditary condition characterized by slowly progressive, nonhemorrhagic, fibrous enlargement of maxillary and mandibular keratinized gingiva caused by increase in submucosal connective tissue elements, mostly associated with some syndrome. This case report describes a case of nonsyndromic generalized IGF in an 18-year-old male patient who presented with generalized gingival enlargement. The enlarged tissue was surgically removed by internal bevel gingivectomy and ledge and wedge procedure. The patient was regularly monitored clinically for improvement in his periodontal condition as well as for any recurrence of gingival overgrowth. PMID:26941525

  20. Idiopathic Gingival Fibromatosis

    PubMed Central

    Nayak, Ullal Anand; Khandelwal, Vishal; Ninave, Nupur

    2011-01-01

    ABSTRACT Idiopathic gingival fibromatosis is a rare heriditary condition characterized by slowly progressive, nonhemorrhagic, fibrous enlargement of maxillary and mandibular keratinized gingiva caused by increase in submucosal connective tissue elements. This case report gives an overview of gingival fibromatosis in a 11-year-old female patient who presented with generalized gingival enlargement. Based on the history and clinical examination, the diagnosis was made and the enlarged tissue was surgically removed. The patient was being regularly monitored clinically for improvement in her periodontal condition as well as for any recurrence of gingival overgrowth. PMID:27616864

  1. [Idiopathic progressive subglottic stenosis].

    PubMed

    Sittel, C

    2014-07-01

    Idiopathic subglottic stenosis is causing a narrowing of the central airway at the laryngotracheal junction. Etiology is remaining unclear at large. There is a marked preponderance for women in the fertile age, an association to estrogene or progesterone metabolism remains doubtful. Suggested treatment varies from repeated endoscopic interventions to primary open resection. Therapy selection in this heterogeneous condition should be based on the individual patient situation as well as surgeon's expertise. This complex entity is prone to complications and should preferably be managed in a referral center.

  2. The History of Mycoplasma pneumoniae Pneumonia.

    PubMed

    Saraya, Takeshi

    2016-01-01

    In the United States in the 1930s, although the pathogen was not known, atypical pneumonia was clinically distinguished from pneumococcal pneumonia by its resistance to sulfonamides. Reimann (1938) reported seven patients with an unusual form of tracheo bronchopneumonia and severe constitutional symptoms. He believed the clinical picture of this disease differed from that of the disease caused by influenza viruses or known bacteria and instead suspected "primary atypical pneumonia." For many years, the responsible infectious agent was tentatively classified as a filterable virus that could pass through a Seitz filter to remove bacteria and was reported to be a psittacosis-like or new virus. After that, Eaton et al. (1942, 1944, 1945) identified an agent that was the principal cause of primary atypical pneumonia using cotton rats, hamsters, and chick embryos. Eaton et al. (1942, 1944, 1945) did not perform an inoculation study in human volunteers. During the 1940s, there were three groups engaged in discovering the etiology of the primary atypical pneumonia. (1) Commission on Acute Respiratory Diseases Diseases directed by John Dingle, (2) Dr. Monroe Eaton's group, the Virus Research Laboratory of the California State Public Health Department, (3) The Hospital of the Rockefeller Institute for Medical Research directed by Horsfall. During 1940s, the members of the Commission on Acute Respiratory Diseases concluded that the bacteria-free filtrates obtained from the patients, presumably containing a virus, could induce primary atypical pneumonia in human volunteers via Pinehurst trials. During 1950s, serological approaches for identification of the Eaton agent developed such as Fluorescent-Stainable Antibody, and at the beginning of the1960s, the Eaton agent successfully grew in media, and finally accepted as a cause of primary atypical pneumonia. Thus, technical difficulties with visualizing the agent and failure to recognize the full significance of the Pinehurst

  3. How Can Pneumonia Be Prevented?

    MedlinePlus

    ... page from the NHLBI on Twitter. How Can Pneumonia Be Prevented? Pneumonia can be very serious and ... t last as long Fewer serious complications Pneumococcal Pneumonia Vaccine A vaccine is available to prevent pneumococcal ...

  4. Adolescent Idiopathic Scoliosis

    PubMed Central

    Choudhry, Muhammad Naghman; Ahmad, Zafar; Verma, Rajat

    2016-01-01

    Background: Scoliosis refers to deviation of spine greater than 10 degrees in the coronal plane. Idiopathic Scoliosis is the most common spinal deformity that develops in otherwise healthy children. The sub types of scoliosis are based on the age of the child at presentation. Adolescent idiopathic scoliosis (AIS) by definition occurs in children over the age of 10 years until skeletal maturity. Objective: The objective of this review is to outline the features of AIS to allow the physician to recognise this condition and commence early treatment, thereby optimizing patient outcome. Method: A thorough literature search was performed using available databases, including Pubmed and Embase, to cover important research published covering AIS. Conclusion: AIS results in higher incidence of back pain and discontent with body image. Curves greater than 50 degrees in thoracic region and greater than 30 degrees in lumbar region progress at a rate of 0.5 to 1 degree per year into adulthood. Curves greater than 60 degrees can lead to pulmonary functional deficit. Therefore once the disease is recognized, effective treatment should be instituted to address the deformity and prevention of its long-term sequelae. PMID:27347243

  5. [Serrapeptase-induced lung injury manifesting as acute eosiniphilic pneumonia].

    PubMed

    Sasaki, S; Kawanami, R; Motizuki, Y; Nakahara, Y; Kawamura, T; Tanaka, A; Watanabe, S

    2000-07-01

    An 84-year-old man was referred to our hospital because of fever, cough, and hemoptysis. The patient had acute respiratory failure (PaO2 < 40 mmHg) on admission, with diffuse interstitial infiltration and bilateral pleural effusion. The bronchoalveolar lavage fluid was bloody, and contained a high percentage of eosinophils (32%). A diagnosis of acute eosinophilic pneumonia was established, and the patient made a rapid recovery after corticosteroids were administered. When the DLST (drug lymphocyte stimulation test) was performed after the corticosteroid therapy was stopped, it was positive for serrapeptase, which had been prescribed for chronic cystitis for 3 months before the onset of the pneumonia. This was a case of drug (serrapeptase)-induced pneumonitis manifesting as acute eosinophilic pneumonia.

  6. The Etiology of Juvenile Idiopathic Arthritis.

    PubMed

    Rigante, Donato; Bosco, Annalisa; Esposito, Susanna

    2015-10-01

    Over the years, the commonly used term to describe juvenile idiopathic arthritis (JIA) has changed. By definition, JIA includes all types of arthritis with no apparent cause, lasting more than 6 weeks, in patients aged less than 16 years at onset. JIA pathogenesis is still poorly understood: the interaction between environmental factors and multiple genes has been proposed as the most relevant working mechanism to the development of JIA. The concept that various microbes that colonize or infect not only the mucosal surfaces, like the oral cavity, but also the airways and gut might trigger autoimmune processes, resulting in chronic arthritides, and JIA was first drafted at the outset of last century. JIA development might be initiated and sustained by the exposure to environmental factors, including infectious agents which affect people at a young age, depending on the underlying genetic predisposition to synovial inflammation. Many data from patients with JIA suggest a scenario in which different external antigens incite multiple antigen-specific pathways, cytotoxic T cell responses, activation of classical complement cascade, and production of proinflammatory cytokines. In this review, emphasis is paid not only to the potential role of parvovirus B19 and Epstein-Barr virus in primis but also to the general involvement of different bacteria as Salmonella spp., Shigella spp., Campylobacter spp., Mycoplasma pneumoniae, Chlamydophila pneumoniae, Bartonella henselae, and Streptococcus pyogenes for the development of immune-mediated arthritides during childhood. No unequivocal evidence favoring or refuting these associations has been clearly proved, and today, the strict definition of JIA etiology remains unknown. The infection can represent a random event in a susceptible individual, or it can be a necessary factor in JIA development, always in combination with a peculiar genetic background. Further studies are needed in order to address the unsolved questions

  7. The Etiology of Juvenile Idiopathic Arthritis.

    PubMed

    Rigante, Donato; Bosco, Annalisa; Esposito, Susanna

    2015-10-01

    Over the years, the commonly used term to describe juvenile idiopathic arthritis (JIA) has changed. By definition, JIA includes all types of arthritis with no apparent cause, lasting more than 6 weeks, in patients aged less than 16 years at onset. JIA pathogenesis is still poorly understood: the interaction between environmental factors and multiple genes has been proposed as the most relevant working mechanism to the development of JIA. The concept that various microbes that colonize or infect not only the mucosal surfaces, like the oral cavity, but also the airways and gut might trigger autoimmune processes, resulting in chronic arthritides, and JIA was first drafted at the outset of last century. JIA development might be initiated and sustained by the exposure to environmental factors, including infectious agents which affect people at a young age, depending on the underlying genetic predisposition to synovial inflammation. Many data from patients with JIA suggest a scenario in which different external antigens incite multiple antigen-specific pathways, cytotoxic T cell responses, activation of classical complement cascade, and production of proinflammatory cytokines. In this review, emphasis is paid not only to the potential role of parvovirus B19 and Epstein-Barr virus in primis but also to the general involvement of different bacteria as Salmonella spp., Shigella spp., Campylobacter spp., Mycoplasma pneumoniae, Chlamydophila pneumoniae, Bartonella henselae, and Streptococcus pyogenes for the development of immune-mediated arthritides during childhood. No unequivocal evidence favoring or refuting these associations has been clearly proved, and today, the strict definition of JIA etiology remains unknown. The infection can represent a random event in a susceptible individual, or it can be a necessary factor in JIA development, always in combination with a peculiar genetic background. Further studies are needed in order to address the unsolved questions

  8. Cryptogenic organising pneumonia.

    PubMed

    Cordier, J-F

    2006-08-01

    Organising pneumonia is defined histopathologically by intra-alveolar buds of granulation tissue, consisting of intermixed myofibroblasts and connective tissue. Although nonspecific, this histopathological pattern, together with characteristic clinical and imaging features, defines cryptogenic organising pneumonia when no cause or peculiar underlying context is found. Rapid clinical and imaging improvement is obtained with corticosteroid treatment, but relapses are common after stopping treatment.

  9. [Pneumonia in the elderly].

    PubMed

    Catherinot, Emilie

    2012-01-01

    Pneumonia is a serious medical pathology frequent in elderly people. The physiological changes of the respiratory system linked with age reduce postural drainage capacities and increase the risk of acute respiratory failure. Associated with other comorbidities, chronic inhalation is a major risk factor of pneumonia in elderly people. Prevention is based on vaccination, nutrition, dental care and an adapted diet.

  10. Early recognition of the 2009 pandemic influenza A (H1N1) pneumonia by chest ultrasound

    PubMed Central

    2012-01-01

    Introduction The clinical picture of the pandemic influenza A (H1N1)v ranges from a self-limiting afebrile infection to a rapidly progressive pneumonia. Prompt diagnosis and well-timed treatment are recommended. Chest radiography (CRx) often fails to detect the early interstitial stage. The aim of this study was to evaluate the role of bedside chest ultrasonography (US) in the early management of the 2009 influenza A (H1N1)v infection. Methods 98 patients who arrived in the Emergency Department complaining of influenza-like symptoms were enrolled in the study. Patients not displaying symptoms of acute respiratory distress were discharged without further investigations. Among patients with clinical suggestion of a community-acquired pneumonia, cases encountering other diagnoses or comorbidities were excluded from the study. Clinical history, laboratory tests, CRx, and computed tomography (CT) scan, if indicated, contributed to define the diagnosis of pneumonia in the remaining patients. Chest US was performed by an emergency physician, looking for presence of interstitial syndrome, alveolar consolidation, pleural line abnormalities, and pleural effusion, in 34 patients with a final diagnosis of pneumonia, in 16 having normal initial CRx, and in 33 without pneumonia, as controls. Results Chest US was carried out without discomfort in all subjects, requiring a relatively short time (9 minutes; range, 7 to 13 minutes). An abnormal US pattern was detected in 32 of 34 patients with pneumonia (94.1%). A prevalent US pattern of interstitial syndrome was depicted in 15 of 16 patients with normal initial CRx, of whom 10 (62.5%) had a final diagnosis of viral (H1N1) pneumonia. Patients with pneumonia and abnormal initial CRx, of whom only four had a final diagnosis of viral (H1N1) pneumonia (22.2%; P < 0.05), mainly displayed an US pattern of alveolar consolidation. Finally, a positive US pattern of interstitial syndrome was found in five of 33 controls (15.1%). False

  11. Adolescent idiopathic scoliosis.

    PubMed

    Cheng, Jack C; Castelein, René M; Chu, Winnie C; Danielsson, Aina J; Dobbs, Matthew B; Grivas, Theodoros B; Gurnett, Christina A; Luk, Keith D; Moreau, Alain; Newton, Peter O; Stokes, Ian A; Weinstein, Stuart L; Burwell, R Geoffrey

    2015-01-01

    Adolescent idiopathic scoliosis (AIS) is the most common form of structural spinal deformities that have a radiological lateral Cobb angle - a measure of spinal curvature - of ≥10(°). AIS affects between 1% and 4% of adolescents in the early stages of puberty and is more common in young women than in young men. The condition occurs in otherwise healthy individuals and currently has no recognizable cause. In the past few decades, considerable progress has been made towards understanding the clinical patterns and the three-dimensional pathoanatomy of AIS. Advances in biomechanics and technology and their clinical application, supported by limited evidence-based research, have led to improvements in the safety and outcomes of surgical and non-surgical treatments. However, the definite aetiology and aetiopathogenetic mechanisms that underlie AIS are still unclear. Thus, at present, both the prevention of AIS and the treatment of its direct underlying cause are not possible. PMID:27188385

  12. Congenital idiopathic clubfoot deformities.

    PubMed

    Kyzer, S P; Stark, S L

    1995-03-01

    Clubfoot is a birth defect that is marked primarily by a deformed talus (ie, ankle) and calcaneous (ie, heel) that give the foot a characteristic "club-like" appearance. In congenital idiopathic clubfoot (ie, talipes equinovarus), the infant's foot points downward (ie, equinus) and turns inward (ie, varus), while the forefoot curls toward the heel (ie, adduction). This congenital disorder has an incidence of 1 in 400 live births, with boys affected twice as often as girls. Unilateral clubfoot is somewhat more common than bilateral clubfoot and may occur as an isolated defect or in association with other disorders (eg, chromosomal aberrations, cerebral palsy, spina bifida, arthrogryposis). Infantile clubfoot deformity is painless and is correctable with early diagnosis and prompt treatment. PMID:7778903

  13. Chronic idiopathic nausea.

    PubMed

    Kovacic, Katja; Chelimsky, Gisela

    2014-04-01

    Chronic nausea is a prevalent but poorly described symptom in adolescents. It often co-occurs with other functional gastrointestinal disorders (FGIDs) but may also present in isolation. A multitude of triggers and complex neural pathways underlie the sensation of nausea. These include gastrointestinal and blood-borne insults, dysmotility, vestibular or central nervous system pathways, an aberrant autonomic nervous system, and psychosocial factors. Although clinical algorithms are lacking, diagnosis is typically made on the basis of a thorough clinical history and without extensive testing. Treatment is mainly empiric and may be directed at comorbid symptoms such as migraine, delayed gastric emptying, orthostatic intolerance, and visceral hypersensitivity. Chronic idiopathic nausea is an increasingly prevalent symptom that needs careful clinical assessment and individualized treatment plans.

  14. Juvenile idiopathic arthritis.

    PubMed

    Espinosa, Maria; Gottlieb, Beth S

    2012-07-01

    Juvenile idiopathic arthrithis (JIA) is the most common rheumatic disease of childhood.JIA is a chronic disease that is associated with periods of disease flares and periods of disease inactivity.Early, aggressive treatment with nonsteroidal anti-inflammatory drugs, intra-articular corticosteroid injections, or methotrexate, has significantly improved the outcome of most children who have JIA. Biologics have been shown to be both safe and effective for the treatment of more aggressive forms of arthritis and for uveitis. Long-term safety data of biologics is still uncertain. In the near future, it is hoped that genetic testing will allow earlier diagnosis of JIA as well as help predict the disease course of children who have JIA. Genetic analysis also may allow physicians to target therapies more effectively. It is hoped that development of more specific therapies will decrease overall immunosuppression and other associated toxicities.

  15. The idiopathic hypereosinophilic syndrome.

    PubMed Central

    Alfaham, M A; Ferguson, S D; Sihra, B; Davies, J

    1987-01-01

    A 14 year old girl with idiopathic hypereosinophilic syndrome is described. In addition to weight loss, anaemia, amenorrhoea, general lethargy, anorexia, mouth ulcers, blisters of hands and feet, and petechial skin rash, she had features of involvement of the cardiovascular system as the major complication. She responded well to treatment. After a comprehensive search of the published reports 18 cases of this syndrome were identified in children under 16 years. Fifteen of these children had involvement of the cardiovascular system as the major source of their morbidity and mortality. Summary of the clinical details and laboratory, biopsy, and necropsy findings of the involvement of the various organ systems of the 18 children is presented. PMID:3619478

  16. Idiopathic laryngotracheal stenosis

    PubMed Central

    Costantino, Christina L.

    2016-01-01

    Idiopathic laryngotracheal stenosis (ILTS) is a rare inflammatory disease of unknown etiology. Infectious, traumatic and immunologic processes must first be excluded. The majority of patients affected are female who present with progressive symptoms of upper airway obstruction, which can extend over a number of years. ILTS is characterized by short segment, circumferential stenotic lesions, located particularly at the level of the cricoid. Bronchoscopic evaluation is essential for establishing the diagnosis and operative planning. Various temporizing interventions have historically been utilized, including dilation and laser ablation, for symptomatic management. However these interventions have demonstrated diminishing returns and poor long-term outcomes. Patients with ILTS should be considered early for definitive surgical intervention to minimize complications and optimize outcomes. Laryngotracheal resection and reconstruction is a viable intervention, which has demonstrated good long-term results and low recurrence rates for this patient population. PMID:26981272

  17. [Idiopathic intracranial hypertension].

    PubMed

    Sergeev, A V

    2016-01-01

    Idiopathic intracranial hypertension (IIH) is a condition due to high intracranial pressure in the absence of an intracranial mass lesion, venous thrombosis or brain infection. It mostly occurs in young obese females. Currently, the incidence of IIH in obese women is estimated to be 12 per 100,000 people per year. Epidemiological data demonstrate the increase in incidence in this group: 323 cases per 100,000. IIH can cause visual loss in 1-2% of the patients during the year before the diagnosis and beginning of treatment. IIH treatment is a complex multidisciplinary problem that includes a body-mass reduction program, conservative pharmacological treatment, prolonged ophthalmological study and, if necessary, timely neurosurgical treatment.

  18. Idiopathic Flushing with Dysesthesia

    PubMed Central

    Fogelman, Joshua P.; Ashinoff, Robin; Soter, Nicholas A.

    2015-01-01

    Objective: The purpose of this study was to analyze the efficacy and safety of the 585nm pulsed dye laser for the treatment of idiopathic flushing with dysesthesia. Design: This was a retrospective study of patients treated with a 585nm pulsed dye laser with fluences ranging from 3.5 to 7.5J/cm2 (purpura threshold fluences), a pulse duration of 450μsec, and a spot size of 5 or 10mm. Setting: The Ronald 0. Perelman Department of Dermatology at New York University Medical Center. Participants: Ten adult subjects who presented with flushing with dysesthesia. Measurements: Participants subjectively evaluated the decrease in dysesthesia and the number of flushing episodes. The objective response to treatment was evaluated by a single physician using pre- and postoperative photographs. The severity of postoperative erythema was compared with baseline using an ordinal scale ranging from zero (resolution of erythema) to four (76-100% of baseline erythema). Results: The mean number of treatments received by the subjects was seven. The mean fluence was 6.66J/cm2. Subjectively, 100 percent of subjects reported a decrease in dysethesia and the number of flushing episodes. Objectively, subjects demonstrated at least a 62.5-percent reduction in erythema. Conclusion: Laser surgery provided subjective relief of dysesthesia and decreased the number of flushing episodes with a greater than 62-percent objective reduction in the severity of erythema. The 585nm pulsed dye laser is a safe, efficacious treatment for the signs and symptoms of idiopathic flushing with dysesthesia. PMID:26345489

  19. The Functional Dysphagia Scale Is a Useful Tool for Predicting Aspiration Pneumonia in Patients With Parkinson Disease

    PubMed Central

    2016-01-01

    Objective To describe the correlation between the functional dysphagia scale and aspiration pneumonia and which characteristics influence the occurrence of aspiration pneumonia in patients with idiopathic Parkinson disease. Methods Fifty-three patients with idiopathic Parkinson disease were prospectively evaluated in this study. Disease severity and functional status were measured by modified Hoehn and Yahr (H&Y) staging, Schwab and England activities of daily living (S-E ADL) scale and Korean version of Mini-Mental State Examination (K-MMSE). Swallowing function was evaluated by the functional dysphagia scale (FDS) and the penetration-aspiration scale (PAS) based on a videofluoroscopic swallowing study. The patients were followed up for 3 months and divided into two groups according to the occurrence of aspiration pneumonia. The correlation between the variables and aspiration pneumonia was analyzed. Results Eight patients of the 53 patients were allocated to the aspiration pneumonia group and 45 patients to the non-aspiration pneumonia group. The patients in the aspiration pneumonia group had significantly higher H&Y staging, and scored lower on S-E ADL scale and K-MMSE. The patients in the aspiration pneumonia group had significantly higher scores on FDS and PAS. A multiple logistic regression analysis showed that the S-E ADL scale and the FDS were associated with the occurrence of aspiration pneumonia in the patients with Parkinson disease. Conclusion Given that the FDS can quantitatively assess the functional problems associated with dysphagia, it can be clinically effective in predicting the occurrence of aspiration pneumonia, and the FDS and the S-E ADL scale could be predictive variables for aspiration pneumonia in patients with Parkinson disease. PMID:27446780

  20. Antibody responses of Chlamydophila pneumoniae pneumonia: Why is the diagnosis of C. pneumoniae pneumonia difficult?

    PubMed

    Miyashita, Naoyuki; Kawai, Yasuhiro; Tanaka, Takaaki; Akaike, Hiroto; Teranishi, Hideto; Wakabayashi, Tokio; Nakano, Takashi; Ouchi, Kazunobu; Okimoto, Niro

    2015-07-01

    The ELNAS Plate Chlamydophila pneumoniae commercial test kit for the detection of anti-C. pneumoniae-specific immunoglobulin M (IgM), IgA and IgG antibodies has become available in Japan recently. To determine the optimum serum collection point for the ELNAS plate in the diagnosis of C. pneumoniae pneumonia, we analyzed the kinetics of the antibody response in patients with laboratory-confirmed C. pneumoniae pneumonia. We enrolled five C. pneumoniae pneumonia cases and collected sera from patients for several months. The kinetics of the IgM and IgG antibody responses were similar among the five patients. Significant increases in IgM and IgG antibody titer between paired sera were observed in all patients. IgM antibodies appeared approximately 2-3 weeks after the onset of illness, reached a peak after 4-5 weeks, and were generally undetectable after 3-5 months. IgG antibodies developed slowly for the first 30 days and reached a plateau approximately 3-4 months after the onset of illness. The kinetics of IgA antibody responses were different among the five patients, and significant increases in IgA antibody titer between paired sera were observed in only two patients. Although the sample size was small, the best serum collection time seemed to be approximately 3-6 weeks after onset of illness when using a single serum sample for the detection of IgM antibodies. Paired sera samples should be obtained at least 4 weeks apart. IgA antibody analysis using ELNAS may not be a useful marker for acute C. pneumoniae pneumonia.

  1. Verminous pneumonia and enteritis due to hyperinfection with Aelurostrongylus abstrusus in a kitten.

    PubMed

    Philbey, A W; Krause, S; Jefferies, R

    2014-05-01

    Severe infestation with Aelurostrongylus abstrusus was identified in the lungs and small intestine of a 2-month-old kitten that died due to verminous pneumonia and enteritis. On clinical examination, the kitten had dyspnoea, pneumonia, pleural effusion, ascites and diarrhoea. An interstitial pattern was evident radiographically in the lungs. The kitten died before treatment could be instituted. On gross and histopathological examination, there was severe interstitial pneumonia and large numbers of A. abstrusus eggs and larvae were present in alveoli, together with fewer adult nematodes in small bronchioles. The mucosa of the small intestine was invaded by large numbers of A. abstrusus larvae. The findings were consistent with a hyperinfection syndrome due to A. abstrusus.

  2. Fibronectin in human bronchopulmonary lavage fluid. Elevation in patients with interstitial lung disease.

    PubMed Central

    Rennard, S I; Crystal, R G

    1982-01-01

    Fibronectin is a major adhesive and opsonic glycoprotein found in plasma and tissues. Because this molecule appears to mediate a number of interactions between cells and extracellular matrix, and because the interstitial lung disease are characterized by marked derangements of the pulmonary extracellular matrix, we evaluated fibronectin in the lower respiratory tract in patients with these disorders. Fibronectin could be detected in the bronchoalveolar lavage fluid of normals (11/11), as well as those with noninterstitial lung diseases (18/18), idiopathic pulmonary fibrosis (21/21), sarcoidosis (20/20), and other interstitial lung diseases (22/22). Compared with normal and those with noninterstitial lung disease, the levels in bronchoalveolar lavage of patients with interstitial disease were significantly higher (P less than 0.01), all comparisons). This was true only for bronchoalveolar lavage fibronectin; plasma levels were similar in all study groups (P greater than 0.2, all comparisons). The lavage fluid fibronectin was intact antigenically and retained collagen binding capability, although in some cases of interstitial disease, the presence of lower molecular weight fragments suggested some degradation. Thus, fibronectin is a normal constituent of the epithelial fluid of the lower respiratory tract and is present in increased amounts in a significant number of individuals with interstitial lung disease. Images PMID:7054232

  3. Interstitial Functionalization in elemental Si

    NASA Astrophysics Data System (ADS)

    Kiefer, Boris; Fohtung, Edwin

    Societies in the 21st century will face many challenges. Materials science and materials design will be essential to address and master some if not all of these challenges. Semiconductors are among the most important technological material classes. Properties such as electrical transport are strongly affected by defects and a central goal continues to be the reduction of defect densities as much as possible in these compounds. Here we present results of interstitial Fe doping in elemental Si using first-principles DFT calculations. The preliminary results show that Fe will only occupy octahedral interstitial sites. The analysis of the electronic structure shows that the compounds are ferromagnetic and that a bandgap opens as interstitial Fe concentrations decrease, with a possible intermittent semi-metallic phase. The formation energy for interstitial Fe is unfavorable, as expected, by ~1.5 eV but becomes favorable as the chemical potential of Fe increases. Therefore, we expect that biasing the system with an external electrical field will lead to the formation of these materials. Thus, our results show that interstitial defects can be beneficial for the design of functionalities that differ significantly from those of the host material.

  4. Predictors of mortality in rheumatoid arthritis-associated interstitial lung disease.

    PubMed

    Solomon, Joshua J; Chung, Jonathan H; Cosgrove, Gregory P; Demoruelle, M Kristen; Fernandez-Perez, Evans R; Fischer, Aryeh; Frankel, Stephen K; Hobbs, Stephen B; Huie, Tristan J; Ketzer, Jill; Mannina, Amar; Olson, Amy L; Russell, Gloria; Tsuchiya, Yutaka; Yunt, Zulma X; Zelarney, Pearlanne T; Brown, Kevin K; Swigris, Jeffrey J

    2016-02-01

    Interstitial lung disease (ILD) is a common pulmonary manifestation of rheumatoid arthritis. There is lack of clarity around predictors of mortality and disease behaviour over time in these patients.We identified rheumatoid arthritis-related interstitial lung disease (RA-ILD) patients evaluated at National Jewish Health (Denver, CO, USA) from 1995 to 2013 whose baseline high-resolution computed tomography (HRCT) scans showed either a nonspecific interstitial pneumonia (NSIP) or a "definite" or "possible" usual interstitial pneumonia (UIP) pattern. We used univariate, multivariate and longitudinal analytical methods to identify clinical predictors of mortality and to model disease behaviour over time.The cohort included 137 subjects; 108 had UIP on HRCT (RA-UIP) and 29 had NSIP on HRCT (RA-NSIP). Those with RA-UIP had a shorter survival time than those with RA-NSIP (log rank p=0.02). In a model controlling for age, sex, smoking and HRCT pattern, a lower baseline % predicted forced vital capacity (FVC % pred) (HR 1.46; p<0.0001) and a 10% decline in FVC % pred from baseline to any time during follow up (HR 2.57; p<0.0001) were independently associated with an increased risk of death.Data from this study suggest that in RA-ILD, disease progression and survival differ between subgroups defined by HRCT pattern; however, when controlling for potentially influential variables, pulmonary physiology, but not HRCT pattern, independently predicts mortality.

  5. Lung transplantation in idiopathic pulmonary fibrosis: a systematic review of the literature

    PubMed Central

    2014-01-01

    Background Idiopathic pulmonary fibrosis (IPF) is a distinct form of interstitial pneumonia with unknown origin and poor prognosis. Current pharmacologic treatments are limited and lung transplantation is a viable option for appropriate patients. The aim of this review was to summarize lung transplantation survival in IPF patients overall, between single (SLT) vs. bilateral lung transplantation (BLT), pre- and post Lung Allocation Score (LAS), and summarize wait-list survival. Methods A systematic review of English-language studies published in Medline or Embase between 1990 and 2013 was performed. Eligible studies were those of observational design reporting survival post-lung transplantation or while on the wait list among IPF patients. Results Median survival post-transplantation among IPF patients is estimated at 4.5 years. From ISHLT and OPTN data, one year survival ranged from 75% - 81%; 3-year: 59% - 64%; and 5-year: 47% - 53%. Post-transplant survival is lower for IPF vs. other underlying pre-transplant diagnoses. The proportion of IPF patients receiving BLT has steadily increased over the last decade and a half. Unadjusted analyses suggest improved long-term survival for BLT vs. SLT; after adjustment for patient characteristics, the differences tend to disappear. IPF patients account for the largest proportion of patients on the wait list and while wait list time has decreased, the number of transplants for IPF patients has increased over time. OPTN data show that wait list mortality is higher for IPF patients vs. other diagnoses. The proportion of IPF patients who died while awaiting transplantation ranged from 14% to 67%. While later transplant year was associated with increased survival, no significant differences were noted pre vs. post LAS implementation; however a high LAS vs low LAS was associated with decreased one-year survival. Conclusions IPF accounts for the largest proportion of patients awaiting lung transplants, and IPF is associated with

  6. Drug Induced Interstitial Lung Disease

    PubMed Central

    Schwaiblmair, Martin; Behr, Werner; Haeckel, Thomas; Märkl, Bruno; Foerg, Wolfgang; Berghaus, Thomas

    2012-01-01

    With an increasing number of therapeutic drugs, the list of drugs that is responsible for severe pulmonary disease also grows. Many drugs have been associated with pulmonary complications of various types, including interstitial inflammation and fibrosis, bronchospasm, pulmonary edema, and pleural effusions. Drug-induced interstitial lung disease (DILD) can be caused by chemotherapeutic agents, antibiotics, antiarrhythmic drugs, and immunosuppressive agents. There are no distinct physiologic, radiographic or pathologic patterns of DILD, and the diagnosis is usually made when a patient with interstitial lung disease (ILD) is exposed to a medication known to result in lung disease. Other causes of ILD must be excluded. Treatment is avoidance of further exposure and systemic corticosteroids in patients with progressive or disabling disease. PMID:22896776

  7. Community-acquired pneumonia.

    PubMed

    Falguera, M; Ramírez, M F

    2015-11-01

    This article not only reviews the essential aspects of community-acquired pneumonia for daily clinical practice, but also highlights the controversial issues and provides the newest available information. Community-acquired pneumonia is considered in a broad sense, without excluding certain variants that, in recent years, a number of authors have managed to delineate, such as healthcare-associated pneumonia. The latter form is nothing more than the same disease that affects more frail patients, with a greater number of risk factors, both sharing an overall common approach. PMID:26186969

  8. Community-acquired pneumonia.

    PubMed

    Falguera, M; Ramírez, M F

    2015-11-01

    This article not only reviews the essential aspects of community-acquired pneumonia for daily clinical practice, but also highlights the controversial issues and provides the newest available information. Community-acquired pneumonia is considered in a broad sense, without excluding certain variants that, in recent years, a number of authors have managed to delineate, such as healthcare-associated pneumonia. The latter form is nothing more than the same disease that affects more frail patients, with a greater number of risk factors, both sharing an overall common approach.

  9. Interstitial brines in playa sediments

    USGS Publications Warehouse

    Jones, B.F.; Van Denburgh, A.S.; Truesdell, A.H.; Rettig, S.L.

    1969-01-01

    Study of several closed drainages in the Great Basin has shown that the interstitial solutions of shallow, fine-grained playa deposits store a large quantity of dissolved solids and are often more concentrated than associated lakes and ponds, except in peripheral zones of stream or ground-water inflow. These interstitial fluids, when compared with local runoff, impoundments, or spring waters, commonly have a distinctive ionic composition which sometimes cannot be explained by either simple mixing of surface and subsurface inflow or by evaporative concentration. At Abert Lake, Oregon, the interstitial solute concentrations increased with depth to values as much as five times greater than the lake, except where springs indicate significant ground-water input. Where Na+, Cl, and CO2 species constitute more than 90% of the solutes, Na+ Cl- ratios in the lake water are lower than in interstitial solutions of bottom cores and higher than in playa fluids. At the same time, Na+ K+ ratios are highest in the fluids of lake bottom muds and lowest in playa interstitials. In deeper playa profiles, interstitial Na+ Cl- tended to decrease with depth (5 ft. maximum). In the Abert Lake area, as in other parts of the western Great Basin, Na+ Cl- ratios are indicative of total CO2 in solution and the effects of organic decay in surficial sediments. These ratios, coupled with data on silica and bulk density, show that higher PCO2 accompanying decay promotes silicate dissolution and hydrogen ion exchange, stripping alkalis from sediment which had preferentially adsorbed K+ when entering the lake. On subsequent loss of pore fluid in the playa regime, silica initially released to solution in the lake environment is readsorbed on dissolution products. ?? 1969.

  10. Equine testicular interstitial cell tumors.

    PubMed

    Gelberg, H B; McEntee, K

    1987-05-01

    Interstitial cell tumors from nine stallions were described. In all but one horse the tumors were found in undescended testes. Five animals had bilateral tumors. Two animals showed increased aggression. Tumors contained two cell types. The first type were large distinctly bordered eosinophilic cells interpreted to be hyperplastic and hypertrophic interstitial cells. They blended with pleomorphic often spindloid neoplastic cells which had fibrillar, vacuolated cytoplasm and indistinct cell borders. This latter cell population was arranged in nodules or broad sheets as endocrine-like packets or interweaving fascicles. Biologic behavior of the neoplasms could not be ascertained from histologic examination. PMID:2885961

  11. Interstitial lung disease in scleroderma.

    PubMed

    Schoenfeld, Sara R; Castelino, Flavia V

    2015-05-01

    Systemic sclerosis is a heterogeneous disease of unknown etiology with limited effective therapies. It is characterized by autoimmunity, vasculopathy, and fibrosis and is clinically manifested by multiorgan involvement. Interstitial lung disease is a common complication of systemic sclerosis and is associated with significant morbidity and mortality. The diagnosis of interstitial lung disease hinges on careful clinical evaluation and pulmonary function tests and high-resolution computed tomography. Effective therapeutic options are still limited. Several experimental therapies are currently in early-phase clinical trials and show promise.

  12. Interstitial nephritis. A brief review.

    PubMed Central

    Heptinstall, R. H.

    1976-01-01

    Interstitial nephritis is a common condition, which in spite of a relatively constant pathologic picture has different etiologic agents and pathogenetic mechanisms. Failure to appreciate this, particularly in the chronic group, has led to considerable confusion and has been largely responsible for the overdiagnosis of chronic pyelonephritis. Although we are still largely ignorant of the causes of interstitial nephritis, it is now possible to define many of them. While experimental studies have not made spectacular contributions to our understanding, an attempt is now being made to develop appropriate models, and we hope these will enable us to still further clarify our understanding of other entities. PMID:776003

  13. How Is Idiopathic Pulmonary Fibrosis Treated?

    MedlinePlus

    ... the NHLBI on Twitter. How Is Idiopathic Pulmonary Fibrosis Treated? Doctors may prescribe medicines, oxygen therapy , pulmonary ... PR), and lung transplant to treat idiopathic pulmonary fibrosis (IPF). Medicines Currently, no medicines are proven to ...

  14. Acute interstitial pneumonitis. Case series and review of the literature.

    PubMed

    Vourlekis, J S; Brown, K K; Cool, C D; Young, D A; Cherniack, R M; King, T E; Schwarz, M I

    2000-11-01

    Acute interstitial pneumonitis (AIP) is an acute, idiopathic interstitial lung disease characterized by rapidly progressive diffuse pulmonary infiltrates and hypoxemia requiring hospitalization. The case-fatality ratio is high. Previous reports suggested that survivors of the acute event have a favorable outcome. We undertook this study to examine the natural history of survivors. We had observed several patients who experienced recurrent episodes of AIP and chronic progressive interstitial lung disease. We sought to determine longitudinal survival in these patients and to compare our experience with that in the medical literature. Overall, we identified 13 biopsy-proven cases of AIP. The mean patient age was 54 years in our review, which is identical to previous reports. Twelve patients were hospitalized and all 12 required mechanical ventilation. Overall hospital survival was 67%. All patients demonstrated abnormalities in gas exchange at presentation. Radiographs typically demonstrated bilateral patchy densities that progressed to a diffuse alveolar filling pattern in nearly all cases. All biopsy specimens showed organizing diffuse alveolar damage. Longitudinal data were available for 7 patients. Two died of AIP recurrences. A third died of complications of heart failure shortly after hospital discharge. One patient progressed to end-stage lung disease and required lung transplantation. Two patients experienced persistent pulmonary symptoms, accompanied in 1 by progressive lung fibrosis. One patient had nearly complete recovery of lung function 2 years following AIP. (Follow-up information was unavailable for 2 survivors.) In our literature review, 5 of 7 patients reported experienced some recovery of lung function. One case of progressive interstitial lung disease requiring lung transplantation was reported. The reported mortality was much higher than in our experience (74% versus 33%). The mean time from symptom onset to death was 26 days, compared with 34

  15. Idiopathic inflammatory myositis.

    PubMed

    Tieu, Joanna; Lundberg, Ingrid E; Limaye, Vidya

    2016-02-01

    Knowledge on idiopathic inflammatory myopathy (IIM) has evolved with the identification of myositis-associated and myositis-specific antibodies, development of histopathological classification and the recognition of how these correlate with clinical phenotype and response to therapy. In this paper, we outline key advances in diagnosis and histopathology, including the more recent identification of antibodies associated with immune-mediated necrotising myopathy (IMNM) and inclusion body myositis (IBM). Ongoing longitudinal observational cohorts allow further classification of these patients with IIM, their predicted clinical course and response to specific therapies. Registries have been developed worldwide for this purpose. A challenging aspect in IIM, a multisystem disease with multiple clinical subtypes, has been defining disease status and clinically relevant improvement. Tools for assessing activity and damage are now recognised to be important in determining disease activity and guiding therapeutic decision-making. The International Myositis Assessment and Clinical Studies (IMACS) group has developed such tools for use in research and clinical settings. There is limited evidence for specific treatment strategies in IIM. With significant development in the understanding of IIM and improved classification, longitudinal observational cohorts and trials using validated outcome measures are necessary, to provide important information for evidence-based care in the clinical setting. PMID:27421222

  16. [Chronic eosinophilic pneumonia].

    PubMed

    Vivero, F; Ciocchini, C; Gandini, M J; Wehbe, L

    2012-03-01

    Chronic eosinophilic pneumonia. The chronic eosinophilic pneumonia is part of Pulmonary Eosinophilic Syndroms. It is presented a 33-years old man, Asmathic, with dry cough, fever, night sweats and fatigue of several weeks. The chest X-ray showed opacity in the right hemithorax. He was treated with antibiotics without response. A chest TC showed multifocal involvement. The patient refused bronchoalveolar lavage (BAL) so treatment antituberculostatic was started. Despite treatment the symptoms worsened. The Chest X-ray showed migration of the infiltrates and the blood smear marked eosinophilia. Finally, bronchoalveolar lavage was carried out and it showed a high percentage of eosinophils (over 50%). The patient was treated with inmmunosuppresive doses of corticosteroids with excellent response. The blood smear in Nonresolving pneumonia is key to consider eosinophilic pneumonia, an uncommon pathology but amenable to treatment.

  17. Hospital-acquired pneumonia

    MedlinePlus

    ... tends to be more serious than other lung infections because: People in the hospital are often very sick and cannot fight off ... prevent pneumonia. Most hospitals have programs to prevent hospital-acquired infections.

  18. Pneumonia in adults - discharge

    MedlinePlus

    ... In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, and Bennett's Principles and Practice of Infectious ... ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 69. Mandell LA. Streptococcus pneumoniae infections. In: Goldman L, Schafer ...

  19. Community-acquired pneumonia.

    PubMed

    Prina, Elena; Ranzani, Otavio T; Torres, Antoni

    2015-09-12

    Community-acquired pneumonia causes great mortality and morbidity and high costs worldwide. Empirical selection of antibiotic treatment is the cornerstone of management of patients with pneumonia. To reduce the misuse of antibiotics, antibiotic resistance, and side-effects, an empirical, effective, and individualised antibiotic treatment is needed. Follow-up after the start of antibiotic treatment is also important, and management should include early shifts to oral antibiotics, stewardship according to the microbiological results, and short-duration antibiotic treatment that accounts for the clinical stability criteria. New approaches for fast clinical (lung ultrasound) and microbiological (molecular biology) diagnoses are promising. Community-acquired pneumonia is associated with early and late mortality and increased rates of cardiovascular events. Studies are needed that focus on the long-term management of pneumonia.

  20. How Is Childhood Interstitial Lung Disease Treated?

    MedlinePlus

    ... the NHLBI on Twitter. How Is Childhood Interstitial Lung Disease Treated? Childhood interstitial lung disease (chILD) is ... prevent acid reflux, which can lead to aspiration. Lung Transplant A lung transplant may be an option ...

  1. Types of Childhood Interstitial Lung Disease

    MedlinePlus

    ... the NHLBI on Twitter. Types of Childhood Interstitial Lung Disease The broad term "childhood interstitial lung disease" ( ... affect are shown in the illustration below. Normal Lungs and Lung Structures Figure A shows the location ...

  2. [CT pathologic correlative study of bronchiolitis obliterans organizing pneumonia].

    PubMed

    Nishimura, K; Kitaichi, M; Izumi, T; Kanaoka, M; Itoh, H

    1989-01-01

    Though bronchiolitis obliterans organizing pneumonia (BOOP) was proposed as a new infiltrative lung disease in 1985, we think it has two radiologic problems. First, in spite of interstitial pneumonia, about half of chest radiographs of BOOP has been reported to show alveolar opacities. Second, because radiologic features of both some cases of BOOP and of usual interstitial pneumonia (UIP) show reticular shadows on chest radiographs, it is sometimes difficult to differentiate between two diseases. We correlated CT images with open lung biopsy specimens and evaluated CT's ability to differentiate BOOP from UIP. CT findings of all cases of BOOP were: (1) markedly increased dense infiltrates of various sizes which demarcated sharply from normal lung field. Air bronchogram was always present. (2) less dense images were seen which also stood out against the normal lung field. The former corresponded to air space consolidation formed by organized exudates and inflammatory cells within alveolar ducts and alveoli, while the latter indicated luminal and mural alveolitis. Both findings were sharply delineated from each other probably because of intervening interlobular septa. Conglomerated small cystic shadows and air bronchography within areas of intense lung density were seen in CT images of most of 28 patients with UIP. Those findings proved to correspond to macroscopic or microscopic honey combing which were not seen in our cases of BOOP. These radiologic and pathologic features of UIP were different. PMID:2724593

  3. Idiopathic Pulmonary Fibrosis and Myasthenia Gravis: An Unusual Association.

    PubMed

    Chogtu, Bharti; Malik, Daliparty Vasudev; Magazine, Rahul

    2016-04-01

    Idiopathic Pulmonary Fibrosis (IPF) is a chronic fibrosing lung condition with high morbidity and mortality, accounting for about 25% of the cases of interstitial lung diseases. It usually has a progressive course resulting in death due to respiratory failure. Myasthenia Gravis (MG) is an autoimmune neuromuscular disease, caused by antibody mediated activity against acetylcholine receptor at the neuromuscular junction. It is characterized by fluctuating muscle weakness and fatigue. Extensive literature search did not reveal any case report of an association between these two conditions. Here we present a case of a patient with IPF who also developed MG. The diagnosis of IPF was based on High Resolution Computed Tomography (HRCT) of the lung and that of MG was based on clinical criteria and electrophysiological testing. The case was successfully managed. PMID:27190866

  4. Aetiology of idiopathic granulomatous mastitis

    PubMed Central

    Altintoprak, Fatih; Kivilcim, Taner; Ozkan, Orhan Veli

    2014-01-01

    Idiopathic granulomatous mastitis is a rare chronic inflammatory lesion of the breast that can clinically and radiographically mimic breast carcinoma. The most common clinical presentation is an unilateral, discrete breast mass, nipple retraction and even a sinus formation often associated with an inflammation of the overlying skin. The etiology of idiopathic granulomatous mastitis is still obscure. Its treatment remains controversial. The cause may be the autoimmune process, infection, a chemical reaction associated with oral contraceptive pills, or even lactation. Various factors, including hormonal imbalance, autoimmunity, unknown microbiological agents, smoking and α 1-antitrypsin deficiency have been suggested to play a role in disease aetiology. In this review, causing factors in the aetiology of idiopathic granulomatous mastitis are reviewed in detail. PMID:25516860

  5. B-Cell Depletion Salvage Therapy in Rapidly Progressive Dermatomyositis Related Interstitial Lung Disease.

    PubMed

    Eissa, Khaled; Palomino, Jaime

    2016-01-01

    Interstitial lung disease (ILD) is a major cause of morbidity and mortality in patients with idiopathic inflammatory myopathies (IIM). Glucocorticoids are the initial standard treatment. However, many patients fail to respond and continue to progress despite treatment with high dose glucocorticoids. The efficacy of rituximab has been suggested in case reports and case series of refractory antisynthetase (AS) syndrome, but data on patients without auto-antibodies or with rapidly progressive ILD are scarce. We report a case of rapidly progressive dermatomyositis (DM) associated ILD treated successfully with B-cell depletion therapy. PMID:27389378

  6. Idiopathic Sporadic Onychomadesis of Toenails

    PubMed Central

    Nitayavardhana, Sunatra

    2016-01-01

    Onychomadesis is a clinical sign of nail plate separation due to transient or permanent arrest of nail matrix activities. Onychomadesis can be considered as a severe form of Beau's line. This condition usually occurs after trauma, causal diseases, or medications, yet it rarely occurs as an idiopathic condition. We report a case of a 38-year-old Thai female who developed recurrence onychomadesis in several toenails in the absence of predisposing factors or associated conditions. To the best of our knowledge, our patient is the first reported case of idiopathic onychomadesis limited to toenails. PMID:27437152

  7. Chronic pulmonary interstitial fibrosis in a blue-fronted Amazon parrot (Amazona aestiva aestiva).

    PubMed

    Amann, Olga; Kik, Marja J L; Passon-Vastenburg, Maartje H A C; Westerhof, Ineke; Lumeij, Johannes T; Schoemaker, Nico J

    2007-03-01

    A 30-yr-old blue-fronted Amazon parrot (Amazon aestiva aestiva) was presented to the clinic with a history of sneezing more often during the last 2 mo. Physical examination revealed only a mild nasal discharge. Complete hematologic and plasma biochemical examination showed no abnormalities. Computerized tomography (CT) of the complete bird showed generalized lung alterations consistent with lung fibrosis. Two lung biopsies were taken. The results of the histologic examination of the biopsies confirmed the tentative CT diagnosis of pulmonary interstitial fibrosis. To our knowledge this is the first reported case of chronic pulmonary interstitial fibrosis diagnosed by means of a lung biopsy in an avian species. The histologic characteristics are discussed and compared with those of human idiopathic pulmonary fibrosis.

  8. Risk of serious infection in patients with rheumatoid arthritis-associated interstitial lung disease.

    PubMed

    Zamora-Legoff, Jorge A; Krause, Megan L; Crowson, Cynthia S; Ryu, Jay H; Matteson, Eric L

    2016-10-01

    The objective of this study is to assess the occurrence of and risk factors for serious infections in rheumatoid arthritis (RA)-associated interstitial lung disease (ILD). All patients with RA-ILD (ACR 1987 criteria for RA) seen at a single center from 1998 to 2014 were identified and manually screened for study inclusion. Follow-up data were abstracted until death or December 31, 2015. Serious infection was defined as requiring antimicrobial therapy and hospitalization. Risk of infection was analyzed by person-year (py) methods using time-dependent covariates started when the medication was first used and stopped 30 days after the medication was discontinued. Of the 181 included patients, 87 (48 %) were female. The mean age at ILD diagnosis was 67.4 (±9.9) years, and median follow-up time was 3.1 (range: 0.01 to 14.8) years. Higher infection rates were observed during the first year after ILD diagnosis (14.1 per 100 py) than subsequently (5.7 per 100 py; p = 0.001). Pneumonia was the most common (3.9 per 100 py). Overall infection risk was higher in organizing pneumonia (OP) (27.1 per 100 py) than usual interstitial pneumonia (7.7 per 100 py) or non-specific interstitial pneumonia (5.5 per 100 py) (p < 0.001). The highest infection rate observed was with a daily prednisone use >10 mg per day (15.4 per 100 py). Patients with RA-ILD are at risk of serious infection. Prednisone use >10 mg per day was associated with higher rates of infection. Immunosuppressive drug use governed by concern for risk of infection in patients with ILD resulting in channeling bias cannot be excluded. PMID:27448151

  9. Disease activity in idiopathic pulmonary fibrosis: CT and pathologic correlation.

    PubMed

    Müller, N L; Staples, C A; Miller, R R; Vedal, S; Thurlbeck, W M; Ostrow, D N

    1987-12-01

    Computed tomography (CT) scans were compared with pathologic determinants of disease activity in 12 patients with idiopathic pulmonary fibrosis. The theory was that intraalveolar and interstitial cellularity would result in areas of opacification of air spaces on CT scans. All patients underwent open lung biopsy, and disease activity was assessed with a pathologic grading system. Seven patients had mild disease activity, five had moderate to marked disease activity. Opacification of air spaces was patchy in distribution, predominantly peripheral, and seen better on 1.5-mm rather than 10-mm collimation scans. Disease activity on CT scans was graded independently from 0 to 3 based on the presence and relative density of the areas of air space consolidation compared with the surrounding parenchyma. The pathologic score was significantly greater in the patients with high CT scores than in those with low CT scores (P = .001). Five patients with marked disease activity and five of seven patients with mild disease activity were correctly identified. CT may be useful in the assessment of disease activity in idiopathic pulmonary fibrosis.

  10. Idiopathic hypercalciuria and formation of calcium renal stones.

    PubMed

    Coe, Fredric L; Worcester, Elaine M; Evan, Andrew P

    2016-09-01

    The most common presentation of nephrolithiasis is idiopathic calcium stones in patients without systemic disease. Most stones are primarily composed of calcium oxalate and form on a base of interstitial apatite deposits, known as Randall's plaque. By contrast some stones are composed largely of calcium phosphate, as either hydroxyapatite or brushite (calcium monohydrogen phosphate), and are usually accompanied by deposits of calcium phosphate in the Bellini ducts. These deposits result in local tissue damage and might serve as a site of mineral overgrowth. Stone formation is driven by supersaturation of urine with calcium oxalate and brushite. The level of supersaturation is related to fluid intake as well as to the levels of urinary citrate and calcium. Risk of stone formation is increased when urine citrate excretion is <400 mg per day, and treatment with potassium citrate has been used to prevent stones. Urine calcium levels >200 mg per day also increase stone risk and often result in negative calcium balance. Reduced renal calcium reabsorption has a role in idiopathic hypercalciuria. Low sodium diets and thiazide-type diuretics lower urine calcium levels and potentially reduce the risk of stone recurrence and bone disease. PMID:27452364

  11. Recent advances in understanding idiopathic pulmonary fibrosis

    PubMed Central

    Daccord, Cécile; Maher, Toby M.

    2016-01-01

    Despite major research efforts leading to the recent approval of pirfenidone and nintedanib, the dismal prognosis of idiopathic pulmonary fibrosis (IPF) remains unchanged. The elaboration of international diagnostic criteria and disease stratification models based on clinical, physiological, radiological, and histopathological features has improved the accuracy of IPF diagnosis and prediction of mortality risk. Nevertheless, given the marked heterogeneity in clinical phenotype and the considerable overlap of IPF with other fibrotic interstitial lung diseases (ILDs), about 10% of cases of pulmonary fibrosis remain unclassifiable. Moreover, currently available tools fail to detect early IPF, predict the highly variable course of the disease, and assess response to antifibrotic drugs. Recent advances in understanding the multiple interrelated pathogenic pathways underlying IPF have identified various molecular phenotypes resulting from complex interactions among genetic, epigenetic, transcriptional, post-transcriptional, metabolic, and environmental factors. These different disease endotypes appear to confer variable susceptibility to the condition, differing risks of rapid progression, and, possibly, altered responses to therapy. The development and validation of diagnostic and prognostic biomarkers are necessary to enable a more precise and earlier diagnosis of IPF and to improve prediction of future disease behaviour. The availability of approved antifibrotic therapies together with potential new drugs currently under evaluation also highlights the need for biomarkers able to predict and assess treatment responsiveness, thereby allowing individualised treatment based on risk of progression and drug response. This approach of disease stratification and personalised medicine is already used in the routine management of many cancers and provides a potential road map for guiding clinical care in IPF. PMID:27303645

  12. Idiopathic necrotising enteritis cases continue.

    PubMed

    2014-09-27

    Cases of idiopathic necrotising enteritis in calves continue Polioencephalitis of unknown cause in lambs Rare types of deformities seen in piglets Colibacillosis in postweaned pigs Rotavirus in gamebirds These are among matters discussed in the Animal Health and Veterinary Laboratories Agency's (AHVLA's) disease surveillance report for June 2014. PMID:25256728

  13. Idiopathic CD4 Lymphocytopenia

    PubMed Central

    Régent, Alexis; Autran, Brigitte; Carcelain, Guislaine; Cheynier, Rémi; Terrier, Benjamin; Charmeteau-De Muylder, Bénédicte; Krivitzky, Alain; Oksenhendler, Eric; Costedoat-Chalumeau, Nathalie; Hubert, Pascale; Lortholary, Olivier; Dupin, Nicolas; Debré, Patrice; Guillevin, Loïc; Mouthon, Luc

    2014-01-01

    Abstract Idiopathic CD4 T lymphocytopenia (ICL) is a rare and severe condition with limited available data. We conducted a French multicenter study to analyze the clinical and immunologic characteristics of a cohort of patients with ICL according to the Centers for Disease Control criteria. We recruited 40 patients (24 female) of mean age 44.2 ± 12.2 (19–70) years. Patients underwent T-lymphocyte phenotyping and lymphoproliferation assay at diagnosis, and experiments related to thymic function and interferon (IFN)-γ release by natural killer (NK) cell were performed. Mean follow-up was 6.9 ± 6.7 (0.14–24.3) years. Infectious, autoimmune, and neoplastic events were recorded, as were outcomes of interleukin 2 therapy. In all, 25 patients had opportunistic infections (12 with human papillomavirus infection), 14 had autoimmune symptoms, 5 had malignancies, and 8 had mild or no symptoms. At the time of diagnosis, the mean cell counts were as follows: mean CD4 cell count: 127/mm3 (range, 4–294); mean CD8: 236/mm3 (range, 1–1293); mean CD19: 113/mm3 (range, 3–547); and mean NK cell count: 122/mm3 (range, 5–416). Most patients had deficiency in CD8, CD19, and/or NK cells. Cytotoxic function of NK cells was normal, and patients with infections had a significantly lower NK cell count than those without (p = 0.01). Patients with autoimmune manifestations had increased CD8 T-cell count. Proliferation of thymic precursors, as assessed by T-cell rearrangement excision circles, was increased. Six patients died (15%). CD4 T-cell count <150/mm3 and NK cell count <100/mm3 were predictors of death. In conclusion, ICL is a heterogeneous disorder often associated with deficiencies in CD8, CD19, and/or NK cells. Long-term prognosis may be related to initial CD4 and NK cell deficiency. PMID:24646462

  14. A Compendium for Mycoplasma pneumoniae.

    PubMed

    Parrott, Gretchen L; Kinjo, Takeshi; Fujita, Jiro

    2016-01-01

    Historically, atypical pneumonia was a term used to describe an unusual presentation of pneumonia. Currently, it is used to describe the multitude of symptoms juxtaposing the classic symptoms found in cases of pneumococcal pneumonia. Specifically, atypical pneumonia is a syndrome resulting from a relatively common group of pathogens including Chlamydophila sp., and Mycoplasma pneumoniae. The incidence of M. pneumoniae pneumonia in adults is less than the burden experienced by children. Transmission rates among families indicate children may act as a reservoir and maintain contagiousness over a long period of time ranging from months to years. In adults, M. pneumoniae typically produces a mild, "walking" pneumonia and is considered to be one of the causes of persistent cough in patients. M. pneumoniae has also been shown to trigger the exacerbation of other lung diseases. It has been repeatedly detected in patients with bronchitis, asthma, chronic obstructive pulmonary disorder, and cystic fibrosis. Recent advances in technology allow for the rapid diagnosis of M. pneumoniae through the use of polymerase chain reaction or rapid antigen tests. With this, more effort has been afforded to identify the causative etiologic agent in all cases of pneumonia. However, previous practices, including the overprescribing of macrolide treatment in China and Japan, have created increased incidence of macrolide-resistant M. pneumoniae. Reports from these countries indicate that >85% of M. pneumoniae pneumonia pediatric cases are macrolide-resistant. Despite its extensively studied past, the smallest bacterial species still inspires some of the largest questions. The developments in microbiology, diagnostic features and techniques, epidemiology, treatment and vaccines, and upper respiratory conditions associated with M. pneumoniae in adult populations are included within this review. PMID:27148202

  15. A Compendium for Mycoplasma pneumoniae

    PubMed Central

    Parrott, Gretchen L.; Kinjo, Takeshi; Fujita, Jiro

    2016-01-01

    Historically, atypical pneumonia was a term used to describe an unusual presentation of pneumonia. Currently, it is used to describe the multitude of symptoms juxtaposing the classic symptoms found in cases of pneumococcal pneumonia. Specifically, atypical pneumonia is a syndrome resulting from a relatively common group of pathogens including Chlamydophila sp., and Mycoplasma pneumoniae. The incidence of M. pneumoniae pneumonia in adults is less than the burden experienced by children. Transmission rates among families indicate children may act as a reservoir and maintain contagiousness over a long period of time ranging from months to years. In adults, M. pneumoniae typically produces a mild, “walking” pneumonia and is considered to be one of the causes of persistent cough in patients. M. pneumoniae has also been shown to trigger the exacerbation of other lung diseases. It has been repeatedly detected in patients with bronchitis, asthma, chronic obstructive pulmonary disorder, and cystic fibrosis. Recent advances in technology allow for the rapid diagnosis of M. pneumoniae through the use of polymerase chain reaction or rapid antigen tests. With this, more effort has been afforded to identify the causative etiologic agent in all cases of pneumonia. However, previous practices, including the overprescribing of macrolide treatment in China and Japan, have created increased incidence of macrolide-resistant M. pneumoniae. Reports from these countries indicate that >85% of M. pneumoniae pneumonia pediatric cases are macrolide-resistant. Despite its extensively studied past, the smallest bacterial species still inspires some of the largest questions. The developments in microbiology, diagnostic features and techniques, epidemiology, treatment and vaccines, and upper respiratory conditions associated with M. pneumoniae in adult populations are included within this review. PMID:27148202

  16. Pneumocystis carinii pneumonia in a Yorkshire terrier dog.

    PubMed

    Cabañes, F J; Roura, X; Majó, N; Bragulat, M R; Domingo, M

    2000-12-01

    A 14-month-old male Yorkshire terrier was presented to the Autonomous University of Barcelona Veterinary Teaching Hospital because of a history of chronic non-productive cough and acute dyspnea. A follow-up radiograph revealed a diffuse, bilaterally interstitial-alveolar lung disease with presence of air bronchograms. The dog died 5 h after admission with severe dyspnea. Histological sections of the necropsy specimens revealed the presence of characteristic Pneumocystis carinii cysts within alveolar spaces. A diagnosis of P. carinii pneumonia (PCP) was made on the basis of these results. To our knowledge, PCP has not been described in a Yorkshire terrier dog.

  17. Chlamydia pneumoniae (TWAR).

    PubMed Central

    Kuo, C C; Jackson, L A; Campbell, L A; Grayston, J T

    1995-01-01

    Chlamydia pneumoniae (TWAR) is a recently recognized third species of the genus Chlamydia that causes acute respiratory disease. It is distinct from the other two chlamydial species that infect humans, C. trachomatis and C. psittaci, in elementary body morphology and shares less than 10% of the DNA homology with those species. The organism has a global distribution, with infection most common among children between the ages of 5 and 14 years. In children, TWAR infection is usually mild or asymptomatic, but it may be more severe in adults. Pneumonia and bronchitis are the most common clinical manifestations of infection, and TWAR is responsible for approximately 10% of cases of pneumonia and 5% of cases of bronchitis in the United States. The microimmunofluorescence serologic assay is specific for TWAR and can distinguish between recent and past infections. The organism can be isolated in cell culture; however, PCR techniques have recently facilitated its detection in tissues and clinical specimens. PMID:8665464

  18. Exogenous lipoid pneumonia.

    PubMed

    Cornacchia, D J; Snyder, C H; Dupont, D C; Yaron, N S

    1989-05-01

    Although a rare form of nonresolving pulmonary infiltrate, exogenous lipoid pneumonia is a great mimicker. It often is mistaken for bacterial pneumonia or cancer. Many cases have been diagnosed only by open lung biopsy or other invasive procedures. Depending on the type of lipid ingested and the degree of inflammation that occurs, damage to the lung can be little to none or can fulminate to necrosis and hemorrhage. Symptoms may range from none to respiratory failure. In the case presented, the patient was ingesting Vaseline Intensive Care Lotion and baby oil as laxatives. This information was elicited only after diagnosis was made by open lung biopsy.

  19. Interstitial lung disease in patients with rheumatoid arthritis: spontaneous and drug induced.

    PubMed

    Hallowell, Robert W; Horton, Maureen R

    2014-03-01

    Rheumatoid arthritis (RA) is an inflammatory autoimmune disease characterized by the destruction of articular joint structures. RA is a systemic condition that often affects multiple organs, including the heart, lungs, and kidneys. Pulmonary complications of RA are relatively common and include pleural effusion, rheumatoid nodules, bronchiectasis, obliterative bronchiolitis, and opportunistic infections. Interstitial lung disease (ILD) is a common occurrence in patients with RA, and can range in severity from an asymptomatic incidental finding to a rapidly progressing life-threatening event. Usual interstitial pneumonia and non-specific interstitial pneumonia are the two most common patterns, though others have been reported. Various disease-modifying anti-rheumatic drugs-in particular, methotrexate and the tumor necrosis factor-alpha inhibitors-have been associated with RA-ILD in numerous case reports and case series, though it is often difficult to distinguish association from causality. Treatment for RA-ILD typically involves the use of high-dose corticosteroids, often in conjunction with alternative immunosuppressant agents such as azathioprine or mycophenolate mofetil, and outcomes vary widely depending on the initial pattern of lung disease. Additional research into the mechanisms driving RA-ILD is needed to guide future therapy.

  20. The role of viruses, inflammation and myocardial macrophages in the development of idiopathic arrhythmia

    NASA Astrophysics Data System (ADS)

    Rebenkova, M.; Gombozhapova, A.; Shurupov, V.; Rogovskaya, Yu.; Botalov, R.; Ryabov, V.; Popov, S.; Karpov, R.

    2015-11-01

    We studied viral antigens, inflammation, and macrophages in the endomyocardial biopsies of patients with idiopathic arrhythmias. Immunohistological study was performed to identify the antigens of cardiotropic viruses and the types of lymphocytes and macrophages. We observed the presence of viral antigens in the myocardium of patients with and without histological criteria of myocarditis. Heart failure and ventricular arrhythmias were associated with small focal infiltration of the myocardium with macrophages. The presence of viral antigens in the myocardium was associated with fewer number of myocardial M2 macrophages. Severity of myocardial interstitial fibrosis correlated with small-focal infiltration of M2 macrophages.

  1. Tocilizumab for refractory organising pneumonia associated with Sjögren's disease.

    PubMed

    Justet, Aurelien; Ottaviani, Sebastien; Dieudé, Philippe; Taillé, Camille

    2015-05-14

    Lung involvement in primary Sjögren syndrome occurs in approximately 10-20% of patients. Tocilizumab, an anti-interleukin-6 receptor antibody, has demonstrated efficacy and safety in small series of systemic sclerosis, and systemic lupus erythematosus, but its effect on interstitial lung manifestations of connective tissue diseases is not well known. We report the use of tocilizumab in a refractory organising pneumonia associated with Sjögren's disease. Our observation suggests that tocilizumab could be an alternative therapeutic in refractory organising pneumonia.

  2. Vaccinating welders against pneumonia

    PubMed Central

    Palmer, Keith T; Cosgrove, Martin P

    2013-01-01

    Background In 2011 the Department of Health in England recommended that welders should each receive a single dose of the 23-valent pneumococcal vaccine (PPV23). This review assesses the evidence behind the advice and its practical implications. Method The review was informed by a systematic search in Medline, which related pneumonia to welding and/or exposure to metal fume, and was supplemented using the personal libraries of the authors. Findings There is consistent evidence that welders die more often of pneumonia, especially lobar pneumonia, are hospitalised more often with lobar and pneumococcal pneumonia, and more often develop invasive pneumococcal disease (IPD). It is estimated that one case of IPD may be prevented over a 10-year period by vaccinating 588 welders against pneumococcal infection. Conclusions A good case exists that employers should offer PPV23 vaccination to welders and other employees exposed to metal fume. Additionally, reasonable measures must be taken to minimise exposure to welding fume and welders should be encouraged not to smoke. PMID:22764269

  3. [Pneumonia in wounded].

    PubMed

    Ovchinnikov, Iu V; Kharitonov, M A; Sadykov, R R; Shelukhin, V A; Gaĭduk, S V; Bogomolov, A B; Ivanov, V V; Dobrovol'skaia, L M

    2015-02-01

    Pneumonia is one of the common complications of wounds of any localization. Therapists are involved into the treatment of lung lesions in wounded in the ICU, in the surgical and if the patient arrives "on follow-up care,"--in the medical ward. The article analyzes the main statistical indicators reflecting the prevalence and clinical and pathogenetic characteristics of lung pathology in wounded during the Great Patriotic War, during the fighting Soviet troops in the Republic of Afghanistan, the 1st and 2nd Chechen campaign. Pneumonia as a manifestation of traumatic disease can occur in two ways. Primary pneumonia is in close connection with the pathogenetic traumatic injury. Secondary lung lesions complicate the injury at a later date and are due to the introduction of a nosocomial infection process flora. We describe the clinical picture of pneumonia in the affected, the basic pathogenesis, principles of therapy. Successful treatment of lung pathology in wounded depends on the performance of a complex of activities involving a wide range of doctors of various specialties. PMID:25920173

  4. Chlamydia pneumoniae and cardiovascular disease.

    PubMed Central

    Campbell, L. A.; Kuo, C. C.; Grayston, J. T.

    1998-01-01

    Chlamydia pneumoniae is a ubiquitous pathogen that causes acute respiratory disease. The spectrum of C. pneumoniae infection has been extended to atherosclerosis and its clinical manifestations. Seroepidemiologic studies have associated C. pneumoniae antibody with coronary artery disease, myocardial infarction, carotid artery disease, and cerebrovascular disease. The association of C. pneumoniae with atherosclerosis is corroborated by the presence of the organism in atherosclerotic lesions throughout the arterial tree and the near absence of the organism in healthy arterial tissue. C. pneumoniae has also been isolated from coronary and carotid atheromatous plaques. To determine whether chronic infection plays a role in initiation or progression of disease, intervention studies in humans have been initiated, and animal models of C. pneumoniae infection have been developed. This review summarizes the evidence for the association and potential role of C. pneumoniae in cardiovascular disease. PMID:9866733

  5. Spectrum of fibrosing diffuse parenchymal lung disease.

    PubMed

    Morgenthau, Adam S; Padilla, Maria L

    2009-02-01

    The interstitial lung diseases are a heterogeneous group of disorders characterized by inflammation and/or fibrosis of the pulmonary interstitium. In 2002, the American Thoracic Society and the European Respiratory Society revised the classification of interstitial lung diseases and introduced the term diffuse parenchymal lung disease. The idiopathic interstitial pneumonias are a subtype of diffuse parenchymal lung disease. The idiopathic interstitial pneumonias are subdivided into usual interstitial pneumonia (with its clinical counterpart idiopathic interstitial pneumonia), nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, desquamative interstitial pneumonia, respiratory bronchiolitis interstitial lung disease, and lymphocytic pneumonia. Sarcoidosis and hypersensitivity pneumonitis are the 2 most common granulomatous diffuse parenchymal lung diseases. Rheumatoid arthritis, systemic sclerosis, and dermatomyositis/polymyositis (causing antisynthetase syndrome) are diffuse parenchymal lung diseases of known association because these conditions are associated with connective tissue disease. Hermansky-Pudlak syndrome is a rare genetic diffuse parenchymal lung disease characterized by the clinical triad of pulmonary disease, oculocutaneous albinism, and bleeding diathesis. This review provides an overview of the chronic fibrosing diffuse parenchymal lung diseases. Its primary objective is to illuminate the clinical challenges encountered by clinicians who manage the diffuse parenchymal lung diseases regularly and to offer potential solutions to those challenges. Treatment for the diffuse parenchymal lung diseases is limited, and for many patients with end-stage disease, lung transplantation remains the best option. Although much has been learned about the diffuse parenchymal lung diseases during the past decade, research in these diseases is urgently needed. PMID:19170214

  6. Klebsiella pneumoniae Bloodstream Infection

    PubMed Central

    Girometti, Nicolò; Lewis, Russell E.; Giannella, Maddalena; Ambretti, Simone; Bartoletti, Michele; Tedeschi, Sara; Tumietto, Fabio; Cristini, Francesco; Trapani, Filippo; Gaibani, Paolo; Viale, Pierluigi

    2014-01-01

    Abstract Multidrug resistance associated with extended-spectrum beta-lactamase (ESBL) and Klebsiella pneumoniae carbapenemase (KPC) among K. pneumoniae is endemic in southern Europe. We retrospectively analyzed the impact of resistance on the appropriateness of empirical therapy and treatment outcomes of K. pneumoniae bloodstream infections (BSIs) during a 2-year period at a 1420-bed tertiary-care teaching hospital in northern Italy. We identified 217 unique patient BSIs, including 92 (42%) KPC-positive, 49 (23%) ESBL-positive, and 1 (0.5%) metallo-beta-lactamase-positive isolates. Adequate empirical therapy was administered in 74% of infections caused by non-ESBL non-KPC strains, versus 33% of ESBL and 23% of KPC cases (p < 0.0001). To clarify the impact of resistance on BSI treatment outcomes, we compared several different models comprised of non-antibiotic treatment-related factors predictive of patients’ 30-day survival status. Acute Physiology and Chronic Health Evaluation (APACHE) II score determined at the time of positive blood culture was superior to other investigated models, correctly predicting survival status in 83% of the study cohort. In multivariate analysis accounting for APACHE II, receipt of inadequate empirical therapy was associated with nearly a twofold higher rate of death (adjusted hazard ratio 1.9, 95% confidence interval 1.1–3.4; p = 0.02). Multidrug-resistant K. pneumoniae accounted for two-thirds of all K. pneumoniae BSIs, high rates of inappropriate empirical therapy, and twofold higher rates of patient death irrespective of underlying illness. PMID:25398065

  7. Granulomatous interstitial nephritis and Crohn's disease.

    PubMed

    Timmermans, Sjoerd A M E G; Christiaans, Maarten H L; Abdul-Hamid, Myrurgia A; Stifft, Frank; Damoiseaux, Jan G M C; van Paassen, Pieter

    2016-08-01

    Granulomatous interstitial nephritis has been observed in <1% of native renal biopsies. Here, we describe two patients with granulomatous interstitial nephritis in relation to Crohn's disease. Circulating helper and cytotoxic T cells were highly activated, and both cell types predominated in the interstitial infiltrate, indicating a cellular autoimmune response. After immunosuppressive treatment, renal function either improved or stabilized in both patients. In conclusion, granulomatous interstitial nephritis is a genuine extraintestinal manifestation of Crohn's disease, the treatment of which should include immunosuppressive agents. PMID:27478596

  8. Case of interstitial lung disease with anti-EJ and anti-CCP antibodies preceding rheumatoid arthritis.

    PubMed

    Tomioka, Hiromi; Kaneko, Masahiro; Kogata, Yoshinori; Katsuyama, Eiji; Ishikawa, Seiko; Fujii, Takao

    2012-06-01

    Autoantibodies against aminoacyl-tRNA synthetases (ARSs) are highly specific for myositis and/or interstitial lung disease. We report a rare case of double positive antibodies (anti-EJ antibody, the least common among anti-aminoacyl-tRNA synthetase antibodies, and anti-cyclic citrullinated peptide antibody, reported to be specific for rheumatoid arthritis) in a patient who presented with interstitial lung disease and later developed rheumatoid arthritis. The patient did not have clinically apparent myositis over a period of careful follow-up of several years. The initial pulmonary pathologic findings showed a nonspecific interstitial pneumonia pattern, with the formation of lymphoid follicles, which should be recognized as the first manifestation of rheumatoid arthritis.

  9. Antisynthetase syndrome: An under-recognized cause of interstitial lung disease

    PubMed Central

    Maturu, Venkata Nagarjuna; Lakshman, Arjun; Bal, Amanjit; Dhir, Varun; Sharma, Aman; Garg, Mandeep; Saikia, Biman; Agarwal, Ritesh

    2016-01-01

    Background: Antisynthetase syndrome (AS) is an uncommon and under-recognised connective tissue disease characterized by the presence of antibodies to anti-aminoacyl t-RNA synthetase along with features of interstitial lung disease (ILD), myositis and arthritis. The aim of the current study is to describe our experience with management of AS. Materials and Methods: This was a 2-year (2013-2014) retrospective analysis of patients diagnosed with anti-Jo-1-related AS. The presence of anti-Jo-1 antibody was tested by the immunoblot assay. All patients underwent high-resolution computed tomography of the chest, transthoracic echocardiography and evaluation for inflammatory myositis. Transbronchial lung biopsies and muscle biopsies were obtained when clinically indicated. Results: Nine patients (mean age: 43.8 years) were diagnosed with anti-Jo-1-related AS. The median duration of symptoms before diagnosis of AS was 6 months. All patients were negative for antinuclear antibodies by indirect immunofluorescence. The prevalence of ILD, myositis and arthritis at presentation was 100%, 77.8% and 55.6%, respectively. The most common ILD pattern was non-specific interstitial pneumonia (n = 6) followed by organizing pneumonia (n = 2) and usual interstitial pneumonia (n = 1). ILD was the sole manifestation in two patients and was subclinical in two patients. Six patients had pleuropericardial effusions, three patients had pulmonary artery hypertension and two patients had venous thromboembolism. Eight of the nine patients improved after treatment with steroids and other immunosuppressants. Conclusion: Antisynthetase syndrome is an important and a treatable cause of ILD. Strong clinical suspicion is needed to achieve an early diagnosis. PMID:26933302

  10. [A case of respiratory distress syndrome complicated by the development of interstitial emphysema and pneumomediastinum].

    PubMed

    Zhidkov, K P; Bogatyr', M N; Chezhin, A N; Leĭman, V A; Smirnova, O R

    2000-01-01

    A 15-year-old patient has been admitted to the intensive care unit for severe respiratory distress syndrome that developed as a result of pneumonia. Interstitial lung edema was confirmed by computer-aided tomography. It was successfully treated by positive pressure ventilation (PPV). Although PEEP did not exceed 7 cm H2O, PPV was complicated by interstitial emphysema, pneumomediastinum, and bilateral pneumothorax as a result of barotrauma. Pulmonary artery pressure (PAP) and pulmonary capillary wedge pressure (PCWP) were monitored. High PCWP values were inconsistent with the diagnosis of acute respiratory distress syndrome. The authors suggest that high PCWP was caused by high intraalveolar pressure, pneumomediastinum, and venule constriction in the hypoxic sites of the lung.

  11. Interstitial lung diseases-can pathologists arrive at an etiology-based diagnosis? A critical update.

    PubMed

    Popper, Helmut H

    2013-01-01

    Interstitial lung diseases (ILD) encompass a group of diseases with a wide range of etiologies and a variety of tissue reactions within the lung. In many instances, a careful evaluation of the tissue reactions will result in a specific diagnosis or at least in a narrow range of differentials, which will assist the clinician to arrive at a definite diagnosis, when combining our interpretation with the clinical presentation of the patient and high-resolution computed tomography. In this review, we will exclude granulomatous pneumonias as well as vascular diseases (primary arterial pulmonary hypertension and vasculitis); however, pulmonary hypertension as a complication of interstitial processes will be mentioned. Few entities of pneumoconiosis presenting as an interstitial process will be included, whereas those with granulomatous reactions will be excluded. Drug reactions will be touched on within interstitial pneumonias, but will not be a major focus. In contrast to the present-day preferred descriptive pattern recognition, it is the author's strong belief that pathologists should always try to dig out the etiology from a tissue specimen and not being satisfied with just a pattern description. It is the difference of sorting tissue reactions into boxes by their main pattern, without recognizing minor or minute reactions, which sometimes will guide one to the correct etiology-oriented interpretation. In the author's personal perspective, tissue reactions can even be sorted by their timeliness, and therefore, ordered by the time of appearance, providing an insight into the pathogenesis and course of a disease. Also, underlying immune mechanisms will be discussed briefly as far as they are essential to understand the disease.

  12. Community-acquired pneumonia.

    PubMed

    Polverino, E; Torres Marti, A

    2011-02-01

    Despite the remarkable advances in antibiotic therapies, diagnostic tools, prevention campaigns and intensive care, community-acquired pneumonia (CAP) is still among the primary causes of death worldwide, and there have been no significant changes in mortality in the last decades. The clinical and economic burden of CAP makes it a major public health problem, particularly for children and the elderly. This issue provides a clinical overview of CAP, focusing on epidemiology, economic burden, diagnosis, risk stratification, treatment, clinical management, and prevention. Particular attention is given to some aspects related to the clinical management of CAP, such as the microbial etiology and the available tools to achieve it, the usefulness of new and old biomarkers, and antimicrobial and other non-antibiotic adjunctive therapies. Possible scenarios in which pneumonia does not respond to treatment are also analyzed to improve clinical outcomes of CAP. PMID:21242952

  13. Acinetobacter Pneumonia: A Review

    PubMed Central

    Hartzell, Joshua D.; Kim, Andrew S.; Kortepeter, Mark G.; Moran, Kimberly A.

    2007-01-01

    Acinetobacter species are becoming a major cause of nosocomial infections, including hospital-acquired and ventilator-associated pneumonia. Acinetobacter species have become increasingly resistant to antibiotics over the past several years and currently present a significant challenge in treating these infections. Physicians now rely on older agents, such as polymyxins (colistin), for treatment. This paper reviews the epidemiology, treatment, and prevention of this emerging pathogen. PMID:18092011

  14. Chlamydia pneumoniae and asthma

    PubMed Central

    Cook, P; Davies, P; Tunnicliffe, W; Ayres, J; Honeybourne, D; Wise, R

    1998-01-01

    BACKGROUND—This study was designed to test the association of Chlamydia pneumoniae infection with asthma in a multiracial population, after adjustments for several potential confounding variables.
METHODS—Antibodies to C pneumoniae were measured by microimmunofluorescence in 123 patients with acute asthma, 1518 control subjects admitted to the same hospital with various non-cardiovascular, non-pulmonary disorders, and 46 patients with severe chronic asthma, including some with "brittle" asthma. Acute infection or reinfection was defined by titres of IgG of ⩾512 or IgM ⩾8 or a fourfold rise in IgG, and previous infection by IgG 64-256 or IgA ⩾8. Logistic regression was used to control for likely confounders, including ethnic origin, age, sex, smoking habit, steroid medication, diabetes mellitus and social deprivation, on antibody levels.
RESULTS—Antibody titres consistent with acute C pneumoniae infection were found in 5.7% of patients with acute asthma and 5.7% of control patients, while 14.6% of patients with acute asthma and 12.7% of control patients had titres suggesting previous infection. These two groups did not differ significantly. However, titres suggesting previous infection were found in 34.8% of patients with severe chronic asthma: the difference between this group and the control group was statistically significant with an adjusted odds ratio of 3.99 (95% confidence interval 1.60 to 9.97).
CONCLUSIONS—These data raise important questions about the previously demonstrated association of C pneumoniae infection with asthma, and suggest that future studies of this association should give particular attention to the presence or absence of a history of severe chronic asthma.

 PMID:9741366

  15. Fungal diagnostics in pneumonia.

    PubMed

    Lease, Erika D; Alexander, Barbara D

    2011-12-01

    Fungal pneumonia is increasingly common, particularly in highly immunosuppressed patients, such as solid organ or hematopoietic stem cell transplant recipients, and the diagnosis is evolving. Although standard techniques such as microscopy and culture remain the mainstays of diagnosis, relatively recent advances in serological and molecular testing are important additions to the field. This article reviews the laboratory tools used to diagnose fungal respiratory disease. PMID:22167394

  16. Fungal Diagnostics in Pneumonia

    PubMed Central

    Lease, Erika D.; Alexander, Barbara D.

    2014-01-01

    Fungal pneumonia is increasingly common, particularly in highly immunosuppressed patients, such as solid organ or hematopoietic stem cell transplant recipients, and the diagnosis is evolving. While standard techniques such as microscopy and culture remain the mainstay of diagnosis, relatively recent advances in serologic and molecular testing are important additions to the field. This chapter will review the laboratory tools used to diagnose fungal respiratory disease. PMID:22167394

  17. [Travel-associated pneumonias].

    PubMed

    Geerdes-Fenge, H F

    2014-10-01

    Respiratory infections are responsible for up to 11% of febrile infections in travellers or immigrants from tropical and subtropical regions. The main pathogens are the same as in temperate climate zones: Streptococcus pneumoniae, Haemophilus influenzae, Mycoplasma pneumoniae, Chlamydophila pneumoniae, influenza viruses, Legionella pneumophila. However, some pulmonary diseases can be attributed to bacterial, parasitic, viral or fungal pathogens that are endemic in tropical and subtropical regions. The most commonly imported infections are malaria, dengue, and tuberculosis. Pulmonary symptoms and eosinophilia in returning travellers and migrants may be caused by several parasitic infections such as Katayama syndrome, Loeffler syndrome, tropical pulmonary eosinophilia, amebiasis, paragonimiasis, echinococcosis, and toxocariasis. In Asia, Tsutsugamushi fever is transmitted by chiggers, spotted fever rickettsiae are transmitted by ticks. Transmission of zoonotic diseases occurs mainly via contact with infected animals or their excretions, human-to-human transmission is generally rare: MERS-CoA (dromedary camels), pulmonary hantavirus infection (rodents), tularemia (rabbits and hares), leptospirosis (rats), Q-fever (sheep and goats), very rarely anthrax (hides of ruminants) and pest (infected rats and wildlife). Inhalation of contaminated dust can cause infections with dimorphic fungi: histoplasmosis (bat guano) and coccidioidomycosis in America and parts of Africa, blastomycosis in America. Some infections can cause symptoms years after a stay in tropical or subtropical regions (melioidosis, tuberculosis, histoplasmosis, schistosomiasis-associated pulmonary hypertension). Noninfectious respiratory diseases caused by inhalation of high amounts of air pollution or toxic dusts may also be considered. PMID:25290923

  18. Hypervirulent (hypermucoviscous) Klebsiella pneumoniae

    PubMed Central

    Shon, Alyssa S.; Bajwa, Rajinder P.S.; Russo, Thomas A.

    2013-01-01

    A new hypervirulent (hypermucoviscous) variant of Klebsiella pneumoniae has emerged. First described in the Asian Pacific Rim, it now increasingly recognized in Western countries. Defining clinical features are the ability to cause serious, life-threatening community-acquired infection in younger healthy hosts, including liver abscess, pneumonia, meningitis and endophthalmitis and the ability to metastatically spread, an unusual feature for enteric Gram-negative bacilli in the non-immunocompromised. Despite infecting a healthier population, significant morbidity and mortality occurs. Although epidemiologic features are still being defined, colonization, particularly intestinal colonization, appears to be a critical step leading to infection. However the route of entry remains unclear. The majority of cases described to date are in Asians, raising the issue of a genetic predisposition vs. geospecific strain acquisition. The traits that enhance its virulence when compared with “classical” K. pneumoniae are the ability to more efficiently acquire iron and perhaps an increase in capsule production, which confers the hypermucoviscous phenotype. An objective diagnostic test suitable for routine use in the clinical microbiology laboratory is needed. If/when these strains become increasingly resistant to antimicrobials, we will be faced with a frightening clinical scenario. PMID:23302790

  19. Motility of Mycoplasma pneumoniae.

    PubMed Central

    Radestock, U; Bredt, W

    1977-01-01

    Cell of Mycoplasma pneumoniae FH gliding on a glass surface in liquid medium were examined by microscopic observation and quantitatively by microcinematography (30 frames per min). Comparisons were made only within the individual experiments. The cells moved in an irregular pattern with numerous narrow bends and circles. They never changed their leading end. The average speed (without pauses) was relatively constant between o.2 and 0.5 mum/s. The maximum speed was about 1.5 to 2.0 mum/s. The movements were interrupted by resting periods of different lengths and frequency. Temperature, viscosity, pH, and the presence of yeast extract in the medium influenced the motility significantly; changes in glucose, calcium ions, and serum content were less effective. The movements were affected by iodoacetate, p-mercuribenzoate, and mitomycin C at inhibitory or subinhibitory concentrations. Sodium fluoride, sodium cyanide, dinitrophenol, chloramphenicol, puromycin, cholchicin, and cytochalasin B at minimal inhibitory concentrations did not affect motility. The movements were effectively inhibited by anti-M. pneumoniae antiserum. Studies with absorbed antiserum suggested that the surface components involved in motility are heat labile. The gliding of M. pneumoniae cells required an intact energy metabolism and the proteins involved seemed to have a low turnover. Images PMID:14925

  20. Prevalence and prognosis of unclassifiable interstitial lung disease.

    PubMed

    Ryerson, Christopher J; Urbania, Thomas H; Richeldi, Luca; Mooney, Joshua J; Lee, Joyce S; Jones, Kirk D; Elicker, Brett M; Koth, Laura L; King, Talmadge E; Wolters, Paul J; Collard, Harold R

    2013-09-01

    The aim of this study was to determine the prevalence, characteristics and outcomes of patients with unclassifiable interstitial lung disease (ILD) and to develop a simple method of predicting disease behaviour. Unclassifiable ILD patients were identified from an ongoing longitudinal cohort. Unclassifiable ILD was diagnosed after a multidisciplinary review did not secure a specific ILD diagnosis. Clinical characteristics and outcomes were compared with idiopathic pulmonary fibrosis (IPF) and non-IPF ILDs. Independent predictors of mortality were determined using Cox proportional-hazards analysis to identify subgroups with distinct disease behaviour. Unclassifiable ILD was diagnosed in 10% of the ILD cohort (132 out of 1370 patients). The most common reason for being unclassifiable was missing histopathological assessment due to a high risk of surgical lung biopsy. Demographic and physiological features of unclassifiable ILD were intermediate between IPF and non-IPF disease controls. Unclassifiable ILD had longer survival rates when compared to IPF on adjusted analysis (hazard ratio 0.62, p = 0.04) and similar survival compared to non-IPF ILDs (hazard ratio 1.54, p = 0.12). Independent predictors of survival in unclassifiable ILD included diffusion capacity of the lung for carbon monoxide (p = 0.001) and a radiological fibrosis score (p = 0.02). Unclassifiable ILD represents approximately 10% of ILD cases and has a heterogeneous clinical course, which can be predicted using clinical and radiological variables.

  1. Incidence and recognition of interstitial pulmonary fibrosis in developing countries.

    PubMed

    Jindal, S K; Gupta, D

    1997-09-01

    Interstitial pulmonary fibrosis in developing countries is now diagnosed with an increased frequency. Increased awareness and more frequent availability of computed tomography and fiberoptic bronchoendoscopy have helped in making the diagnosis more often. The spectrum of diseases causing pulmonary fibrosis is broadly similar to that seen in the West. Connective tissue disorders such as systemic sclerosis and rheumatoid arthritis and sarcoidosis are more common causes. Idiopathic fibrosis is seen in approximately half the patients. Pneumoconiosis such as silicosis are also important. Diagnosis is often established on the basis of clinical features and radiologic findings alone. Transbronchial lung biopsy is used as a frequent method to make histologic diagnosis. Some of the causes described from India are rather rare. One of the interesting examples included a patient in whom pulmonary fibrosis was related to his ascent to very high altitude. Extreme cold, solar radiation, and other factors complicating low atmospheric oxygen pressure were implicated as causative factors. Lung fibrosis, secondary to exposure to toxic gas (methyl isocyanate), is reported in survivors of the Bhopal gas leakage tragedy of 1984. Serial bronchoalveolar studies have show elevated fibronectin levels and the presence of macrophage-neutrophilic exudate in the lavage fluid.

  2. Hemoglobin variant (hemoglobin Aalborg) mimicking interstitial pulmonary disease.

    PubMed

    Panou, Vasiliki; Jensen, Peter-Diedrich Mathias; Pedersen, Jan Freddy; Thomsen, Lars Pilegaard; Weinreich, Ulla Møller

    2014-01-01

    Hemoglobin Aalborg is a moderately unstable hemoglobin variant with no affiliation to serious hematological abnormality or major clinical symptoms under normal circumstances. Our index person was a healthy woman of 58, not previously diagnosed with hemoglobinopathy Aalborg, who developed acute respiratory failure after a routine cholecystectomy. Initially she was suspected of idiopathic interstitial lung disease, yet a series of tests uncovered various abnormal physiological parameters and set the diagnosis of hemoglobinopathy Aalborg. This led us to examine a group of the index person's relatives known with hemoglobinopathy Aalborg in order to study whether the same physiological abnormalities would be reencountered. They were all subjected to spirometry and body plethysmography, six-minute walking test, pulse oximetry, and arterial blood gas samples before and after the walking test. The entire study population presented the same physiological anomalies: reduction in diffusion capacity, and abnormalities in P(a)O2 and p50 values; the latter could not be presented by the arterial blood gas analyzer; furthermore there was concordance between pulse oximetry and arterial blood gas samples regarding saturation. These data suggest that, based upon the above mentioned anomalies in physiological parameters, the diagnosis of hemoglobinopathy Aalborg should be considered.

  3. Hemoglobin Variant (Hemoglobin Aalborg) Mimicking Interstitial Pulmonary Disease

    PubMed Central

    Panou, Vasiliki; Jensen, Peter-Diedrich Mathias; Pedersen, Jan Freddy; Thomsen, Lars Pilegaard; Weinreich, Ulla Møller

    2014-01-01

    Hemoglobin Aalborg is a moderately unstable hemoglobin variant with no affiliation to serious hematological abnormality or major clinical symptoms under normal circumstances. Our index person was a healthy woman of 58, not previously diagnosed with hemoglobinopathy Aalborg, who developed acute respiratory failure after a routine cholecystectomy. Initially she was suspected of idiopathic interstitial lung disease, yet a series of tests uncovered various abnormal physiological parameters and set the diagnosis of hemoglobinopathy Aalborg. This led us to examine a group of the index person's relatives known with hemoglobinopathy Aalborg in order to study whether the same physiological abnormalities would be reencountered. They were all subjected to spirometry and body plethysmography, six-minute walking test, pulse oximetry, and arterial blood gas samples before and after the walking test. The entire study population presented the same physiological anomalies: reduction in diffusion capacity, and abnormalities in PaO2 and p50 values; the latter could not be presented by the arterial blood gas analyzer; furthermore there was concordance between pulse oximetry and arterial blood gas samples regarding saturation. These data suggest that, based upon the above mentioned anomalies in physiological parameters, the diagnosis of hemoglobinopathy Aalborg should be considered. PMID:25400945

  4. Acute interstitial nephritis – a reappraisal and update

    PubMed Central

    Raghavan, Rajeev; Eknoyan, Garabed

    2014-01-01

    Acute interstitial nephritis (AIN) is an under recognized and under diagnosed cause of acute kidney injury (AKI). It is estimated to account for 15 – 20% of cases of AKI; it is the reported diagnosis in 2.8% of all kidney biopsies, and 13.5% of biopsies done specifically for acute renal failure. Considerable evidence implicates antigen initiated cell-mediated injury in the pathogenesis of AIN. Drugs account for 70% of all cases, with over 150 different agents incriminated. The remaining cases are due to infections, autoimmune diseases, and rarely idiopathic. The central component of renal injury in AIN is altered tubular function, which usually precedes decrements in filtration rate. The key to early diagnosis is vigilance for the presence of tubular dysfunction in non-oliguric individuals, especially in patients with modest but gradual increments in creatinine level. The utility of urinary biomarkers to diagnose AIN in its early nascent and potentially reversible stage remains to be determined. Prompt recognition, elimination of the offending source of antigen, and use of a limited course of steroid therapy where indicated, will result in complete resolution in ~ 65% of cases, partial resolution in up to 20%, and irreversible damage in the rest. PMID:25079860

  5. Chitotriosidase in the Pathogenesis of Inflammation, Interstitial Lung Diseases and COPD.

    PubMed

    Cho, Soo Jung; Weiden, Michael D; Lee, Chun Geun

    2015-01-01

    As a member of 18 glycosyl hydrolase (GH) family, chitotriosidase (Chitinase 1, CHIT1) is a true chitinase mainly expressed in the differentiated and polarized macrophages. CHIT1 is an innate immune mediator that digests the cell walls of chitin-containing eukaryotic pathogens, such as fungi. However, CHIT1 is dysregulated in granulomatous and fibrotic interstitial lung diseases characterized by inflammation and tissue remodeling. These include tuberclosis, sarcoidosis, idiopathic pulmonary fibrosis, scleroderma-associated interstitial lung diseases (SSc-ILD), and chronic obstructive lung diseases (COPD). CHIT1 serum concentration correlates with the progression or the severity of these diseases, suggesting a potential use of CHIT1 as a biomarker or a therapeutic target. Recent studies with genetically modified mice demonstrate that CHIT1 enhances TGF-β1 receptor expression and signaling, suggesting a role in initiating or amplifying the response to organ injury and repair. This additional CHIT1 activity is independent of its enzymatic activity. These studies suggest that CHIT1 serves a bridging function; it is both an innate immune mediator and a regulator of tissue remodeling. This review will focus on recent data linking CHIT1 to the pathogenesis of inflammation, interstitial lung disease, and COPD. PMID:25553258

  6. Chitotriosidase in the Pathogenesis of Inflammation, Interstitial Lung Diseases and COPD.

    PubMed

    Cho, Soo Jung; Weiden, Michael D; Lee, Chun Geun

    2015-01-01

    As a member of 18 glycosyl hydrolase (GH) family, chitotriosidase (Chitinase 1, CHIT1) is a true chitinase mainly expressed in the differentiated and polarized macrophages. CHIT1 is an innate immune mediator that digests the cell walls of chitin-containing eukaryotic pathogens, such as fungi. However, CHIT1 is dysregulated in granulomatous and fibrotic interstitial lung diseases characterized by inflammation and tissue remodeling. These include tuberclosis, sarcoidosis, idiopathic pulmonary fibrosis, scleroderma-associated interstitial lung diseases (SSc-ILD), and chronic obstructive lung diseases (COPD). CHIT1 serum concentration correlates with the progression or the severity of these diseases, suggesting a potential use of CHIT1 as a biomarker or a therapeutic target. Recent studies with genetically modified mice demonstrate that CHIT1 enhances TGF-β1 receptor expression and signaling, suggesting a role in initiating or amplifying the response to organ injury and repair. This additional CHIT1 activity is independent of its enzymatic activity. These studies suggest that CHIT1 serves a bridging function; it is both an innate immune mediator and a regulator of tissue remodeling. This review will focus on recent data linking CHIT1 to the pathogenesis of inflammation, interstitial lung disease, and COPD.

  7. Chitotriosidase in the Pathogenesis of Inflammation, Interstitial Lung Diseases and COPD

    PubMed Central

    Cho, Soo Jung; Weiden, Michael D.

    2015-01-01

    As a member of 18 glycosyl hydrolase (GH) family, chitotriosidase (Chitinase 1, CHIT1) is a true chitinase mainly expressed in the differentiated and polarized macrophages. CHIT1 is an innate immune mediator that digests the cell walls of chitin-containing eukaryotic pathogens, such as fungi. However, CHIT1 is dysregulated in granulomatous and fibrotic interstitial lung diseases characterized by inflammation and tissue remodeling. These include tuberclosis, sarcoidosis, idiopathic pulmonary fibrosis, scleroderma-associated interstitial lung diseases (SSc-ILD), and chronic obstructive lung diseases (COPD). CHIT1 serum concentration correlates with the progression or the severity of these diseases, suggesting a potential use of CHIT1 as a biomarker or a therapeutic target. Recent studies with genetically modified mice demonstrate that CHIT1 enhances TGF-β1 receptor expression and signaling, suggesting a role in initiating or amplifying the response to organ injury and repair. This additional CHIT1 activity is independent of its enzymatic activity. These studies suggest that CHIT1 serves a bridging function; it is both an innate immune mediator and a regulator of tissue remodeling. This review will focus on recent data linking CHIT1 to the pathogenesis of inflammation, interstitial lung disease, and COPD. PMID:25553258

  8. Burden of Severe Pneumonia, Pneumococcal Pneumonia and Pneumonia Deaths in Indian States: Modelling Based Estimates

    PubMed Central

    Farooqui, Habib; Jit, Mark; Heymann, David L.; Zodpey, Sanjay

    2015-01-01

    The burden of severe pneumonia in terms of morbidity and mortality is unknown in India especially at sub-national level. In this context, we aimed to estimate the number of severe pneumonia episodes, pneumococcal pneumonia episodes and pneumonia deaths in children younger than 5 years in 2010. We adapted and parameterized a mathematical model based on the epidemiological concept of potential impact fraction developed CHERG for this analysis. The key parameters that determine the distribution of severe pneumonia episode across Indian states were state-specific under-5 population, state-specific prevalence of selected definite pneumonia risk factors and meta-estimates of relative risks for each of these risk factors. We applied the incidence estimates and attributable fraction of risk factors to population estimates for 2010 of each Indian state. We then estimated the number of pneumococcal pneumonia cases by applying the vaccine probe methodology to an existing trial. We estimated mortality due to severe pneumonia and pneumococcal pneumonia by combining incidence estimates with case fatality ratios from multi-centric hospital-based studies. Our results suggest that in 2010, 3.6 million (3.3–3.9 million) episodes of severe pneumonia and 0.35 million (0.31–0.40 million) all cause pneumonia deaths occurred in children younger than 5 years in India. The states that merit special mention include Uttar Pradesh where 18.1% children reside but contribute 24% of pneumonia cases and 26% pneumonia deaths, Bihar (11.3% children, 16% cases, 22% deaths) Madhya Pradesh (6.6% children, 9% cases, 12% deaths), and Rajasthan (6.6% children, 8% cases, 11% deaths). Further, we estimated that 0.56 million (0.49–0.64 million) severe episodes of pneumococcal pneumonia and 105 thousand (92–119 thousand) pneumococcal deaths occurred in India. The top contributors to India’s pneumococcal pneumonia burden were Uttar Pradesh, Bihar, Madhya Pradesh and Rajasthan in that order. Our

  9. Burden of Severe Pneumonia, Pneumococcal Pneumonia and Pneumonia Deaths in Indian States: Modelling Based Estimates.

    PubMed

    Farooqui, Habib; Jit, Mark; Heymann, David L; Zodpey, Sanjay

    2015-01-01

    The burden of severe pneumonia in terms of morbidity and mortality is unknown in India especially at sub-national level. In this context, we aimed to estimate the number of severe pneumonia episodes, pneumococcal pneumonia episodes and pneumonia deaths in children younger than 5 years in 2010. We adapted and parameterized a mathematical model based on the epidemiological concept of potential impact fraction developed CHERG for this analysis. The key parameters that determine the distribution of severe pneumonia episode across Indian states were state-specific under-5 population, state-specific prevalence of selected definite pneumonia risk factors and meta-estimates of relative risks for each of these risk factors. We applied the incidence estimates and attributable fraction of risk factors to population estimates for 2010 of each Indian state. We then estimated the number of pneumococcal pneumonia cases by applying the vaccine probe methodology to an existing trial. We estimated mortality due to severe pneumonia and pneumococcal pneumonia by combining incidence estimates with case fatality ratios from multi-centric hospital-based studies. Our results suggest that in 2010, 3.6 million (3.3-3.9 million) episodes of severe pneumonia and 0.35 million (0.31-0.40 million) all cause pneumonia deaths occurred in children younger than 5 years in India. The states that merit special mention include Uttar Pradesh where 18.1% children reside but contribute 24% of pneumonia cases and 26% pneumonia deaths, Bihar (11.3% children, 16% cases, 22% deaths) Madhya Pradesh (6.6% children, 9% cases, 12% deaths), and Rajasthan (6.6% children, 8% cases, 11% deaths). Further, we estimated that 0.56 million (0.49-0.64 million) severe episodes of pneumococcal pneumonia and 105 thousand (92-119 thousand) pneumococcal deaths occurred in India. The top contributors to India's pneumococcal pneumonia burden were Uttar Pradesh, Bihar, Madhya Pradesh and Rajasthan in that order. Our results

  10. Lung and pleural fibrosis in asbestos-exposed workers: a risk factor for pneumonia mortality.

    PubMed

    Vehmas, T; Pallasaho, P; Oksa, P

    2012-11-01

    Lungs exposed to occupational dust may be especially vulnerable to fatal infections. We followed up asbestos-exposed workers (n=590) originally screened for lung cancer with computed tomography and scored for pleuropulmonary fibrosis. We checked these workers' influenza and pneumonia mortality data (ICD-10 codes J10-J18) in the national register. In total, 191 deaths, including 43 deaths from infectious pneumonia, occurred in 6158 person-years of follow-up (mean follow-up time 10·44 years). 'Some interstitial fibrosis' [hazard ratio (HR) 2·26, 95% confidence interval (CI) 0·98-5·19, P=0·06] and 'definite interstitial fibrosis' (HR 3·70, 95% CI 1·22-11·23, P=0·02) were associated with an increased risk of death from pneumonia compared to no fibrosis. Asbestosis patients, i.e. those with both asbestos exposure and lung fibrosis, therefore appear to be particularly at risk for death from pneumonia. These patients should be vaccinated against influenza and Pneumococcus. PMID:22214835

  11. [Oral therapy of interstitial cystitis].

    PubMed

    Sievert, K D; Edenfeld, K D; Oberpenning, F; Piechota, H J

    2000-11-01

    Up to now there is no specific treatment targeting the ultimate cause of interstitial cystitis (IC), since its pathogenesis and etiology are still unknown. Most studies focussing on oral medication have not been randomized, double-blinded or placebo-controlled. Numerous case reports and intent-to-treat trials are lacking a systematic approach and do not meet evidence-based medicine criteria. Consequently there is as yet no standard oral therapy available for the treatment of IC. However, only a few oral substances have shown a potential to improve symptoms such as frequency and pain. The best results were obtained from monotherapeutic use of pentosanpolysulfate, amitriptylin and hydroxycin. The true benefit of these substances alone should be compared to analgesics and anticholinergics in the course of controlled clinical trials.

  12. Interstitial cystitis--an update.

    PubMed

    Thompson, A C; Christmas, T J

    1996-12-01

    Interstitial cystitis is a rare chronic debilitating condition predominantly affecting middle-aged women. The diagnosis, made from the combination of symptoms, cystoscopic findings and bladder biopsies, is often delayed because GPs are often unaware of the condition. The precise aetiology remains obscure and it is clear that there is much research that needs to be undertaken to shed any light on the various current aetiological theories. There is a wide assortment of therapies already available and many more currently under trial. Until there is further progress in the pathogenesis of IC, it seems unlikely that non-operative treatment will consist of anything but palliation. The only potential cures currently available are cystectomy and urinary diversion or surgical replacement of the bladder by enterocystoplasty.

  13. Glucocorticoids in juvenile idiopathic arthritis.

    PubMed

    Malattia, Clara; Martini, Alberto

    2014-05-01

    Although the use of corticosteroids in juvenile idiopathic arthritis (JIA) is now much more limited owing to the availability of methotrexate and biological agents, there are clinical scenarios where it is still indicated. For example, corticosteroids may be indicated for intraarticular injections to prevent joint deformities, as a "bridge" drug to relieve symptoms in polyarticular disease while waiting for methotrexate and biologics to exert their full therapeutic effects, and in the treatment of chronic iridocyclitis, macrophage activation syndrome, and systemic JIA, although the advent of interleukin (IL)-1 and IL-6 blockers has greatly reduced the latter indication.

  14. Idiopathic internal mammary artery aneurysm

    PubMed Central

    Heyn, Jens; Zimmermann, Hanna; Klose, Alexander; Luchting, Benjamin; Hinske, Christian; Sadeghi-Azandaryani, Mojtaba

    2014-01-01

    Aneurysms of the internal mammary artery are extremely rare, and their presentation and treatment are variable. Since these aneurysms often tend to rupture and cause haemothorax and life-threatening conditions, the knowledge of secure treatment options is indispensable. We here report the case of an idiopathic internal mammary aneurysm in a 46-year-old man. Open surgical resection of the aneurysm was performed in this case without any complications. The postoperative course was uneventful and the patient was in a good physical condition without any vascular or neurological abnormalities during follow-up. PMID:25452261

  15. Chronic idiopathic pulmonary hilar fibrosis

    PubMed Central

    Yacoub, Magdi H.; Thompson, Vernon C.

    1971-01-01

    Idiopathic pulmonary hilar fibrosis is a condition related to mediastinal fibrosis, characterized by localization of the fibrosing process to one or both pulmonary hila. This results in pulmonary hypertension and bronchial narrowing. Three patients suffering from this disease, in whom the diagnosis has been confirmed by thoracotomy, are reported. The clinical and pathological features are described and previously reported cases are reviewed. The syndrome is classified into two types, according to whether the obstruction affects mainly the pulmonary artery or veins. The disease is a self-limiting one but may lead to organic changes in the lungs causing severe disability. Images PMID:5565782

  16. The clinical phenotypes of the juvenile idiopathic inflammatory myopathies.

    PubMed

    Shah, Mona; Mamyrova, Gulnara; Targoff, Ira N; Huber, Adam M; Malley, James D; Rice, Madeline Murguia; Miller, Frederick W; Rider, Lisa G

    2013-01-01

    The juvenile idiopathic inflammatory myopathies (JIIM) are systemic autoimmune diseases characterized by skeletal muscle weakness, characteristic rashes, and other systemic features. Although juvenile dermatomyositis (JDM), the most common form of JIIM, has been well studied, the other major clinical subgroups of JIIM, including juvenile polymyositis (JPM) and juvenile myositis overlapping with another autoimmune or connective tissue disease (JCTM), have not been well characterized, and their similarity to the adult clinical subgroups is unknown. We enrolled 436 patients with JIIM, including 354 classified as JDM, 33 as JPM, and 49 as JCTM, in a nationwide registry study. The aim of the study was to compare demographics; clinical features; laboratory measures, including myositis autoantibodies; and outcomes among these clinical subgroups, as well as with published data on adult patients with idiopathic inflammatory myopathies (IIM) enrolled in a separate natural history study. We used random forest classification and logistic regression modeling to compare clinical subgroups, following univariate analysis. JDM was characterized by typical rashes, including Gottron papules, heliotrope rash, malar rash, periungual capillary changes, and other photosensitive and vasculopathic skin rashes. JPM was characterized by more severe weakness, higher creatine kinase levels, falling episodes, and more frequent cardiac disease. JCTM had more frequent interstitial lung disease, Raynaud phenomenon, arthralgia, and malar rash. Differences in autoantibody frequency were also evident, with anti-p155/140, anti-MJ, and anti-Mi-2 seen more frequently in patients with JDM, anti-signal recognition particle and anti-Jo-1 in JPM, and anti-U1-RNP, PM-Scl, and other myositis-associated autoantibodies more commonly present in JCTM. Mortality was highest in patients with JCTM, whereas hospitalizations and wheelchair use were highest in JPM patients. Several demographic and clinical features

  17. The clinical phenotypes of the juvenile idiopathic inflammatory myopathies.

    PubMed

    Shah, Mona; Mamyrova, Gulnara; Targoff, Ira N; Huber, Adam M; Malley, James D; Rice, Madeline Murguia; Miller, Frederick W; Rider, Lisa G

    2013-01-01

    The juvenile idiopathic inflammatory myopathies (JIIM) are systemic autoimmune diseases characterized by skeletal muscle weakness, characteristic rashes, and other systemic features. Although juvenile dermatomyositis (JDM), the most common form of JIIM, has been well studied, the other major clinical subgroups of JIIM, including juvenile polymyositis (JPM) and juvenile myositis overlapping with another autoimmune or connective tissue disease (JCTM), have not been well characterized, and their similarity to the adult clinical subgroups is unknown. We enrolled 436 patients with JIIM, including 354 classified as JDM, 33 as JPM, and 49 as JCTM, in a nationwide registry study. The aim of the study was to compare demographics; clinical features; laboratory measures, including myositis autoantibodies; and outcomes among these clinical subgroups, as well as with published data on adult patients with idiopathic inflammatory myopathies (IIM) enrolled in a separate natural history study. We used random forest classification and logistic regression modeling to compare clinical subgroups, following univariate analysis. JDM was characterized by typical rashes, including Gottron papules, heliotrope rash, malar rash, periungual capillary changes, and other photosensitive and vasculopathic skin rashes. JPM was characterized by more severe weakness, higher creatine kinase levels, falling episodes, and more frequent cardiac disease. JCTM had more frequent interstitial lung disease, Raynaud phenomenon, arthralgia, and malar rash. Differences in autoantibody frequency were also evident, with anti-p155/140, anti-MJ, and anti-Mi-2 seen more frequently in patients with JDM, anti-signal recognition particle and anti-Jo-1 in JPM, and anti-U1-RNP, PM-Scl, and other myositis-associated autoantibodies more commonly present in JCTM. Mortality was highest in patients with JCTM, whereas hospitalizations and wheelchair use were highest in JPM patients. Several demographic and clinical features

  18. Klebsiella pneumoniae FimK Promotes Virulence in Murine Pneumonia.

    PubMed

    Rosen, David A; Hilliard, Julia K; Tiemann, Kristin M; Todd, Elizabeth M; Morley, S Celeste; Hunstad, David A

    2016-02-15

    Klebsiella pneumoniae, a chief cause of nosocomial pneumonia, is a versatile and commonly multidrug-resistant human pathogen for which further insight into pathogenesis is needed. We show that the pilus regulatory gene fimK promotes the virulence of K. pneumoniae strain TOP52 in murine pneumonia. This contrasts with the attenuating effect of fimK on urinary tract virulence, illustrating that a single factor may exert opposing effects on pathogenesis in distinct host niches. Loss of fimK in TOP52 pneumonia was associated with diminished lung bacterial burden, limited innate responses within the lung, and improved host survival. FimK expression was shown to promote serum resistance, capsule production, and protection from phagocytosis by host immune cells. Finally, while the widely used K. pneumoniae model strain 43816 produces rapid dissemination and death in mice, TOP52 caused largely localized pneumonia with limited lethality, thereby providing an alternative tool for studying K. pneumoniae pathogenesis and control within the lung.

  19. Pneumonia caused by Pittsburgh pneumonia agent: radiologic manifestations

    SciTech Connect

    Muder, R.R.; Reddy, S.C.; Yu, V.L.; Kroboth, F.J.

    1984-03-01

    Using an objective scoring system, chest radiographs were reviewed in 23 cases of pneumonia due to the Pittsburgh pneumonia agent (PPA, Tatlockia micdadei, Legionella micdadei), including six cases of pneumonia with simultaneous isolation of PPA and L pneumophila (Legionnaires' disease). Infiltrates were typically segmental to lobar; nodular infiltrates were noted in three cases. Spread to additional lobes after presentation occurred in four of 17 PPA infections. Pneumonia caused by both PPA and L pneumophila was unusually severe, with involvement of all lobes occurring in four of six cases, compared with one of 17 cases of PPA infection (p>0.02). Radiographic severity did not correlate with underlying disease, immune status, or outcome. The majority of patients receiving erythromycin demonstrated objective radiologic improvement. In a patients, population that included nonimmunosuppressed patient, nodule formation and rapid radiologic progression were not found to be characteristic of PPA pneumonia.

  20. [Annual review of Archivos de Neumología in interventional pneumology, interstitial diseases and lung transplantation].

    PubMed

    Disdier, Carlos; Bollo, Elena; Morales, Pilar; Montero, Carmen

    2009-01-01

    A review has been made of original articles on invasive pneumology techniques, interstitial diseases and lung transplantation, published in the Archivos de Bronconeumología during the year 2008. We have selected the publication by Martínez-Olondrins et al on the mediastinal staging of bronchogenic carcinoma by transbronchial needle aspiration to highlight the role of this simple, safe and cost-effective technique at a time when aspiration by ultrasound-guided bronchoscopy is profiled as an alternative to staging by mediastinoscopy. Besides its usefulness in the study of lymph nodes, transbronchial needle aspiration increases the overall performance of bronchoscopy by 20%, which means that it should be considered as a basic tool in the study of lung cancer. We also comment on the work by Galvis-Caravajal et al, who describe percutaneous radiofrequency as an alternative to radiotherapy in small lung or metastasic tumours. In diffuse interstitial disease, Morell et al analysed the diagnostic methods in 500 patients with this clinical-radiological presentation in which a definitive diagnosis was achieved in 85%, with 25% of them being obtained by non-invasively. Baloira et al analysed the characteristics of 19 patients with desquamative interstitial pneumonia and respiratory bronchiolitis associated-interstitial lung disease obtained from the National register of Interstitial Diseases.

  1. [Annual review of Archivos de Neumología in interventional pneumology, interstitial diseases and lung transplantation].

    PubMed

    Disdier, Carlos; Bollo, Elena; Morales, Pilar; Montero, Carmen

    2009-01-01

    A review has been made of original articles on invasive pneumology techniques, interstitial diseases and lung transplantation, published in the Archivos de Bronconeumología during the year 2008. We have selected the publication by Martínez-Olondrins et al on the mediastinal staging of bronchogenic carcinoma by transbronchial needle aspiration to highlight the role of this simple, safe and cost-effective technique at a time when aspiration by ultrasound-guided bronchoscopy is profiled as an alternative to staging by mediastinoscopy. Besides its usefulness in the study of lymph nodes, transbronchial needle aspiration increases the overall performance of bronchoscopy by 20%, which means that it should be considered as a basic tool in the study of lung cancer. We also comment on the work by Galvis-Caravajal et al, who describe percutaneous radiofrequency as an alternative to radiotherapy in small lung or metastasic tumours. In diffuse interstitial disease, Morell et al analysed the diagnostic methods in 500 patients with this clinical-radiological presentation in which a definitive diagnosis was achieved in 85%, with 25% of them being obtained by non-invasively. Baloira et al analysed the characteristics of 19 patients with desquamative interstitial pneumonia and respiratory bronchiolitis associated-interstitial lung disease obtained from the National register of Interstitial Diseases. PMID:19303530

  2. The Myositis Autoantibody Phenotypes of the Juvenile Idiopathic Inflammatory Myopathies

    PubMed Central

    Shah, Mona; Mamyrova, Gulnara; Huber, Adam M.; Rice, Madeline Murguia; Targoff, Ira N.; Miller, Frederick W.

    2013-01-01

    Abstract The juvenile idiopathic inflammatory myopathies (JIIM) are systemic autoimmune diseases characterized by skeletal muscle weakness, characteristic rashes, and other systemic features. In follow-up to our study defining the major clinical subgroup phenotypes of JIIM, we compared demographics, clinical features, laboratory measures, and outcomes among myositis-specific autoantibody (MSA) subgroups, as well as with published data on adult idiopathic inflammatory myopathy patients enrolled in a separate natural history study. In the present study, of 430 patients enrolled in a nationwide registry study who had serum tested for myositis autoantibodies, 374 had either a single specific MSA (n = 253) or no identified MSA (n = 121) and were the subject of the present report. Following univariate analysis, we used random forest classification and exact logistic regression modeling to compare autoantibody subgroups. Anti-p155/140 autoantibodies were the most frequent subgroup, present in 32% of patients with juvenile dermatomyositis (JDM) or overlap myositis with JDM, followed by anti-MJ autoantibodies, which were seen in 20% of JIIM patients, primarily in JDM. Other MSAs, including anti-synthetase, anti-signal recognition particle (SRP), and anti-Mi-2, were present in only 10% of JIIM patients. Features that characterized the anti-p155/140 autoantibody subgroup included Gottron papules, malar rash, “shawl-sign” rash, photosensitivity, cuticular overgrowth, lowest creatine kinase (CK) levels, and a predominantly chronic illness course. The features that differed for patients with anti-MJ antibodies included muscle cramps, dysphonia, intermediate CK levels, a high frequency of hospitalization, and a monocyclic disease course. Patients with anti-synthetase antibodies had higher frequencies of interstitial lung disease, arthralgia, and “mechanic’s hands,” and had an older age at diagnosis. The anti-SRP group, which had exclusively juvenile polymyositis, was

  3. The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies.

    PubMed

    Rider, Lisa G; Shah, Mona; Mamyrova, Gulnara; Huber, Adam M; Rice, Madeline Murguia; Targoff, Ira N; Miller, Frederick W

    2013-07-01

    The juvenile idiopathic inflammatory myopathies (JIIM) are systemic autoimmune diseases characterized by skeletal muscle weakness, characteristic rashes, and other systemic features. In follow-up to our study defining the major clinical subgroup phenotypes of JIIM, we compared demographics, clinical features, laboratory measures, and outcomes among myositis-specific autoantibody (MSA) subgroups, as well as with published data on adult idiopathic inflammatory myopathy patients enrolled in a separate natural history study. In the present study, of 430 patients enrolled in a nationwide registry study who had serum tested for myositis autoantibodies, 374 had either a single specific MSA (n = 253) or no identified MSA (n = 121) and were the subject of the present report. Following univariate analysis, we used random forest classification and exact logistic regression modeling to compare autoantibody subgroups. Anti-p155/140 autoantibodies were the most frequent subgroup, present in 32% of patients with juvenile dermatomyositis (JDM) or overlap myositis with JDM, followed by anti-MJ autoantibodies, which were seen in 20% of JIIM patients, primarily in JDM. Other MSAs, including anti-synthetase, anti-signal recognition particle (SRP), and anti-Mi-2, were present in only 10% of JIIM patients. Features that characterized the anti-p155/140 autoantibody subgroup included Gottron papules, malar rash, "shawl-sign" rash, photosensitivity, cuticular overgrowth, lowest creatine kinase (CK) levels, and a predominantly chronic illness course. The features that differed for patients with anti-MJ antibodies included muscle cramps, dysphonia, intermediate CK levels, a high frequency of hospitalization, and a monocyclic disease course. Patients with anti-synthetase antibodies had higher frequencies of interstitial lung disease, arthralgia, and "mechanic's hands," and had an older age at diagnosis. The anti-SRP group, which had exclusively juvenile polymyositis, was characterized by high

  4. The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies.

    PubMed

    Rider, Lisa G; Shah, Mona; Mamyrova, Gulnara; Huber, Adam M; Rice, Madeline Murguia; Targoff, Ira N; Miller, Frederick W

    2013-07-01

    The juvenile idiopathic inflammatory myopathies (JIIM) are systemic autoimmune diseases characterized by skeletal muscle weakness, characteristic rashes, and other systemic features. In follow-up to our study defining the major clinical subgroup phenotypes of JIIM, we compared demographics, clinical features, laboratory measures, and outcomes among myositis-specific autoantibody (MSA) subgroups, as well as with published data on adult idiopathic inflammatory myopathy patients enrolled in a separate natural history study. In the present study, of 430 patients enrolled in a nationwide registry study who had serum tested for myositis autoantibodies, 374 had either a single specific MSA (n = 253) or no identified MSA (n = 121) and were the subject of the present report. Following univariate analysis, we used random forest classification and exact logistic regression modeling to compare autoantibody subgroups. Anti-p155/140 autoantibodies were the most frequent subgroup, present in 32% of patients with juvenile dermatomyositis (JDM) or overlap myositis with JDM, followed by anti-MJ autoantibodies, which were seen in 20% of JIIM patients, primarily in JDM. Other MSAs, including anti-synthetase, anti-signal recognition particle (SRP), and anti-Mi-2, were present in only 10% of JIIM patients. Features that characterized the anti-p155/140 autoantibody subgroup included Gottron papules, malar rash, "shawl-sign" rash, photosensitivity, cuticular overgrowth, lowest creatine kinase (CK) levels, and a predominantly chronic illness course. The features that differed for patients with anti-MJ antibodies included muscle cramps, dysphonia, intermediate CK levels, a high frequency of hospitalization, and a monocyclic disease course. Patients with anti-synthetase antibodies had higher frequencies of interstitial lung disease, arthralgia, and "mechanic's hands," and had an older age at diagnosis. The anti-SRP group, which had exclusively juvenile polymyositis, was characterized by high

  5. Klebsiella pneumoniae splenic abscess.

    PubMed

    Gill, V; Marzocca, F J; Cunha, B A

    1994-01-01

    Splenic abscesses may be solitary or multiple and are unusual infections. Signs and symptoms are variable and do not always include left upper quadrant pain or tenderness, as the Case Report illustrate. Abscesses of the spleen may occur as a result of endocarditis or from hematogenous seeding from a distant focus of infection. Computed tomographic scan of the spleen is the diagnostic method of choice. We report a case of multiple splenic abscesses caused by Klebsiella pneumoniae that resulted from a Klebsiella urinary tract infection and was successfully managed with antibiotic therapy and splenectomy. PMID:8039997

  6. Enteral Tube Feeding and Pneumonia

    ERIC Educational Resources Information Center

    Gray, David Sheridan; Kimmel, David

    2006-01-01

    To determine the effects of enteral tube feeding on the incidence of pneumonia, we performed a retrospective review of all clients at our institution who had gastrostomy or jejunostomy tubes placed over a 10-year period. Ninety-three subjects had a history of pneumonia before feeding tube insertion. Eighty had gastrostomy and 13, jejunostomy…

  7. Vitamin D deficiency in chronic idiopathic urticaria.

    PubMed

    Movahedi, Masoud; Tavakol, Marzieh; Hirbod-Mobarakeh, Armin; Gharagozlou, Mohammad; Aghamohammadi, Asghar; Tavakol, Zahra; Momenzadeh, Kaveh; Nabavi, Mohammad; Dabbaghzade, Abbas; Mosallanejad, Asieh; Rezaei, Nima

    2015-04-01

    Chronic urticaria is the most common skin diseases, characterized by chronic cutaneous lesions which severely debilitates patients in several aspects of their everyday life. Vitamin D is known to exert several actions in the immune system and to influence function and differentiation of mast cells, central role players in the pathogenesis of chronic idiopathic urticaria. This study was performed to evaluate the relationship between vitamin D levels and susceptibility to chronic idiopathic urticaria. One hundred and fourteen patients with chronic idiopathic urticaria were recruited in this study along with one hundred and eighty seven sex-matched and age-matched healthy volunteers as the control group. For each patient, urticaria activity score was calculated and autologous serum skin test was done. Vitamin D metabolic statue was measured in serum as 25 hydroxyvitamin D using enzyme immunoassay method. Patients with chronic idiopathic urticaria significantly showed lower levels of vitamin D. Vitamin D deficiency was significantly associated with increased susceptibility to chronic idiopathic urticaria. There was a significant positive correlation between vitamin D levels and urticaria activity score. This study showed that patients with chronic idiopathic urticaria had reduced levels of vitamin D, while vitamin D deficiency could increase susceptibility to chronic idiopathic urticaria.

  8. Bronchiolitis obliterans organising pneumonia and primary biliary cirrhosis-like lung involvement in a patient with primary biliary cirrhosis.

    PubMed

    Strobel, E S; Bonnet, R B; Werner, P; Schaefer, H E; Peter, H H

    1998-01-01

    A 55-year-old woman with a 6-year history of primary biliary cirrhosis presented with an acute onset of fever, dyspnoea, crackles over both lower lung fields, and diffuse interstitial and bibasilar patchy pulmonary opacities. After exclusion of an infectious aetiology, an open lung biopsy was performed which revealed two histopathological features: (1) bronchiolitis obliterans organising pneumonia and (2) lympho-histiocytic interstitial pneumonitis and destructive bronchiolitis. Treatment response to corticosteroids and azathioprine followed a bimodal pattern with immediate resolution of her initial presenting symptoms and late resolution of residual gas exchange defects.

  9. Animal models of polymicrobial pneumonia

    PubMed Central

    Hraiech, Sami; Papazian, Laurent; Rolain, Jean-Marc; Bregeon, Fabienne

    2015-01-01

    Pneumonia is one of the leading causes of severe and occasionally life-threatening infections. The physiopathology of pneumonia has been extensively studied, providing information for the development of new treatments for this condition. In addition to in vitro research, animal models have been largely used in the field of pneumonia. Several models have been described and have provided a better understanding of pneumonia under different settings and with various pathogens. However, the concept of one pathogen leading to one infection has been challenged, and recent flu epidemics suggest that some pathogens exhibit highly virulent potential. Although “two hits” animal models have been used to study infectious diseases, few of these models have been described in pneumonia. Therefore the aims of this review were to provide an overview of the available literature in this field, to describe well-studied and uncommon pathogen associations, and to summarize the major insights obtained from this information. PMID:26170617

  10. Idiopathic subglottic stenosis: a familial predisposition.

    PubMed

    Dumoulin, Elaine; Stather, David R; Gelfand, Gary; Maranda, Bruno; Maceachern, Paul; Tremblay, Alain

    2013-03-01

    Idiopathic subglottic stenosis is a narrowing of the trachea at the level of the cricoid cartilage of unknown etiology. It is a rare condition for which the real incidence has never been established owing to the difficulty of making the diagnosis. Although there is a female preponderance, no familial cases have been reported in the literature. We describe two pairs of sisters as well as a mother and daughter presenting with idiopathic subglottic stenosis. All known causes of tracheal stenosis were excluded, including prolonged intubation, surgery, autoimmune and inflammatory disorders, infection and gastroesophageal reflux disease. These are the first cases reported in the literature that suggest a genetic predisposition for idiopathic subglottic stenosis.

  11. Bronchoscopic diagnosis of pneumonia.

    PubMed Central

    Baselski, V S; Wunderink, R G

    1994-01-01

    Lower respiratory tract infections are characterized by significant morbidity and mortality but also by a relative inability to establish a specific etiologic agent on clinical grounds alone. With the recognized shortcomings of expectorated or aspirated secretions toward establishing an etiologic diagnosis, clinicians have increasingly used bronchoscopy to obtain diagnostic samples. A variety of specimen types may be obtained, including bronchial washes or brushes, protected specimen brushings, bronchoalveolar lavage, and transbronchial biopsies. Bronchoscopy has been applied in three primary clinical settings, including the immunocompromised host, especially human immunodeficiency virus-infected and organ transplant patients; ventilator-associated pneumonia; and severe, nonresolving community- or hospital-acquired pneumonia in nonventilated patients. In each clinical setting, and for each specimen type, specific laboratory protocols are required to provide maximal information. These protocols should provide for the use of a variety of rapid microscopic and quantitative culture techniques and the use of a variety of specific stains and selective culture to detect unusual organism groups. PMID:7834604

  12. Granzyme A impairs host defense during Streptococcus pneumoniae pneumonia.

    PubMed

    van den Boogaard, Florry E; van Gisbergen, Klaas P J M; Vernooy, Juanita H; Medema, Jan P; Roelofs, Joris J T H; van Zoelen, Marieke A D; Endeman, Henrik; Biesma, Douwe H; Boon, Louis; Van't Veer, Cornelis; de Vos, Alex F; van der Poll, Tom

    2016-08-01

    Streptococcus pneumoniae is the most common causative pathogen in community-acquired pneumonia (CAP). Granzyme A (GzmA) is a serine protease produced by a variety of cell types involved in the immune response. We sought to determine the role of GzmA on the host response during pneumococcal pneumonia. GzmA was measured in bronchoalveolar lavage fluid (BALF) harvested from CAP patients from the infected and contralateral uninfected side and in lung tissue slides from CAP patients and controls. In CAP patients, GzmA levels were increased in BALF obtained from the infected lung. Human lungs showed constitutive GzmA expression by both parenchymal and nonparenchymal cells. In an experimental setting, pneumonia was induced in wild-type (WT) and GzmA-deficient (GzmA(-/-)) mice by intranasal inoculation of S. pneumoniae In separate experiments, WT and GzmA(-/-) mice were treated with natural killer (NK) cell depleting antibodies. Upon infection with S. pneumoniae, GzmA(-/-) mice showed a better survival and lower bacterial counts in BALF and distant body sites compared with WT mice. Although NK cells showed strong GzmA expression, NK cell depletion did not influence bacterial loads in either WT or GzmA(-/-) mice. These results implicate that GzmA plays an unfavorable role in host defense during pneumococcal pneumonia by a mechanism that does not depend on NK cells. PMID:27343190

  13. Fractionalization of interstitials in curved colloidal crystals

    NASA Astrophysics Data System (ADS)

    Irvine, William T. M.; Bowick, Mark J.; Chaikin, Paul M.

    2012-11-01

    Understanding the effect of curvature and topological frustration in crystals yields insights into the fragility of the ordered state. For instance, a one-dimensional crystal of identical charged particles can accommodate an extra particle (interstitial) if all the particle positions are readjusted, yet in a planar hexagonal crystal interstitials remain trapped between lattice sites and diffuse by hopping. Using optical tweezers operated independently of three-dimensional imaging, we inserted interstitials in a lattice of similar colloidal particles sitting on flat or curved oil/glycerol interfaces, and imaged the ensuing dynamics. We find that, unlike in flat space, the curved crystals self-heal through a collective particle rearrangement that redistributes the increased density associated with the interstitial. This process can be interpreted in terms of the out-of-equilibrium interaction of topological defects with each other and with the underlying curvature. Our observations suggest the existence of particle fractionalization on curved surface crystals.

  14. Thermodynamic processes of si-interstitial clusters

    SciTech Connect

    Kim, J.; Birner, S.; Richie, D. A.; Voter, A. F.

    2001-01-01

    Structural and dynamical properties of silicon interstitial defects are extracted from extensive atomistic simulations using ab initio total energy calculations. With increasing number of interstitials, the stable defect shape evolves from compact to chain-like to rod-like. The rodlike {l_brace}311{r_brace} defect, formed from (011) interstitial chains, is stabilized as it grows, elongating in the chain direction. We utilize new accelerated dynamics algorithms based on the parallel-replica method and reliable empirical potentials to efficiently explore large configurational space involving many degrees of freedom. We evaluate the empirical potentials that have been widely used for bulk silicon in light of the energetic and structural properties of interstitial defects.

  15. Mathematical model of renal interstitial fibrosis

    PubMed Central

    Hao, Wenrui; Rovin, Brad H.; Friedman, Avner

    2014-01-01

    Lupus nephritis (LN) is an autoimmune disease that occurs when autoantibodies complex with self-antigen and form immune complexes that accumulate in the glomeruli. These immune complexes initiate an inflammatory response resulting in glomerular injury. LN often concomitantly affects the tubulointerstitial compartment of the kidney, leading first to interstitial inflammation and subsequently to interstitial fibrosis and atrophy of the renal tubules if not appropriately treated. Presently the only way to assess interstitial inflammation and fibrosis is through kidney biopsy, which is invasive and cannot be repeated frequently. Hence, monitoring of disease progression and response to therapy is suboptimal. In this paper we describe a mathematical model of the progress from tubulointerstitial inflammation to fibrosis. We demonstrate how the model can be used to monitor treatments for interstitial fibrosis in LN with drugs currently being developed or used for nonrenal fibrosis. PMID:25225370

  16. Interstitial fibrosis and growth factors.

    PubMed Central

    Lasky, J A; Brody, A R

    2000-01-01

    Interstitial pulmonary fibrosis (IPF) is scarring of the lung caused by a variety of inhaled agents including mineral particles, organic dusts, and oxidant gases. The disease afflicts millions of individuals worldwide, and there are no effective therapeutic approaches. A major reason for this lack of useful treatments is that few of the molecular mechanisms of disease have been defined sufficiently to design appropriate targets for therapy. Our laboratory has focused on the molecular mechanisms through which three selected peptide growth factors could play a role in the development of IPF. Hundreds of growth factors and cytokines could be involved in the complex disease process. We are studying platelet-derived growth factor because it is the most potent mesenchymal cell mitogen yet described, transforming growth factor beta because it is a powerful inducer of extracellular matrix (scar tissue) components by mesenchymal cells, and tumor necrosis factor alpha because it is a pleiotropic cytokine that we and others have shown is essential for the development of IPF in animal models. This review describes some of the evidence from studies in humans, in animal models, and in vitro, that supports the growth factor hypothesis. The use of modern molecular and transgenic technologies could elucidate those targets that will allow effective therapeutic approaches. Images Figure 1 Figure 2 PMID:10931794

  17. Interstitial zinc clusters in zinc oxide

    NASA Astrophysics Data System (ADS)

    Gluba, M. A.; Nickel, N. H.; Karpensky, N.

    2013-12-01

    Doped zinc oxide (ZnO) exhibits anomalous Raman modes in the range of 270 to 870 cm-1. Commonly, the resonance at 275 cm-1 is attributed to the local vibration of Zn atoms in the vicinity of extrinsic dopants. We revisit this assignment by investigating the influence of isotopically purified zinc oxide thin films on the frequency of the vibrational mode around 275 cm-1. For this purpose, undoped and nitrogen-doped ZnO thin-films with Zn isotope compositions of natural Zn, 64Zn, 68Zn, and a 1:1 mixture of 64Zn and 68Zn were grown by pulsed laser deposition. The isotopic shift and the line shape of the Raman resonance around 275 cm-1 are analyzed in terms of three different microscopic models, which involve the vibration of (i) interstitial zinc atoms bound to extrinsic defects, (ii) interstitial diatomic Zn molecules, and (iii) interstitial zinc clusters. The energy diagram of interstitial Zn-Zn bonds in a ZnO matrix is derived from density functional theory calculations. The interstitial Zn-Zn bond is stabilized by transferring electrons from the antibonding orbital into the ZnO conduction band. This mechanism facilitates the formation of interstitial Zn clusters and fosters the common n-type doping asymmetry of ZnO.

  18. Guidelines or guidance for better idiopathic pulmonary fibrosis management?

    PubMed

    Behr, Jürgen

    2016-02-10

    Idiopathic pulmonary fibrosis (IPF) is a rare fibrotic interstitial lung disease with a relentlessly progressive course and fatal outcome. Guidelines summarizing the current evidence and providing evidence-based recommendations for the treatment of rare diseases such as IPF are important since individual physicians often have limited experience. Nevertheless, the available evidence is often scarce and, therefore, evidence-based recommendations are prone to being vague or with low confidence, thus creating uncertainty instead of guidance. Moreover, the effect of guidelines themselves on clinical practice has not been sufficiently evaluated. On the other hand, expert opinion may be biased and lead to the misinterpretation of evidence, resulting in misleading recommendations and a potential harm to patients. This editorial focuses on the advantages and disadvantages of evidence-based guidelines and professional experience in the context of a rare disease such as IPF and tries to assess the optimum combination of both approaches.Please see related commentary articles: http://dx.doi.org/10.1186/s12916-016-0562-1 and http://dx.doi.org/10.1186/s12916-016-0563-0.

  19. Hodgkin's disease presenting as idiopathic thrombocytopenic purpura.

    PubMed Central

    Murphy, W. G.; Allan, N. C.; Perry, D. J.; Stockdill, G.

    1984-01-01

    A case of Hodgkin's disease presenting as idiopathic thrombocytopenic purpura in a 23-year-old male is reported. This is a rare presentation of Hodgkin's disease having been previously described in only two cases. PMID:6541338

  20. Genetics Home Reference: idiopathic pulmonary fibrosis

    MedlinePlus

    ... However, the course of the disease is highly variable; some affected people become seriously ill within a ... idiopathic pulmonary fibrosis: an observational cohort study with independent validation. Lancet Respir Med. 2014 Jul;2(7): ...

  1. Epigenomics of idiopathic pulmonary fibrosis.

    PubMed

    Yang, Ivana V

    2012-04-01

    Idiopathic pulmonary fibrosis (IPF) is a complex lung disease of unknown etiology. Development of IPF is influenced by both genetic and environmental factors. Gene-expression profiling studies have taught us quite a bit about the biology of this fatal disease, but epigenetic marks may be the missing link that connects the environmental exposure in genetically predisposed individuals to transcriptome changes associated with the development of IPF. This review will begin with an introduction to the disease, followed by brief summaries of studies of gene expression in IPF and epigenetic marks associated with exposures relevant to IPF. The majority of the discussion will focus on epigenetic studies conducted so far in IPF, the limitations, challenges nd future directions in this field.

  2. Epidemiology of idiopathic pulmonary fibrosis

    PubMed Central

    Ley, Brett; Collard, Harold R

    2013-01-01

    Idiopathic pulmonary fibrosis is a chronic fibrotic lung disease of unknown cause that occurs in adults and has a poor prognosis. Its epidemiology has been difficult to study because of its rarity and evolution in diagnostic and coding practices. Though uncommon, it is likely underappreciated both in terms of its occurrence (ie, incidence, prevalence) and public health impact (ie, health care costs and resource utilization). Incidence and mortality appear to be on the rise, and prevalence is expected to increase with the aging population. Potential risk factors include occupational and environmental exposures, tobacco smoking, gastroesophageal reflux, and genetic factors. An accurate understanding of its epidemiology is important, especially as novel therapies are emerging. PMID:24348069

  3. Molecular etiology of idiopathic cardiomyopathy

    PubMed Central

    Arimura, T; Hayashi, T; Kimura, A

    2007-01-01

    Summary Idiopathic cardiomyopathy (ICM) is a primary cardiac disorder associated with abnormalities of ventricular wall thickness, size of ventricular cavity, contraction, relaxation, conduction and rhythm. Over the past two decades, molecular genetic analyses have revealed that mutations in the various genes cause ICM and such information concerning the genetic basis of ICM enables us to speculate the pathogenesis of this heterogeous cardiac disease. This review focuses on the molecular pathogenesis, i.e., genetic abnormalities and functional alterations due to the mutations especially in sarcomere/cytoskeletal components, in three characteristic features of ICM, hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM) and restrictive cardiomyopathy (RCM). Understanding the functional abnormalities of the sarcomere/cytoskeletal components, in ICM, has unraveled the function of these components not only as a contractile unit but also as a pivot for transduction of biochemical signals. PMID:18646564

  4. Cough in idiopathic pulmonary fibrosis.

    PubMed

    van Manen, Mirjam J G; Birring, Surinder S; Vancheri, Carlo; Cottin, Vincent; Renzoni, Elisabetta A; Russell, Anne-Marie; Wijsenbeek, Marlies S

    2016-09-01

    Many patients with idiopathic pulmonary fibrosis (IPF) complain of chronic refractory cough. Chronic cough is a distressing and disabling symptom with a major impact on quality of life. During recent years, progress has been made in gaining insight into the pathogenesis of cough in IPF, which is most probably "multifactorial" and influenced by mechanical, biochemical and neurosensory changes, with an important role for comorbidities as well. Clinical trials of cough treatment in IPF are emerging, and cough is increasingly included as a secondary end-point in trials assessing new compounds for IPF. It is important that such studies include adequate end-points to assess cough both objectively and subjectively. This article summarises the latest insights into chronic cough in IPF. It describes the different theories regarding the pathophysiology of cough, reviews the different methods to assess cough and deals with recent and future developments in the treatment of cough in IPF. PMID:27581827

  5. [Physiotherapy for juvenile idiopathic arthritis].

    PubMed

    Spamer, M; Georgi, M; Häfner, R; Händel, H; König, M; Haas, J-P

    2012-07-01

    Control of disease activity and recovery of function are major issues in the treatment of children and adolescents suffering from juvenile idiopathic arthritis (JIA). Functional therapies including physiotherapy are important components in the multidisciplinary teamwork and each phase of the disease requires different strategies. While in the active phase of the disease pain alleviation is the main focus, the inactive phase requires strategies for improving motility and function. During remission the aim is to regain general fitness by sports activities. These phase adapted strategies must be individually designed and usually require a combination of different measures including physiotherapy, occupational therapy, massage as well as other physical procedures and sport therapy. There are only few controlled studies investigating the effectiveness of physical therapies in JIA and many strategies are derived from long-standing experience. New results from physiology and sport sciences have contributed to the development in recent years. This report summarizes the basics and main strategies of physical therapy in JIA.

  6. Treatment of rheumatoid arthritis-associated interstitial lung disease: a perspective review

    PubMed Central

    Iqbal, Kundan; Kelly, Clive

    2015-01-01

    Rheumatoid arthritis (RA) is a systemic autoimmune disease affecting 0.5–1% of the worldwide population. Whilst predominantly causing chronic pain and inflammation in synovial joints, it is also associated with significant extra-articular manifestations in a large proportion of patients. Among the various pulmonary manifestations, interstitial lung disease (ILD), a progressive fibrotic disease of the lung parenchyma, is the commonest and most important, contributing significantly to increased morbidity and mortality. The most frequent patterns of RA-associated ILD (RA-ILD) are usual interstitial pneumonia and nonspecific interstitial pneumonia. New insights during the past several years have highlighted the epidemiological impact of RA-ILD and have begun to identify factors contributing to its pathogenesis. Risk factors include smoking, male sex, human leukocyte antigen haplotype, rheumatoid factor and anticyclic citrullinated protein antibodies (ACPAs). Combined with clinical information, chest examination and pulmonary function testing, high-resolution computed tomography of the chest forms the basis of investigation and allows assessment of subtype and disease extent. The management of RA-ILD is a challenge. Several therapeutic agents have been suggested in the literature but as yet no large randomized controlled trials have been undertaken to guide clinical management. Therapy is further complicated by commonly prescribed drugs of proven articular benefit such as methotrexate, leflunomide (LEF) and anti-tumour necrosis factor α agents having been implicated in both ex novo occurrence and acceleration of existing ILD. Agents that offer promise include immunomodulators such as mycophenolate and rituximab as well as newly studied antifibrotic agents. In this review, we discuss the current literature to evaluate recommendations for the management of RA-ILD and discuss key gaps in our knowledge of this important disease. PMID:26622326

  7. Treatment of rheumatoid arthritis-associated interstitial lung disease: a perspective review.

    PubMed

    Iqbal, Kundan; Kelly, Clive

    2015-12-01

    Rheumatoid arthritis (RA) is a systemic autoimmune disease affecting 0.5-1% of the worldwide population. Whilst predominantly causing chronic pain and inflammation in synovial joints, it is also associated with significant extra-articular manifestations in a large proportion of patients. Among the various pulmonary manifestations, interstitial lung disease (ILD), a progressive fibrotic disease of the lung parenchyma, is the commonest and most important, contributing significantly to increased morbidity and mortality. The most frequent patterns of RA-associated ILD (RA-ILD) are usual interstitial pneumonia and nonspecific interstitial pneumonia. New insights during the past several years have highlighted the epidemiological impact of RA-ILD and have begun to identify factors contributing to its pathogenesis. Risk factors include smoking, male sex, human leukocyte antigen haplotype, rheumatoid factor and anticyclic citrullinated protein antibodies (ACPAs). Combined with clinical information, chest examination and pulmonary function testing, high-resolution computed tomography of the chest forms the basis of investigation and allows assessment of subtype and disease extent. The management of RA-ILD is a challenge. Several therapeutic agents have been suggested in the literature but as yet no large randomized controlled trials have been undertaken to guide clinical management. Therapy is further complicated by commonly prescribed drugs of proven articular benefit such as methotrexate, leflunomide (LEF) and anti-tumour necrosis factor α agents having been implicated in both ex novo occurrence and acceleration of existing ILD. Agents that offer promise include immunomodulators such as mycophenolate and rituximab as well as newly studied antifibrotic agents. In this review, we discuss the current literature to evaluate recommendations for the management of RA-ILD and discuss key gaps in our knowledge of this important disease.

  8. Idiopathic Adult Growth Hormone Deficiency

    PubMed Central

    2013-01-01

    GH secretion is controlled by hypothalamic as well as intrapituitary and peripheral signals, all of which converge upon the somatotroph, resulting in integrated GH synthesis and secretion. Enabling an accurate diagnosis of idiopathic adult GH deficiency (IAGHD) is challenged by the pulsatility of GH secretion, provocative test result variability, and suboptimal GH assay standardization. The spectrum between attenuated GH secretion associated with the normal aging process and with obesity and truly well-defined IAGHD is not distinct and may mislead the diagnosis. Adult-onset GHD is mainly caused by an acquired pituitary deficiency, commonly including prior head/neck irradiation, or an expanding pituitary mass causing functional somatotroph compression. To what extent rare cryptic causes account for those patients seemingly classified as IAGHD is unclear. About 15% of patients with adult GHD and receiving GH replacement in open-label surveillance studies are reported as being due to an idiopathic cause. These patients may also reflect a pool of subjects with an as yet to be determined occult defect, or those with unclear or incomplete medical histories (including forgotten past sports head injury or motor vehicle accident). Therefore, submaximal diagnostic evaluation likely leads to an inadvertent diagnosis of IAGHD. In these latter cases, adherence to rigorous biochemical diagnostic criteria and etiology exclusion may result in reclassification of a subset of these patients to a distinct known acquired etiology, or as GH-replete. Accordingly, rigorously verified IAGHD likely comprises less than 10% of adult GHD patients, an already rare disorder. Regardless of etiology, patients with adult GHD, including those with IAGHD, exhibit a well-defined clinical phenotype including increased fat mass, loss of lean muscle mass, decreased bone mass, and enhanced cardiac morbidity. Definition of unique efficacy and dosing parameters for GH replacement and resultant therapeutic

  9. Carbapenemase-producing Klebsiella pneumoniae

    PubMed Central

    Deresinski, Stan

    2014-01-01

    The continuing emergence of infections due to multidrug resistant bacteria is a serious public health problem. Klebsiella pneumoniae, which commonly acquires resistance encoded on mobile genetic elements, including ones that encode carbapenemases, is a prime example. K. pneumoniae carrying such genetic material, including both blaKPC and genes encoding metallo-β-lactamases, have spread globally. Many carbapenemase-producing K. pneumoniae are resistant to multiple antibiotic classes beyond β-lactams, including tetracyclines, aminoglycosides, and fluoroquinolones. The optimal treatment, if any, for infections due to these organisms is unclear but, paradoxically, appears to often require the inclusion of an optimally administered carbapenem. PMID:25343037

  10. Urine markers of interstitial cystitis.

    PubMed

    Erickson, D R

    2001-06-01

    This article describes the current state of the art with regard to urine markers of interstitial cystitis (IC), and describes the areas that need continuing research. Articles referenced in MEDLINE that describe urine alterations in IC were reviewed. Additional articles were identified by cross-referencing. The different marker alterations were tabulated. The relevant articles were discussed, considering different purposes for urine markers including: (1) diagnosing IC; (2) confirming a specific pathophysiology for IC; and (3) predicting or following response to a specific treatment. Currently, 2 markers (glycoprotein-51 and antiproliferative factor [APF]) clearly separate IC and control subjects, with minimal overlap. Markers that correlate with specific bladder biopsy features include 1,4-methylimidazole acetic acid and eosinophil cationic protein (ECP), which correlate with mast cell density, and interleukin (IL)-6, which correlates with mononuclear inflammation. Markers that changed after treatment were as follows: (1) nitric oxide synthase and cyclic guanosine monophosphate increased with oral L-arginine; (2) ECP decreased with subcutaneous heparin; (3) prostaglandin E(2) and kallikrein decreased after bladder distention; (4) neutrophil chemotactic activity decreased after dimethyl sulfoxide; (5) IL-2 inhibitor decreased after oral nifedipine; (6) IL-2, IL-6, and IL-8 decreased after bacille Calmette-Guérin (BCG) vaccine; and (7) APF and heparin-binding epidermal growth factor changed to or toward normal levels after bladder distention or sacral nerve stimulation. A larger number of urine alterations have been reported, and a few are being pursued further by correlating with bladder biopsy findings or treatment responses. Further research is needed.

  11. Bronchoalveolar lavage: role in the pathogenesis, diagnosis, and management of interstitial lung disease

    SciTech Connect

    Daniele, R.P.; Elias, J.A.; Epstein, P.E.; Rossman, M.D.

    1985-01-01

    Bronchoalveolar lavage has emerged as a useful technique for the study of pulmonary interstitial disorders. Several types of information are provided by the evaluation of lavage fluid. First, the identification of cellular constituents helps to separate inflammatory processes in which lymphocytes predominate (for example, sarcoidosis, hypersensitivity pneumonitis, and berylliosis) from those in which neutrophils or macrophages predominate (for example, idiopathic pulmonary fibrosis and histiocytosis X). Second, the cells removed during lavage can be studied for their immune properties and function; tested with specific antigens, in diseases such as berylliosis and hypersensitivity pneumonitis; and examined for the presence of unique surface antigens with monoclonal antibodies (for example, histiocytosis X). Third, in conjunction with scanning electron microscopy and electron probe analysis, lavage makes possible the identification of inorganic particles in alveolar macrophages of patients with pneumoconiotic lung disease. Finally, although lavage is still an investigative procedure for most pulmonary disorders, it has an established role in the diagnosis of opportunistic infections in the immunocompromised patient.

  12. Idiopathic pancreatitis in a patient with a STAT3 mutation

    PubMed Central

    Peppers, Brian; Frith, John; Tcheurekdjian, Haig; Hostoffer, Robert

    2016-01-01

    Background: Hyperimmunoglobulin E syndrome (HIES) is a rare primary immunodeficiency characterized by recurrent skin infections with abscesses, recurrent pneumonias with pneumatoceles, and immunoglobulin E levels of >10 times the upper limit of normal. Case: The patient described herein had a classic case of signal transducer and activator of transcription 3 (STAT3) deficiency associated with HIES diagnosed several years before this particular presentation. He demonstrated extraimmune manifestations of the disease as well, including characteristic facies and a history of skeletal fractures. In addition, the patient had several distinct episodes of idiopathic pancreatitis for which a full gastrointestinal workup had been performed. STAT3 mutation was confirmed by genotyping at the time of diagnosis of HIES. Conclusions: STAT3, a mammalian protein that regulates cell growth, survival, and differentiation, has been linked to human pancreatic carcinogenesis as well as the above-mentioned immune deficiency. Mouse studies demonstrated that genetic ablation of STAT3 exacerbates the course of acute pancreatitis, whereas normal pancreatic STAT3 seems to have a protective effect against necrotizing pancreatitis. An association between STAT3 mutations and pancreatitis has not yet been revealed in humans. Here we describe a case of acute pancreatitis that presented in a patient with STAT3 mutation. PMID:27103560

  13. Clinical features of idiopathic megarectum and idiopathic megacolon.

    PubMed Central

    Gattuso, J M; Kamm, M A

    1997-01-01

    BACKGROUND: Dilatation of the rectum and/or colon, in the absence of demonstrable organic disease, is an uncommon and poorly characterised condition. AIMS: To characterise the clinical and diagnostic features, and response to treatment, of patients with idiopathic megarectum (IMR) and idiopathic megacolon (IMC). METHODS: A retrospective review was undertaken of all patients operated on for these conditions over a 23 year period. In addition all patients treated over a three year period were prospectively studied by means of a questionnaire, contrast studies of the upper and lower intestine, spine x rays to exclude spinal dysraphism, anorectal physiological studies, and assessment of clinical outcome. Patients with Hirschsprung's disease and other known causes of gut dilatation were excluded. RESULTS: (i) Retrospective study: Of 63 operated patients, 22 had IMR, 23 had IMR and IMC, and 18 had IMC only. Five patients with IMC had previous sigmoid volvulus, and three had associated non-gastrointestinal congenital abnormalities. Faecal incontinence was always associated with rectal impaction and 14 patients (82%) with IMR alone had had manual disimpaction. (ii) Prospective study: Twenty two patients had IMR, with a median rectal diameter of 10 cm (normal < 6.5 cm). Six patients had IMC and one patient had IMR and IMC. Patients with IMR were significantly (p = 0.0007) younger than patients with IMC. All patients with IMR became symptomatic in childhood, compared with half the patients with IMC who developed symptoms as adults. Patients with IMR all presented with soiling and impaction, compared with patients with IMC whose symptoms were variable and included constipation or increased bowel frequency, pain, and variable need for laxatives. No upper gut dilatation was seen in either group of patients. Spinal dysraphism was seen in two of 18 patients with IMR and two of four with IMC, suggesting extrinsic denervation as a possible cause in a minority. Twelve of 22 patients

  14. Interstitial Cells: Regulators of Smooth Muscle Function

    PubMed Central

    Sanders, Kenton M.; Ward, Sean M.; Koh, Sang Don

    2014-01-01

    Smooth muscles are complex tissues containing a variety of cells in addition to muscle cells. Interstitial cells of mesenchymal origin interact with and form electrical connectivity with smooth muscle cells in many organs, and these cells provide important regulatory functions. For example, in the gastrointestinal tract, interstitial cells of Cajal (ICC) and PDGFRα+ cells have been described, in detail, and represent distinct classes of cells with unique ultrastructure, molecular phenotypes, and functions. Smooth muscle cells are electrically coupled to ICC and PDGFRα+ cells, forming an integrated unit called the SIP syncytium. SIP cells express a variety of receptors and ion channels, and conductance changes in any type of SIP cell affect the excitability and responses of the syncytium. SIP cells are known to provide pacemaker activity, propagation pathways for slow waves, transduction of inputs from motor neurons, and mechanosensitivity. Loss of interstitial cells has been associated with motor disorders of the gut. Interstitial cells are also found in a variety of other smooth muscles; however, in most cases, the physiological and pathophysiological roles for these cells have not been clearly defined. This review describes structural, functional, and molecular features of interstitial cells and discusses their contributions in determining the behaviors of smooth muscle tissues. PMID:24987007

  15. [Modern Views on Children's Interstitial Lung Disease].

    PubMed

    Boĭtsova, E V; Beliashova, M A; Ovsiannikov, D Iu

    2015-01-01

    Interstitial lung diseases (ILD, diffuse lung diseases) are a heterogeneous group of diseases in which a pathological process primarily involved alveoli and perialveolar interstitium, resulting in impaired gas exchange, restrictive changes of lung ventilation function and diffuse interstitial changes detectable by X-ray. Children's interstitial lung diseases is an topical problem ofpediatricpulmonoogy. The article presents current information about classification, epidemiology, clinical presentation, diagnostics, treatment and prognosis of these rare diseases. The article describes the differences in the structure, pathogenesis, detection of various histological changes in children's ILD compared with adult patients with ILD. Authors cite an instance of registers pediatric patients with ILD. The clinical semiotics of ILD, the possible results of objective research, the frequency of symptoms, the features of medical history, the changes detected on chest X-rays, CT semiotics described in detail. Particular attention was paid to interstitial lung diseases, occurring mainly in newborns and children during the first two years of life, such as congenital deficiencies of surfactant proteins, neuroendocrine cell hyperplasia of infancy, pulmonary interstitial glycogenosis. The diagnostic program for children's ILD, therapy options are presented in this article.

  16. [Methods and clinical results of interstitial thermoradiotherapy].

    PubMed

    Seegenschmiedt, M H; Sauer, R

    1989-05-01

    Besides percutaneous hyperthermia techniques, some new interstitial procedures have been developed and included into the arsenal of radiotherapists. As compared to percutaneous techniques, considerable benefits are offered by interstitial methods: a more homogeneous distribution of therapeutical temperatures, a better sparing of normal tissue, the possibility to treat deep tumors, and better therapy control and evaluation by extensive "thermal mapping" within the target volume. This study presents the technical principles and the clinical possibilities of the interstitial hyperthermia methods developed hitherto: resistive radiofrequency hyperthermia, radiative microwave hyperthermia, inductive ferromagnetic seed hyperthermia, and conductive hot-water perfusion hyperthermia. Until now, interstitial thermo-radiotherapy was only performed during some phase I/II studies. It was applied for palliative reasons in persisting and recurrent tumors accessible for implantation and situated in superficial to semi-deep locations with no or insufficient response to differently combined treatment modalities (surgery, radiotherapy, or chemotherapy). The preliminary clinical results obtained in almost 300 patients are quite promising: complete remission rates up to 70% have been achieved. Interstitial hyperthermia represents an effective and safe therapy modality, especially when combined with radiotherapy in palliative tumor therapy. At present, several prospective randomized multicentric studies are conducted to investigate its value as adjuvant therapy modality.

  17. [Modern Views on Children's Interstitial Lung Disease].

    PubMed

    Boĭtsova, E V; Beliashova, M A; Ovsiannikov, D Iu

    2015-01-01

    Interstitial lung diseases (ILD, diffuse lung diseases) are a heterogeneous group of diseases in which a pathological process primarily involved alveoli and perialveolar interstitium, resulting in impaired gas exchange, restrictive changes of lung ventilation function and diffuse interstitial changes detectable by X-ray. Children's interstitial lung diseases is an topical problem ofpediatricpulmonoogy. The article presents current information about classification, epidemiology, clinical presentation, diagnostics, treatment and prognosis of these rare diseases. The article describes the differences in the structure, pathogenesis, detection of various histological changes in children's ILD compared with adult patients with ILD. Authors cite an instance of registers pediatric patients with ILD. The clinical semiotics of ILD, the possible results of objective research, the frequency of symptoms, the features of medical history, the changes detected on chest X-rays, CT semiotics described in detail. Particular attention was paid to interstitial lung diseases, occurring mainly in newborns and children during the first two years of life, such as congenital deficiencies of surfactant proteins, neuroendocrine cell hyperplasia of infancy, pulmonary interstitial glycogenosis. The diagnostic program for children's ILD, therapy options are presented in this article. PMID:26234096

  18. The Significance of Interstitial Cells in Neurogastroenterology

    PubMed Central

    Blair, Peter J; Rhee, Poong-Lyul; Sanders, Kenton M; Ward, Sean M

    2014-01-01

    Smooth muscle layers of the gastrointestinal tract consist of a heterogeneous population of cells that include enteric neurons, several classes of interstitial cells of mesenchymal origin, a variety of immune cells and smooth muscle cells (SMCs). Over the last number of years the complexity of the interactions between these cell types has begun to emerge. For example, interstitial cells, consisting of both interstitial cells of Cajal (ICC) and platelet-derived growth factor receptor alpha-positive (PDGFRα+) cells generate pacemaker activity throughout the gastrointestinal (GI) tract and also transduce enteric motor nerve signals and mechanosensitivity to adjacent SMCs. ICC and PDGFRα+ cells are electrically coupled to SMCs possibly via gap junctions forming a multicellular functional syncytium termed the SIP syncytium. Cells that make up the SIP syncytium are highly specialized containing unique receptors, ion channels and intracellular signaling pathways that regulate the excitability of GI muscles. The unique role of these cells in coordinating GI motility is evident by the altered motility patterns in animal models where interstitial cell networks are disrupted. Although considerable advances have been made in recent years on our understanding of the roles of these cells within the SIP syncytium, the full physiological functions of these cells and the consequences of their disruption in GI muscles have not been clearly defined. This review gives a synopsis of the history of interstitial cell discovery and highlights recent advances in structural, molecular expression and functional roles of these cells in the GI tract. PMID:24948131

  19. The significance of interstitial cells in neurogastroenterology.

    PubMed

    Blair, Peter J; Rhee, Poong-Lyul; Sanders, Kenton M; Ward, Sean M

    2014-07-31

    Smooth muscle layers of the gastrointestinal tract consist of a heterogeneous population of cells that include enteric neurons, several classes of interstitial cells of mesenchymal origin, a variety of immune cells and smooth muscle cells (SMCs). Over the last number of years the complexity of the interactions between these cell types has begun to emerge. For example, interstitial cells, consisting of both interstitial cells of Cajal (ICC) and platelet-derived growth factor receptor alpha-positive (PDGFRα(+)) cells generate pacemaker activity throughout the gastrointestinal (GI) tract and also transduce enteric motor nerve signals and mechanosensitivity to adjacent SMCs. ICC and PDGFRα(+) cells are electrically coupled to SMCs possibly via gap junctions forming a multicellular functional syncytium termed the SIP syncytium. Cells that make up the SIP syncytium are highly specialized containing unique receptors, ion channels and intracellular signaling pathways that regulate the excitability of GI muscles. The unique role of these cells in coordinating GI motility is evident by the altered motility patterns in animal models where interstitial cell networks are disrupted. Although considerable advances have been made in recent years on our understanding of the roles of these cells within the SIP syncytium, the full physiological functions of these cells and the consequences of their disruption in GI muscles have not been clearly defined. This review gives a synopsis of the history of interstitial cell discovery and highlights recent advances in structural, molecular expression and functional roles of these cells in the GI tract.

  20. Diagnosis and treatment of adolescent idiopathic scoliosis.

    PubMed

    Burton, Monique S

    2013-11-01

    Scoliosis is defined as a lateral curvature of the spine greater than 10 degrees on radiography that is typically associated with trunk rotation. The three major types of scoliosis are congenital, idiopathic, and neuromuscular. Idiopathic scoliosis is divided into three subcategories based on the age of onset. Infantile idiopathic scoliosis affects patients younger than 3 years, juvenile idiopathic scoliosis appears in children between 3 and 10 years, and adolescent idiopathic scoliosis (AIS) occurs in skeletally immature patients older than 10 years. AIS is the most common form of idiopathic scoliosis. Approximately 2% to 4% of children aged 10 to 16 years have some degree of spinal curvature. Although some researchers view routine screening for AIS as controversial, well-child examinations and sports physicals are an optimal time to evaluate for AIS in the clinical setting. In 2008, the American Academy of Orthopaedic Surgeons, the Scoliosis Research Society, the Pediatric Orthopaedic Society of North America, and the American Academy of Pediatrics convened a task force to review the issues related to scoliosis screening and issued an information statement concluding that although screening has limitations, the potential benefits that patients with idiopathic scoliosis receive from early treatment can be substantial. Recommendations are now that females are screened twice, at age 10 and 12 years, and males once at age 13 or 14 years. Screening during routine well-child examinations and/or school-based evaluations will help identify patients who need ongoing monitoring. The evaluation of curvatures in conjunction with the level of skeletal maturity will help to guide the management of the curvature.

  1. Idiopathic Gingival Fibromatosis: Case Report and Its Management

    PubMed Central

    Jaju, Prashant P.; Desai, Ankit; Desai, Rajiv S.; Jaju, Sushma P.

    2009-01-01

    Idiopathic gingival fibromatosis is a rare condition. We present a case of idiopathic gingival fibromatosis with its multidisciplinary approach of management. The clinical, radiographic, and histopathological features have been described in detail. PMID:20339448

  2. Interstitial granulomatous dermatitis secondary to soy.

    PubMed

    Dyson, Senait W; Hirsch, Ann; Jaworsky, Christine

    2004-08-01

    A healthy 58-year-old woman developed an asymptomatic papular eruption of the neck, cheek, abdomen, arms, and flexures. There was an 8-year history of the lesions, which had erupted when the patient started a strict vegetarian diet. Lesions lasted 3 to 5 days, cleared without scarring, and were associated with burning and increased tearing of the eyes. The biopsy specimen showed an interstitial granulomatous dermatitis without vascular injury, collagen alteration, or mononuclear atypia. The eruption cleared when the patient omitted soy products from her diet. It subsequently recurred with intake of even minimal amounts of soy. Interstitial granulomatous dermatitis is a histologic pattern of inflammation that generates a broad differential diagnosis. No previous reports of interstitial granulomatous dermatitis related to soy products are available in the literature.

  3. [Karyomegalic interstitial nephritis: A new French case].

    PubMed

    Verine, Jérôme; Reade, Richard; Janin, Anne; Droz, Dominique

    2010-06-01

    Karyomegalic interstitial nephritis (KIN) is a rare and slowly progressive chronic interstitial nephritis (CIN) (28 cases reported), described for the first time by Mihatsch et al. in 1979. Here, we report on a 50-year-old woman who presented with asymptomatic renal failure and mild proteinuria without hematuria. Renal biopsy showed large tubulo-interstitial fibrosis and massively enlarged tubular epithelial cell nuclei, without viral inclusion. KIN is a rare CIN defined by a karyomegaly of tubular epithelial cell nuclei. Its pathogenesis remains obscure. Nevertheless, an exogenous factor is suspected, ochratoxin A particularly. The familial clustering of patients and the frequency of HLA-A9 and HLA-B35 haplotypes suggest the presence of a possible genetic susceptibility to this disorder.

  4. Di-interstitial defect in silicon revisited

    SciTech Connect

    Londos, C. A.; Antonaras, G.; Chroneos, A.

    2013-11-21

    Infrared spectroscopy was used to study the defect spectrum of Cz-Si samples following fast neutron irradiation. We mainly focus on the band at 533 cm{sup −1}, which disappears from the spectra at ∼170 °C, exhibiting similar thermal stability with the Si-P6 electron paramagnetic resonance (EPR) spectrum previously correlated with the di-interstitial defect. The suggested structural model of this defect comprises of two self-interstitial atoms located symmetrically around a lattice site Si atom. The band anneals out following a first-order kinetics with an activation energy of 0.88 ± 0.3 eV. This value does not deviate considerably from previously quoted experimental and theoretical values for the di-interstitial defect. The present results indicate that the 533 cm{sup −1} IR band originates from the same structure as that of the Si-P6 EPR spectrum.

  5. An overview of interstitial brachytherapy and hyperthermia

    SciTech Connect

    Brandt, B.B.; Harney, J.

    1989-11-01

    Interstitial thermoradiotherapy, an experimental cancer treatment that combines interstitial radiation implants (brachytherapy) and interstitial hyperthermia, is in the early stages of investigation. In accordance with the procedure used in a current national trial protocol, a 60-minute hyperthermia treatment is administered after catheters are placed into the tumor area while the patient is under general anesthesia. This is immediately followed by loading of radioactive Iridium-192 seeds into the catheters for a defined period of time. Once the prescribed radiation dose is delivered, the radioactive sources are removed and a second, 60-minute hyperthermia treatment is administered. Clinical trials with hyperthermia in combination with radiation have increased in recent years. Nurses caring for these patients need to become more knowledgeable about this investigational therapy. This paper provides an overview of the biologic rationale for this therapy, as well as a description of the delivery method and clinical application. Specific related nursing interventions are defined in a nursing protocol.23 references.

  6. Wear evaluation of high interstitial stainless steel

    SciTech Connect

    Rawers, J.C.; Tylczak, J.H.

    2008-07-01

    A new series of high nitrogen-carbon manganese stainless steel alloys are studied for their wear resistance. High nitrogen and carbon concentrations were obtained by melting elemental iron-chromium-manganese (several with minor alloy additions of nickel, silicon, and molybdenum) in a nitrogen atmosphere and adding elemental graphite. The improvement in material properties (hardness and strength) with increasing nitrogen and carbon interstitial concentration was consistent with previously reported improvements in similar material properties alloyed with nitrogen only. Wear tests included: scratch, pin-on-disk, sand-rubber-wheel, impeller, and jet erosion. Additions of interstitial nitrogen and carbon as well as interstitial nitrogen and carbide precipitates were found to greatly improve material properties. In general, with increasing nitrogen and carbon concentrations, strength, hardness, and wear resistance increased.

  7. Interstitial Pneumonitis from Treatment with Gemcitabine

    PubMed Central

    Poole, Brolin B.; Brockman, Megan M.; Byrd, Debbie C.

    2014-01-01

    Introduction: The use of gemcitabine may lead to numerous adverse effects ranging from mild to very severe, such as interstitial pneumonitis. The diagnosis of this complication is based on multiple laboratory findings, radiographic evidence, and high clinical suspicion. Presented is a case report of a patient who met these criteria and had onset consistent with drug-induced interstitial pneumonitis. Case Presentation: A 76-year-old White female was treated with gemcitabine for pancreatic cancer. Two months after the initiation of therapy, she was admitted to the hospital for worsening dyspnea and cough. High clinical suspicion, bilateral interstitial opacities on chest x-ray, worsening pulmonary status, and onset 2 months after initiation of therapy led to the diagnosis of gemcitabine-induced interstitial pneumonitis. Steroid therapy with prednisone was initiated, and the patient’s clinical symptoms and radiographic findings improved. Discussion: Gemcitabine-induced interstitial pneumonitis is well described in the literature. It is a rare but serious complication associated with gemcitabine therapy in which patients present with worsening dyspnea. Most patients only require supportive care and discontinuation of the drug for treatment, but in severe cases supplemental oxygen and steroid therapy must be used before resolution of symptoms. It is important to obtain an accurate medication history to evaluate for other potentially pulmonary toxic medications. Radiographic findings such as bilateral infiltrates should be completely resolved after therapy. Conclusion: Radiographic findings, clinical symptoms, and clinical suspicion can lead to early recognition of interstitial pneumonitis from gemcitabine. Physician awareness of this adverse effect and early recognition are keys to providing prompt treatment in resolving symptoms and decreasing mortality. PMID:25477616

  8. What Are the Signs and Symptoms of Pneumonia?

    MedlinePlus

    ... Twitter. What Are the Signs and Symptoms of Pneumonia? The signs and symptoms of pneumonia vary from ... have sudden changes in mental awareness. Complications of Pneumonia Often, people who have pneumonia can be successfully ...

  9. Familial idiopathic normal pressure hydrocephalus.

    PubMed

    Huovinen, Joel; Kastinen, Sami; Komulainen, Simo; Oinas, Minna; Avellan, Cecilia; Frantzen, Janek; Rinne, Jaakko; Ronkainen, Antti; Kauppinen, Mikko; Lönnrot, Kimmo; Perola, Markus; Pyykkö, Okko T; Koivisto, Anne M; Remes, Anne M; Soininen, Hilkka; Hiltunen, Mikko; Helisalmi, Seppo; Kurki, Mitja; Jääskeläinen, Juha E; Leinonen, Ville

    2016-09-15

    Idiopathic normal pressure hydrocephalus (iNPH) is a late-onset surgically alleviated, progressive disease. We characterize a potential familial subgroup of iNPH in a nation-wide Finnish cohort of 375 shunt-operated iNPH-patients. The patients were questionnaired and phone-interviewed, whether they have relatives with either diagnosed iNPH or disease-related symptomatology. Then pedigrees of all families with more than one iNPH-case were drawn. Eighteen patients (4.8%) from 12 separate pedigrees had at least one shunt-operated relative whereas 42 patients (11%) had relatives with two or more triad symptoms. According to multivariate logistic regression analysis, familial iNPH-patients had up to 3-fold risk of clinical dementia compared to sporadic iNPH patients. This risk was independent from diagnosed Alzheimer's disease and APOE ε4 genotype. This study describes a familial entity of iNPH offering a novel approach to discover the potential genetic characteristics of iNPH. Discovered pedigrees offer an intriguing opportunity to conduct longitudinal studies targeting potential preclinical signs of iNPH. PMID:27538594

  10. Scrotal calcinosis: idiopathic or dystrophic?

    PubMed

    Dubey, Suparna; Sharma, Rajeev; Maheshwari, Veena

    2010-01-01

    Scrotal calcinosis is a rare benign local process characterized by multiple, painless, hard scrotal nodules in the absence of any systemic metabolic disorder. Histological examination reveals extensive deposition of calcium in the dermis, which may be surrounded by histiocytes and an inflammatory giant cell reaction. Numerous theories have been propounded to explain the pathogenesis of this condition, but the principal debate revolves around whether the calcium is deposited at the site of previous epithelial cysts or the calcified nodules are purely idiopathic. This is the largest study of scrotal calcinosis to date with 100 cases, on which clinical, biochemical, radiological, cytopathological, and histopathological examinations were conducted. The histological picture shows a continuous spectrum of changes ranging from intact epithelial cysts (41.0%) - both normal and inflamed; through inflamed cysts containing calcific material in the lumen but with intact cyst wall (53.0%); calcified inflamed cysts with partial epithelial lining (11.0%); to 'naked' calcium deposits lying in the dermis (100%), sometimes compressing surrounding collagen fibres to form a pseudocyst (56.0%). The presence of normal values of calcium and phosphorus along with this spectrum of changes in histology both support the theory that these form by dystrophic calcification of epithelial cysts in a progression that involves inflammation, rupture, calcification and obliteration of the cyst wall. PMID:20178701

  11. Interstitial lung disease in an adult with Fanconi anemia: Clues to the pathogenesis

    SciTech Connect

    Rubinstein, W.S.; Wenger, S.L.; Hoffman, R.M.

    1997-03-31

    We have studied a 38-year-old man with a prior diagnosis of Holt-Oram syndrome, who presented with diabetes mellitus. He had recently taken prednisone for idiopathic interstitial lung disease and trimethoprim-sulfamethoxazole for sinusitis. Thrombocytopenia progressed to pancytopenia. The patient had skeletal, cardiac, renal, cutaneous, endocrine, hepatic, neurologic, and hematologic manifestations of Fanconi anemia (FA). Chest radiographs showed increased interstitial markings at age 25, dyspnea began in his late 20s, and he stopped smoking at age 32. At age 38, computerized tomography showed bilateral upper lobe fibrosis, lower lobe honeycombing, and bronchiectasis. Pulmonary function tests, compromised at age 29, showed a moderately severe obstructive and restrictive pattern by age 38. Serum alpha-1 antitrypsin level was 224 (normal 85-213) mg/dL and PI phenotype was M1. Karyotype was 46,X-Y with a marked increase in chromosome aberrations induced in vitro by diepoxybutane. The early onset and degree of pulmonary disease in this patient cannot be fully explained by environmental or known genetic causes. The International Fanconi Anemia Registry (IFAR) contains no example of a similar pulmonary presentation. Gene-environment (ecogenetic) interactions in FA seem evident in the final phenotype. The pathogenic mechanism of lung involvement in FA may relate to oxidative injury and cytokine anomalies. 49 refs., 2 figs., 1 tab.

  12. αvβ6 integrin may be a potential prognostic biomarker in interstitial lung disease.

    PubMed

    Saini, Gauri; Porte, Joanne; Weinreb, Paul H; Violette, Shelia M; Wallace, William A; McKeever, Tricia M; Jenkins, Gisli

    2015-08-01

    Idiopathic pulmonary fibrosis (IPF) and fibrotic nonspecific interstitial pneumonitis are progressive interstitial lung diseases (ILDs) with limited treatment options and poor survival. However, the rate of disease progression is variable, implying there may be different endotypes of disease. We hypothesised that immunophenotyping biopsies from ILD patients might reveal distinct endotypes of progressive fibrotic disease, which may facilitate stratification when undertaking clinical trials of novel therapies for IPF.43 paraffin-embedded, formalin-fixed lung tissue sections were immunostained for five molecules implicated in the pathogenesis of the fibrosis: α-smooth muscle actin (αSMA), αvβ6 integrin, pro-surfactant protein C (SP-C), hepatocyte growth factor (HGF) and tenascin-C (TenC). Levels of immunostaining and numbers of fibroblastic foci were quantified using operator-dependent and -independent methods. The relationship of all these markers to overall survival was analysed.Staining revealed high levels of αSMA, αvβ6 integrin, pro-SP-C, HGF and TenC, and fibroblastic foci. Immunostaining varied across samples for all molecules but only the extent of αvβ6 integrin immunostaining was associated with increased mortality. There was no association with the other markers measured.Our data suggest high levels of αvβ6 integrin may identify a specific endotype of progressive fibrotic lung disease.

  13. Effect of Stem Cell Therapy on Amiodarone Induced Fibrosing Interstitial Lung Disease in Albino Rat

    PubMed Central

    Zaglool, Somaya Saad; Zickri, Maha Baligh; Abd El Aziz, Dalia Hussein; Mabrouk, Doaa; Metwally, Hala Gabr

    2011-01-01

    Background and Objectives: The fibrosing forms of interstitial lung disease (ILD) are associated with significant morbidity and mortality. ILD may be idiopathic, secondary to occupational, infection, complicate rheumatic diseases or drug induced. Efficacy of antifibrotic agents is as far as, limited and uncertain. No effective treatment was confirmed for pulmonary fibrosis except lung transplantation. The present study aimed at investigating the possible effect of human cord blood mesenchymal stem cell (MSC) therapy on fibrosing ILD. This was accomplished by using amiodarone as a model of induced lung damage in albino rat. Methods and Results: Seventeen adult male albino rats were divided into 3 groups. Rats of amiodarone group were given 30 mg/kg of amiodarone orally 6 days/ week for 6 weeks. Rats of stem cell therapy group were injected with stem cells in the tail vein following confirmation of lung damage and left for 4 weeks before sacrifice. Obstructed bronchioles, thickened interalveolar septa and thickened wall of pulmonary vessels were found and proved morphometrically. Reduced type I pneumocytes and increased area% of collagen fibers were recorded. All findings regressed on stem cell therapy. Conclusions: Cord blood MSC therapy proved definite amelioration of fibrosing interstitial lung disease provided therapy starts early in the development of the pathogenesis. PMID:24298346

  14. [Interstitial cystitis. A challenge for the clinician].

    PubMed

    Young, Pablo; Finn, Bárbara C; González, Martín; Comercio, Laura P; Quezel, Mariano; Bruetman, Julio E

    2010-01-01

    Interstitial cystitis is characterized by over 6 months of chronic pain, pressure and discomfort felt in the lower pelvis or bladder. It is often relieved with voiding, along with daytime frequency and nocturia in the absence of an urinary tract infection. The disorder can be divided clinically into two groups -ulcerative and non-ulcerative- based on cystoscopic findings and response to treatment. Management follows an approach of applying the least invasive therapy that affords sufficient relief of symptoms. We report a case of a patient with interstitial cystitis. The diagnosis was performed by symptoms and lesion in the cystoscopy and excellent response to amitriptyline.

  15. [Interstitial cystitis. A challenge for the clinician].

    PubMed

    Young, Pablo; Finn, Bárbara C; González, Martín; Comercio, Laura P; Quezel, Mariano; Bruetman, Julio E

    2010-01-01

    Interstitial cystitis is characterized by over 6 months of chronic pain, pressure and discomfort felt in the lower pelvis or bladder. It is often relieved with voiding, along with daytime frequency and nocturia in the absence of an urinary tract infection. The disorder can be divided clinically into two groups -ulcerative and non-ulcerative- based on cystoscopic findings and response to treatment. Management follows an approach of applying the least invasive therapy that affords sufficient relief of symptoms. We report a case of a patient with interstitial cystitis. The diagnosis was performed by symptoms and lesion in the cystoscopy and excellent response to amitriptyline. PMID:20679058

  16. Interstitial irradiation of brain tumors: a review

    SciTech Connect

    Bernstein, M.; Gutin, P.H.

    1981-12-01

    As an adjuvant to surgery, radiation therapy has consistently proven to be the most successful form of treatment for primary and secondary malignant brain tumors and possibly for inoperable benign tumors. Because the risk of radiation necrosis of normal brain limits the amount of radiation that can be given by external beam therapy at conventional dose rates, interstitial radiation of brain tumors is a logical alternative treatment approach. We discuss the radiobiological advantages of low dose rate irradiation and intratumoral placement of sources that make interstitial irradiation an attractive treatment for brain tumors and review the history of clinical brachytherapy for intracranial neoplasia.

  17. Role of epithelial cells in idiopathic pulmonary fibrosis: from innocent targets to serial killers.

    PubMed

    Selman, Moisés; Pardo, Annie

    2006-06-01

    Idiopathic pulmonary fibrosis (IPF), a progressive and relentless lung scarring of unknown etiology, has been recognized as the most lethal interstitial lung disease. Despite the growing interest in IPF, the precise molecular mechanisms underlying the development of fibrosis and leading to the irreversible destruction of the lung are still unknown. Recently, it has been proposed that IPF, instead of being a chronic inflammatory disorder, results from multiple cycles of epithelial cell injury and activation. In turn, active alveolar epithelial cells provoke the migration, proliferation, and activation of mesenchymal cells with the formation of fibroblastic/myofibroblastic foci and the exaggerated accumulation of extracellular matrix, mirroring abnormal wound repair. In this article, some characteristics of the alveolar epithelium are briefly outlined, and the fibrogenic mechanisms specifically operated by active abnormal epithelial cells are examined.

  18. Anti-endothelial cell antibody, thrombomodulin, and von Willebrand factor in idiopathic inflammatory myopathies.

    PubMed Central

    Salojin, K V; Bordron, A; Nassonov, E L; Shtutman, V Z; Guseva, N G; Baranov, A A; Targoff, I N; Youinou, P

    1997-01-01

    Sera from 19 patients with idiopathic inflammatory myopathy (IIM) were examined for the presence of anti-endothelial cell antibodies (AECA) by an immunoglobulin G-specific cellular enzyme-linked immunosorbent assay. The mean binding index of AECA was found to be 37.7% +/- 26.5% for the patients, compared with a mean of 7.2% +/- 2.7% for normal controls (P < 0.04). Levels of thrombomodulin, von Willebrand factor antigen, and serum creatine kinase were also shown to be augmented. Interestingly, positive correlations between AECA on the one hand and Raynaud's phenomenon and interstitial lung disease on the other were demonstrated. Given that the pathogenesis of IIM remains uncertain, these findings may be of importance. PMID:9302198

  19. Malignant Hemispheric Cerebral Infarction Associated with Idiopathic Systemic Capillary Leak Syndrome

    PubMed Central

    Miyata, Kei; Mikami, Takeshi; Mikuni, Nobuhiro; Aisaka, Wakiko; Irifune, Hideto; Narimatsu, Eichi

    2013-01-01

    Idiopathic systemic capillary leak syndrome (ISCLS) is a rare condition that is characterized by unexplained episodic capillary hyperpermeability due to a shift of fluid and protein from the intravascular to the interstitial space. This results in diffuse general swelling, fetal hypovolemic shock, hypoalbuminemia, and hemoconcentration. Although ISCLS rarely induces cerebral infarction, we experienced a patient who deteriorated and was comatose as a result of massive cerebral infarction associated with ISCLS. In this case, severe hypotensive shock, general edema, hemiparesis, and aphasia appeared after serious antecedent gastrointestinal symptoms. Progressive life-threatening ischemic cerebral edema required decompressive hemicraniectomy. The patient experienced another episode of severe hypotension and limb edema that resulted in multiple extremity compartment syndrome. Treatment entailed forearm and calf fasciotomies. Cerebral edema in the ischemic brain progresses rapidly in patients suffering from ISCLS. Strict control of fluid volume resuscitation and aggressive diuretic therapy may be needed during the post-leak phase of fluid remobilization. PMID:24163674

  20. Diffuse Neuroendocrine Hyperplasia with Obliterative Bronchiolitis and Usual Interstitial Pneumonia: An Unusual "Headcheese Pattern" with Nodules.

    PubMed

    Pietrangeli, V; Piciucchi, S; Tomassetti, S; Ravaglia, C; Gurioli, C; Gurioli, Ch; Cavazza, A; Dubini, A; Poletti, V

    2015-12-01

    A 74-year-old non-smoker female presented to our attention with a history of dyspnea and cough. CT scan revealed multiple areas of patchy ground glass attenuation associated to a diffuse mosaic oligoemia. Scattered bilateral subcentimetric pulmonary nodules were also present. Patient underwent a surgical lung biopsy. Specimens showed features of diffuse neuroendocrine hyperplasia, microhoneycombing, fibroblast foci. A final diagnosis of diffuse neuroendocrine hyperplasia with obliterative bronchiolitis and UIP was rendered. PMID:26446675

  1. Natural Antioxidant Betanin Protects Rats from Paraquat-Induced Acute Lung Injury Interstitial Pneumonia

    PubMed Central

    Ma, Deshun; Zhang, Miao; Yang, Xuelian; Tan, Dehong

    2015-01-01

    The effect of betanin on a rat paraquat-induced acute lung injury (ALI) model was investigated. Paraquat was injected intraperitoneally at a single dose of 20 mg/kg body weight, and betanin (25 and 100 mg/kg/d) was orally administered 3 days before and 2 days after paraquat administration. Rats were sacrificed 24 hours after the last betanin dosage, and lung tissue and bronchoalveolar lavage fluid (BALF) were collected. In rats treated only with paraquat, extensive lung injury characteristic of ALI was observed, including histological changes, elevation of lung : body weight ratio, increased lung permeability, increased lung neutrophilia infiltration, increased malondialdehyde (MDA) and myeloperoxidase (MPO) activity, reduced superoxide dismutase (SOD) activity, reduced claudin-4 and zonula occluden-1 protein levels, increased BALF interleukin (IL-1) and tumor necrosis factor (TNF)-α levels, reduced BALF IL-10 levels, and increased lung nuclear factor kappa (NF-κB) activity. In rats treated with betanin, paraquat-induced ALI was attenuated in a dose-dependent manner. In conclusion, our results indicate that betanin attenuates paraquat-induced ALI possibly via antioxidant and anti-inflammatory mechanisms. Thus, the potential for using betanin as an auxilliary therapy for ALI should be explored further. PMID:25861636

  2. Natural antioxidant betanin protects rats from paraquat-induced acute lung injury interstitial pneumonia.

    PubMed

    Han, Junyan; Ma, Deshun; Zhang, Miao; Yang, Xuelian; Tan, Dehong

    2015-01-01

    The effect of betanin on a rat paraquat-induced acute lung injury (ALI) model was investigated. Paraquat was injected intraperitoneally at a single dose of 20 mg/kg body weight, and betanin (25 and 100 mg/kg/d) was orally administered 3 days before and 2 days after paraquat administration. Rats were sacrificed 24 hours after the last betanin dosage, and lung tissue and bronchoalveolar lavage fluid (BALF) were collected. In rats treated only with paraquat, extensive lung injury characteristic of ALI was observed, including histological changes, elevation of lung : body weight ratio, increased lung permeability, increased lung neutrophilia infiltration, increased malondialdehyde (MDA) and myeloperoxidase (MPO) activity, reduced superoxide dismutase (SOD) activity, reduced claudin-4 and zonula occluden-1 protein levels, increased BALF interleukin (IL-1) and tumor necrosis factor (TNF)-α levels, reduced BALF IL-10 levels, and increased lung nuclear factor kappa (NF-κB) activity. In rats treated with betanin, paraquat-induced ALI was attenuated in a dose-dependent manner. In conclusion, our results indicate that betanin attenuates paraquat-induced ALI possibly via antioxidant and anti-inflammatory mechanisms. Thus, the potential for using betanin as an auxilliary therapy for ALI should be explored further.

  3. Inflammation-inducing Factors of Mycoplasma pneumoniae

    PubMed Central

    Shimizu, Takashi

    2016-01-01

    Mycoplasma pneumoniae, which causes mycoplasmal pneumonia in human, mainly causes pneumonia in children, although it occasionally causes disease in infants and geriatrics. Some pathogenic factors produced by M. pneumoniae, such as hydrogen peroxide and Community-Acquired Respiratory Distress Syndrome (CARDS) toxin have been well studied. However, these factors alone cannot explain this predilection. The low incidence rate of mycoplasmal pneumonia in infants and geriatrics implies that the strong inflammatory responses induced by M. pneumoniae coordinate with the pathogenic factors to induce pneumonia. However, M. pneumoniae lacks a cell wall and does not possess an inflammation-inducing endotoxin, such as lipopolysaccharide (LPS). In M. pneumoniae, lipoproteins were identified as an inflammation-inducing factor. Lipoproteins induce inflammatory responses through Toll-like receptors (TLR) 2. Because Mycoplasma species lack a cell wall and lipoproteins anchored in the membrane are exposed, lipoproteins and TLR2 have been thought to be important for the pathogenesis of M. pneumoniae. However, recent reports suggest that M. pneumoniae also induces inflammatory responses also in a TLR2-independent manner. TLR4 and autophagy are involved in this TLR2-independent inflammation. In addition, the CARDS toxin or M. pneumoniae cytadherence induces inflammatory responses through an intracellular receptor protein complex called the inflammasome. In this review, the inflammation-inducing factors of M. pneumoniae are summarized. PMID:27065977

  4. Asbestos bodies in bronchoalveolar lavage fluid. A study of 20 asbestos-exposed individuals and comparison to patients with other chronic interstitial lung diseases

    SciTech Connect

    Roggli, V.L.; Piantadosi, C.A.; Bell, D.Y.

    1986-09-01

    We studied the asbestos body (AB) content of bronchoalveolar lavage fluid from 20 patients with a history of occupational asbestos exposure, 31 patients with sarcoidosis and 5 patients with idiopathic pulmonary fibrosis. The cellular lavage pellet was digested in sodium hypochlorite and filtered onto Nuclepore filters for AB quantification by light microscopy. ABs were found in 15 of 20 asbestos-exposed individuals, 9 of 31 sarcoidosis cases and 2 of 5 patients with idiopathic pulmonary fibrosis. There was a statistically significant difference in the number of ABs per million cells recovered or per milliliter of recovered lavage fluid in the asbestos-exposed group as compared to the other categories of chronic interstitial lung disease. The highest levels occurred in patients with asbestosis. Large numbers of asbestos bodies in the lavage fluid (greater than 1 AB/10(6) cells) were indicative of considerable occupational asbestos exposure, whereas occasional bodies were a nonspecific finding.

  5. Computer-assisted interstitial laser coagulation for BPH

    NASA Astrophysics Data System (ADS)

    Ho, Gideon; Barrett, Adrian R. W.; Ng, Wan S.; Lim, Liam G.; Cheng, Wai S.

    2001-06-01

    Interstitial laser thermotherapy is a minimally invasive surgical procedure that utilizes laser to coagulate and treat benign prostatic hyperplasia. This study explores the use of a computer-assisted interstitial laser coagulation system to aid surgeons in performing this procedure.

  6. Nonsurgical Management of Adolescent Idiopathic Scoliosis.

    PubMed

    Gomez, Jaime A; Hresko, M Timothy; Glotzbecker, Michael P

    2016-08-01

    Pediatric patient visits for spinal deformity are common. Most of these visits are for nonsurgical management of scoliosis, with approximately 600,000 visits for adolescent idiopathic scoliosis (AIS) annually. Appropriate management of scoliotic curves that do not meet surgical indication parameters is essential. Renewed enthusiasm for nonsurgical management of AIS (eg, bracing, physical therapy) exists in part because of the results of the Bracing in Adolescent Idiopathic Scoliosis Trial, which is the only randomized controlled trial available on the use of bracing for AIS. Bracing is appropriate for idiopathic curves between 20° and 40°, with successful control of these curves reported in >70% of patients. Patient adherence to the prescribed duration of wear is essential to maximize the effectiveness of the brace. The choice of brace type must be individualized according to the deformity and the patient's personality as well as the practice setting and brace availability. PMID:27388720

  7. [Helicobacter pylori infection and idiopathic thrombocytopenic purpura].

    PubMed

    Franchini, Massimo; Veneri, Dino

    2005-03-01

    There are increasing data on the association between Helicobacter pylori infection and idiopathic thrombocytopenic purpura and the significant increase in platelet count after bacterial eradication. The aim of this review was to consider the studies so far published on Helicobacter pylori infection and idiopathic thrombocytopenic purpura in order to evaluate a possible correlation between these two conditions. A review of the literature showed that 306 out of the 524 patients investigated (54.8%) were positive for Helicobacter pylori infection and that the bacterium was eradicated in 83% of cases. Eradication therapy was accompanied by a complete or partial platelet response in approximately half the cases. Overall, these data show that Helicobacter pylori eradication in patients with idiopathic thrombocytopenic purpura is effective in increasing platelet count. However, since the studies so far published are few, sometimes controversial and involve small series of patients, further studies on larger numbers of patients with longer followup are needed to confirm these preliminary findings.

  8. Macrolide Resistance in Streptococcus pneumoniae

    PubMed Central

    Schroeder, Max R.; Stephens, David S.

    2016-01-01

    Streptococcus pneumoniae is a common commensal and an opportunistic pathogen. Suspected pneumococcal upper respiratory infections and pneumonia are often treated with macrolide antibiotics. Macrolides are bacteriostatic antibiotics and inhibit protein synthesis by binding to the 50S ribosomal subunit. The widespread use of macrolides is associated with increased macrolide resistance in S. pneumoniae, and the treatment of pneumococcal infections with macrolides may be associated with clinical failures. In S. pneumoniae, macrolide resistance is due to ribosomal dimethylation by an enzyme encoded by erm(B), efflux by a two-component efflux pump encoded by mef (E)/mel(msr(D)) and, less commonly, mutations of the ribosomal target site of macrolides. A wide array of genetic elements have emerged that facilitate macrolide resistance in S. pneumoniae; for example erm(B) is found on Tn917, while the mef (E)/mel operon is carried on the 5.4- or 5.5-kb Mega element. The macrolide resistance determinants, erm(B) and mef (E)/mel, are also found on large composite Tn916-like elements most notably Tn6002, Tn2009, and Tn2010. Introductions of 7-valent and 13-valent pneumococcal conjugate vaccines (PCV-7 and PCV-13) have decreased the incidence of macrolide-resistant invasive pneumococcal disease, but serotype replacement and emergence of macrolide resistance remain an important concern. PMID:27709102

  9. Common variable immune deficiency in a Pomeranian with Pneumocystis carinii pneumonia

    PubMed Central

    KANEMOTO, Hideyuki; MORIKAWA, Rei; CHAMBERS, James Kenn; KASAHARA, Koichi; HANAFUSA, Yasuko; UCHIDA, Kazuyuki; OHNO, Koichi; NAKAYAMA, Hiroyuki

    2015-01-01

    A Pomeranian dog, 1 year- and 8 month-old neutered female, was presented with persistent respiratory distress and recurrent generalized demodicosis. Physical examination revealed cyanosis, rough respiratory sounds, multifocal alopecia and dermal erosions on the dorsal side of the forelimbs, perineal area and skin around the eyes. A severe diffuse interstitial lung pattern was observed on thoracic radiographs. The blood examination revealed neutrophilia and hypoglobulinemia. Serum immunoglobulin concentrations of IgG and IgA were low. Histopathological examination revealed severe diffuse interstitial pneumonia with Pneumocystis carinii infection. Severe lymphoid depletion was observed in the spleen and other organs with lymphoid follicles consisted mainly of CD3-positive T cells and few cells of B-cell lineage. B-cell hypoplasia with subsequent antibody deficiency was suspected. PMID:25715954

  10. [Cough syncope caused by a possible Chlamydia pneumoniae pneumonia].

    PubMed

    Cinotti, R; Moubarak, G; Gervais, R; Mabo, P

    2009-09-01

    We report the case of a 61-year-old man who presented with coughing fits followed by sinus pauses and syncope. Cardiac and neurological diagnostic work-up was negative and the patient was considered to have cough syncope. As this occurred within the context of febrile pneumonia, an infectious disease was suspected but diagnostic work-up only revealed an increase of antibodies against Chlamydia pneumoniae. The responsibility of this agent is discussed. Clinical recovery was obtained with the prescription of antitussive medication.

  11. Studies of an outbreak of Corynebacterium equi pneumonia in foals.

    PubMed

    Smith, B P; Robinson, R C

    1981-10-01

    Five out of 6 foals between 2 and 4 months old, on a ranch in northern California, developed pneumonia within a 3 week period in June and July 1978. Corynebacterium equi was recovered from each of the 5 foals by transtracheal aspiration. Clinical signs were variable but included increased respiratory rate, fever, cough, nasal discharge, harsh airway sounds over middle sized airways and wheezing over small airways. Cyanosis was present in the most severely affected foal. Radiographic findings included diffusely increased interstitial and peribronchial densities, areas of consolidation and, in 3 cases, dense focal areas indicating abscessation. Foals were treated with several different antimicrobial agents. Most were treated with penicillin and gentamicin. Four of the 5 affected foals recovered within 2 to 3 weeks but the first foal to be affected died 2 days after first receiving veterinary attention. At postmortem examination, pulmonary changes considered typical of C equi pneumonia were found, including wet, heavy dark red lungs which failed to collapse and numerous 1 to 7 cm thin-walled abscesses throughout the parenchyma, containing inspissated exudate. C equi was cultured from the exudate. Samples of soil and dust from 9 of 20 areas inhabited by infected foals yielded C equi of the same serological group as found in the foals. Eight paddocks in which foals had not been kept were negative for C equi. The organism was recovered from cobwebs in the stalls occupied by infected foals. Aerosol infection via dust was considered to be the route of infection. Pharyngeal, vaginal and faecal cultures from the dams of 3 affected foals were negative for C equi. Early diagnosis by transtracheal aspiration and appropriate therapy are considered to be extremely important in the successful treatment of C equi pneumonia. Preventive therapy should include control of environmental dust. PMID:7318800

  12. Edemagenic gain and interstitial fluid volume regulation.

    PubMed

    Dongaonkar, R M; Quick, C M; Stewart, R H; Drake, R E; Cox, C S; Laine, G A

    2008-02-01

    Under physiological conditions, interstitial fluid volume is tightly regulated by balancing microvascular filtration and lymphatic return to the central venous circulation. Even though microvascular filtration and lymphatic return are governed by conservation of mass, their interaction can result in exceedingly complex behavior. Without making simplifying assumptions, investigators must solve the fluid balance equations numerically, which limits the generality of the results. We thus made critical simplifying assumptions to develop a simple solution to the standard fluid balance equations that is expressed as an algebraic formula. Using a classical approach to describe systems with negative feedback, we formulated our solution as a "gain" relating the change in interstitial fluid volume to a change in effective microvascular driving pressure. The resulting "edemagenic gain" is a function of microvascular filtration coefficient (K(f)), effective lymphatic resistance (R(L)), and interstitial compliance (C). This formulation suggests two types of gain: "multivariate" dependent on C, R(L), and K(f), and "compliance-dominated" approximately equal to C. The latter forms a basis of a novel method to estimate C without measuring interstitial fluid pressure. Data from ovine experiments illustrate how edemagenic gain is altered with pulmonary edema induced by venous hypertension, histamine, and endotoxin. Reformulation of the classical equations governing fluid balance in terms of edemagenic gain thus yields new insight into the factors affecting an organ's susceptibility to edema.

  13. Edemagenic gain and interstitial fluid volume regulation.

    PubMed

    Dongaonkar, R M; Quick, C M; Stewart, R H; Drake, R E; Cox, C S; Laine, G A

    2008-02-01

    Under physiological conditions, interstitial fluid volume is tightly regulated by balancing microvascular filtration and lymphatic return to the central venous circulation. Even though microvascular filtration and lymphatic return are governed by conservation of mass, their interaction can result in exceedingly complex behavior. Without making simplifying assumptions, investigators must solve the fluid balance equations numerically, which limits the generality of the results. We thus made critical simplifying assumptions to develop a simple solution to the standard fluid balance equations that is expressed as an algebraic formula. Using a classical approach to describe systems with negative feedback, we formulated our solution as a "gain" relating the change in interstitial fluid volume to a change in effective microvascular driving pressure. The resulting "edemagenic gain" is a function of microvascular filtration coefficient (K(f)), effective lymphatic resistance (R(L)), and interstitial compliance (C). This formulation suggests two types of gain: "multivariate" dependent on C, R(L), and K(f), and "compliance-dominated" approximately equal to C. The latter forms a basis of a novel method to estimate C without measuring interstitial fluid pressure. Data from ovine experiments illustrate how edemagenic gain is altered with pulmonary edema induced by venous hypertension, histamine, and endotoxin. Reformulation of the classical equations governing fluid balance in terms of edemagenic gain thus yields new insight into the factors affecting an organ's susceptibility to edema. PMID:18056984

  14. Reversible Fluindione-Induced Chronic Interstitial Nephritis

    PubMed Central

    Crepin, Thomas; Bamoulid, Jamal; Courivaud, Cécile; Dahmani, Omar; Felix, Sophie; Ducloux, Didier

    2016-01-01

    Fluindione is well known to induce acute drug-induced interstitial nephritis (IN). Most cases occurred soon after the onset of treatment. We report a unique case of severe subacute fluindione-induced IN diagnosed 2 years after the treatment was begun. Renal function dramatically improved after fluindione withdrawal and steroid therapy. PMID:27127666

  15. Nerve fibre proliferation in interstitial cystitis.

    PubMed

    Christmas, T J; Rode, J; Chapple, C R; Milroy, E J; Turner-Warwick, R T

    1990-01-01

    The aetiology of pain in interstitial cystitis is not understood, although it has been reported to be due to release of mediators from mast cell granules. Cystolysis and intravesical instillation of dimethyl sulphoxide have been shown to relieve pain in this condition. We have studied the nerve population within the bladder wall using immunohistochemical stains for protein gene product 9.5. A group of 18 cases of chronic interstitial cystitis and 12 controls; neuropathic bladder (n = 1), chronic bacterial cystitis (n = 3), systemic lupus erythematosus cystitis (n = 2) and normals (n = 6), were investigated. There were significantly more nerve fibres within the sub-urothelial and detrusor muscle layers in chronic interstitial cystitis than there were in normals. Patients with chronic cystitis of other aetiology did not have a significant increase in nerve fibre density within the bladder wall suggesting a specific association between nerve fibre proliferation and interstitial cystitis. Cystolysis is shown to deplete selectively the submucosal nerve plexuses without altering the nerve density within detrusor muscle. This finding explains the desensitisation of the bladder without impairment of detrusor function after this procedure.

  16. Idiopathic congenital chylothorax treated with octreotide

    PubMed Central

    Sousa, Paulo Rego; Leitão, Henrique; Camacho, Maria Carmo; Nunes, José Luis

    2010-01-01

    Idiopathic congenital chylothorax is defined as an abnormal accumulation of lymphatic fluid within the pleural space and is a relatively rare condition. It is a cause of progressive respiratory distress with nutritional and immunological consequences. Treatment of congenital chylothorax has been conservative management and cases unresponsive usually require surgery. We report a case of idiopathic congenital chylothorax treated with octreotide (a somatostatin analogue), avoiding surgery after failed conservative medical treatment. The patient promptly improved after initiation of endovenous octreotide treatment (10 µg/kg/h) with no observed side effects. PMID:22750919

  17. Idiopathic Parkinson's disease: epidemiology, diagnosis and management.

    PubMed Central

    Ben-Shlomo, Y; Sieradzan, K

    1995-01-01

    Since the introduction of levodopa therapy for idiopathic Parkinson's disease over 20 years ago, there has been an awakening of research interest in this chronic neuro-degenerative disorder. This paper describes current understanding of the role of genetic and environmental factors in the aetiology of idiopathic Parkinson's disease and problems associated with both diagnosis and management. It briefly outlines both pharmacological and non-pharmacological options for treatment. Despite an increasing armoury of available treatments, the optimum management for this condition remains controversial. PMID:7619574

  18. Glucocorticoid pharmacogenetics in pediatric idiopathic nephrotic syndrome.

    PubMed

    Cuzzoni, Eva; De Iudicibus, Sara; Franca, Raffaella; Stocco, Gabriele; Lucafò, Marianna; Pelin, Marco; Favretto, Diego; Pasini, Andrea; Montini, Giovanni; Decorti, Giuliana

    2015-01-01

    Idiopathic nephrotic syndrome represents the most common type of primary glomerular disease in children: glucocorticoids (GCs) are the first-line therapy, even if considerable interindividual differences in their efficacy and side effects have been reported. Immunosuppressive and anti-inflammatory effects of these drugs are mainly due to the GC-mediated transcription regulation of pro- and anti-inflammatory genes. This mechanism of action is the result of a complex multistep pathway that involves the glucocorticoid receptor and several other proteins, encoded by polymorphic genes. Aim of this review is to highlight the current knowledge on genetic variants that could affect GC response, particularly focusing on children with idiopathic nephrotic syndrome.

  19. A Case of Idiopathic Thyrotropin (TSH) Deficiency

    PubMed Central

    Ichida, Tatsuya; Kajita, Yoshihiro

    1997-01-01

    The first case of idiopathic thyrotropin (TSH) deficiency in an old woman with thyroid functioning adenoma was reported. She got subtotal thyroidectomy before about four years of her admission to our hospital because of fatigability, puffy face and leg edema. At that time, she had low TSH and free T4 levels despite replacement therapy with desiccated thyroid. No response of only serum TSH after adminstration of combined stimulant containing TRH and repeated TRH suggested the failure of TSH secretion. CT MRI did not show any abnormality. These results indicated that her hypothyroidism was due to acquired idiopathic TSH deficiency. PMID:9159047

  20. A surgical challenge: Idiopathic scrotal elephantiasis

    PubMed Central

    Brotherhood, Hilary Laurel; Metcalfe, Michael; Goldenberg, Larry; Pommerville, Peter; Bowman, Cameran; Naysmith, David

    2014-01-01

    Scrotal elephantiasis is a condition rarely encountered in developed nations. It is endemic in tropical regions due to the presence of filariasis (Wucheria bancrofti). We report 2 cases of idiopathic scrotal elephantiasis in Canadian citizens with no history of travel to endemic filariasis regions, malignancy, surgery or radiation. Both patients underwent complete excision of the involved tissue with reconstruction. We found that for advanced cases of scrotal lymphedema, surgery is currently the only solution. In our cases of advanced idiopathic disease, surgical treatment combining the expertise of a plastic surgeon and a urologist provided a successful functional and cosmetic result. PMID:25132898

  1. Idiopathic Pulmonary Fibrosis and the Elderly: Diagnosis and Management Considerations.

    PubMed

    Jo, Helen E; Randhawa, Sharan; Corte, Tamera J; Moodley, Yuben

    2016-05-01

    Idiopathic pulmonary fibrosis (IPF) is a severe and progressive fibrosing interstitial lung disease, which ultimately results in respiratory failure and death. The median age at diagnosis is 66 years, and the incidence increases with age, making this a disease that predominantly affects the elderly population. IPF can often be difficult to diagnose, as its symptoms--cough, dyspnoea and fatigue--are non-specific and can often be attributed to co-morbidities such as heart failure and chronic obstructive pulmonary disease. Making an accurate diagnosis of IPF is imperative, as new treatments that appear to slow the progression of IPF have recently become available. Pirfenidone and nintedanib are two such treatments, which have shown efficacy in randomised controlled trials. As with all new treatments, caution must be advocated in the elderly, as these patients often lie outside the narrow clinical trial cohorts that are studied, and the benefits of therapy must be weighed against potential toxicities. Both medications, while relatively safe, have been associated with adverse effects, particularly gastrointestinal symptoms such as nausea, diarrhoea and anorexia. In this review, we highlight measures to improve recognition and accurate diagnosis of IPF, as well as co-morbidities that often affect the diagnosis and disease course. The gold standard for IPF diagnosis is a multidisciplinary meeting whereby clinicians, radiologists and histopathologists reach a consensus after interactive discussion. In many cases, a lung biopsy may not be available because of high risk or patient choice, particularly in the elderly. In these cases, there is debate as to whether a biopsy is required, given the high rates of IPF in patients over the age of 70 years with interstitial changes on computed tomography. We also discuss the management of IPF, drawing particular attention to specific issues affecting the elderly population, especially with regard to polypharmacy and end-of-life care

  2. The Clinical Phenotypes of the Juvenile Idiopathic Inflammatory Myopathies

    PubMed Central

    Shah, Mona; Mamyrova, Gulnara; Targoff, Ira N.; Huber, Adam M.; Malley, James D.; Rice, Madeline Murguia; Miller, Frederick W.; Rider, Lisa G.

    2015-01-01

    The juvenile idiopathic inflammatory myopathies (JIIM) are systemic autoimmune diseases characterized by skeletal muscle weakness, characteristic rashes and other systemic features. Although juvenile dermatomyositis (JDM), the most common form of JIIM, has been well-studied, the other major clinical subgroups of JIIM, including juvenile polymyositis (JPM) and juvenile myositis overlapping with another autoimmune or connective tissue disease (JCTM), have not been well characterized, and their similarity to the adult clinical subgroups is also unknown. We enrolled 436 patients with JIIM, including 354 classified as JDM, 33 as JPM and 49 as JCTM, in a nationwide registry study. The aim of this study was to compare demographics, clinical features, laboratory measures, including myositis autoantibodies, and outcomes, among these clinical subgroups, as well as with published data on adult IIM patients enrolled in a separate natural history study. Random forest classification and logistic regression modeling were used to compare clinical subgroups, following univariate analysis. JDM was characterized by typical rashes, including Gottron’s papules, heliotrope rash, malar rash, periungual capillary changes and other photosensitive and vasculopathic skin rashes. JPM was characterized by more severe weakness, higher creatine kinase levels, falling episodes and more frequent cardiac disease. JCTM had more frequent interstitial lung disease, Raynaud’s phenomenon, arthralgia and malar rash. Differences in autoantibody frequency were also evident, with anti-p155, anti-MJ and anti-Mi2 seen more frequently in patients with JDM, anti-signal recognition particle and anti-Jo1 in JPM, and anti-U1RNP, PM-Scl and other myositis-associated autoantibodies more commonly present in JCTM. Mortality was highest in JCTM, whereas hospitalizations and wheelchair usage were highest in JPM patients. Several demographic and clinical features were shared between juvenile and adult IIM subgroups

  3. Equid herpesvirus 1 and rhodococcus equi coinfection in a foal with bronchointerstitial pneumonia

    PubMed Central

    PEREZ-ECIJA, Alejandro; MENDOZA, Francisco Javier; ESTEPA, José Carlos; BAUTISTA, María José; PÉREZ, José

    2016-01-01

    A 2-month-old foal with septic shock and severe respiratory distress was referred to the Veterinary Teaching Hospital. Due to poor prognosis, the foal was euthanized. Histopathology showed lesions suggestive of Rhodococcus equi infection associated with a diffuse interstitial infiltrate of foamy macrophages and syncytial cells presenting large acidophilic intranuclear inclusion bodies, fibrin exudates and hyaline membranes. Bacteriological examination from lung and respiratory exudates confirmed R. equi infection, whilst immunohistochemistry and PCR yielded a positive result for Equid herpesvirus type 1 (EHV-1). Several etiologies have been proposed for bronchointerstitial pneumonia in foals, although a multifactorial origin for this lesional pattern could be possible. This work is the first one describing a combined EHV-1 and R. equi infection in a foal affected with bronchointerstitial pneumonia. PMID:27264610

  4. Experimental aerosolized guinea pig-adapted Zaire ebolavirus (variant: Mayinga) causes lethal pneumonia in guinea pigs.

    PubMed

    Twenhafel, N A; Shaia, C I; Bunton, T E; Shamblin, J D; Wollen, S E; Pitt, L M; Sizemore, D R; Ogg, M M; Johnston, S C

    2015-01-01

    Eight guinea pigs were aerosolized with guinea pig-adapted Zaire ebolavirus (variant: Mayinga) and developed lethal interstitial pneumonia that was distinct from lesions described in guinea pigs challenged subcutaneously, nonhuman primates challenged by the aerosol route, and natural infection in humans. Guinea pigs succumbed with significant pathologic changes primarily restricted to the lungs. Intracytoplasmic inclusion bodies were observed in many alveolar macrophages. Perivasculitis was noted within the lungs. These changes are unlike those of documented subcutaneously challenged guinea pigs and aerosolized filoviral infections in nonhuman primates and human cases. Similar to findings in subcutaneously challenged guinea pigs, there were only mild lesions in the liver and spleen. To our knowledge, this is the first report of aerosol challenge of guinea pigs with guinea pig-adapted Zaire ebolavirus (variant: Mayinga). Before choosing this model for use in aerosolized ebolavirus studies, scientists and pathologists should be aware that aerosolized guinea pig-adapted Zaire ebolavirus (variant: Mayinga) causes lethal pneumonia in guinea pigs.

  5. Unsuspected Pneumocystis carinii pneumonia and vertically acquired HIV infection in infants requiring intensive care.

    PubMed Central

    Tasker, R. C.; Wilkinson, K.; Slater, T. J.; Novelli, V.

    1994-01-01

    When an infant develops acute respiratory failure of sufficient severity to necessitate supportive mechanical ventilation a cause should always be sought. A chest radiograph showing predominantly interstitial lung disease and an infant's failure to respond to standard antibiotic treatment are indications for non-bronchoscopic bronchoalveolar lavage. If P carinii pneumonia is diagnosed a congenital immunodeficiency should be sought and the parents counselled about HIV infection. Earlier investigation may be indicated by features of immunodeficiency when taking a history, performing a general examination, or analysing the results of basic haematological testing. Images p462-a PMID:8124183

  6. Respiratory syncytial virus pneumonia in a lung transplant recipient: case report.

    PubMed

    Doud, J R; Hinkamp, T; Garrity, E R

    1992-01-01

    A 29-year-old man underwent bilateral lung transplantation and received maintenance immunosuppressive therapy. He was readmitted 11 months later with symptoms of cough, sneezing, and rhinorrhea. The physical examination was normal. Laboratory results were significant for a reduction of FEV1 and an interstitial infiltrate on chest films. The patient had recently undergone bronchoscopy for rejection surveillance, and 2 days before admission the bronchoalveolar lavage cultures returned positive for respiratory syncytial virus. The patient was treated with aerosolized ribavirin with complete resolution of symptoms. Respiratory syncytial virus must now be included in the list of pathogens causing pneumonia in the lung transplant recipient. PMID:1540615

  7. Acute fibrinous and organising pneumonia.

    PubMed

    Guimarães, Catarina; Sanches, Inês; Ferreira, Catarina

    2012-03-20

    Acute fibrinous and organising pneumonia (AFOP) was recently described as an unusual pattern of diffuse lung disease. Particular characteristics make the differential diagnosis with the well recognised clinical patterns of diffuse alveolar damage, cryptogenic organising pneumonia or eosinophilic pneumonia. The lack of hyaline membranes, the presence of intra-alveolar fibrin, absence of noticeable eosinophils and patchy distribution suggests that AFOP define a distinct histological pattern. The authors describe the case of a woman diagnosed with AFOP after surgical lung biopsy, in association with primary biliary cirrhosis. The patient presented dyspnoea, fatigue, dry cough and thoracic pain. The CT scan showed bilateral patchy infiltrates predominantly in the lower lobes. Flexible bronchoscopy and subsidiary techniques were inconclusive and biopsy through video-assisted thoracoscopic surgery led to anatomopathological diagnosis of AFOP. The patient is having a good clinical response to prednisone.

  8. Hyaluronan in Tubular and Interstitial Nephrocalcinosis

    NASA Astrophysics Data System (ADS)

    Verkoelen, Carl F.

    2007-04-01

    Hyaluronan (HA) is the major glycosaminoglycan (GAG) component of the renal medullary interstitium. HA is extremely large (up to 104 kDa) and composed of thousands repeating disaccharides of glucuronic acid (GlcUA) and N-acetylglucosamine (GlcNAc). HA is synthesized by hyaluronan synthases (HASs) and degraded by hyaluronidases (Hyals). The production of HA by renomedullary interstitial cells is mediated by local osmolality. When excess water needs to be excreted, increased interstitial HA seems to antagonize water reabsorption, while the opposite occurs during water conservation. Hence, papillary interstitial HA is low and Hyal high during anti-diuresis, whereas during diuresis HA is high and Hyal low. The polyanion HA plays a role in the reabsorption of hypotonic fluid by immobilizing cations (Na+) via the carboxylate (COO-) groups of GlcUA. The binding of Ca2+ to anionic HA is probably also responsible for the fact that the papilla does not become a stone despite the extremely high interstitial phosphate and oxalate. HA is also an excellent crystal binding molecule. The expression of HA at the luminal surface of renal tubular cells leads to tubular nephrocalcinosis (tubular NC). Calcium staining methods (Von Kossa, Yasue) demonstrated that crystallization inhibitors cannot avoid the occasional precipitation of calcium phosphate in the papillary interstitium (interstitial NC). These crystals are probably immediately immobilized by the gel-like HA matrix. After ulcerating through the pelvic wall the calcified matrix becomes a Randall's plaque. The attachment of calcium oxalate crystals from the primary urine to plaque may ultimately lead to the development of clinical stones in the renal calyces (nephrolithiasis).

  9. Clinical features and prognosis of patients with idiopathic inflammatory myopathies and anti-Jo-1 antibodies.

    PubMed

    Mielnik, Pawel; Wiesik-Szewczyk, Ewa; Olesinska, Marzena; Chwalinska-Sadowska, Hanna; Zabek, Jakub

    2006-05-01

    The idiopathic inflammatory myopathies are a heterogeneous group of diseases that can involve various systems. Antibodies directed against aminoacyl-tRNA synthetases, such as anti-Jo-1 antibodies, are strongly associated with a syndrome which consists of myositis, interstitial lung disease (ILD), arthritis and Raynaud's phenomenon. Forty-one patients with various forms of idiopathic inflammatory myopathies were assessed: 14 patients with anti-Jo-1 antibodies and 27 patients without anti-Jo-1 antibodies as a control group. We retrospectively analysed clinical symptoms, treatment and outcome in both groups. Patients with anti-Jo-1 antibodies more often had ILD (64.2 vs. 11.1%), arthritis (64.2 vs. 18.1%) and Raynaud's phenomenon (38 vs. 0%). Patients without the anti-Jo-1 antibody presented worse muscle strength and more frequently myalgia (37 vs. 21%), cutaneous rash (18.5 vs. 7%), heliotrope rash (29% vs. 7%) and periungueal changes (22 vs. 0%) than the anti-Jo-1-positive patients. Outcome was good in both groups. Improvement was achieved in the 14 (100%) Jo-1 positive patients, and in 25 (92.5%) controls. Two (7.5%) patients from control group achieved remission.

  10. [Severe haemolysis caused by Mycoplasma pneumoniae].

    PubMed

    Bohr, Anne Lisbeth; Aagaard, Thomas Granum; Birgens, Henrik; Søborg, Christian

    2015-10-01

    Mycoplasma pneumoniae is naturally resistant to betalactamase antibiotics but is sensitive to macrolides. Occasionally, infections with M. pneumoniae can lead to severe anaemia due to its ability to cause haemolysis when cold agglutination occurs. Increasing bacterial resistance to macrolid antibiotics is a growing concern worldwide. We present two cases where infection with M. pneumoniae caused severe haemolysis, one of which was macrolide-resistant.

  11. How Radiotherapy Was Historically Used To Treat Pneumonia: Could It Be Useful Today?

    PubMed Central

    Calabrese, Edward J.; Dhawan, Gaurav

    2013-01-01

    X-ray therapy was used to treat pneumonia during the first half of the 20th century. Fifteen studies report that approximately 700 cases of bacterial (lobar and bronchopneumonia), sulfanilamide non-responsive, interstitial, and atypical pneumonia were effectively treated by low doses of X-rays, leading to disease resolution, based on clinical symptoms, objective disease biomarkers, and mortality incidence. The capacity of the X-ray treatment to reduce mortality was similar to serum therapy and sulfonamide treatment during the same time period. Studies with four experimental animal models (i.e., mice, guinea pig, cat, and dog) with bacterial and viral pneumonia supported the clinical findings. The mechanism by which the X-ray treatment acts upon pneumonia involves the induction of an anti-inflammatory phenotype that leads to a rapid reversal of clinical symptoms, facilitating disease resolution. The capacity of low doses of X-rays to suppress inflammatory responses is a significant new concept with widespread biomedical and therapeutic applications. PMID:24348219

  12. [Current Status of Preoperative Professional Oral Care by Dentists for Elderly Patients Undergoing Lung Resection and Occurrence of Postoperative Pneumonia].

    PubMed

    Hoshikawa, Yasushi; Tanda, Naoko; Matsuda, Yasushi; Katsumata, Hiroshi; Notsuda, Hirotsugu; Watanabe, Tatsuaki; Niikawa, Hiromichi; Noda, Masafumi; Sakurada, Akira; Kondo, Takashi; Okada, Yoshinori

    2016-01-01

    Pneumonia in elderly people is mainly caused by silent aspiration due to an age-related impairment of cough and swallowing reflexes. Because most of the patients with lung cancer are elderly people, we hypothesized that the age-related impairment of these protective reflexes might exist or occur in patients undergoing lung surgery, and cause postoperative pneumonia. We revealed that many elderly patients showed depressed swallowing reflex even before surgery and transient attenuation of cough reflex after surgery, and that postoperative pneumonia occurred only in the patients whose cough and/or swallowing reflex was abnormal postoperatively. Then, we prospectively showed that 30 elderly patients who received perioperative intensive oral care, including professional assessment of oral status, dental cleaning, and patient education for self-oral care by dentists, followed by intensive oral care by intensive care unit nurses, and encouragement of self-oral care by floor nurses, did not develop pneumonia after lung resection. In this study, we retrospectively reviewed the execution status of professional oral care by dentists and the occurrence of postoperative pneumonia in 159 consecutive patients aged 65 or older undergoing lung resection from 2013 to 2014. Thoracic surgeons in our institute asked dentists to provide professional oral care before lung resection only in 30.3% of the subjects in 2013, and 45.8% in 2014. Postoperative pneumonia occurred in 3 out of 76 subjects(3.9%)in 2013, and 1 out of 83(1.2%) in 2014. In 2013, 1 patient who did not receive preoperative professional oral care developed aspiration pneumonia postoperatively followed by acute exacerbation of idiopathic pulmonary fibrosis and in-hospital death. We need to make an effective system to provide preoperative professional oral care by dentists especially for elderly patients and high-risk patients before lung resection.

  13. Nocardia brasiliensis infection mimicking juvenile idiopathic arthritis in a 4-year-old girl.

    PubMed

    Kapur, Nitin; Adib, Navid; Grimwood, Keith

    2013-11-01

    Nocardia are ubiquitous environmental saprophytes that cause pneumonia and disseminated disease in immunocompromised patients. They can also cause localized cutaneous and soft tissue infections in healthy people after direct percutaneous inoculation. Nocardia arthritis is rare in both forms of the disease. Here we present the first published case of a child with septic arthritis caused by N brasiliensis. Importantly, this otherwise well 4-year-old girl had no known history of trauma but presented with transient cutaneous lesions and a 6-week history of arthritis involving the right fourth digit proximal interphalangeal joint without accompanying fever or raised systemic inflammatory markers. She received a diagnosis of juvenile idiopathic arthritis and underwent antiinflammatory and immunosuppressant therapy. After 2 months she developed frank septic arthritis, which necessitated a surgical joint washout, from which an intraoperative swab grew N brasiliensis. The patient received 6 months of high-dose trimethoprim-sulfamethoxazole and remains well more than 4 years after treatment. This unusual case highlights the importance of considering an indolent infection from slow-growing organisms, including Nocardia, when diagnosing the oligoarthritis subtype of juvenile idiopathic arthritis. This is especially relevant when a single joint is involved and response to antiinflammatory therapy is suboptimal because antiinflammatory agents may mask evolving signs of infection.

  14. Idiopathic segmental sclerosis of vertebral bodies

    SciTech Connect

    McCarthy, E.F.; Dorfman, H.D.

    1982-12-01

    Five cases of idiopathic vetebral sclerosis are presented. The features of this condition are segmental vertebral sclerosis of a single lumbar vertebra in a young adult without disc space narrowing or alteration of vertebral contour. The differential diagnosis is discussed. Lumbar vertebra biopsies of three patients showed reactive nonspecific osteosclerosis.

  15. Idiopathic Arterial Calcification of Infancy: Case Report.

    PubMed

    Attia, Tarek Hamed; Abd Alhamed, Mohamed Maisara; Selim, Mohamed Fouad; Haggag, Mohamed Salah; Fathalla, Diaa

    2015-11-01

    Idiopathic arterial calcification of infancy is a rare autosomal recessive disease, characterized by deposition of calcium along the internal elastic membrane of arteries, accompanied by fibrous thickening of the intima which causes luminal narrowing. Here we are reporting a case of idiopathic arterial calcification of infancy in a Saudi female newborn of non-consanguineous pregnant woman who had polyhydramnios. The newborn baby had severe respiratory distress, systemic hypertension and persistent pulmonary hypertension of newborn. She was admitted to Neonatal Intensive Care Unit, where she was ventilated and proper treatment was provided. Molecular genetic testing was positive for mutations of ectonucleotide pyrophosphatase/phosphodiesterase1 gene which is reported in 80% of cases of Idiopathic arterial calcification of infancy. The baby died at about 5 month of age because of myocardial ischemia and cardiorespiratory arrest. Idiopathic Arterial Calcification of Infancy should be considered in any newborn who presented with persistent pulmonary hypertension of newborn, severe systemic hypertension and echogenic vessels on any radiological study. Calcifications of large and medium-sized arteries are important diagnostic finding.

  16. [Idiopathic hypereosinophilic syndrome presenting with oculomotor paresis].

    PubMed

    Rafik, R; Bourazza, A; Karouache, A; Mounach, J; Boutaleb, N; Ouhabi, H; Mossadeq, R

    2004-12-01

    A 42-year-old patient with a known depressive syndrome developed diplopia. The neurological examination revealed a peripheral neurogenic syndrome with incoordination of the left arm. Biological and radiological findings were in agreement with the diagnosis of idiopathic hypereosinophilic syndrome. Treatment with corticosteroids was effective.

  17. Resistin in idiopathic inflammatory myopathies

    PubMed Central

    2012-01-01

    Introduction The purpose of this study was to evaluate and compare the serum levels and local expression of resistin in patients with idiopathic inflammatory myopathies to controls, and to determine the relationship between resistin levels, inflammation and disease activity. Methods Serum resistin levels were determined in 42 patients with inflammatory myopathies and 27 healthy controls. The association among resistin levels, inflammation, global disease activity and muscle strength was examined. The expression of resistin in muscle tissues from patients with inflammatory myopathies and healthy controls was evaluated. Gene expression and protein release from resistin-stimulated muscle and mononuclear cells were assessed. Results In patients with inflammatory myopathies, the serum levels of resistin were significantly higher than those observed in controls (8.53 ± 6.84 vs. 4.54 ± 1.08 ng/ml, P < 0.0001) and correlated with C-reactive protein (CRP) levels (r = 0.328, P = 0.044) and myositis disease activity assessment visual analogue scales (MYOACT) (r = 0.382, P = 0.026). Stronger association was observed between the levels of serum resistin and CRP levels (r = 0.717, P = 0.037) as well as MYOACT (r = 0.798, P = 0.007), and there was a trend towards correlation between serum resistin and myoglobin levels (r = 0.650, P = 0.067) in anti-Jo-1 positive patients. Furthermore, in patients with dermatomyositis, serum resistin levels significantly correlated with MYOACT (r = 0.667, P = 0.001), creatine kinase (r = 0.739, P = 0.001) and myoglobin levels (r = 0.791, P = 0.0003) and showed a trend towards correlation with CRP levels (r = 0.447, P = 0.067). Resistin expression in muscle tissue was significantly higher in patients with inflammatory myopathies compared to controls, and resistin induced the expression of interleukins (IL)-1β and IL-6 and monocyte chemoattractant protein (MCP)-1 in mononuclear cells but not in myocytes. Conclusions The results of this study

  18. Idiopathic pulmonary fibrosis in BRIC countries: the cases of Brazil, Russia, India, and China.

    PubMed

    Richeldi, Luca; Rubin, Adalberto Sperb; Avdeev, Sergey; Udwadia, Zarir F; Xu, Zuo Jun

    2015-09-24

    Idiopathic pulmonary fibrosis (IPF), the prototype of interstitial lung diseases, has the worst prognosis and is the only interstitial lung disease for which approved pharmacological treatments are available. Despite being considered a rare disease, IPF patients pose major challenges to both physicians and healthcare systems. It is estimated that a large number of IPF patients reside in BRIC countries (Brazil, Russia, India, and China) given their overall total population of approximately 3 billion inhabitants. Nevertheless, the limited availability of chest imaging in BRIC countries is considered a chief obstacle to diagnosis, since high-resolution computed tomography of the chest is the key diagnostic test for IPF. Further, obtaining reliable lung function tests and providing treatment access is difficult in the more rural areas of these countries. However, IPF might represent an opportunity for BRIC countries: the exponentially increasing demand for the enrollment of IPF patients in clinical trials of new drugs is predicted to face a shortage of patients - BRIC countries may thus play a crucial role in advancing towards a cure for IPF.

  19. Comparison of Active Drug Concentrations in the Pulmonary Epithelial Lining Fluid and Interstitial Fluid of Calves Injected with Enrofloxacin, Florfenicol, Ceftiofur, or Tulathromycin

    PubMed Central

    Foster, Derek M.; Martin, Luke G.; Papich, Mark G.

    2016-01-01

    Bacterial pneumonia is the most common reason for parenteral antimicrobial administration to beef cattle in the United States. Yet there is little information describing the antimicrobial concentrations at the site of action. The objective of this study was to compare the active drug concentrations in the pulmonary epithelial lining fluid and interstitial fluid of four antimicrobials commonly used in cattle. After injection, plasma, interstitial fluid, and pulmonary epithelial lining fluid concentrations and protein binding were measured to determine the plasma pharmacokinetics of each drug. A cross-over design with six calves per drug was used. Following sample collection and drug analysis, pharmacokinetic calculations were performed. For enrofloxacin and metabolite ciprofloxacin, the interstitial fluid concentration was 52% and 78% of the plasma concentration, while pulmonary fluid concentrations was 24% and 40% of the plasma concentration, respectively. The pulmonary concentrations (enrofloxacin + ciprofloxacin combined) exceeded the MIC90 of 0.06 μg/mL at 48 hours after administration. For florfenicol, the interstitial fluid concentration was almost 98% of the plasma concentration, and the pulmonary concentrations were over 200% of the plasma concentrations, exceeding the breakpoint (≤ 2 μg/mL), and the MIC90 for Mannheimia haemolytica (1.0 μg/mL) for the duration of the study. For ceftiofur, penetration to the interstitial fluid was only 5% of the plasma concentration. Pulmonary epithelial lining fluid concentration represented 40% of the plasma concentration. Airway concentrations exceeded the MIC breakpoint for susceptible respiratory pathogens (≤ 2 μg/mL) for a short time at 48 hours after administration. The plasma and interstitial fluid concentrations of tulathromcyin were lower than the concentrations in pulmonary fluid throughout the study. The bronchial concentrations were higher than the plasma or interstitial concentrations, with over 900

  20. Bacterial Pneumonia in Older Adults.

    PubMed

    Marrie, Thomas J; File, Thomas M

    2016-08-01

    Community-acquired pneumonia is common in the elderly person; its presentation in this population is often confounded by multiple comorbid illnesses, including those that result in confusion. Although severity-of-illness scoring systems might aid decision-making, clinical judgment following a careful assessment is key in deciding on the site of care and appropriate therapy.

  1. Eosinophilic pneumonia induced by daptomycin.

    PubMed

    Hayes, Don; Anstead, Michael I; Kuhn, Robert J

    2007-04-01

    We present a case of drug-induced eosinophilic pneumonia resulting from intravenous daptomycin being used as therapy for recurrent methicillin-sensitive Staphlococcus aureus endocarditis. The patient developed hypoxic respiratory failure requiring intubation and mechanical ventilation. Daptomycin therapy was discontinued immediately, and the patient improved significantly after the administration of intravenous corticosteroids allowing for extubation 3 days later.

  2. Etanercept in Treating Young Patients With Idiopathic Pneumonia Syndrome After Undergoing a Donor Stem Cell Transplant

    ClinicalTrials.gov

    2016-02-23

    Accelerated Phase Chronic Myelogenous Leukemia; Blastic Phase Chronic Myelogenous Leukemia; Childhood Acute Lymphoblastic Leukemia in Remission; Childhood Acute Myeloid Leukemia in Remission; Childhood Chronic Myelogenous Leukemia; Childhood Myelodysplastic Syndromes; Chronic Phase Chronic Myelogenous Leukemia; de Novo Myelodysplastic Syndromes; Disseminated Neuroblastoma; Juvenile Myelomonocytic Leukemia; Previously Treated Childhood Rhabdomyosarcoma; Previously Treated Myelodysplastic Syndromes; Pulmonary Complications; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Neuroblastoma; Recurrent Wilms Tumor and Other Childhood Kidney Tumors; Recurrent/Refractory Childhood Hodgkin Lymphoma; Relapsing Chronic Myelogenous Leukemia; Secondary Acute Myeloid Leukemia; Secondary Myelodysplastic Syndromes

  3. Exogenous lipoid pneumonia caused by herbicide inhalation.

    PubMed

    Hotta, Takamasa; Tsubata, Yukari; Okimoto, Tamio; Hoshino, Teppei; Hamaguchi, Shun-Ichi; Isobe, Takeshi

    2016-09-01

    Exogenous lipoid pneumonia is caused by aspiration or inhalation of oily substances. Generally, lipoid pneumonia has non-specific clinical and radiological presentations and may be misdiagnosed as bacterial pneumonia. Our patient, a 68-year-old man who had been diagnosed with pneumonia on three previous occasions, was admitted to our hospital with a fourth similar episode. Computed tomography of the chest revealed extensive consolidations with air bronchograms in lung fields on the right side. The bronchoalveolar lavage fluid (BALF) increased ghost-like macrophages that stained positive for lipid. Our patient reported that he had sprayed herbicide in large quantities without wearing a mask. We analysed the BALF and herbicide by gas chromatography and diagnosed exogenous lipoid pneumonia caused by inhalation of herbicide. Clinicians should be aware of lipoid pneumonia, which may present as infectious pneumonia. PMID:27516888

  4. Interstitial pregnancy management and subsequent pregnancy outcome.

    PubMed

    Sagiv, Ron; Debby, Abraham; Keidar, Ran; Kerner, Ram; Golan, Abraham

    2013-11-01

    We report on management and subsequent fertility outcome of interstitial pregnancy in a retrospective cohort study (Canadian Task Force classification II-3) at a university affiliated teaching hospital. Of 706 women with extrauterine pregnancy, 14 consecutive women with interstitial pregnancy were treated by methotrexate, laparotomy or laparoscopy between 1997 and 2007. The first four women, with significant hemoperitoneum, were treated by laparotomy. Of the next 10 women, four were selected for medical treatment with methotrexate. Only one case was treated successfully. The other six women had laparoscopic treatment. Of nine laparoscopies, one was converted to laparotomy due to excessive blood loss during the procedure. Of nine women desiring a child, three were infertile, whereas six conceived with an intrauterine pregnancy. A change from diagnosis later in pregnancy and laparotomy to more conservative treatment, mainly by laparoscopy, suggests a possibly better subsequent pregnancy rate.

  5. Interstitial laser thermotherapy in neurosurgery: a review.

    PubMed

    Menovsky, T; Beek, J F; van Gemert, M J; Roux, F X; Bown, S G

    1996-01-01

    One of the most recent laser treatment modalities in neurosurgery is interstitial laser thermotherapy (ILTT). In this review, experimental and clinical studies concerning intracranial ILTT are discussed. Two methods for intra-operative control of the laser induced lesions are described; i.e., computer-controlled power delivery, using a thermocouple that is positioned interstitially at the periphery of the tumour to maintain the desired temperature at that point, and MRI, to visualise the extent of the thermal lesions induced by ILTT. The results show that ILTT using a Nd: YAG laser is easy and relatively effective in the treatment of small deep-seated brain tumours with minimal risk and complications. This review is concluded with suggestions for further improvement of this treatment modality.

  6. Noninvasive NIR monitoring of interstitial ethanol concentration

    NASA Astrophysics Data System (ADS)

    Ridder, Trent D.; Ver Steeg, Benjamin J.; Vanslyke, Stephen J.; Way, Jeff F.

    2009-02-01

    A practical limitation encountered in alcohol research is the relatively small number of body compartments (e.g. blood, liver, tissue) that can be directly interrogated. In this work, an NIR spectroscopic device was investigated that provided a direct measurement of alcohol concentration in skin tissue (interstitial fluid). This work is intended to characterize the relationship of forearm interstitial fluid alcohol concentration relative to capillary blood using a first order kinetic model. Concurrent blood and tissue alcohol concentrations were collected on 101 test subjects while consuming alcohol. Estimates of the first order kinetic rate constant were calculated for each of the subjects. It is hoped that this characterization will lead to further improvements in optical based alcohol monitors for impairment detection.

  7. Pancreas tumor interstitial pressure catheter measurement

    NASA Astrophysics Data System (ADS)

    Nieskoski, Michael D.; Gunn, Jason; Marra, Kayla; Trembly, B. Stuart; Pogue, Brian W.

    2016-03-01

    This paper highlights the methodology in measuring interstitial pressure in pancreatic adenocarcinoma tumors. A Millar Mikrotip pressure catheter (SPR-671) was used in this study and a system was built to amplify and filter the output signal for data collection. The Millar pressure catheter was calibrated prior to each experiment in a water column at 37°C, range of 0 to 60 inH2O (112 mmHg), resulting in a calibration factor of 33 mV / 1 inH2O. The interstitial pressures measured in two orthotopically grown pancreatic adenocarcinoma tumor were 57 mmHg and 48 mmHg, respectively. Verteporfin uptake into the pancreatic adenocarcinoma tumor was measured using a probe-based experimental dosimeter.

  8. Pneumonia in stroke patients: a retrospective study.

    PubMed

    Ding, R; Logemann, J A

    2000-01-01

    This is a retrospective study of 378 consecutive stroke patients who were referred between June 1994 and June 1997 for videofluorographic study of oropharyngeal swallow. Patients who had radiation therapy, brain tumor, brain surgery, head and/or spinal cord trauma, oral-pharyngeal disease or surgery, or other neurologic diseases in addition to the stroke were excluded from the study. Patients were assigned to two groups: one with pneumonia and one without pneumonia. One hundred one patients were included in the pneumonia group, and 277 patients were included in the nonpneumonia group. Within the pneumonia group, patients were assigned to an acute pneumonia group (pneumonia within 6 months poststroke) and a chronic pneumonia group (pneumonia more than 6 months poststroke). Variables examined in the study included patients' medical history and the findings from the videofluorographic studies. Pearson chi-square analysis was used to identify those variables that were significantly different between the pneumonia and nonpneumonia patient groups and between the acute and chronic pneumonia groups. Results showed that stroke patients who developed pneumonia had a significantly higher incidence of multiple-location and unspecified lesion strokes, chronic airway disease in their medical history, and aspiration during the videofluorographic studies when compared with patients who did not develop pneumonia. Within the pneumonia group, the acute pneumonia group was found to have a significantly higher incidence of hypertension and diabetes in their medical history and a significantly higher incidence of aspiration and reduced laryngeal elevation during the videofluorographic studies. Between 48% and 55% of all stroke patients in the study aspirated. Patients who suffered multiple strokes, brainstem stroke, or subcortical stroke had the greatest frequency of aspiration.

  9. Interstitial devices for treating deep seated tumors

    NASA Astrophysics Data System (ADS)

    Lafon, Cyril; Cathignol, Dominique; Prat, Frédéric; Melodelima, David; Salomir, Rares; Theillère, Yves; Chapelon, Jean-Yves

    2006-05-01

    Techniques using intracavitary or interstitial applicators have been proposed because extracorporeal HIFU techniques are not always suitable for deep-seated tumors. Bones or gaseous pockets may indeed be located in the intervening tissue. The objective is to bring the ultrasound source as close as possible to the target through natural routes in order to minimize the effects of attenuation and phase aberration along the ultrasound pathway. Under these circumstances, it becomes possible to use higher frequency, thus increasing the ultrasonic absorption coefficient and resulting in more efficient heating of the treatment region. In contrast to extra-corporeal applicators, the design of interstitial probes imposes additional constraints relative to size and ergonomy. The goal of this paper is to present the range of miniature interstitial applicators we developed at INSERM for various applications. The sources are rotating plane water-cooled transducers that operate at a frequency between 3 and 10 MHz depending on the desired therapeutic depth. The choice of a plane transducer rather than divergent sources permits to extend the therapeutic depth and to enhance the angular selectivity of the treatment Rotating single element flat transducer can also be replaced by cylindrical arrays for rotating electronically a reconstructed plane wave. When extended zone of coagulation are required, original therapeutic modalities combining cavitation and thermal effects are used. These methods consist in favoring in depth heating by increasing the acoustic attenuation away from the transducer with the presence of bubbles. When associated to modern imaging modalities, these minimally invasive therapeutic devices offer very promising options for cancer treatment. For examples, two versions of an image-guided esophageal applicator are designed: one uses a retractable ultrasound mini probe for the positioning of the applicator, while the other is MRI compatible and offers on line

  10. Interstitial cystitis/painful bladder syndrome.

    PubMed

    Panzera, Alis Kolter

    2007-02-01

    Interstitial cystitis (IC) is a chronic, painful bladder syndrome primarily found in women. Although the direct cause(s) of IC are unknown, several theories exist. Common symptoms include urinary urgency, frequency, and pain. Treatment options include behavioral therapies, use of pharmacologic agents, and surgery. Patients benefit from prompt diagnosis and initiation of treatments. Important clinical features of IC in women including the pathology, common symptoms, and recommended evaluation and management strategies are reviewed.

  11. Predictors of mortality in rheumatoid arthritis-related interstitial lung disease.

    PubMed

    Assayag, Deborah; Lubin, Molly; Lee, Joyce S; King, Talmadge E; Collard, Harold R; Ryerson, Christopher J

    2014-05-01

    Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) has a heterogeneous clinical presentation and disease course. Establishing prognosis for these patients is challenging. Identifying the factors that predict mortality in patients with RA-ILD could help guide management. A detailed systematic review was conducted in order to identify individual variables that predict mortality in RA-ILD. A literature review was performed using keywords and medical subject headings to identify all articles relating to the prognosis of RA-ILD. Studies were included if they identified predictors of mortality in adults with RA-ILD, were published in English, and included at least 10 patients with RA-ILD. Two authors independently reviewed each citation and extracted data from all studies meeting inclusion criteria. Any differences were then resolved by consensus. A total of 10 studies met our inclusion criteria. All were observational cohort studies of variable quality. Mean age of reported patients ranged from 55 to 69 years, and 41.7% of all patients were male. Median survival ranged from 3.2 to 8.1 years. Significant predictors of mortality on multivariate analysis were older age, male gender, lower diffusion capacity for carbon monoxide, extent of fibrosis, and the presence of usual interstitial pneumonia pattern. Mortality in RA-ILD is associated with several patient- and ILD-specific variables; however, previous studies are of low quality.

  12. Epidemiology of Mycoplasma pneumoniae Infections in Japan and Therapeutic Strategies for Macrolide-Resistant M. pneumoniae.

    PubMed

    Yamazaki, Tsutomu; Kenri, Tsuyoshi

    2016-01-01

    Pneumonia caused by Mycoplasma pneumoniae (M. pneumoniae pneumonia) is a major cause of community-acquired pneumonia worldwide. The surveillance of M. pneumoniae pneumonia is important for etiological and epidemiological studies of acute respiratory infections. In Japan, nation-wide surveillance of M. pneumoniae pneumonia has been conducted as a part of the National Epidemiological Surveillance of Infectious Diseases (NESID) program. This surveillance started in 1981, and significant increases in the numbers of M. pneumoniae pneumonia patients were noted in 1984, 1988, 2006, 2010, 2011, 2012, and 2015. The epidemics in 2011 and 2012 were particularly widespread and motivated researchers to conduct detailed epidemiological studies, including genotyping and drug resistance analyses of M. pneumoniae isolates. The genotyping studies based on the p1 gene sequence suggested that the p1 gene type 1 lineage has been dominant in Japan since 2003, including the epidemic period during 2011-2012. However, more detailed p1 typing analysis is required to determine whether the type 2 lineages become more relevant after the dominance of the type 1 lineage. There has been extensive research interest in implications of the p1 gene types on the epidemiology of M. pneumoniae infections. Serological characterizations of sera from patients have provided a glimpse into these associations, showing the presence of type specific antibody in the patient sera. Another important epidemiological issue of M. pneumoniae pneumonia is the emergence of macrolide-resistant M. pneumoniae (MRMP). MRMPs were noted among clinical isolates in Japan after 2000. At present, the isolation rate of MRMPs from pediatric patients is estimated at 50-90% in Japan, depending on the specific location. In view of the situation, Japanese societies have issued guiding principles for treating M. pneumoniae pneumonia. In these guiding principles, macrolides are still recommended as the first-line drug, however, if the

  13. Epidemiology of Mycoplasma pneumoniae Infections in Japan and Therapeutic Strategies for Macrolide-Resistant M. pneumoniae

    PubMed Central

    Yamazaki, Tsutomu; Kenri, Tsuyoshi

    2016-01-01

    Pneumonia caused by Mycoplasma pneumoniae (M. pneumoniae pneumonia) is a major cause of community-acquired pneumonia worldwide. The surveillance of M. pneumoniae pneumonia is important for etiological and epidemiological studies of acute respiratory infections. In Japan, nation-wide surveillance of M. pneumoniae pneumonia has been conducted as a part of the National Epidemiological Surveillance of Infectious Diseases (NESID) program. This surveillance started in 1981, and significant increases in the numbers of M. pneumoniae pneumonia patients were noted in 1984, 1988, 2006, 2010, 2011, 2012, and 2015. The epidemics in 2011 and 2012 were particularly widespread and motivated researchers to conduct detailed epidemiological studies, including genotyping and drug resistance analyses of M. pneumoniae isolates. The genotyping studies based on the p1 gene sequence suggested that the p1 gene type 1 lineage has been dominant in Japan since 2003, including the epidemic period during 2011–2012. However, more detailed p1 typing analysis is required to determine whether the type 2 lineages become more relevant after the dominance of the type 1 lineage. There has been extensive research interest in implications of the p1 gene types on the epidemiology of M. pneumoniae infections. Serological characterizations of sera from patients have provided a glimpse into these associations, showing the presence of type specific antibody in the patient sera. Another important epidemiological issue of M. pneumoniae pneumonia is the emergence of macrolide-resistant M. pneumoniae (MRMP). MRMPs were noted among clinical isolates in Japan after 2000. At present, the isolation rate of MRMPs from pediatric patients is estimated at 50–90% in Japan, depending on the specific location. In view of the situation, Japanese societies have issued guiding principles for treating M. pneumoniae pneumonia. In these guiding principles, macrolides are still recommended as the first-line drug, however, if

  14. Interstitial loop transformations in FeCr

    SciTech Connect

    Béland, Laurent Karim; Osetsky, Yuri N.; Stoller, Roger E.; Xu, Haixuan

    2015-03-27

    Here, we improve the Self-Evolving Atomistic Kinetic Monte Carlo (SEAKMC) algorithm by integrating the Activation Relaxation Technique nouveau (ARTn), a powerful open-ended saddle-point search method, into the algorithm. We use it to investigate the reaction of 37-interstitial 1/2[1 1 1] and 1/2[View the MathML source] loops in FeCr at 10 at.% Cr. They transform into 1/2[1 1 1], 1/2[View the MathML source], [1 0 0] and [0 1 0] 74-interstitial clusters with an overall barrier of 0.85 eV. We find that Cr decoration locally inhibits the rotation of crowdions, which dictates the final loop orientation. Moreover, the final loop orientation depends on the details of the Cr decoration. Generally, a region of a given orientation is favored if Cr near its interface with a region of another orientation is able to inhibit reorientation at this interface more than the Cr present at the other interfaces. Also, we find that substitutional Cr atoms can diffuse from energetically unfavorable to energetically favorable sites within the interlocked 37-interstitial loops conformation with barriers of less than 0.35 eV.

  15. Interstitial loop transformations in FeCr

    DOE PAGES

    Béland, Laurent Karim; Osetsky, Yuri N.; Stoller, Roger E.; Xu, Haixuan

    2015-03-27

    Here, we improve the Self-Evolving Atomistic Kinetic Monte Carlo (SEAKMC) algorithm by integrating the Activation Relaxation Technique nouveau (ARTn), a powerful open-ended saddle-point search method, into the algorithm. We use it to investigate the reaction of 37-interstitial 1/2[1 1 1] and 1/2[View the MathML source] loops in FeCr at 10 at.% Cr. They transform into 1/2[1 1 1], 1/2[View the MathML source], [1 0 0] and [0 1 0] 74-interstitial clusters with an overall barrier of 0.85 eV. We find that Cr decoration locally inhibits the rotation of crowdions, which dictates the final loop orientation. Moreover, the final loop orientationmore » depends on the details of the Cr decoration. Generally, a region of a given orientation is favored if Cr near its interface with a region of another orientation is able to inhibit reorientation at this interface more than the Cr present at the other interfaces. Also, we find that substitutional Cr atoms can diffuse from energetically unfavorable to energetically favorable sites within the interlocked 37-interstitial loops conformation with barriers of less than 0.35 eV.« less

  16. Breast conservation treatment with perioperative interstitial irradiation

    SciTech Connect

    Krishnan, L.; Mansfield, C.M.; Jewell, W.R.; Reddy, E.K.; Thomas, J.H.; Krishnan, E.C.

    1987-10-01

    Limited resection of the breast combined with radiation has proved to be as effective as more radical surgery in treating early breast cancer. At the University of Kansas Medical Center, the radiotherapy consists of an interstitial implant at the time of lumpectomy to deliver an interstitial boost dose to the tumor bed with iridium-192 immediately following the surgical procedure. An axillary node dissection is also performed at the time of lumpectomy. A dose of 2000 cGy is delivered to the tumor bed between 40 and 60 h. Two to three weeks later, 4500-5000 cGy is delivered to the entire breast with external beam radiation over 5-5.5 weeks. One hundred breasts in 98 patients were so treated between June 1982 and February 1986, with 2 carcinomas in situ, 40 stage I, 51 stage II, and 7 stage III cancers, consisting of 2 TIS, 54 T1, 39 T2, and 5 T3 lesions. Locoregional control with a median follow-up of 31 months was 98%. One recurrence was in a different quadrant, and the other revealed predominantly the in situ component. Immediate implant did not compromise wound healing or cosmesis. The cosmetic result was graded as good to excellent in 88% of the breasts. Our preliminary results appear to suggest a better local control with immediate interstitial irradiation.

  17. INTERSTITIAL IMMUNE COMPLEX THYROIDITIS IN MICE

    PubMed Central

    Clagett, James A.; Wilson, Curtis B.; Weigle, William O.

    1974-01-01

    Mice immunized with soluble heterologous thyroglobulins developed autoantibody that cross-reacted with autologous thyroglobulin. There was a direct correlation between the temporal appearance and quantity of serum autoantibody and the presumed in situ formation of immune complexes in the interstitium of the thyroid glands. Immediately after the formation of interstitial immune complexes containing antibody of the IgG complement-fixing type, the thyroids were invaded by a transient but intense neutrophil infiltrate which within 1 wk was replaced by chronic mononuclear elements. By the combination of fluorescence microscopy and autoradiography, thyroglobulin was demonstrated to be one, if not the sole, antigen in the interstitial immune complexes. The interstitial immune complexes were granular to lumpy in appearance and formed at the basal area of the follicular cells in intimate association with the follicular basement membrane. Electron microscopy revealed electron dense deposits, presumably immune complexes, between the follicular basement membrane and the plasma membrane. The presumed in situ formation of immune complexes in this model is similar to that which occurs in the Arthus reaction and is a different mechanism of immune complex injury than that caused by tissue deposition of circulating immune complexes as occurs in serum sickness. PMID:4279269

  18. Mycoplasma pneumoniae associated organising pneumonia in a 10 year old boy.

    PubMed

    Wachowski, O; Demirakça, S; Müller, K-M; Scheurlen, W

    2003-03-01

    We describe a 10 year old boy with organising pneumonia associated with acute Mycoplasma pneumoniae infection. The diagnosis of organising pneumonia was made by open lung biopsy and the M pneumoniae infection was proven serologically. Antibiotic and long term corticosteroid treatment resulted in steadily improving pulmonary function monitored by spirometry. The introduction of anti-inflammatory treatment with NSAIDs/immunosuppressive agents in order to spare steroids was well tolerated and resulted in further improvement of the pulmonary function. To our knowledge this is the first documented case of Mycoplasma pneumoniae associated organising pneumonia to be reported in a child.

  19. Cardiopulmonary and Gastrointestinal Manifestations of Eosinophil- associated Diseases and Idiopathic Hypereosinophilic Syndromes: Multimodality Imaging Approach.

    PubMed

    Katre, Rashmi S; Sunnapwar, Abhijit; Restrepo, Carlos S; Katabathina, Venkata S; Mumbower, Amy; Baxi, Ameya; Sonavane, Sushilkumar

    2016-01-01

    Eosinophil-associated diseases (EADs) are a diverse group of disorders characterized by an increase in circulating or tissue eosinophils. Cardiopulmonary and gastrointestinal system involvement can be due to primary EAD with no known cause or can be secondary to known systemic disease. The cardiopulmonary spectrum of EADs comprises simple pulmonary eosinophilia, acute eosinophilic pneumonia, chronic eosinophilic pneumonia, Churg-Strauss syndrome, allergic bronchopulmonary aspergillosis, bronchocentric granulomatosis, parasitic infections, and idiopathic hypereosinophilic syndrome. Eosinophilic gastrointestinal disorders include eosinophilic esophagitis, eosinophilic gastroenteritis, and eosinophilic colitis. Diagnosis is often challenging and requires a combination of clinical and imaging features along with laboratory findings. The absolute eosinophil count in peripheral blood and the percentage of eosinophils in bronchoalveolar lavage fluid are crucial in evaluation of various eosinophilic lung diseases. Although chest radiography is the initial imaging modality used in suspected cases of pulmonary EAD, multidetector computed tomography may demonstrate more characteristic pulmonary patterns, nodules, and subtle parenchymal abnormalities. Barium esophagography is used to assess mucosal abnormalities and the length and diameter of esophageal strictures. Magnetic resonance imaging is superior in providing valuable information in select patients, especially in evaluation of cardiac and gastrointestinal system involvement. Many patients require a multimodality imaging approach to enable diagnosis, guide treatment, and assess treatment response. Knowledge of the clinical features and imaging findings of the spectrum of EADs involving the lungs, heart, and gastrointestinal tract permits optimal patient care. PMID:26963455

  20. Interstitial space and collagen alterations of the developing rat diaphragm

    NASA Technical Reports Server (NTRS)

    Gosselin, L. E.; Martinez, D. A.; Vailas, A. C.; Sieck, G. C.

    1993-01-01

    The effect of growth on the relative interstitial space [%total cross-sectional area (CSA)] and collagen content of the rat diaphragm muscle was examined at postnatal ages of 0, 7, 14, and 21 days as well as in adult males. The proportion of interstitial space relative to total muscle CSA was determined by computerized image analysis of lectin-stained cross sections of diaphragm muscle. To assess collagen content and extent of collagen maturation (i.e., cross-linking), high-pressure liquid chromatography analysis was used to measure hydroxyproline concentration and the nonreducible collagen cross-link hydroxylysylpyridinoline (HP), respectively. At birth, interstitial space accounted for approximately 47% of total diaphragm muscle CSA. During postnatal growth, the relative contribution of interstitial space decreased such that by adulthood the interstitial space accounted for approximately 18% of total muscle CSA. The change in relative interstitial space occurred without a concomitant change in hydroxyproline concentration. However, the concentration of HP markedly increased with age such that the adult diaphragm contained approximately 17 times more HP than at birth. These results indicate that during development the relative CSA occupied by interstitial space decreases as muscle fiber size increases. However, the reduction in relative interstitial space is not associated with a change in collagen concentration. Thus collagen density in the interstitial space may increase with age. It is possible that the observed changes in relative interstitial space and collagen influence the passive length-force properties of the diaphragm.

  1. Experimental depletion of different renal interstitial cell populations

    SciTech Connect

    Bohman, S.O.; Sundelin, B.; Forsum, U.; Tribukait, B.

    1988-04-01

    To define different populations of renal interstitial cells and investigate some aspects of their function, we studied the kidneys of normal rats and rats with hereditary diabetes insipidus (DI, Brattleboro) after experimental manipulations expected to alter the number of interstitial cells. DI rats showed an almost complete loss of interstitial cells in their renal papillae after treatment with a high dose of vasopressin. In spite of the lack of interstitial cells, the animals concentrated their urine to the same extent as vasopressin-treated normal rats, indicating that the renomedullary interstitial cells do not have an important function in concentrating the urine. The interstitial cells returned nearly to normal within 1 week off vasopressin treatment, suggesting a rapid turnover rate of these cells. To further distinguish different populations of interstitial cells, we studied the distribution of class II MHC antigen expression in the kidneys of normal and bone-marrow depleted Wistar rats. Normal rats had abundant class II antigen-positive interstitial cells in the renal cortex and outer medulla, but not in the inner medulla (papilla). Six days after 1000 rad whole body irradiation, the stainable cells were almost completely lost, but electron microscopic morphometry showed a virtually unchanged volume density of interstitial cells in the cortex and outer medulla, as well as the inner medulla. Thus, irradiation abolished the expression of the class II antigen but caused no significant depletion of interstitial cells.

  2. Optimal management of idiopathic scoliosis in adolescence

    PubMed Central

    Kotwicki, Tomasz; Chowanska, Joanna; Kinel, Edyta; Czaprowski, Dariusz; Tomaszewski, Marek; Janusz, Piotr

    2013-01-01

    Idiopathic scoliosis is a three-dimensional deformity of the growing spine, affecting 2%–3% of adolescents. Although benign in the majority of patients, the natural course of the disease may result in significant disturbance of body morphology, reduced thoracic volume, impaired respiration, increased rates of back pain, and serious esthetic concerns. Risk of deterioration is highest during the pubertal growth spurt and increases the risk of pathologic spinal curvature, increasing angular value, trunk imbalance, and thoracic deformity. Early clinical detection of scoliosis relies on careful examination of trunk shape and is subject to screening programs in some regions. Treatment options are physiotherapy, corrective bracing, or surgery for mild, moderate, or severe scoliosis, respectively, with both the actual degree of deformity and prognosis being taken into account. Physiotherapy used in mild idiopathic scoliosis comprises general training of the trunk musculature and physical capacity, while specific physiotherapeutic techniques aim to address the spinal curvature itself, attempting to achieve self-correction with active trunk movements developed in a three-dimensional space by an instructed adolescent under visual and proprioceptive control. Moderate but progressive idiopathic scoliosis in skeletally immature adolescents can be successfully halted using a corrective brace which has to be worn full time for several months or until skeletal maturity, and is able to prevent more severe deformity and avoid the need for surgical treatment. Surgery is the treatment of choice for severe idiopathic scoliosis which is rapidly progressive, with early onset, late diagnosis, and neglected or failed conservative treatment. The psychologic impact of idiopathic scoliosis, a chronic disease occurring in the psychologically fragile period of adolescence, is important because of its body distorting character and the onerous treatment required, either conservative or surgical

  3. Community-onset Klebsiella pneumoniae pneumonia in Taiwan: clinical features of the disease and associated microbiological characteristics of isolates from pneumonia and nasopharynx

    PubMed Central

    Lin, Yi-Tsung; Wang, Yu-Ping; Wang, Fu-Der; Fung, Chang-Phone

    2015-01-01

    Klebsiella pneumoniae is an important cause of community-onset pneumonia in Asian countries and South Africa. We investigated the clinical characteristics of K. pneumoniae causing community-onset pneumonia, and the associated microbiological features between K. pneumoniae isolates from pneumonia and those from the nasopharynx in Taiwan. This study was conducted at the Taipei Veterans General Hospital during July, 2012 to February, 2014. The clinical characteristics in patients with community-onset K. pneumoniae pneumonia were analyzed. K. pneumoniae isolates from the nasopharynx of adults attending otorhinolaryngology outpatient clinics were collected to compare their microbiological features with those from pneumonia. Capsular genotypes, antimicrobial susceptibility, and multilocus sequence type (MLST) were determined among these strains. Ninety-one patients with community-onset K. pneumoniae pneumonia were enrolled. We found a high mortality (29.7%) among these patients. Capsular types K1, K2, K5, K20, K54, and K57 accounted for ∼70% of the K. pneumoniae isolates causing pneumonia, and ∼70% of all the K. pneumoniae strains isolated from the nasopharynx of patients in outpatient clinics. The MLST profiles further demonstrated the genetic relatedness between most pneumonia isolates and those from the nasopharynx. In conclusion, our results show that community-onset pneumonia caused by K. pneumoniae was associated with high mortality and could have a reservoir in the nasopharynx. To tackle this high-mortality disease, the distribution of capsular types in the nasopharynx might have implications for future vaccine development. PMID:25741336

  4. Community-onset Klebsiella pneumoniae pneumonia in Taiwan: clinical features of the disease and associated microbiological characteristics of isolates from pneumonia and nasopharynx.

    PubMed

    Lin, Yi-Tsung; Wang, Yu-Ping; Wang, Fu-Der; Fung, Chang-Phone

    2015-01-01

    Klebsiella pneumoniae is an important cause of community-onset pneumonia in Asian countries and South Africa. We investigated the clinical characteristics of K. pneumoniae causing community-onset pneumonia, and the associated microbiological features between K. pneumoniae isolates from pneumonia and those from the nasopharynx in Taiwan. This study was conducted at the Taipei Veterans General Hospital during July, 2012 to February, 2014. The clinical characteristics in patients with community-onset K. pneumoniae pneumonia were analyzed. K. pneumoniae isolates from the nasopharynx of adults attending otorhinolaryngology outpatient clinics were collected to compare their microbiological features with those from pneumonia. Capsular genotypes, antimicrobial susceptibility, and multilocus sequence type (MLST) were determined among these strains. Ninety-one patients with community-onset K. pneumoniae pneumonia were enrolled. We found a high mortality (29.7%) among these patients. Capsular types K1, K2, K5, K20, K54, and K57 accounted for ∼70% of the K. pneumoniae isolates causing pneumonia, and ∼70% of all the K. pneumoniae strains isolated from the nasopharynx of patients in outpatient clinics. The MLST profiles further demonstrated the genetic relatedness between most pneumonia isolates and those from the nasopharynx. In conclusion, our results show that community-onset pneumonia caused by K. pneumoniae was associated with high mortality and could have a reservoir in the nasopharynx. To tackle this high-mortality disease, the distribution of capsular types in the nasopharynx might have implications for future vaccine development.

  5. Persistent Pneumonia in an Infant

    PubMed Central

    Padilla, Kristen; Logan, Latania; Codispoti, Christopher; Jones, Carolyn

    2015-01-01

    A 4-month-old boy with past medical history of eczema presented with fever and cough; a chest radiograph showed lung consolidation, and he was initially treated with amoxicillin for presumed community-acquired pneumonia. After several days, his fever persisted. He was also profoundly anemic. Antibiotic coverage was broadened because of the concern for resistant organisms; he began to improve and was discharged from the hospital. However, at 5 months of age, his fever returned, and he continued to demonstrate lung consolidation on chest radiograph. Additionally, he had lost weight and continued to be anemic. Splenic cysts were noted on abdominal ultrasound. He was diagnosed with an unusual etiology for his pneumonia and improved with the appropriate therapy. An underlying immunodeficiency was suspected, but initial testing was nondiagnostic. At 12 months of age, he presented with another infection, and the final diagnosis was made. PMID:26122810

  6. Persistent Pneumonia in an Infant.

    PubMed

    Padilla, Kristen; Logan, Latania; Codispoti, Christopher; Jones, Carolyn; Van Opstal, Elizabeth

    2015-07-01

    A 4-month-old boy with past medical history of eczema presented with fever and cough; a chest radiograph showed lung consolidation, and he was initially treated with amoxicillin for presumed community-acquired pneumonia. After several days, his fever persisted. He was also profoundly anemic. Antibiotic coverage was broadened because of the concern for resistant organisms; he began to improve and was discharged from the hospital. However, at 5 months of age, his fever returned, and he continued to demonstrate lung consolidation on chest radiograph. Additionally, he had lost weight and continued to be anemic. Splenic cysts were noted on abdominal ultrasound. He was diagnosed with an unusual etiology for his pneumonia and improved with the appropriate therapy. An underlying immunodeficiency was suspected, but initial testing was nondiagnostic. At 12 months of age, he presented with another infection, and the final diagnosis was made. PMID:26122810

  7. Idiopathic noncirrhotic portal hypertension: current perspectives.

    PubMed

    Riggio, Oliviero; Gioia, Stefania; Pentassuglio, Ilaria; Nicoletti, Valeria; Valente, Michele; d'Amati, Giulia

    2016-01-01

    The term idiopathic noncirrhotic portal hypertension (INCPH) has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals), splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis. Physicians should learn to look for this condition in a number of clinical settings, including cryptogenic cirrhosis, a disease known to be associated with INCPH, drug administration, and even chronic alterations in liver function tests. Once INCPH is clinically suspected, liver histology becomes mandatory for the correct diagnosis. However, pathologists should be familiar with the histological features of INCPH, especially in cases in which histology is not only requested to exclude liver cirrhosis. PMID:27555800

  8. Idiopathic noncirrhotic portal hypertension: current perspectives

    PubMed Central

    Riggio, Oliviero; Gioia, Stefania; Pentassuglio, Ilaria; Nicoletti, Valeria; Valente, Michele; d’Amati, Giulia

    2016-01-01

    The term idiopathic noncirrhotic portal hypertension (INCPH) has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals), splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis. Physicians should learn to look for this condition in a number of clinical settings, including cryptogenic cirrhosis, a disease known to be associated with INCPH, drug administration, and even chronic alterations in liver function tests. Once INCPH is clinically suspected, liver histology becomes mandatory for the correct diagnosis. However, pathologists should be familiar with the histological features of INCPH, especially in cases in which histology is not only requested to exclude liver cirrhosis. PMID:27555800

  9. Idiopathic head tremor in English bulldogs.

    PubMed

    Guevar, Julien; De Decker, Steven; Van Ham, Luc M L; Fischer, Andrea; Volk, Holger A

    2014-02-01

    Idiopathic head tremor (IHT) syndrome is a recognized but poorly characterized movement disorder in English bulldogs (EBs). The data analyzed were collected via a detailed online questionnaire and video recordings. Thirty-eight percent of the population demonstrated IHT. The first presentation was early in life. There was no sex or neutered status predisposition. The condition disappeared with time in 50% of the cases. The direction of the head movement was vertical or horizontal. The number of episodes per day and the duration of the episodes were greatly variable. The majority of episodes occurred at rest. Most of the episodes were unpredictable. And there was no alteration of the mental status for most dogs during the episodes. Stress has been reported as a suspected trigger factor. IHT in EBs can be considered an idiopathic paroxysmal movement disorder. PMID:24375621

  10. Genetic Aspects of Congenital and Idiopathic Scoliosis

    PubMed Central

    Giampietro, Philip F.

    2012-01-01

    Congenital and idiopathic scoliosis represent disabling conditions of the spine. While congenital scoliosis (CS) is caused by morphogenic abnormalities in vertebral development, the cause(s) for idiopathic scoliosis is (are) likely to be varied, representing alterations in skeletal growth, neuromuscular imbalances, disturbances involving communication between the brain and spine, and others. Both conditions are characterized by phenotypic and genetic heterogeneities, which contribute to the difficulties in understanding their genetic basis that investigators face. Despite the differences between these two conditions there is observational and experimental evidence supporting common genetic mechanisms. This paper focuses on the clinical features of both CS and IS and highlights genetic and environmental factors which contribute to their occurrence. It is anticipated that emerging genetic technologies and improvements in phenotypic stratification of both conditions will facilitate improved understanding of the genetic basis for these conditions and enable targeted prevention and treatment strategies. PMID:24278672

  11. Dental manifestation of primary idiopathic hypoparathyroidism.

    PubMed

    Srirangarajan, Sridharan; Satyanarayan, Aparna; Ravindra, Shivamurthy; Thakur, Srinath

    2014-07-01

    Idiopathic hypoparathyroidism (IHP) is a rare endocrinopathic disorder, of idiopathic nature, characterized by a deficiency of parathyroid hormone causing low serum calcium (Ca) and high serum phosphorus concentrations. We present a case report ofa 40-year-old woman with complaint of bleeding gums and tingling sensation in fingers and toes reported to our department in March 2005 with no tangible diagnosis from previous medical examinations. Oral findings included abnormally short roots, cemental hyperplasia, widening of the periodontal ligament space and root resorption. Laboratory investigations revealed a low serum Ca level and along with the clinical findings, a diagnosis of IHP was reached. Oral prophylaxis was performed and Ca supplements were prescribed. We believe that this case report is a first of its kind reporting cemental hyperplasia, altered alveolar bone patterns and periodontal ligament widening in a patient with IHP.

  12. [Pathogenesis of the idiopathic inflammatory myopathies].

    PubMed

    Riebeling-Navarro, Carlos; Nava, Arnulfo

    2009-11-01

    The inflammatory myopathies, commonly described as idiopathic, are a group of acquired diseases characterized by an inflammatory infiltrate of the skeletal muscle. On the basis of clinical and immuno-pathological features, three major diseases can be identified: dermatomiositis (DM), polymyositis (PM) and inclusion body myositis (IBM). Immunopathogenesis mechanisms are crucial for discriminating between the three different subsets of inflammatory myopathies. DM is a complement-mediated microangiopathy affecting skin and muscle. PM and IBM are T cell-mediated disorders, where CD8-positive cytotoxic T cells invade muscle fibres expressing MHC class I antigens. This article summarizes the main immunopathological markers. The impact of this new knowledge must be defined in relation to potential therapeutic targets for idiopathic inflammatory myopathies.

  13. Idiopathic gingival enlargement and its management

    PubMed Central

    Shetty, Arvind K.; Shah, Hardik J.; Patil, Mallika A.; Jhota, Komal N.

    2010-01-01

    Idiopathic gingival enlargement is a proliferative fibrous lesion of the gingival tissue that causes esthetic and functional problems. Both genetically and pharmacologically induced forms of gingival enlargement exist. This case report addresses the diagnosis and treatment of a case of idiopathic gingival enlargement in a 13-year-old female. The patient presented with generalized diffuse gingival enlargement involving the maxillary and mandibular arches extending on buccal and lingual/palatal surfaces and covering incisal / occlusal third of the tooth resulting in difficulty in speech and mastication since last three years. Patient also gave a history of surgical treatment being carried out four years back in upper anterior region suggesting of recurrence. Biopsy report confirmed the diagnosis of gingival hyperplasia. Gingivectomy was carried out in all four quadrants by using four different methods. PMID:21731254

  14. Spontaneous Idiopathic Unilateral Adrenal Haemorrhage (SIAH).

    PubMed

    Naqvi, Syed Ali; Zaman, Shamas; Ahmed, Irfan

    2015-04-01

    Spontaneous Idiopathic Adrenal Haemorrhage (SIAH) is an unusual surgical emergency which can present with life threatening massive retroperitoneal bleeding. Most of the cases reported in the literature are associated with use of anticoagulation or underlying adrenal pathology such as tumors or cysts. Since this clinical entity is uncommon and clinical presentation is very indistinct, the diagnosis can be easily missed and can be challenging for the treating physicians. Nevertheless a raised clinical suspicion coupled with advances in radiological imaging have considerably improved the detection of SIAH in recent times. We report an unusual case of a 20 years old healthy female student who presented to our hospital with sudden onset of abdominal pain and shock. She was diagnosed as a case of massive spontaneous idiopathic unilateral adrenal haemorrhage, unaccompanied by any hematologic disorder, trauma or underlying pathology. Although patient was hemodynamically unstable at presentation, she was resuscitated promptly, investigated appropriately, hence recovered uneventfully with conservative management alone.

  15. "Idiopathic" mental retardation and new chromosomal abnormalities

    PubMed Central

    2010-01-01

    Mental retardation is a heterogeneous condition, affecting 1-3% of general population. In the last few years, several emerging clinical entities have been described, due to the advent of newest genetic techniques, such as array Comparative Genomic Hybridization. The detection of cryptic microdeletion/microduplication abnormalities has allowed genotype-phenotype correlations, delineating recognizable syndromic conditions that are herein reviewed. With the aim to provide to Paediatricians a combined clinical and genetic approach to the child with cognitive impairment, a practical diagnostic algorithm is also illustrated. The use of microarray platforms has further reduced the percentage of "idiopathic" forms of mental retardation, previously accounted for about half of total cases. We discussed the putative pathways at the basis of remaining "pure idiopathic" forms of mental retardation, highlighting possible environmental and epigenetic mechanisms as causes of altered cognition. PMID:20152051

  16. [Juvenile idiopathic arthritis: Definition and classification].

    PubMed

    Deslandre, C

    2016-04-01

    Juvenile idiopathic arthritis (JIA) is a group of diseases defined by the presence of arthritis of more than 6 weeks duration in patients aged less than 16 years and with unknown etiology. The international classification based on clinical and biological criteria define each type of JIA: systemic, oligoarticular, polyarticular with and without rheumatoid factor, enthesitis-related arthritis, and psoriatic arthritis. However, some discussions persist concerning systemic-onset juvenile idiopathic arthritis, whose clinical symptoms and pathogenic mechanisms are quite similar to those observed in autoinflammatory diseases, arthritis with antinuclear factors (poly- and oligoarticular) that could be considered as a homogenous group, and a family history of psoriasis that frequently led to unclassified arthritis. Better knowledge of the pathogenic mechanisms should improve the initial clinical classification with more homogeneous groups of patients and reduce the number of unclassified cases of arthritis. PMID:26968301

  17. Respiratory failure due to infliximab induced interstitial lung disease.

    PubMed

    Kakavas, Sotiris; Balis, Evangelos; Lazarou, Vasiliki; Kouvela, Marousa; Tatsis, Georgios

    2013-01-01

    Although poorly understood, interstitial lung disease has been reported as a possible complication of tumor necrosis factor alpha inhibitors. We report a case of interstitial lung disease in a 64-year-old man with psoriasis 3 weeks after the initiation of infliximab treatment. The patient had received two fortnightly infusions of infliximab following a short course of methotrexate. Thoracic computed tomography showed bilateral ground glass and interstitial infiltrates, while the results of microbiology and immunologic workup were negative. Likewise, bronchoalveolar lavage detected neither typical nor atypical pathogens. Infliximab-induced interstitial lung injury was suspected and corticosteroid therapy was administered which resulted in rapid clinical and radiological improvement. This is one of the few reported cases of interstitial lung disease due to infliximab in the psoriasis population. The patient had no pre-existing lung pathology, while his previous exposure to methotrexate was minimal and was not temporally associated with the induction of interstitial lung disease.

  18. Cognitive function of idiopathic childhood epilepsy

    PubMed Central

    2012-01-01

    Most children with epilepsy are of normal intelligence. However, a significant subset will have temporary or permanent cognitive impairment. Factors that affect cognitive function are myriad and include the underlying neuropathology of the epilepsy, seizures, epileptiform discharges, psychosocial problems, age at seizure onset, duration of epilepsy, and side effects associated with antiepileptic drugs. This review article discusses cognitive function in children with idiopathic epilepsy and the effects of antiepileptic drugs on cognitive function in children. PMID:22670150

  19. Treatment strategies for pediatric idiopathic hypercalciuria.

    PubMed

    Lau, Keith Kwong; Butani, Lavjay

    2009-06-01

    Idiopathic hypercalciuria (IH) is a common metabolic disorder in children and is associated with the development of renal calculi, nephrocalcinosis, hematuria and osteopenia. The effect of various dietary modifications and available pharmacologic therapies on reducing urinary calcium excretion and/or urinary supersaturation is discussed in this article. The importance of a multidisciplinary approach involving the patient, their families, and health-care professionals is also addressed.

  20. Interventional Radiologic Treatment for Idiopathic Portal Hypertension

    SciTech Connect

    Hirota, Shozo; Ichikawa, Satoshi; Matsumoto, Shinichi; Motohara, Tomofumi; Fukuda, Tetsuya; Yoshikawa, Takeshi

    1999-07-15

    Purpose: To evaluate the usefulness of interventional radiological treatment for idiopathic portal hypertension. Methods: Between 1995 and 1998, we performed an interventional radiological treatment in five patients with idiopathic portal hypertension, four of whom had refused surgery and one of whom had undergone surgery. Three patients with gastroesophageal varices (GEV) were treated by partial splenic embolization (PSE), one patient with esophageal varices (EV) and massive ascites by transjugular intrahepatic portosytemic shunt (TIPS) and PSE, and one patient with GEV by percutaneous transhepatic obliteration (PTO). Midterm results were analyzed in terms of the effect on esophageal and/or gastric varices. Results: In one woman with severe GEV who underwent three sessions of PSE, there was endoscopic confirmation that the GEV had disappeared. In one man his EV shrunk markedly after two sessions of PSE. In two patients slight reduction of the EV was obtained with one application of PSE combined with endoscopic variceal ligation therapy. PTO for GV in one patient resulted in good control of the varices. All patients have survived for 16-42 months since the first interventional treatment, and varices are well controlled. Conclusion: Interventional radiological treatment is effective for patients with idiopathic portal hypertension, whether or not they have undergone surgery.