Science.gov

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. Genetics and Idiopathic Interstitial Pneumonias.

    PubMed

    Chu, Sarah G; El-Chemaly, Souheil; Rosas, Ivan O

    2016-06-01

    Significant progress has been made in elucidating the genetics of parenchymal lung diseases, particularly idiopathic interstitial pneumonias (IIPs). IIPs are a heterogeneous group of diffuse interstitial lung diseases of uncertain etiology, diagnosed only after known causes of interstitial lung disease have been excluded. Idiopathic pulmonary fibrosis is the most common IIP. Through candidate gene approaches and genome wide association studies, much light has been shed on the genetic origins of IIPs, enhancing our understanding of risk factors and pathogenesis. However, significant work remains to be accomplished in identifying novel genetic variants and characterizing the function of validated candidate genes in lung pathobiology, their interplay with environmental factors, and ultimately translating these discoveries to patient care. PMID:27231858

  3. Idiopathic Interstitial Pneumonia

    PubMed Central

    Flaherty, Kevin R.; Andrei, Adin-Cristian; King, Talmadge E.; Raghu, Ganesh; Colby, Thomas V.; Wells, Athol; Bassily, Nadir; Brown, Kevin; du Bois, Roland; Flint, Andrew; Gay, Steven E.; Gross, Barry H.; Kazerooni, Ella A.; Knapp, Robert; Louvar, Edmund; Lynch, David; Nicholson, Andrew G.; Quick, John; Thannickal, Victor J.; Travis, William D.; Vyskocil, James; Wadenstorer, Frazer A.; Wilt, Jeffrey; Toews, Galen B.; Murray, Susan; Martinez, Fernando J.

    2007-01-01

    Rationale: Treatment and prognoses of diffuse parenchymal lung diseases (DPLDs) varies by diagnosis. Obtaining a uniform diagnosis among observers is difficult. Objectives: Evaluate diagnostic agreement between academic and community-based physicians for patients with DPLDs, and determine if an interactive approach between clinicians, radiologists, and pathologists improved diagnostic agreement in community and academic centers. Methods: Retrospective review of 39 patients with DPLD. A total of 19 participants reviewed cases at 2 community locations and 1 academic location. Information from the history, physical examination, pulmonary function testing, high-resolution computed tomography, and surgical lung biopsy was collected. Data were presented in the same sequential fashion to three groups of physicians on separate days. Measurements and Main Results: Each observer's diagnosis was coded into one of eight categories. A κ statistic allowing for multiple raters was used to assess agreement in diagnosis. Interactions between clinicians, radiologists, and pathologists improved interobserver agreement at both community and academic sites; however, final agreement was better within academic centers (κ = 0.55–0.71) than within community centers (κ = 0.32–0.44). Clinically significant disagreement was present between academic and community-based physicians (κ = 0.11–0.56). Community physicians were more likely to assign a final diagnosis of idiopathic pulmonary fibrosis compared with academic physicians. Conclusions: Significant disagreement exists in the diagnosis of DPLD between physicians based in communities compared with those in academic centers. Wherever possible, patients should be referred to centers with expertise in diffuse parenchymal lung disorders to help clarify the diagnosis and provide suggestions regarding treatment options. PMID:17255566

  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. PMID:27123874

  5. Invasive diagnostic techniques in idiopathic interstitial pneumonias.

    PubMed

    Poletti, Venerino; Ravaglia, Claudia; Gurioli, Carlo; Piciucchi, Sara; Dubini, Alessandra; Cavazza, Alberto; Chilosi, Marco; Rossi, Andrea; Tomassetti, Sara

    2016-01-01

    Fibrosing interstitial lung diseases (f-ILDs) represent a heterogeneous group of disorders in which the aetiology may be identified or, not infrequently, remain unknown. Establishing a correct diagnosis of a distinct f-ILD requires a multidisciplinary approach, integrating clinical profile, physiological and laboratory data, radiological appearance and, when appropriate, histological findings. Surgical lung biopsy is still considered the most important diagnostic tool as it is able to provide lung samples large enough for identification of complex patterns such as usual interstitial pneumonitis (UIP) and nonspecific interstitial pneumonitis. However, this procedure is accompanied by significant morbidity and mortality. Bronchoalveolar lavage is still a popular diagnostic tool allowing identification of alternative diagnoses in patients with suspected idiopathic pulmonary fibrosis (IPF) when an increase in lymphocytes is detected. Conventional transbronchial lung biopsy has a very low sensitivity in detecting the UIP pattern and its role in this clinical-radiological context is marginal. The introduction of less invasive methods such as transbronchial cryobiopsy show great promise to clinical practice as they can be used to obtain samples large enough to morphologically support a diagnosis of IPF or other idiopathic interstitial pneumonias, along with fewer complications. Recent advances in the field suggest that less invasive methods of lung sampling, without significant side effects, in combination with other diagnostic methods could replace the need for surgical lung biopsy in the future. Indeed, these new multidisciplinary procedures may become the main diagnostic work-up method for patients with suspected idiopathic interstitial pneumonia. PMID:26682637

  6. 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

  7. Idiopathic interstitial pneumonias: progress in classification, diagnosis, pathogenesis and management.

    PubMed Central

    King, Talmadge E.

    2004-01-01

    The idiopathic interstitial pneumonias are a heterogeneous group of poorly understood diseases with often devastating consequences for those afflicted. Subclassification of the idiopathic interstitial pneumonia based on clinical-radiological-pathological criteria has highlighted important pathogenic, therapeutic and prognostic implications. The most critical distinction is the presence of usual interstitial pneumonia, the histopathological pattern seen in idiopathic pulmonary fibrosis. Idiopathic pulmonary fibrosis has a worse response to therapy and prognosis. New insight into the pathophysiology of usual interstitial pneumonia suggests a distinctly fibroproliferative process, and antifibrotic therapies show promise. While the clinical and radiographic diagnosis of idiopathic interstitial pneumonias can be made confidently in some cases, many patients require surgical lung biopsy to determine their underlying histopathology. A structured, clinical-radiological-pathological approach to the diagnosis of the idiopathic interstitial pneumonias, with particular attention to the identification of idiopathic pulmonary fibrosis, insures proper therapy, enhances prognostication, and allows for further investigation of therapies aimed at distinct pathophysiology. Images Fig. 2 Fig. 4 Fig. 5 Fig. 6 PMID:17060957

  8. Smoking-related idiopathic interstitial pneumonia: A review.

    PubMed

    Margaritopoulos, George A; Harari, Sergio; Caminati, Antonella; Antoniou, Katerina M

    2016-01-01

    For many years, cigarette smoking has been considered as the leading cause of chronic obstructive pulmonary disease and lung cancer. Recently, however, it has also been associated with the development of diffuse interstitial lung diseases. In the latest classification of the major idiopathic interstitial pneumonias (IIP), the term smoking-related IIP has been introduced, including two entities, namely desquamative interstitial pneumonia (DIP) and respiratory bronchiolitis-interstitial lung disease (RB-ILD). Other entities in which smoking has a definite or suggested role include pulmonary Langerhan's cell histiocytosis, smoking-related interstitial fibrosis, combined pulmonary fibrosis and emphysema syndrome and idiopathic pulmonary fibrosis. In this review, we will focus on the mechanisms of smoking-related lung damage and on the clinical aspects of these disorders with the exception of idiopathic pulmonary fibrosis, which will be reviewed elsewhere in this review series. PMID:26138798

  9. Improvement in idiopathic nonspecific interstitial pneumonia after smoking cessation.

    PubMed

    Shinohara, Tsutomu; Kadota, Naoki; Hino, Hiroyuki; Naruse, Keishi; Ohtsuki, Yuji; Ogushi, Fumitaka

    2015-01-01

    Although cigarette smoking has been recognized as a risk factor for the development of several interstitial lung diseases, the relationship between smoking and nonspecific interstitial pneumonia (NSIP) has not yet been fully elucidated. We here present a case of fibrotic NSIP with mild emphysema in an elderly male with normal pulmonary function, whose symptoms, serum KL-6 level, and high-resolution computed tomography findings of interstitial changes markedly improved without medication following the cessation of smoking. Our case suggests that smoking may be an etiological factor in some patients with NSIP and that early smoking cessation before a clinically detectable decline in pulmonary function may be critical for smokers with idiopathic NSIP. PMID:26029566

  10. Improvement in idiopathic nonspecific interstitial pneumonia after smoking cessation

    PubMed Central

    Shinohara, Tsutomu; Kadota, Naoki; Hino, Hiroyuki; Naruse, Keishi; Ohtsuki, Yuji; Ogushi, Fumitaka

    2014-01-01

    Although cigarette smoking has been recognized as a risk factor for the development of several interstitial lung diseases, the relationship between smoking and nonspecific interstitial pneumonia (NSIP) has not yet been fully elucidated. We here present a case of fibrotic NSIP with mild emphysema in an elderly male with normal pulmonary function, whose symptoms, serum KL-6 level, and high-resolution computed tomography findings of interstitial changes markedly improved without medication following the cessation of smoking. Our case suggests that smoking may be an etiological factor in some patients with NSIP and that early smoking cessation before a clinically detectable decline in pulmonary function may be critical for smokers with idiopathic NSIP. PMID:26029566

  11. 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.

  12. 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

  13. 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

  14. 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

  15. 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

  16. Abnormal deposition of collagen/elastic vascular fibres and prognostic significance in idiopathic interstitial pneumonias

    PubMed Central

    Parra, Edwin Roger; Kairalla, Ronaldo Adib; de Carvalho, Carlos Roberto Ribeiro; Capelozzi, Vera Luiza

    2007-01-01

    Background Vascular remodelling has recently been shown to be a promising pathogenetic indicator in idiopathic interstitial pneumonias (IIPs). Aim To validate the importance of the collagen/elastic system in vascular remodelling and to study the relationships between the collagen/elastic system, survival and the major histological patterns of IIPs. Methods Collagen/elastic system fibres were studied in 25 patients with acute interstitial pneumonia/diffuse alveolar damage, 22 with non‐specific interstitial pneumonia/non‐specific interstitial pneumonia and 55 with idiopathic pulmonary fibrosis/usual interstitial pneumonia. The Picrosirius polarisation method and Weigert's resorcin–fuchsin histochemistry and morphometric analysis were used to evaluate the amount of vascular collagen/elastic system fibres and their association with the histological pattern of IIPs. The association between vascular remodelling and the degree of parenchymal fibrosis in usual interstitial pneumonia (UIP) was also considered. Results The vascular measurement of collagen/elastic fibres was significantly higher in UIP than in the lungs of controls, and in those with diffuse alveolar damage and those with non‐specific interstitial pneumonia. In addition, the increment of collagen/elastic fibres in UIP varied according to the degree and activity of the parenchymal fibrosis. The most important predictors of survival in UIP were vascular remodelling classification and vascular collagen deposition. Conclusion A progressive vascular fibroelastosis occurs in IIP histological patterns, probably indicating evolutionarily adapted responses to parenchymal injury. The vascular remodelling classification and the increase in vascular collagen were related to survival in IIP and possibly play a role in its pathogenesis. Further studies are needed to determine whether this relationship is causal or consequential. PMID:17251318

  17. Idiopathic pleuroparenchymal fibroelastosis, a new idiopathic interstitial pneumonia: A case report.

    PubMed

    Hurtado, Enrique Javier Soto; González, Maria Luisa Amaya; Soto, Maria Del Mar Elena; Rueda, Francisco Jose Cabello; Nadal, Francisco Javier Pérez; Cantero, Alberto Ruíz

    2016-08-01

    Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disease, idiopathic in most of the cases described in the literature. We report the case of a 55-year-old patient, non-smoker with tuberculosis treated in his youth, starting with progressive dyspnoea and cough, with radiographic abnormalities consisting of pleural thickening, bronchiectasis and structural distortion predominantly in the upper lobes. Due to functional impairment and persistent radiographic abnormalities, biopsy by video-assisted thoracoscopic surgical was decided. The presence of striking elastosis, absence of interstitial damage and abrupt boundary of the fibrous lesion with healthy lung allowed the diagnosis of IPPFE. Currently, the patient has no specific treatment and is in follow-up in the Transplant Unit. PMID:26810762

  18. Exercise pathophysiology and the role of oxygen therapy in idiopathic interstitial pneumonia.

    PubMed

    Troy, Lauren K; Young, Iven H; Lau, Edmund M T; Corte, Tamera J

    2016-08-01

    Exercise limitation is a common feature in idiopathic interstitial pneumonia (IIP). There are multiple contributing pathophysiological mechanisms, including ventilatory mechanical limitation, impaired gas exchange, pulmonary vascular insufficiency and peripheral muscle dysfunction. Progressive exertional dyspnoea and functional incapacity impact significantly on quality of life. Exercise-induced desaturation is frequently observed and is predictive of poorer outcomes. Tests to assess the cardiorespiratory system under stress (e.g. cardiopulmonary exercise testing and the 6-min walk test) can provide important physiologic and prognostic information as adjuncts to resting measurements of lung function. Despite many advances in understanding disease mechanisms, therapies to improve exercise capacity, symptom burden and quality of life are lacking. Exercise training and supplemental oxygen are two potential interventions that require closer evaluation in patients with IIP. PMID:26416262

  19. 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

  20. 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

  1. 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. PMID:26893034

  2. Germline SFTPA1 mutation in familial idiopathic interstitial pneumonia and lung cancer.

    PubMed

    Nathan, Nadia; Giraud, Violaine; Picard, Clément; Nunes, Hilario; Dastot-Le Moal, Florence; Copin, Bruno; Galeron, Laurie; De Ligniville, Alice; Kuziner, Nathalie; Reynaud-Gaubert, Martine; Valeyre, Dominique; Couderc, Louis-Jean; Chinet, Thierry; Borie, Raphaël; Crestani, Bruno; Simansour, Maud; Nau, Valérie; Tissier, Sylvie; Duquesnoy, Philippe; Mansour-Hendili, Lamisse; Legendre, Marie; Kannengiesser, Caroline; Coulomb-L'Hermine, Aurore; Gouya, Laurent; Amselem, Serge; Clement, Annick

    2016-04-15

    Idiopathic interstitial pneumonias (IIPs) comprise a heterogeneous group of rare lung parenchyma disorders with high morbidity and mortality, which can occur at all ages. In adults, the most common form of IIPs, idiopathic pulmonary fibrosis (IPF), has been associated with an increased frequency of lung cancer. The molecular basis of IIPs remains unknown in most cases. This study investigates IIP pathophysiology in 12 families affected by IPF and lung cancer. We identified, in a multigenerational family, nine members carrying a heterozygous missense mutation with evidence of pathogenicity in SFTPA1 that encodes the surfactant protein (SP)-A1. The mutation (p.Trp211Arg), which segregates with a disease phenotype characterized by either isolated IIP/IPF, or IPF associated with lung adenocarcinoma, is located in the carbohydrate recognition domain (CRD) of SP-A1 and involves a residue invariant throughout evolution, not only in SP-A1, but also in its close paralog SP-A2 and other CRD-containing proteins. As shown through functional studies, the p.Trp211Arg mutation impairs SP-A1 secretion. Immunohistochemistry studies on patient alveolar epithelium showed an altered SP-A expression pattern. Overall, this first report of a germline molecular defect in SFTPA1 unveils the key role of SP-A1 in the occurrence of several chronic respiratory diseases, ranging from severe respiratory insufficiency occurring early in life to the association of lung fibrosis and cancer in adult patients. These data also clearly show that, in spite of their structural and functional similarities, SP-A1 and SP-A2 are not redundant. PMID:26792177

  3. 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.

  4. 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

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

    PubMed

    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

  6. 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

  7. 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

  8. Efficacy of thrombomodulin for acute exacerbation of idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia: a nonrandomized prospective study

    PubMed Central

    Abe, Mitsuhiro; Tsushima, Kenji; Matsumura, Takuma; Ishiwata, Tsukasa; Ichimura, Yasunori; Ikari, Jun; Terada, Jiro; Tada, Yuji; Sakao, Seiichirou; Tanabe, Nobuhiro; Tatsumi, Koichiro

    2015-01-01

    Purpose Acute exacerbation (AE) is an important outcome of idiopathic pulmonary fibrosis (IPF) and nonspecific interstitial pneumonia (NSIP). Recombinant human soluble thrombomodulin (rhTM) is a new drug for the treatment of disseminated intravascular coagulation in Japan. The objective of this study was to evaluate the efficacy of rhTM for AE of IPF/NSIP. Methods Twenty-two patients with AE-idiopathic interstitial pneumonia (16 patients with IPF and six patients with NSIP) were enrolled in our study. Among them, eleven patients were treated with rhTM (rhTM group), and eleven patients were treated without rhTM (non-rhTM group). Patients admitted to our hospital prior to December 2013 were treated with rhTM, while those admitted after January 2014 were treated without rhTM. The primary endpoint was mortality at 90 days after AE treatment. The secondary endpoint was the safety of rhTM for AE-IPF/AE-NSIP. In addition, we examined prognostic factors of AE-IPF/AE-NSIP. Results The mortality rate was significantly lower in the rhTM group than in the non-rhTM group (mortality rate at 90 days: 36% vs 90%, P=0.023; median survival time: not reached vs 15.0 days, P=0.019). A univariate analysis revealed the respiratory rate (hazard ratio [HR] 1.09, 95% confidence interval [CI] 1.00–1.18, P=0.039) and rhTM administration (HR 0.21, 95% CI 0.06–0.77, P=0.013) as predictors of mortality at 90 days, and a multivariate analysis identified rhTM administration (HR 0.025, 95% CI 0.0006–0.94, P=0.046) as an independent predictor of mortality at 90 days. No serious adverse events were observed. Conclusion The administration of rhTM is associated with reductions in mortality in patients with AE-IPF/NSIP, without causing adverse events. PMID:26566367

  9. Scoring of the radiological picture of idiopathic interstitial pneumonia: a study to verify the reliability of the method

    PubMed Central

    Vanasek, Jiri; Koblizek, Vladimir; Novosad, Jakub; Elias, Pavel; Bartos, Vladimir; Sterclova, Martina

    2015-01-01

    Background Idiopathic pulmonary fibrosis (IPF) is a clinical form of usual interstitial pneumonia (UIP). Computed chest tomography (CT) has a fundamental role in the multidisciplinary diagnostics. However, it has not been verified if and how a subjective opinion of a radiologists or pneumologists can influence the assessment and overall diagnostic summary. Purpose To verify the reliability of the scoring system. Material and Methods Assessment of conformity of the radiological score of high-resolution CT (HRCT) of lungs in patients with IPF was performed by a group of radiologists and pneumologists. Personal data were blinded and the assessment was performed independently using the Dutka/Vasakova scoring system (modification of the Gay system). The final score of the single assessors was then evaluated by means of the paired Spearman’s correlation and analysis of the principal components. Results Two principal components explaining cumulatively a 62% or 73% variability of the assessment of the single assessors were extracted during the analysis. The groups did not differ both in terms of specialty and experience with the assessment of the HRCT findings. Conclusion According to our study, scoring of a radiological image using the Dutka/Vasakova system is a reliable method in the hands of experienced radiologists. Significant differences occur during the assessment performed by pneumologists especially during the evaluation of the alveolar changes. PMID:26600944

  10. Idiopathic Interstitial Pneumonias

    MedlinePlus

    ... ALL NEWS > Resources First Aid Videos Figures Images Audio Pronunciations The One-Page Manual of Health Quizzes ... ALL NEWS > Resources First Aid Videos Figures Images Audio Pronunciations The One-Page Manual of Health Quizzes ...

  11. 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. PMID:26253266

  12. The Ratio KL-6 to SLX in Serum for Prediction of the Occurrence of Drug-Induced Interstitial Lung Disease in Lung Cancer Patients with Idiopathic Interstitial Pneumonias Receiving Chemotherapy.

    PubMed

    Kashiwabara, Kosuke; Semba, Hiroshi; Fujii, Shinji; Tsumura, Shinsuke; Aoki, Ryota

    2015-01-01

    We retrospectively evaluated whether the ratio KL-6 to SLX in serum (K/S ratio) before chemotherapy was a predictor for the occurrence of drug-induced interstitial lung disease (D-ILD) in lung cancer patients with idiopathic interstitial pneumonias (IIPs). D-ILD occurred in 8 of 20 IIPs-positive cases and in 14 of 100 IIPs-negative cases (40 vs. 14%, p = .015). In IIPs-positive cases, the high K/S ratio (>20) before first-line chemotherapy had a tendency to increase the risk of D-ILD (p = .085). Serum K/S ratio may be a useful predictor for the occurrence of D-ILD in lung cancer patients with IIPs. PMID:26305851

  13. Role of α1 and α2 chains of type IV collagen in early fibrotic lesions of idiopathic interstitial pneumonias and migration of lung fibroblasts.

    PubMed

    Urushiyama, Hirokazu; Terasaki, Yasuhiro; Nagasaka, Shinya; Terasaki, Mika; Kunugi, Shinobu; Nagase, Takahide; Fukuda, Yuh; Shimizu, Akira

    2015-08-01

    Early fibrotic lesions are thought to be the initial findings of fibrogenesis in idiopathic interstitial pneumonias, but little is known about their properties. Type IV collagen comprises six gene products, α1-α6, and although it is known as a major basement membrane component, its abnormal deposition is seen in fibrotic lesions of certain organs. We studied the expression of type I and III collagen and all α chains of type IV collagen in lung specimens from patients with usual interstitial pneumonia (UIP) or organizing pneumonia (OP) via immunohistochemistry. With cultured lung fibroblasts, we analyzed the expression and function of all α chains of type IV collagen via immunohistochemistry, western blotting, real-time quantitative PCR, and a Boyden chamber migration assay after the knockdown of α1 and α2 chains. Although we observed type I and III collagens in early fibrotic lesions of both UIP and OP, we found type IV collagen, especially α1 and α2 chains, in early fibrotic lesions of UIP but not OP. Fibroblasts enhanced the expression of α1 and α2 chains of type IV collagen after transforming growth factor-β1 stimulation. Small interfering RNA against α1 and α2 chains increased fibroblast migration, with upregulated phosphorylation of focal adhesion kinase (FAK), and adding medium containing fibroblast-produced α1 and α2 chains reduced the increased levels of fibroblast migration and phosphorylation of FAK. Fibroblasts in OP were positive for phosphorylated FAK but fibroblasts in UIP were not. These results suggest that fibroblasts in UIP with type IV collagen deposition, especially α1 and α2 chains, have less ability to migrate from early fibrotic lesions than fibroblasts in OP without type IV collagen deposition. Thus, type IV collagen deposition in early fibrotic lesions of UIP may be implicated in refractory pathophysiology including migration of lesion fibroblasts via a FAK pathway. PMID:26006016

  14. Characterisation of patients with interstitial pneumonia with autoimmune features.

    PubMed

    Oldham, Justin M; Adegunsoye, Ayodeji; Valenzi, Eleanor; Lee, Cathryn; Witt, Leah; Chen, Lena; Husain, Aliya N; Montner, Steven; Chung, Jonathan H; Cottin, Vincent; Fischer, Aryeh; Noth, Imre; Vij, Rekha; Strek, Mary E

    2016-06-01

    Patients with interstitial lung disease (ILD) may have features of connective tissue disease (CTD), but lack findings diagnostic of a specific CTD. A recent European Respiratory Society/American Thoracic Society research statement proposed criteria for patients with interstitial pneumonia with autoimmune features (IPAF).We applied IPAF criteria to patients with idiopathic interstitial pneumonia and undifferentiated CTD-ILD (UCTD). We then characterised the clinical, serological and morphological features of the IPAF cohort, compared outcomes to other ILD cohorts and validated individual IPAF domains using survival as an endpoint.Of 422 patients, 144 met IPAF criteria. Mean age was 63.2 years with a slight female predominance. IPAF cohort survival was marginally better than patients with idiopathic pulmonary fibrosis, but worse than CTD-ILD. A non-usual interstitial pneumonia pattern was associated with improved survival, as was presence of the clinical domain. A modified IPAF cohort of those meeting the clinical domain and a radiographic or histological feature within the morphological domain displayed survival similar to those with CTD-ILD.IPAF is common among patients with idiopathic interstitial pneumonia and UCTD. Specific IPAF features can identify subgroups with differential survival. Further research is needed to replicate these findings and determine whether patients meeting IPAF criteria benefit from immunosuppressive therapy. PMID:27103387

  15. A case of DIPNECH presenting as usual interstitial pneumonia.

    PubMed

    Chatterjee, Kshitij; Kamimoto, Jorge Jo; Dunn, Andrew; Mittadodla, Enchala; Joshi, Manish

    2016-01-01

    Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare disease that is classically described as presenting with cough, dyspnea, and wheezing in non-smoker middle aged females. Pulmonary function tests commonly demonstrate an obstructive pattern and CT of chest usually reveals diffuse air trapping with mosaic pattern. We present a case of patient with DIPNECH manifesting with restrictive pattern and as usual interstitial pneumonia on imaging. PMID:27238180

  16. Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparison of the clinicopathologic features and prognosis

    PubMed Central

    Xu, Jinfu; Liu, Jinming; Yi, Xianghua; Sun, Xiwen; Shi, Jingyun

    2014-01-01

    Background Nonspecific interstitial pneumonia (NSIP) has recently been proposed as a histologic type of idiopathic interstitial pneumonia (IIP), but its broad spectrum of clinicopathologic findings and variable prognosis are poorly understood. It is particularly unclear how NSIP and usual interstitial pneumonia (UIP) are related. The present study investigated the clinicopathologic features and prognosis of NSIP, and its differential diagnosis from UIP. Methods The clinicopathologic findings and prognosis in 21 NSIP and 18 UIP patients who underwent surgical or video-assisted thoracoscopic lung biopsy were reviewed. Results NSIP was more frequent in women and showed nonspecific clinical manifestations. High-resolution computed tomography (HRCT) demonstrated ground-glass, net-like, and patchy attenuation in both lungs. Semiquantitative HRCT showed a median fibrosis score of 3 (range, 0 to 7) in NSIP patients and 5 (range, 2 to 7) in UIP patients (P<0.01). On histopathologic examination, NSIP cases were heterogeneous and the findings could be categorized into cellular and fibrosing patterns. The mean age of the NSIP and UIP patients was 48 and 60 years, respectively. The frequencies of fibroblast foci, myogelosis, honeycomb lesions, and pulmonary structural destruction in NSIP and UIP patients were 16.7% and 100% (P<0.001), 22.2% and 85.7% (P<0.05), 16.7% and 92.9% (P<0.001), and 27.8% and 100% (P<0.05), respectively. The responses to glucocorticoid treatment and the prognosis were significantly greater in NSIP than those in UIP. Conclusions NSIP was difficult to be differentiated from UIP by general clinical manifestations, but HRCT can be helpful for this purpose. Definitive diagnosis depends on the results of surgical lung biopsy. PMID:25364525

  17. Significance of granulomatous inflammation in usual interstitial pneumonia.

    PubMed

    Tomic, Rade; Kim, Hyun J; Perlman, David M; Bors, Melinda; Allen, Tadashi; Ritter, Jon; Dincer, H Erhan; Bhargava, Maneesh

    2015-01-01

    Sarcoidosis is a systemic granulomatous disease of unclear etiology with characteristic pulmonary lesions. We describe 2 unique cases of sarcoidosis where after approximately 20 years of clinical quiescence, patients developed interstitial opacities on chest CT scan and an increase in shortness of breath. With lack of therapeutic response to a course of prednisone, both patients underwent a surgical lung biopsy that revealed a pattern consistent with Usual Interstitial Pneumonia (UIP) with honeycombing and fibroblastic foci. Postoperatively, the course of the disease was consistent with what would be expected in Idiopathic Pulmonary Fibrosis. Ultimately the disease progressed with one patient needed lung transplantation and the other requiring high-flow oxygen supplementation. In conclusion, we present two patients in whom a diagnosis of sarcoidosis preceded the diagnosis of UIP by 20 years or more. The subsequent course of disease in both patients was consistent with Idiopathic Pulmonary Fibrosis. PMID:26278696

  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. PMID:27231862

  19. 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

  20. [Bronchiolitis obliterans with organizing pneumonia associated with idiopathic thrombocytopenic purpura].

    PubMed

    Presas, J L; Piriz, J; Serra, S L; Paz, E D; Allievi, A; Kartin, D; Olmedo, G

    1998-01-01

    We report a case of a 35 year-old woman with idiopathic thrombocytopenic purpura (ITP) who, under treatment with immunosuppressive drugs, developed bilateral interstitial pulmonary disease. Previously she had been splenectomized and treated with corticosteroids and cyclosporin. During the clinical course, the patient developed alterations of the hepatogram and presented a positive serology for Epstein-Barr virus. The lung biopsy showed the histologic pattern of obliterative bronchiolitis, interstitial inflammatory infiltration and intraalveolar pneumonia (BOOP). We could not find in the literature a previous report in which ITP was associated with BOOP. Of interest was the spontaneous remission of the pulmonary disease after suppression of cyclosporin and positive serology for Epstein-Barr virus. PMID:9674210

  1. Idiopathic lipoid pneumonia successfully treated with prednisolone.

    PubMed

    Lococo, Filippo; Cesario, Alfredo; Porziella, Venanzio; Mulè, Antonino; Petrone, Gianluigi; Margaritora, Stefano; Granone, Pierluigi

    2012-01-01

    Lipoid pneumonia (LP) is a rare type of pneumonia that is radiologically characterized by lung infiltrates, although imaging alone may not be diagnostic. We describe an unusual 61-year-old patient with idiopathic LP presenting as a solitary pulmonary nodule mimicking lung cancer because of its rapid growth. After treatment with oral prednisone, a control chest radiogram indicated complete normalization of the radiologic features. This case shows that LP should be considered in the diagnostic assessment of any undefined pulmonary mass, after malignancy has been pathologically excluded. PMID:21419490

  2. 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

  3. Pulmonary Vein Stenosis Mimicking Nonspecific Interstitial Pneumonia

    PubMed Central

    Linga, Karthika R.; Khoor, Andras; Phelan, Jonathan A.; Mira-Avendano, Isabel

    2015-01-01

    Pulmonary vein stenosis (PVS) is a known complication after catheter ablation of arrhythmias. Surprisingly, little information is available on its manifestations in the lung. We describe the case of a 39-year-old woman who presented from an outside hospital with worsening shortness of breath after catheter ablation of pulmonary veins for atrial fibrillation. After an initial diagnosis of pneumonia and its nonimprovement with antibiotics, a surgical lung biopsy was done and interpreted as nonspecific interstitial pneumonia (NSIP) with vascular changes consistent with pulmonary arterial hypertension. Later, she was admitted to our institution where a transthoracic echocardiogram (TTE) and subsequent computed tomography (CT) angiogram of the heart showed severe stenosis of all four pulmonary veins. The previous lung biopsy was rereviewed and reinterpreted as severe parenchymal congestion mimicking NSIP. Our case demonstrates that PVS is an underrecognized complication of catheter ablation, and increased awareness among both clinicians and pathologists is necessary to avoid misdiagnosis. PMID:26779359

  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. Normal Lung Quantification in Usual Interstitial Pneumonia Pattern: The Impact of Threshold-based Volumetric CT Analysis for the Staging of Idiopathic Pulmonary Fibrosis

    PubMed Central

    Ohkubo, Hirotsugu; Kanemitsu, Yoshihiro; Uemura, Takehiro; Takakuwa, Osamu; Takemura, Masaya; Maeno, Ken; Ito, Yutaka; Oguri, Tetsuya; Kazawa, Nobutaka; Mikami, Ryuji; Niimi, Akio

    2016-01-01

    Background Although several computer-aided computed tomography (CT) analysis methods have been reported to objectively assess the disease severity and progression of idiopathic pulmonary fibrosis (IPF), it is unclear which method is most practical. A universal severity classification system has not yet been adopted for IPF. Objective The purpose of this study was to test the correlation between quantitative-CT indices and lung physiology variables and to determine the ability of such indices to predict disease severity in IPF. Methods A total of 27 IPF patients showing radiological UIP pattern on high-resolution (HR) CT were retrospectively enrolled. Staging of IPF was performed according to two classification systems: the Japanese and GAP (gender, age, and physiology) staging systems. CT images were assessed using a commercially available CT imaging analysis workstation, and the whole-lung mean CT value (MCT), the normally attenuated lung volume as defined from −950 HU to −701 Hounsfield unit (NL), the volume of the whole lung (WL), and the percentage of NL to WL (NL%), were calculated. Results CT indices (MCT, WL, and NL) closely correlated with lung physiology variables. Among them, NL strongly correlated with forced vital capacity (FVC) (r = 0.92, P <0.0001). NL% showed a large area under the receiver operating characteristic curve for detecting patients in the moderate or advanced stages of IPF. Multivariable logistic regression analyses showed that NL% is significantly more useful than the percentages of predicted FVC and predicted diffusing capacity of the lungs for carbon monoxide (Japanese stage II/III/IV [odds ratio, 0.73; 95% confidence intervals (CI), 0.48 to 0.92; P < 0.01]; III/IV [odds ratio. 0.80; 95% CI 0.59 to 0.96; P < 0.01]; GAP stage II/III [odds ratio, 0.79; 95% CI, 0.56 to 0.97; P < 0.05]). Conclusion The measurement of NL% by threshold-based volumetric CT analysis may help improve IPF staging. PMID:27031615

  6. Idiopathic granulomatous interstitial nephritis responsive to mycophenolate mofetil therapy.

    PubMed

    Leeaphorn, Napat; Stokes, Michael B; Ungprasert, Patompong; Lecates, William

    2014-04-01

    Granulomatous interstitial nephritis (GIN) is a rare histologic disease. Various causes have been reported in the literature, including drugs, sarcoidosis, and infections. Other incidents have no discernible cause and are identified as idiopathic. We report a 68-year-old white man who presented with acute kidney injury and was given a diagnosis of idiopathic GIN. Mycophenolate mofetil treatment was elected because of steroid toxicity. He responded well to mycophenolate mofetil and has been in remission for more than 3 years. To our knowledge, this is the first report of successful treatment with mycophenolate mofetil of an adult patient with idiopathic GIN. PMID:24315767

  7. 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

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

    PubMed

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

    2016-02-01

    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

  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. Hermansky-Pudlak syndrome type 4 with interstitial pneumonia.

    PubMed

    Sakata, Yoshihiko; Kawamura, Kodai; Ichikado, Kazuya; Suga, Moritaka; Yoshioka, Masakazu

    2013-01-01

    Hermansky-Pudlak syndrome (HPS) is an autosomal recessive disorder characterized by oculocutaneous albinism, bleeding tendency, and lysosomal accumulation of ceroid-like material, with occasional development of interstitial pneumonia (IP). Nine genetically distinct subtypes of HPS are known in humans; IP develops primarily in types 1 and 4. Most reported cases of HPS with IP are type 1, and there are no published reports of type 4 in Japanese individuals. A 58-year-old man with congenital oculocutaneous albinism and progressive dyspnea for 1 month was admitted to our hospital. We administered high-dose corticosteroids on the basis of a diagnosis of acute exacerbation of interstitial pneumonia. Respiratory symptoms and the findings of high-resolution computed tomography (CT) showed improvement. He was diagnosed with HPS type 4 with interstitial pneumonia on the basis of gene analysis. He has been receiving pirfenidone for 1 year and his condition is stable. This is the first report on the use of pirfenidone for HPS with IP caused by a novel mutation in the HPS4 gene. We conclude that HPS should be suspected in patients with albinism and interstitial pneumonia. High-dose corticosteroid treatment may be useful in cases of acute exacerbation of interstitial pneumonia due to HPS-4, and pirfenidone may be useful and well tolerated in patients with HPS-4. PMID:26029628

  11. Hermansky-Pudlak syndrome with nonspecific interstitial pneumonia.

    PubMed

    Furuhashi, Kazuki; Enomoto, Noriyuki; Fujisawa, Tomoyuki; Hashimoto, Dai; Inui, Naoki; Nakamura, Yutaro; Suda, Takafumi

    2014-01-01

    We herein report a case of Hermansky-Pudlak syndrome (HPS) with nonspecific interstitial pneumonia (NSIP). A 58-year-old Japanese woman presented with oculocutaneous albinism and dyspnea on exertion. A high resolution computed tomography scan showed areas of reticular and ground glass opacity in the lungs, and a surgical lung biopsy revealed fibrotic NSIP. Foamy type 2 pneumocytes and the absence of dense granules in platelets were also observed, consistent with a diagnosis of HPS. Ultimately, a genetic analysis revealed a mutation in the HPS1 gene. The interstitial pneumonia progressed despite treatment with prednisolone, cyclosporine A and pirfenidone. In this report, we discuss the pathological lung features and treatment of HPS associated with interstitial pneumonia. PMID:24583434

  12. Normalization of lung function following treatment of secondary usual interstitial pneumonia: a case report.

    PubMed

    Hohberger, Laurie A; Montero-Arias, Felicia; Roden, Anja C; Vassallo, Robert

    2015-01-01

    Usual interstitial pneumonia (UIP) is the most common idiopathic interstitial pneumonia (IIP) and is associated with a poor prognosis and poor responsiveness to immunosuppressive therapy. We present a case of a woman with steroid-responsive biopsy-proven UIP with significant and sustained improvement in pulmonary function. A female in her 40s presented following a one-year history of progressive dyspnea, a 20 lb weight loss, and fatigue. Imaging of the chest with computed tomography (CT) showed bibasilar subpleural reticular opacities and minimal peripheral honeycombing. Comprehensive connective tissue disease (CTD) antibody testing was negative. Pulmonary function testing showed moderate impairment with reduction in forced vital capacity (FVC, 69% predicted), forced expiratory volume in one second (FEV1 73% predicted), and diffusing capacity for carbon monoxide (DLCO, 52% predicted). Surgical lung biopsy showed UIP with prominent inflammatory infiltrates. Following treatment with prednisone and azathioprine, the patient's symptoms resolved, while objective pulmonary function testing showed normalization of lung function, which is sustained at >4 years of follow-up. Improvement in lung function following immunosuppressive therapy is distinctly uncommon in either idiopathic or secondary UIP. This report suggests that occasionally, patients with secondary UIP occurring in the context of otherwise undefinable autoimmune clinical syndromes may be responsive to immunosuppressive therapy. PMID:25922588

  13. Normalization of Lung Function Following Treatment of Secondary Usual Interstitial Pneumonia: A Case Report

    PubMed Central

    Hohberger, Laurie A; Montero-Arias, Felicia; Roden, Anja C; Vassallo, Robert

    2015-01-01

    Usual interstitial pneumonia (UIP) is the most common idiopathic interstitial pneumonia (IIP) and is associated with a poor prognosis and poor responsiveness to immunosuppressive therapy. We present a case of a woman with steroid-responsive biopsy-proven UIP with significant and sustained improvement in pulmonary function. A female in her 40s presented following a one-year history of progressive dyspnea, a 20 lb weight loss, and fatigue. Imaging of the chest with computed tomography (CT) showed bibasilar subpleural reticular opacities and minimal peripheral honeycombing. Comprehensive connective tissue disease (CTD) antibody testing was negative. Pulmonary function testing showed moderate impairment with reduction in forced vital capacity (FVC, 69% predicted), forced expiratory volume in one second (FEV1 73% predicted), and diffusing capacity for carbon monoxide (DLCO, 52% predicted). Surgical lung biopsy showed UIP with prominent inflammatory infiltrates. Following treatment with prednisone and azathioprine, the patient’s symptoms resolved, while objective pulmonary function testing showed normalization of lung function, which is sustained at >4 years of follow-up. Improvement in lung function following immunosuppressive therapy is distinctly uncommon in either idiopathic or secondary UIP. This report suggests that occasionally, patients with secondary UIP occurring in the context of otherwise undefinable autoimmune clinical syndromes may be responsive to immunosuppressive therapy. PMID:25922588

  14. 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.

  15. A case of vildagliptin-induced interstitial pneumonia.

    PubMed

    Kuse, Naoyuki; Abe, Shinji; Kuribayashi, Hidehiko; Inomata, Minoru; Saito, Hitoshi; Fukuda, Yuh; Gemma, Akihiko

    2016-01-01

    A 65-year-old Japanese male with type 2 diabetes mellitus was admitted to our hospital with a productive cough and worsening dyspnea. He had started receiving vildagliptin, which is one of the dipeptideylpeptidase-4 (DPP-4) inhibitors, several days before the appearance of his symptoms. Laboratory findings revealed markedly elevated levels of immunoglobulin E and Krebs von den Lungen-6. Chest computed tomography revealed ground-glass opacity with irregular reticulation throughout both lungs. Biopsy specimens by transbronchial lung biopsy showed subacute interstitial pneumonia and an organizing pneumonia pattern with acute alveolar injury. The drug lymphocyte stimulation test showed a positive result for vildagliptin. Withdrawal of vildagliptin and administration of glucocorticoid treatment improved his respiratory condition and radiological findings. Therefore, we diagnosed the patient with vildagliptin-induced interstitial pneumonia based on both his clinical course and pathological findings. Interstitial pneumonia as a side effect of vildagliptin is rare. It may be necessary to monitor the respiratory condition of patients upon administration of DPP-4 inhibitors until further evidence is obtained. PMID:27144110

  16. A case of vildagliptin-induced interstitial pneumonia

    PubMed Central

    Kuse, Naoyuki; Abe, Shinji; Kuribayashi, Hidehiko; Inomata, Minoru; Saito, Hitoshi; Fukuda, Yuh; Gemma, Akihiko

    2016-01-01

    A 65-year-old Japanese male with type 2 diabetes mellitus was admitted to our hospital with a productive cough and worsening dyspnea. He had started receiving vildagliptin, which is one of the dipeptideylpeptidase-4 (DPP-4) inhibitors, several days before the appearance of his symptoms. Laboratory findings revealed markedly elevated levels of immunoglobulin E and Krebs von den Lungen-6. Chest computed tomography revealed ground-glass opacity with irregular reticulation throughout both lungs. Biopsy specimens by transbronchial lung biopsy showed subacute interstitial pneumonia and an organizing pneumonia pattern with acute alveolar injury. The drug lymphocyte stimulation test showed a positive result for vildagliptin. Withdrawal of vildagliptin and administration of glucocorticoid treatment improved his respiratory condition and radiological findings. Therefore, we diagnosed the patient with vildagliptin-induced interstitial pneumonia based on both his clinical course and pathological findings. Interstitial pneumonia as a side effect of vildagliptin is rare. It may be necessary to monitor the respiratory condition of patients upon administration of DPP-4 inhibitors until further evidence is obtained. PMID:27144110

  17. Ultrastructural evidence of alveolar epithelial injury in idiopathic bronchiolitis obliterans-organizing pneumonia.

    PubMed Central

    Myers, J. L.; Katzenstein, A. L.

    1988-01-01

    The ultrastructural features of idiopathic bronchiolitis obliterans-organizing pneumonia (BOOP) were studied in 9 patients. As expected, the characteristic air space fibrosis was composed of spindled fibroblasts and myofibroblasts arranged concentrically within an electron-lucent stroma. In 6 patients there was evidence of incorporation of air space fibrosis into the interstitium. A surprising finding in all patients was the presence of extensive epithelial damage involving peribronchiolar alveolar septa. Necrosis and sloughing of alveolar lining cells resulted in denuding of epithelial basal laminae. Complex infoldings and deep invaginations of the denuded basal laminae into alveolar septa were common. These ultrastructural changes involving the interstitium are similar to those occurring in the interstitial pneumonias, and suggest that BOOP also results from acute epithelial injury. The different clinical manifestations and prognosis of these entities may relate to the peribronchiolar localization of the epithelial damage in BOOP compared with more diffuse involvement of distal lung in the interstitial pneumonias. Images Figure 2 Figure 3 Figure 1 Figure 4 Figure 5 Figure 6 Figure 7 PMID:3394793

  18. [An autopsy case of pulmonary tumor thrombotic microangiopathy complicated with interstitial pneumonia and lipoid pneumonia].

    PubMed

    Ota, Kyoko; Matsuyama, Masashi; Kokuho, Nariaki; Masuko, Hironori; Hayashi, Hiroki; Iizuka, Takashi; Hayashibara, Kenji; Saito, Takefumi; Kawabata, Yoshinori

    2009-06-01

    A 67-year-old woman was admitted to our hospital because of progressive dyspnea, cough, bloody sputum, and backache. Chest radiography and CT scans showed bilateral diffuse interstitial shadows, bilateral pleural effusion and dilatation of the pulmonary artery. Echocardiography indicated pulmonary hypertension, and the serum tumor marker levels were elevated. We performed right cardiac catheterization, and withdrew some blood from a pulmonary artery catheter in the wedge position. We confirmed moderate pulmonary hypertension, and adenocarcinoma-like malignant cells were seen in the aspirated blood. The patient died of progressive respiratory failure despite supportive care. In addition to PTTM and lymphangiosis carcinomatosa, autopsy of the right lung revealed interstitial pneumonia and lipoid pneumonia, both of which were not reported before to be associated with PTTM. PMID:19601530

  19. Proteomic analysis in usual and nonspecific interstitial pneumonia.

    PubMed

    Ohara, Ichiyo; Aida, Shinsuke; Shimazaki, Hideyuki; Kobayashi, Hideo; Tsuda, Hitoshi; Toda, Tosifusa; Nakanishi, Kuniaki; Tamai, Seiichi

    2014-03-01

    Differentiating nonspecific interstitial pneumonia (NSIP) from usual interstitial pneumonia (UIP) is important for the determination of both treatment and prognosis. Using two-dimensional fluorescence difference gel electrophoresis (2D-DIGE), we examined 8 UIPs, 8 NSIPs, and 30 normal lung tissues. Comparisons with control in 2D-DIGE showed that (a) in UIP, nine protein spots were significantly upregulated and seven were significantly downregulated, (b) in NSIP, four protein spots were significantly upregulated and nine were significantly downregulated. The detected proteins were analyzed by MALDI-TOF mass spectrometry, allowing qualitative differences in vimentin subtypes to be characterized. One vimentin subtype was upregulated in UIP, while another one was downregulated in NSIP (vs. control). These different characteristics were partially supported by the results of Western blot analysis. Our immunohistochemistry revealed vimentin expression within fibroblasts (a) in fibroblastic foci in UIP and (b) in fibrotic alveolar walls in NSIP. Differences in vimentin subtypes may provide useful biomarkers for separating NSIP from UIP, alongside differences in histological characteristics. PMID:24048960

  20. Radiologic-pathologic discordance in biopsy-proven usual interstitial pneumonia.

    PubMed

    Yagihashi, Kunihiro; Huckleberry, Jason; Colby, Thomas V; Tazelaar, Henry D; Zach, Jordan; Sundaram, Baskaran; Pipavath, Sudhakar; Schwarz, Marvin I; Lynch, David A

    2016-04-01

    The aim of this study was to compare the clinical, radiological and histological findings in a large population of subjects enrolled during a multicentre study of idiopathic pulmonary fibrosis, with a focus on discordance between imaging and histologic diagnoses of usual interstitial pneumonia (UIP).Two independent radiologists retrospectively reviewed 241 subjects who underwent high-resolution computed tomography (HRCT) and surgical lung biopsies. HRCT findings were classified as UIP, possible UIP and inconsistent with UIP. Histological findings were classified as definite, probable, possible and not UIP.Of the 241 cases, 102 (42.3%) had HRCT findings of UIP, 64 (26.6%) had possible UIP and 75 (31.1%) were inconsistent with UIP. Among those with UIP on HRCT, 99 (97.1%) had histologically definite or probable UIP (concordant group), and 71 (94.7%) of those with "inconsistent" HRCT features had histologically definite or probable UIP (discordant group). Discordant subjects were slightly younger and less likely to be smokers than concordant subjects, but no survival differences were identified.In this population of patients enrolled with a diagnosis of idiopathic pulmonary fibrosis, 94.7% of those with HRCT findings "inconsistent with UIP" demonstrated histological UIP. This suggests that the term "inconsistent with UIP" is misleading. PMID:26917616

  1. [A Case of Gastric Cancer Associated with Sjögren's Syndrome and Interstitial Pneumonia].

    PubMed

    Ido, Mirai; Mishima, Hideyuki; Kimura, Kengo; Iwata, Tsutomu; Kiyota, Yoshiharu; Komaya, Kenichi; Saito, Takuya; Ohashi, Norifumi; Arikawa, Takashi; Ishiguro, Seiji; Komatsu, Shunichiro; Miyachi, Masahiko; Sano, Tsuyoshi

    2015-11-01

    A 77-year-old man presented with poor appetite and dyspnea. A gastroendoscopy showed an advanced gastric cancer and a CT scan demonstrated diffuse interstitial infiltrative shadows in both lungs. Laboratory data showed high level of anti-SSA and anti-SSB antibodies, suggestive of interstitial pneumonia associated with Sjögren's syndrome. Although the levels of KL-6 and SP-D, markers of interstitial pneumonia, decreased after steroid and immunosuppressive therapy, the CT findings of interstitial pneumonia showed no remarkable change. Surgery was performed 2 months after the administration of prednisolone since the respiratory function had improved, allowing the administration of general anesthesia. A CT scan revealed remarkable improvement of the lung lesions after the surgery. Therefore, it is likely that Sjögren's syndrome and interstitial pneumonia manifested as paraneoplastic syndromes in the presented case. PMID:26805235

  2. [Lung transplantation in patients with interstitial lung disease/idiopathic pulmonary fibrosis].

    PubMed

    Murer, Christian; Benden, Christian

    2016-01-01

    Lung transplantation is an established therapy for advanced lung disease. Among the common disease indications for lung transplantation, patients with interstitial lung disease, in particular, idiopathic pulmonary fibrosis (IPF), have the worst prognosis. Thus referral to a transplant center should ideally be realised at the time of diagnosis of usual interstitial pneumonitis (UIP), regardless of lung function, in order to carry out a through initial assessment and evaluation. PMID:26884220

  3. 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. PMID:26617847

  4. [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

  5. Do you really know precise radiologic-pathologic correlation of usual interstitial pneumonia?

    PubMed

    Johkoh, Takeshi; Sumikawa, Hiromotsu; Fukuoka, Junya; Tanaka, Tomonori; Fujimoto, Kiminori; Takahashi, Masashi; Tomiyama, Noriyuki; Kondo, Yasuhiro; Taniguchi, Hiroyuki

    2014-01-01

    Although usual interstitial pneumonia (UIP) is the most common chronic interstitial pneumonia, understanding of pathologic backgrounds of CT findings has still not been enough. Since honeycombing on either scanning microgram or CT is essential for diagnosis of UIP in 2010 ATS-ERS-JRS-ALAT guide line, the role of radiologists has become much more important. We will summarize common and uncommon CT findings with radiologic-pathological correlation. PMID:23806534

  6. [Idiopathic Pulmonary Fibrosis].

    PubMed

    Prasse, A

    2015-10-01

    Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and a disease of the elderly. Cigarette smoking and longterm exposure to substances harming alveolar epithelial cells are risk factors for the development of IPF. There is also evidence for a genetic susceptibility. IPF is defined as the idiopathic variant of Usual Interstitial Pneumonitis (UIP). Diagnosis of IPF is complex and based on the exclusion of other diseases associated with an UIP pattern. The only cure is lung transplantation. In the last years there was a breakthrough in the treatment of IPF. With pirfenidone and nintedanib there are now two compounds approved for the treatment of IPF. PMID:26444136

  7. Bevacizumab‐induced chronic interstitial pneumonia during maintenance therapy in non‐small cell lung cancer

    PubMed Central

    Sekimoto, Yasuhito; Shukuya, Takehiko; Koyama, Ryo; Nagaoka, Tetsutaro; Takahashi, Kazuhisa

    2016-01-01

    Abstract Bevacizumab is a monoclonal antibody targeting the vascular endothelial growth factor receptor and a key drug for advanced non‐small cell lung cancer. There are few reports describing bevacizumab‐induced chronic interstitial pneumonia. A 62‐year‐old man with advanced non‐small cell lung cancer was admitted to our hospital with dyspnea. He previously received four courses of carboplatin plus paclitaxel with bevacizumab combination therapy and thereafter received four courses of maintenance bevacizumab monotherapy. A chest‐computed tomography scan on admission revealed diffuse ground glass opacity. He had not received any other drugs and did not have pneumonia. Thus, he was diagnosed with bevacizumab‐induced chronic interstitial pneumonia and was treated with a high dose of corticosteroids. After steroid treatment, his dyspnea and radiological findings improved. This case report is the first description of bevacizumab‐induced chronic interstitial pneumonia during maintenance therapy in a patient with non‐small cell lung cancer. PMID:27081491

  8. 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

  9. Interstitial pneumonia caused by inhalation of fumes of nickel and chrome.

    PubMed

    Hisatomi, Keiko; Ishii, Hiroshi; Hashiguchi, Koji; Seki, Masafumi; Ide, Mioko; Sugiyama, Kanako; Ishimoto, Hiroshi; Nakayama, Seiko; Mukae, Hiroshi; Kohno, Shigeru

    2006-11-01

    Two male industrial painters were admitted to hospital with dry cough and dyspnoea on exertion following a tank coating operation using a high-temperature spray paint consisting of a nickel-chromium alloy. Both patients showed hypoxaemia, peripheral leukocytosis, high levels of serum cytokines and bilateral ground-glass opacities on a chest CT scan. They were diagnosed with interstitial pneumonia caused by inhalation of nickel and chrome fumes and successfully treated with corticosteroid. These are rare cases of interstitial pneumonia associated with nickel/chromium inhalation. PMID:17052314

  10. 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

  11. 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. PMID:24567239

  12. Interstitial pneumonia induced by sorafenib in a patient with hepatocellular carcinoma: An autopsy case report

    PubMed Central

    YAMAGUCHI, TAKASHI; SEKI, TOSHIHITO; MIYASAKA, CHIKA; INOKUCHI, RYOSUKE; KAWAMURA, RINAKO; SAKAGUCHI, YUUTAKU; MURATA, MIKI; MATSUZAKI, KOICHI; NAKANO, YORIKA; UEMURA, YOSHIKO; OKAZAKI, KAZUICHI

    2015-01-01

    Sorafenib is a multikinase inhibitor currently approved in Japan for the treatment of unresectable hepatocellular carcinoma. Interstitial pneumonia induced by sorafenib may have a fatal outcome, and therefore, has recently been the focus of many studies. The current report presents an autopsy case of diffuse alveolar damage (DAD) that occurred in a 59-year-old male, who had been treated with sorafenib. The patient had been given sorafenib for six months and had exhibited no respiratory symptoms during this time. However, 19 days after sorafenib treatment was resumed, acute interstitial pneumonia developed. In previously reported cases, the first symptoms of pulmonary toxicity appeared following a limited treatment duration with sorafenib; this was in contrast to the patient in the current study, who developed the first symptoms after eight months. We therefore conclude that physicians must be aware of interstitial pneumonia as a potential pulmonary toxicity associated with sorafenib treatment when treatment with sorafenib is resumed, even after prolonged use. In addition, to best of our knowledge, this is the first case of a postmortem examination reported in patient with interstitial pneumonia induced by sorafenib treatment. PMID:25789013

  13. 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.

  14. Reversed Halo Sign on CT as a Presentation of Lymphocytic Interstitial Pneumonia

    PubMed Central

    Freeman, Marcus D; Grajo, Joseph R; Karamsadkar, Neel D; Steffensen, Thora S; Hazelton, Todd R

    2013-01-01

    A 52 year-old African American female with a past medical history of symptomatic uterine fibroids and increasing abdominal circumference underwent abdominal computed tomography (CT) as part of her workup. Because of an abnormality in the left lower lobe, CT of the chest was subsequently performed and showed a focal region of discontinuous crescentic consolidation with central ground glass opacification in the right lower lobe, suggestive of the reversed halo sign. The patient underwent percutaneous CT-guided core biopsy of the lesion, which demonstrated lymphocytic interstitial pneumonia, a benign lymphoproliferative disease characterized histologically by small lymphocytes and plasma cells. This case report describes the first histologically confirmed presentation of lymphocytic interstitial pneumonia with the reversed halo sign on CT. PMID:24421923

  15. 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

  16. Giant cell interstitial pneumonia in patients without hard metal exposure: analysis of 3 cases and review of the literature.

    PubMed

    Khoor, Andras; Roden, Anja C; Colby, Thomas V; Roggli, Victor L; Elrefaei, Mohamed; Alvarez, Francisco; Erasmus, David B; Mallea, Jorge M; Murray, David L; Keller, Cesar A

    2016-04-01

    Giant cell interstitial pneumonia is a rare lung disease and is considered pathognomonic for hard metal lung disease, although some cases with no apparent hard metal (tungsten carbide cobalt) exposure have been reported. We aimed to explore the association between giant cell interstitial pneumonia and hard metal exposure. Surgical pathology files from 2001 to 2004 were searched for explanted lungs with the histopathologic diagnosis of giant cell interstitial pneumonia, and we reviewed the associated clinical histories. Mass spectrometry, energy-dispersive x-ray analysis, and human leukocyte antigen typing data were evaluated. Of the 455 lung transplants, 3 met the histologic criteria for giant cell interstitial pneumonia. Patient 1 was a 36-year-old firefighter, patient 2 was a 58-year-old welder, and patient 3 was a 45-year-old environmental inspector. None reported exposure to hard metal or cobalt dust. Patients 1 and 2 received double lung transplants; patient 3 received a left single-lung transplant. Histologically, giant cell interstitial pneumonia presented as chronic interstitial pneumonia with fibrosis, alveolar macrophage accumulation, and multinucleated giant cells of both alveolar macrophage and type 2 cell origin. Energy-dispersive x-ray analysis revealed no cobalt or tungsten particles in samples from the explanted lungs. None of the samples had detectable tungsten levels, and only patient 2 had elevated cobalt levels. The lack of appropriate inhalation history and negative analytical findings in the tissue from 2 of the 3 patients suggests that giant cell interstitial pneumonia is not limited to individuals with hard metal exposure, and other environmental factors may elicit the same histologic reaction. PMID:26997453

  17. A case of interstitial pneumonia associated with Pneumocystis carinii in a foal.

    PubMed

    Perron Lepage, M F; Gerber, V; Suter, M M

    1999-11-01

    Subacute interstitial pneumonia with diffuse alveolar damage, marked macrophage infiltration, and intracellular Pneumocystis carinii cysts is described in a 3-month-old Swiss warmblood foal. Clinically, the disease was characterized by sudden onset of respiratory distress with fatal outcome. Based on serum immunoglobulin G (IgG), IgA, and IgM values, no humoral immunosuppression was detected. Spleen, thymus, and bronchial lymph nodes did not reveal lymphoid depletion, as assessed by immunohistochemical staining of CD-3-positive cells. Immunopathogenesis of pulmonary infections with intracellular agents in foals is discussed. PMID:10568448

  18. Idiopathic pneumonia syndrome after hematopoietic cell transplantation: evidence of occult infectious etiologies.

    PubMed

    Seo, Sachiko; Renaud, Christian; Kuypers, Jane M; Chiu, Charles Y; Huang, Meei-Li; Samayoa, Erik; Xie, Hu; Yu, Guixia; Fisher, Cynthia E; Gooley, Ted A; Miller, Steven; Hackman, Robert C; Myerson, David; Sedlak, Ruth H; Kim, Yae-Jean; Fukuda, Takahiro; Fredricks, David N; Madtes, David K; Jerome, Keith R; Boeckh, Michael

    2015-06-11

    Newer diagnostic methods may link more idiopathic pneumonia syndrome (IPS) cases to an infectious agent. Bronchoalveolar lavage (BAL) samples from 69 hematopoietic cell transplant (HCT) recipients with IPS diagnosed between 1992 and 2006 were tested for 28 pathogens (3 bacteria and 25 viruses) by quantitative polymerase chain reaction and for Aspergillus by galactomannan assay. Research BALs from 21 asymptomatic HCT patients served as controls. Among 69 HCT patients with IPS, 39 (56.5%) had a pathogen detected. The most frequent pathogens were human herpesvirus-6 (HHV-6) (N = 20 [29%]) followed by human rhinovirus (HRV), cytomegalovirus (CMV), and Aspergillus (N = 8 [12%] in each). HHV-6 and HRV were rarely detected in controls, whereas CMV and Aspergillus were occasionally detected with low pathogen load. Patients with pathogens had worse day-100 survival than those without (hazard ratio, 1.88; P = .03). Mortality in patients with only pathogens of "uncertain" significance in lung was similar to that in patients with pathogens of "established" significance. Metagenomic next-generation sequencing did not reveal additional significant pathogens. Our study demonstrated that approximately half of patients with IPS had pathogens detected in BAL, and pathogen detection was associated with increased mortality. Thus, an expanded infection detection panel can significantly increase the diagnostic precision for idiopathic pneumonia. PMID:25918347

  19. [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

  20. 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

  1. An observational study of giant cell interstitial pneumonia and lung fibrosis in hard metal lung disease

    PubMed Central

    Tanaka, Junichi; Moriyama, Hiroshi; Terada, Masaki; Takada, Toshinori; Suzuki, Eiichi; Narita, Ichiei; Kawabata, Yoshinori; Yamaguchi, Tetsuo; Hebisawa, Akira; Sakai, Fumikazu; Arakawa, Hiroaki

    2014-01-01

    Background Hard metal lung disease has various pathological patterns including giant cell interstitial pneumonia (GIP) and usual interstitial pneumonia (UIP). Although the UIP pattern is considered the prominent feature in advanced disease, it is unknown whether GIP finally progresses to the UIP pattern. Objectives To clarify clinical, pathological and elemental differences between the GIP and UIP patterns in hard metal lung disease. Methods A cross-sectional study of patients from 17 institutes participating in the 10th annual meeting of the Tokyo Research Group for Diffuse Parenchymal Lung Diseases, 2009. Nineteen patients (seven female) diagnosed with hard metal lung disease by the presence of tungsten in lung specimens were studied. Results Fourteen cases were pathologically diagnosed as GIP or centrilobular inflammation/fibrosing. The other five cases were the UIP pattern or upper lobe fibrosis. Elemental analyses of lung specimens of GIP showed tungsten throughout the centrilobular fibrotic areas. In the UIP pattern, tungsten was detected in the periarteriolar area with subpleural fibrosis, but no association with centrilobular fibrosis or inflammatory cell infiltration. The GIP group was younger (43.1 vs 58.6 years), with shorter exposure duration (73 vs 285 months; p<0.01), lower serum KL-6 (398 vs 710 U/mL) and higher lymphocyte percentage in bronchoalveolar lavage fluid (31.5% vs 3.22%; p<0.05) than the fibrosis group. Conclusions The UIP pattern or upper lobe fibrosis is remarkably different from GIP in distribution of hard metal elements, associated interstitial inflammation and fibrosis, and clinical features. In hard metal lung disease, the UIP pattern or upper lobe fibrosis may not be an advanced form of GIP. PMID:24674995

  2. The importance of determining surgical indications in cases of lung cancer and interstitial pneumonia with multiple intrapulmonary lymph nodes

    PubMed Central

    Nakajima, Yuki; Akiyama, Hirohiko; Kinoshita, Hiroyasu; Atari, Maiko; Fukuhara, Mitsuro; Sakai, Hiroshi; Uramoto, Hidetaka

    2015-01-01

    Introduction When considering treatment for pulmonary cancer complicated by interstitial pneumonia, the greatest problem is acute exacerbation. We report two cases of pulmonary cancer complicated by interstitial pneumonia in which multiple intrapulmonary lymph nodes were noted. Case presentations Cases 1 and 2 were 76- and 62-year-old males, respectively. Both cases were diagnosed as pulmonary cancer complicated by interstitial pneumonia, and upon chest CT, it was noted that, in addition to the primary lesions, there were multiple nodule shadows under the pleura in the lung lobe, which required identification in order to rule out lung metastasis. In Case 1, the pulmonary nodules were first resected during surgery and a swift diagnosis was made, determining them to be intrapulmonary lymph nodes, which were then operated on curatively. In Case 2, the patient underwent thoracoscopic observation during surgery, the shadows were diagnosed visually as intrapulmonary lymph nodes, and curative surgery was implemented. Discussion and conclusions The greatest problem in treating pulmonary cancer complicated with interstitial pneumonia is acute exacerbation, wherein, in the absence of any surgical indications, alternative treatment is limited. Thus, contra-indicating surgery for a patient due to a diagnosis of metastasis within the lungs, based only on nodule images, should be avoided. If nodules are noted in the area of the pleura, the possibility exists that these could be intrapulmonary lymph nodes, along with metastasis within the lung, and thoracoscopic surgery should be implemented proactively while keeping these in mind. PMID:26656149

  3. 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: 40L/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

  4. Giant cell interstitial pneumonia: an unusual finding in a case of preoperative death.

    PubMed

    Sisodia, Shantilal M; Bendale, Kiran; Khan, Wasif Ali Zafarali; Sanklecha, Vandana

    2013-06-01

    Giant cell interstitial pneumonia (GIP) is an exceedingly rare, debatable, perplexing, occupational lung disease, which most commonly affects individuals exposed to hard metal dust. We report a case of GIP in a 60-year-old man, scheduled for coronary artery bypass graft surgery and died during induction of general anesthesia despite all efforts to resuscitate him. Patient's relatives lodged complaint with the police alleging the negligence by the attending physicians. Despite inaccessible data pertaining to the occupation, clinical history, and radiographic findings, the diagnosis was GIP due to the presence of intra-alveolar, bizarre, "cannibalistic" multinucleated giant cells-the histologic sine qua non of GIP. To the best of our knowledge, this is the first case report of GIP in the world literature that was diagnosed on histopathologic examination of lung tissue obtained at medicolegal autopsy. PMID:23629398

  5. Cellular non-specific interstitial pneumonia masquerading as congestive heart failure.

    PubMed

    Saraya, Takeshi; Takata, Saori; Fujiwara, Masachika; Takei, Hidefumi

    2013-01-01

    A 66-year-old woman with a history of myocardial infarction 2 months prior presented to our respiratory department with several days of dry cough and night sweats. Chest X-ray and thoracic CT showed ground glass opacities or consolidation spreading from the hilar area to the peripheral area, suggesting central redistribution. Although neither rales nor abnormal heart sounds were noted, she was tentatively diagnosed with congestive heart failure based on those radiological findings. However, radiographic lung lesions and her symptoms were refractory to intensive diuretic treatment. Thereafter, video-assisted thoracoscopic surgery was performed, resulting in a diagnosis of cellular non-specific interstitial pneumonia (c-NSIP). After initiating treatment with prednisolone, her symptoms and the radiological findings resolved. In patients with NSIP, a radiological central distribution could rarely occur, especially in cases of c-NSIP. No rales were detected because of its paucity of fibrous components in the lung. PMID:24001730

  6. 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

  7. Idiopathic Pulmonary Fibrosis.

    PubMed

    Martin, Maria D; Chung, Jonathan H; Kanne, Jeffrey P

    2016-05-01

    Idiopathic pulmonary fibrosis (IPF) is the most common fibrosing lung disease and is associated with a very poor prognosis. IPF manifests histopathologically as usual interstitial pneumonia (UIP) and as subpleural and basal predominant reticulation with honeycombing on high-resolution computed tomography (HRCT) of the chest. When a high-confidence radiologic diagnosis of UIP is made on HRCT, surgical biopsy is rarely required. Therefore, radiologists should recognize a UIP pattern on HRCT as well as recognize other patterns of fibrosing lung disease such as nonspecific interstitial pneumonia or chronic hypersensitivity pneumonitis, both of which can be mistaken for UIP. This article reviews the clinical, CT, and histopathologic features of IPF, discusses the impact of CT findings on prognosis, and describes complications associated with IPF. PMID:27043425

  8. Quantitative diagnosis of connective tissue disease-associated interstitial pneumonia using thoracic computed tomography images.

    PubMed

    Ozuno, Nobuko Tosaka; Akamatsu, Hokuto; Takahashi, Hiroshi; Fujii, Naoko; Yoshida, Shunji

    2015-12-01

    Patients with polymyositis (PM) or dermatomyositis (DM) frequently show interstitial pneumonia (IP), which is sometimes rapidly progressive or resistant to treatment, thereby significantly affecting the prognosis. The diagnosis and response evaluation of IP are commonly performed qualitatively based on imaging findings, which may cause disagreement among rheumatologists in the evaluation of early lesions and atypical interstitial changes. To determine whether IP could be diagnosed in a quantitative manner during the early stage of PM/DM using a workstation that allows quantitative image processing. Thoracic computed tomography (CT) images of 20 PM/DM patients were reconstructed into a three-dimensional (3D) image using an image processing workstation. The CT values of the constituent voxels were arranged in a histogram of -1000 to +1000 Hounsfield units (HU). The most frequent lung field density was -900 to -801 HU, and relative size was as follows: IP (+) group 0.45 and IP (-) group 0.53. Between -1000 and -701 HU, relative size was not significantly different between the IP (+) group and IP (-) group. Between -700 and -1 HU, the relative size of the lung field was significantly larger in the IP (+) than in the IP (-) group, demonstrating its IP-diagnosing ability. Particularly, within the range from -700 to -301 HU, the macroscopically-assessed ground glass opacity was consistent with the CT value, which, in turn, was closely correlated with KL-6, the pre-existing marker for IP diagnosis. The results of this study may lead to the establishment of quantitative methods of evaluating IP and possible elucidation of the pathogenesis of IP. PMID:26519047

  9. [Anesthetic management of posterior lumbar spinal fusion in a patient suspected of having acute exacerbation of chronic interstitial pneumonia].

    PubMed

    Sakai, Mayuko; Takenami, Tamie; Otsuka, Tomohisa; Hayashi, Norito; Yoshino, Kazuhisa; Matsumoto, Shigehiro; Okamoto, Hirotsugu

    2014-02-01

    A patient complicated with interstitial pneumonia required emergency posterior lumbar spinal fusion. The blood gas analysis showed relatively benign values (PaO2 81 torr, PaCO2 44 torr, under room air), but the honeycombing lungs were noted in the bilateral lung fields on CT, and the KL-6 level was high (1,000 U x ml(-1)), for which the acute exacerbation of interstitial pneumonia was suspected. Sivelestat sodium administration was initiated during the surgery and continued postoperatively. During surgery, setting the FIO2 at 0.34, the P/F ratio and intra-airway pressure could be maintained at 500 and 25 mmHg, respectively. To reduce postoperative respiratory complication, anesthesia was maintained with desflurane, which is dissipated easily, and 0.5% ropivacaine 15 ml was subcutaneously injected to the surgical field at the time of wound closure to reduce the total doses of intraoperative fentanyl and postoperative analgesics. After the completion of surgery, the endotracheal tube was removed with head elevated position, and the patient was transported back to the ward. No acute exacerbation occurred thereafter, and the patient was discharged 67 days after surgery. The prediction of acute exacerbation of interstitial pneumonia is difficult. Moreover, there is no established preventive method, although the mortality is high. Therefore, physicians should be thoroughly informed about the currently available evidence, including developmental factors. PMID:24601111

  10. 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

  11. 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

  12. IgG4-Related Lung Disease Associated with Usual Interstitial Pneumonia.

    PubMed

    Schneider, Frank; Veraldi, Kristen L; Levesque, Marc C; Colby, Thomas V; S Yi, Eunhee

    2016-01-01

    We report a case of immunoglobulin(Ig)G4-related disease with the radiologic and histopathological manifestations resembling usual interstitial pneumonia (UIP). The patient was a 62-year-old man who presented with progressive dyspnea of insidious onset. High resolution computed tomography of the chest showed lower-lobe predominant peripheral reticulation and traction bronchiectasis but no honeycomb change. Microscopic examination of the surgical lung biopsy showed characteristic features of UIP including architectural distortion by fibrosis with peripheral and paraseptal accentuation, scattered fibroblast foci and microscopic honeycomb change. In addition there were prominent multifocal lymphoplasmacytic infiltrates with a marked increase of IgG4-positive plasma cells (79 per high power field in hot spots) and high IgG4/IgG ratio (up to 67%). The serum IgG4 level was elevated at 760 mg/dl (reference range 9-89), with normal levels for the other IgG subclasses and negative serologic markers for autoimmune diseases. The patient's symptoms improved significantly with oral corticosteroid treatment. PMID:27053971

  13. [A case of interstitial pneumonia exacerbated by kampo-induced pneumonitis].

    PubMed

    Suzuki, Kazuo; Kinebuchi, Shinichi; Sugiyama, Kentaro; Satoh, Hiroshi; Tango, Masuo; Moriyama, Hirochi; Terada, Masaki; Ooi, Hidemi; Hasegawa, Takashi; Igarashi, Kenichi; Satoh, Makoto; Suzuki, Eiichi; Gejyo, Fumitake

    2002-07-01

    A 65-year-old woman who had complained of non-productive cough since May 1998 visited our hospital on November 5, 2000. She had been treated at another hospital with Kampo (Chinese herbal medicine), including Moku-boui-to, Bakumon-do-to, and Saiko-keishi-kankyo-to for chronic non-productive cough. Chest radiographs and CT films showed the reticular shadows that had been present in 1998, in both lower lung fields, and also demonstrated new reticular shadows in the right upper lung field and left lingular segment. Laboratory data revealed hypoxemia and pulmonary function tests revealed restrictive ventilatory disturbance, so she was admitted to our hospital on November 9, 2000. After the cessation of Kampo treatment, her symptoms disappeared, and the hypoxemia, restrictive disturbance, and reticular shadows in the chest radiograph gradually improved. Video-assisted lung biopsy specimens showed thickened alveolar walls with lymphocyte and eosinophil infiltration. A leukocyte migration test was positive for Moku-boui-to, Bakumon-do-to, and weakly positive for Saiko-keishi-kankyo-to. Although no challenge test for Kampo was performed, we diagnosed this case as interstitial pneumonia exacerbated Kampo-induced pneumonitis based on these clinical, laboratory and histological findings. PMID:12382427

  14. IgG4-Related Lung Disease Associated with Usual Interstitial Pneumonia

    PubMed Central

    Schneider, Frank; Veraldi, Kristen L.; Levesque, Marc C.; Colby, Thomas V.; S. Yi, Eunhee

    2016-01-01

    We report a case of immunoglobulin(Ig)G4-related disease with the radiologic and histopathological manifestations resembling usual interstitial pneumonia (UIP). The patient was a 62-year-old man who presented with progressive dyspnea of insidious onset. High resolution computed tomography of the chest showed lower-lobe predominant peripheral reticulation and traction bronchiectasis but no honeycomb change. Microscopic examination of the surgical lung biopsy showed characteristic features of UIP including architectural distortion by fibrosis with peripheral and paraseptal accentuation, scattered fibroblast foci and microscopic honeycomb change. In addition there were prominent multifocal lymphoplasmacytic infiltrates with a marked increase of IgG4-positive plasma cells (79 per high power field in hot spots) and high IgG4/IgG ratio (up to 67%). The serum IgG4 level was elevated at 760 mg/dl (reference range 9-89), with normal levels for the other IgG subclasses and negative serologic markers for autoimmune diseases. The patient’s symptoms improved significantly with oral corticosteroid treatment. PMID:27053971

  15. Nonspecific Interstitial Pneumonia/Fibrosis: Clinical Manifestations, Histologic and Radiologic Features

    PubMed Central

    Park, Choon Sik; Jeon, Jin Woo; Park, Sung Woo; Lim, Gune-Il; Jeong, Seong Hwan; Uh, Soo-Taek; Park, Jai Soung; Choi, Deuk Lin; Jin, So Young; Kang, Chang Hee

    1996-01-01

    Objectives Customarily used classification of IPF did not satisfy a sizable group of patients with IPF that in the past had been lumped with UIP and now currently has begun to be recognized as nonspecific interstitial pneumonia/fibrosis (NIP). There are few reports about the clinical features of NIP. Methods The pathologic slides of 66 patients having open lung biopsy (OLB) for the differential diagnosis of interstitial lung diseases (ILD) from 1984 to 1995 were reviewed. Seven cases were confirmed as NIP. The clinical record, pulmonay function tests (PFT), chest PA and HRCT were reviewed and analysed retrospectively. Results Six patients with NIP were female. The median age was 56 years. Mean duration of symptoms was 4 months. Five patients had systemic flu-like symptoms. the most common respiratory symptom was gradual dyspnea Two patients revealed a mild degree of anemia Four cases had leukocytois of more than 10,000/mm3. ESR and CRP O.K. elevated in all measured cases. Anti-nuclear antibody (ANA) was positive in three of six patients and ds-DNA antibody was positive in one of six patients Restrictive pattern of PFT was predominant. Diffusion capacity of carbonmonoxide (DLCO) decreased markedly. In bronchoalveolar lavage (BAL), total cell counts elevated about three times of normal value. On differntial counts of BAL cells, lymphocytes, neutrophils and eosinophils were higher than those of normal controls. The prominent finding of chest radiology was bilaterally patchy opacifications in parenchyme of lower lung zones. On HRCT, bilaterally patchy areas of ground-glass attenuation and/or areas of alveolar consolidation were commonly shown. The number of pathologic type was one case of group I, four cases of group II and two cases of group III. The average period from diagnosis to the last follow-up was 24.8 months. Five patients were clinically recovered to the previously well-being state. Conclusion Patients with NIP had different clinical features from UIP, AIP

  16. 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

  17. 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.

  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. Granulomatous interstitial pneumonia induced by the intake of rice bran pickles: a rare subtype of pulmonary manifestation associated with food allergy.

    PubMed

    Kinoshita, Yoshiaki; Sakamoto, Atsuhiko; Aishima, Shinichi; Hidaka, Kouko

    2015-01-01

    Pulmonary manifestations associated with food allergy are rich in variety. We report the first case of food-induced granulomatous interstitial pneumonia mimicking hypersensitivity pneumonitis (HP). A 77-year-old woman with respiratory symptoms was referred to our hospital. We performed a surgical lung biopsy, which showed the features of granulomatous interstitial pneumonia. Her clinical history resembled those observed in HP. However, avoidance of exposure to the causative antigens did not improve her symptoms. Moreover, the patient had some features inconsistent with HP, such as elevated serum IgE levels, blood eosinophilia, intrathoracic lymphadenopathies and pleural effusion. Therefore, we pursued another extrinsic non-inhaled antigen as the cause of pulmonary involvements. We noted that she had been eating homemade rice bran pickles, and pulmonary involvements were induced by an ingestion challenge test. We suggest that granulomatous interstitial pneumonia may be a rare subtype of the pulmonary manifestations associated with food allergy. PMID:25858927

  20. 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.

  1. Cutaneous Vasculitis, Interstitial Pneumonia with Crazy-Paving Appearance, and Positive pANCA in a Patient with Severe Crohn's Disease

    PubMed Central

    Chen, Guang-liang; Wang, Juan; Li, Li-mei; Mo, Han-you; Ye, Shuang

    2014-01-01

    Cutaneous vasculitis, interstitial pneumonia with crazy-paving appearance on high-resolution computed tomography, and repeated positive perinuclear anti-neutrophil cytoplasmic antibodies (pANCA) are rarely found together in patients with inflammatory bowel disease in the existing literature. We report the case of a Chinese patient previously diagnosed with cutaneous vasculitis and interstitial pneumonia, who presented with acute pain and mass in his right lower quadrant a couple of years later. The terminal ileum biopsy and postoperative pathology confirmed Crohn's disease (CD). PMID:25371834

  2. [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

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

    PubMed

    Shah, Aditya; Patel, Samir R

    2016-09-01

    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

  4. 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

  5. An Official American Thoracic Society Research Statement: Noninfectious Lung Injury after Hematopoietic Stem Cell Transplantation: Idiopathic Pneumonia Syndrome

    PubMed Central

    Panoskaltsis-Mortari, Angela; Griese, Matthias; Madtes, David K.; Belperio, John A.; Haddad, Imad Y.; Folz, Rodney J.; Cooke, Kenneth R.

    2011-01-01

    Rationale: Acute lung dysfunction of noninfectious etiology, known as idiopathic pneumonia syndrome (IPS), is a severe complication following hematopoietic stem cell transplantation (HSCT). Several mouse models have been recently developed to determine the underlying causes of IPS. A cohesive interpretation of experimental data and their relationship to the findings of clinical research studies in humans is needed to better understand the basis for current and future clinical trials for the prevention/treatment of IPS. Objectives: Our goal was to perform a comprehensive review of the preclinical (i.e., murine models) and clinical research on IPS. Methods: An ATS committee performed PubMed and OVID searches for published, peer-reviewed articles using the keywords “idiopathic pneumonia syndrome” or “lung injury” or “pulmonary complications” AND “bone marrow transplant” or “hematopoietic stem cell transplant.” No specific inclusion or exclusion criteria were determined a priori for this review. Measurements and Main Results: Experimental models that reproduce the various patterns of lung injury observed after HSCT have identified that both soluble and cellular inflammatory mediators contribute to the inflammation engendered during the development of IPS. To date, 10 preclinical murine models of the IPS spectrum have been established using various donor and host strain combinations used to study graft-versus-host disease (GVHD). This, as well as the demonstrated T cell dependency of IPS development in these models, supports the concept that the lung is a target of immune-mediated attack after HSCT. The most developed therapeutic strategy for IPS involves blocking TNF signaling with etanercept, which is currently being evaluated in clinical trials. Conclusions: IPS remains a frequently fatal complication that limits the broader use of allogeneic HSCT as a successful treatment modality. Faced with the clinical syndrome of IPS, one can categorize the

  6. [Characteristics of and risk factors for interstitial pneumonia due to TS-1(R) capsule administration-case reviews from a drug use results survey on Non-Small Cell Lung cancer patients and spontaneous reports].

    PubMed

    Ito, Kunio; Jin, Zhe

    2015-05-01

    A drug use results survey for the TS-1(R)capsule was conducted on patients with non-small cell lung cancer. A total of 1,784 patients were registered for the survey, 1,669 of whomwere evaluated for safety. The incidence of adverse drug reactions was 67.9%(1,134/1,669). The overall incidence of interstitial pneumonia was 1.38%(23/1,669), while it was 1.34% in patients treated with TS-1(R) alone(14/1,046). The risk factors for interstitial pneumonia according to this survey included allergic predisposition, concomitant diseases(including interstitial pneumonia), past medical history(including interstitial pneumonia), and combination with anticancer drugs(excluding cisplatin). Among the spontaneous reports of interstitial pneumonia as an adverse drug reaction from physicians, test images were available in 39 cases. These images were analyzed by the Safety Review Committee for interstitial pneumonia, consisting of external medical experts. In addition, the imaging patterns, settings of occurrence, and patient's backgrounds were reviewed. Twenty-five of the 39 cases were assessed to be possible interstitial pneumonia, of which 10 were confirmed to have an imaging pattern of diffuse alveolar damage. Based on the above findings, it is essential to check the patient's medical history for conditions such as lung disorder through a medical interview and an imaging test, prior to starting treatment with TS-1(R). PMID:25981654

  7. 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

  8. 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

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

    PubMed

    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

  10. 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.

  11. Update on diagnosis and treatment of idiopathic pulmonary fibrosis.

    PubMed

    Baddini-Martinez, José; Baldi, Bruno Guedes; Costa, Cláudia Henrique da; 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

  12. 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

  13. [Case of polymer fume fever with interstitial pneumonia caused by inhalation of polytetrafluoroethylene (Teflon)].

    PubMed

    Son, Masami; Maruyama, Eiichi; Shindo, Yuichiro; Suganuma, Nobukazu; Sato, Shinji; Ogawa, Masahiro

    2006-07-01

    A 30-year old man was admitted to our hospital with cough, slight fever, and dyspnea that he had developed several hours after inhaling the fumes produced from a Teflon-coated pan, after evaporation of the water in the pan. Chest radiography revealed diffuse infiltrations, and a computed tomography (CT) scan revealed patchy interstitial shadows in both lungs. In pulmonary function tests, the diffusing capacity of the lungs showed a moderate decrease. Leukocytosis and slight hypoxemia were observed. The patient recovered clinically in a few days without any specific treatment. We speculated that the pulmonary problems in this patient may have been induced by the products of thermal degradation of Teflon that were present in the fumes. When Teflon is heated, the fumes generated cause an influenza like syndrome (polymer fume fever) or cause severe toxic effects such as pulmonary edema, pneumonitis, and death in the exposed individual. PMID:16922460

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

    PubMed

    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

  15. 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

  16. Studies on the sequential development of acute interstitial pneumonia caused by Aleutian disease virus in mink kits.

    PubMed Central

    Alexandersen, S; Bloom, M E

    1987-01-01

    We studied different parameters during the development of acute interstitial pneumonia in mink kits caused by neonatal infection with Aleutian disease virus (ADV). When histological lesions, presence of intranuclear inclusion bodies, and intranuclearly localized ADV antigen were correlated with levels of single-stranded virion and duplex replicative forms of ADV DNA in the different tissues, it was concluded that the lung, probably alveolar type II cells, is the major primary target for viral replication and cytopathology. The presence of the duplex dimeric replicative-form DNA, a strong marker of parvovirus replication, was also observed in low amount in the mesenteric lymph node, suggesting replication of ADV in this organ, although no viral cytopathology could be demonstrated. Moreover, a few intranuclear inclusion bodies were demonstrated in kidney and liver from affected kits, but intranuclearly localized ADV antigen could not be demonstrated in liver sections, and neither could duplex dimer replicative-form DNA, suggesting that these organs are nevertheless not a major site of ADV replication. When the data were compared with results previously reported for ADV-infected adult mink and ADV-infected permissive cell cultures, the data suggested that the pattern of ADV replication in alveolar type II cells is similar to that seen in infected cell cultures but that the replication in the other kit organs resembles the restricted pattern seen in adult mink. Images PMID:3023709

  17. 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) ...

  18. 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

  19. Pneumonia

    MedlinePlus

    ... the flu Your doctor will use your medical history, a physical exam, and lab tests to diagnose pneumonia. Treatment depends on what kind you have. If bacteria are the cause, antibiotics should help. If you ...

  20. Pneumocystis carinii causes a distinctive interstitial pneumonia in immunocompetent laboratory rats that had been attributed to "rat respiratory virus".

    PubMed

    Henderson, K S; Dole, V; Parker, N J; Momtsios, P; Banu, L; Brouillette, R; Simon, M A; Albers, T M; Pritchett-Corning, K R; Clifford, C B; Shek, W R

    2012-05-01

    A prevalent and distinctive infectious interstitial pneumonia (IIP) of immunocompetent laboratory rats was suspected to be caused by a putative virus, termed rat respiratory virus, but this was never substantiated. To study this disease, 2 isolators were independently populated with rats from colonies with endemic disease, which was perpetuated by the regular addition of naive rats. After Pneumocystis was demonstrated by histopathology and polymerase chain reaction (PCR) in the lungs of rats from both isolators and an earlier bedding transmission study, the relationship between Pneumocystis and IIP was explored further by analyzing specimens from 3 contact transmission experiments, diagnostic submissions, and barrier room breeding colonies, including 1 with and 49 without IIP. Quantitative (q) PCR and immunofluorescence assay only detected Pneumocystis infection and serum antibodies in rats from experiments or colonies in which IIP was diagnosed by histopathology. In immunocompetent hosts, the Pneumocystis concentration in lungs corresponded to the severity and prevalence of IIP; seroconversion occurred when IIP developed and was followed by the concurrent clearance of Pneumocystis from lungs and resolution of disease. Experimentally infected immunodeficient RNU rats, by contrast, did not seroconvert to Pneumocystis or recover from infection. qPCR found Pneumocystis at significantly higher concentrations and much more often in lungs than in bronchial and nasal washes and failed to detect Pneumocystis in oral swabs. The sequences of a mitochondrial ribosomal large-subunit gene region for Pneumocystis from 11 distinct IIP sources were all identical to that of P. carinii. These data provide substantial evidence that P. carinii causes IIP in immunocompetent rats. PMID:22308234

  1. AKR1B10 in usual interstitial pneumonia: expression in squamous metaplasia in association with smoking and lung cancer.

    PubMed

    Li, Chih-Ping; Goto, Akiteru; Watanabe, Akira; Murata, Kengo; Ota, Satoshi; Niki, Toshiro; Aburatani, Hiroyuki; Fukayama, Masashi

    2008-01-01

    The incidence of lung cancer (LC) is markedly increased among patients with usual interstitial pneumonia (UIP), and tobacco smoking is its superimposed risk factor. AKR1B10 (aldo-keto reductase 1B10) is frequently overexpressed in pulmonary squamous cell carcinoma and adenocarcinoma in smokers. To investigate the role of AKR1B10 in the pulmonary carcinogenesis in UIP with correlation to tobacco smoking, we examined 13 UIP cases with LC, 13 UIP cases without LC, and 30 cases of non-UIP LC using AKR1B10 immunohistochemistry. AKR1B10 immunoreactivity was confined to squamous metaplasia in honeycomb lesions of UIP and neoplastic cells of LC. Squamous metaplastic foci showed AKR1B10 immunoreactivity more frequently in UIP with LC (24/36 foci, 67%) than in UIP without LC (16/44 foci, 37%) (P<0.01). AKR1B10 expression in UIP was also more frequent in squamous metaplastic foci in smokers (38/67 foci, 57%) than in non-smokers (2/13 foci, 15%) (P<0.01). AKR1B10 expression was frequently observed in both UIP-associated LC (10/13 foci, 77%) and non-UIP LC (18/30 foci, 60%). Ki-67 labeling index was significantly higher in AKR1B10-positive squamous metaplasia of UIP than in AKR1B10-negative squamous metaplasia of UIP. Our results demonstrate that AKR1B10 is involved in the development of LC in UIP in association with smoking. AKR1B10 might be useful as a new marker for identification of high LC risk patients in UIP. PMID:18358633

  2. Intraluminal fibrosis in interstitial lung disorders.

    PubMed Central

    Basset, F.; Ferrans, V. J.; Soler, P.; Takemura, T.; Fukuda, Y.; Crystal, R. G.

    1986-01-01

    The histopathologic and ultrastructural features of intraluminal organizing and fibrotic changes were studied in open lung biopsies and autopsy specimens from 373 patients with interstitial lung disorders, including hypersensitivity pneumonitis (n = 44), idiopathic pulmonary fibrosis (n = 92), collagen-vascular diseases (n = 20), chronic eosinophilic pneumonia (n = 10), pulmonary histiocytosis X (n-90), pulmonary sarcoidosis (n = 62), pneumoconioses (n = 25), Legionnaire's disease (n = 5), drug- and toxin-induced pneumonitis (n = 4), radiation-induced pneumonitis (n = 2), lymphangioleiomyomatosis (n = 11), and chronic organizing pneumonia of unknown cause (n = 8). Three patterns of intraluminal organization and fibrosis were recognized: 1) intraluminal buds, which partially filled the alveoli, alveolar ducts and/or distal bronchioles; 2) obliterative changes, in which loose connective tissue masses obliterated the lumens of alveoli, alveolar ducts or distal bronchioles, and 3) mural incorporation of previously intraluminal connective tissue masses, which fused with alveolar, alveolar ductal, or bronchiolar structures and frequently became reepithelialized. All three patterns had common morphologic features, suggesting that, regardless of their severity, they resulted from a common pathogenetic mechanism, ie, the migration of activated connective tissue cells, through defects in the epithelial lining and its basement membrane, from the interstitial into the intraluminal compartment. Intraluminal buds were observed most frequently in hypersensitivity pneumonitis, chronic eosinophilic pneumonia, and organizing pneumonia of unknown cause. Mural incorporation and, to a lesser extent, obliterative changes were observed in most interstitial disorders and were very prominent in idiopathic pulmonary fibrosis. Mural incorporation and obliterative changes play an important role in pulmonary remodeling, especially when several adjacent alveoli and/or other air spaces are

  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. Outcome Measures for Clinical Trials in Interstitial Lung Diseases

    PubMed Central

    Lammi, Matthew R.; Baughman, Robert P.; Birring, Surinder S.; Russell, Anne-Marie; Ryu, Jay H.; Scholand, Marybeth; Distler, Oliver; LeSage, Daphne; Sarver, Catherine; Antoniou, Katerina; Highland, Kristin B.; Kowal-Bielecka, Otylia; Lasky, Joseph A.; Wells, Athol U.; Saketkoo, Lesley Ann

    2015-01-01

    The chronic fibrosing idiopathic interstitial pneumonias (IIPs) are a group of heterogeneous pulmonary parenchymal disorders described by radiologic and histological patterns termed usual interstitial pneumonia (UIP) and non-specific interstitial pneumonia (NSIP). These include idiopathic pulmonary fibrosis (IPF) and those related to connective tissue disease (CTD) and are associated with substantial morbidity and mortality. Beyond the importance of establishing an appropriate diagnosis, designing optimal clinical trials for IIPs has been fraught with difficulties in consistency of clinical endpoints making power analyses, and the establishment of efficacy and interpretation of results across trials challenging. Preliminary recommendations, developed by rigorous consensus methods, proposed a minimum set of outcome measures, a ‘core set’, to be incorporated into future clinical trials (Saketkoo et al, THORAX. 2014.). This paper sets out to examine the candidate instruments for each domain (Dyspnea, Cough, Health Related Quality of Life, Imaging, Lung Physiology and Function, Mortality). Candidate measures that were not selected as well as measures that were not available for examination at the time of the consensus process will also be discussed. PMID:27019654

  5. 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

  6. Favorable outcome with hemoperfusion of polymyxin B-immobilized fiber column for rapidly progressive interstitial pneumonia associated with clinically amyopathic dermatomyositis: report of three cases.

    PubMed

    Ichiyasu, Hidenori; Horio, Yuko; Tsumura, Shinsuke; Hirosako, Susumu; Sakamoto, Yasumiko; Sakata, Shinya; Nakashima, Kei; Komatsu, Taiyo; Kojima, Keisuke; Masunaga, Aiko; Fujii, Kazuhiko; Saita, Naoki; Kohrogi, Hirotsugu

    2014-03-01

    We present 3 cases of rapidly progressive interstitial pneumonia (RPIP) associated with clinically amyopathic dermatomyositis (C-ADM) that were treated with two courses of direct hemoperfusion with polymyxin B-immobilized fiber column (PMX-DHP). Despite initial treatment with high-dose corticosteroids, pulsed cyclophosphamide, and cyclosporine, the lung disease and hypoxemia deteriorated in all the patients. After PMX-DHP treatment, the PaO2/FiO2 ratio and serum LDH and KL-6 were improved, the abnormal shadows in chest high-resolution computed tomography (HRCT) scans gradually decreased, and, finally, all patients survived. These findings indicate that PMX-DHP treatment could be effective in the management of RPIP in patients with C-ADM in combination with conventional therapy. PMID:24593215

  7. Idiopathic hypersomnia

    MedlinePlus

    Hypersomnia - idiopathic; Drowsiness - idiopathic; Somnolence - idiopathic ... extremely sleepy. It is different from narcolepsy because idiopathic hypersomnia does not usually involve suddenly falling asleep (sleep ...

  8. Bosentan for pulmonary hypertension secondary to idiopathic pulmonary fibrosis

    PubMed Central

    Onda, Naomi; Tanaka, Yosuke; Hino, Mitsunori; Gemma, Akihiko

    2014-01-01

    Pulmonary hypertension is a poor prognostic factor in patients with interstitial lung disease. No established treatment exists for pulmonary hypertension secondary to interstitial pneumonia. We describe the case of an 81-year-old woman with idiopathic pulmonary fibrosis (IPF), who was admitted to our hospital due to aggravation of dyspnea and decreased oxygen saturation, as well as onset of orthopnea and rapidly progressing edema. The transthoracic echocardiography and right heart catheterization showed the mean pulmonary artery pressure was 39 mmHg and the mean pulmonary capillary wedge pressure was 9 mmHg. After various examinations, the diagnoses of pulmonary hypertension (PH) due to IPF and of congestive heart failure secondary to PH were established. Diuretic therapy was started, but the patient's condition showed poor improvement. Subsequent initiation of oral bosentan therapy led to improvement in symptoms and findings. At the follow-up assessment one year later her pulmonary function showed no significant changes and no apparent worsening of arterial blood gases, with evident improvement of PH, WHO functional class, maximum exercise tolerance on treadmill exercise testing, right heart catheterization, and transthoracic echocardiography. This report describes a case of successful treatment with bosentan for severe pulmonary hypertension in a patient with idiopathic pulmonary fibrosis. We also present a review of the literature on treatment of pulmonary hypertension in patients with chronic lung disease. Bosentan appears to be efficacious in some patients with pulmonary hypertension secondary to idiopathic interstitial pneumonitis. PMID:26029570

  9. 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

  10. [Massive pulmonary thromboembolism found at autopsy in a patient with a 14-year history of polymyositis-associated nonspecific interstitial pneumonia].

    PubMed

    Takagi, Yusuke; Hagiwara, Kiyofumi; Sato, Takeo; Akiyama, Osamu; Ogura, Takashi; Tsugata, Mikiko; Takemura, Tamiko

    2008-10-01

    A 64-year-old woman with a 14-year history of refractory nonspecific interstitial pneumonia (NSIP) associated with polymyositis was admitted urgently to our hospital due to acute exacerbation of dyspnea. The first episode of dyspnea had occurred 14 years earlier and the diagnosis of cellular and fibrotic NSIP had been made by radiographic examinations and open lung biopsy. Her NSIP initially responded well to high-dose prednisolone therapy (40mg/day), but flared when the dose of prednisolone was tapered. Three years after the diagnosis of NSIP, a diagnosis of polymyositis was made based on her elevated serum level of creatinine phosphokinase and muscle biopsy findings. Her polymyositis was controlled well by prednisolone therapy, but her NSIP gradually worsened, despite treatment with several courses of methylprednisolone pulse therapy and immunosuppressive agents. She was brought to our hospital by ambulance due to acute progression of dyspnea and was admitted. Chest X-ray film revealed diffuse interstitial shadows and marked enlargement of the right atrium and bilateral pulmonary arteries. Transthoracic echocardiogram revealed severe dilatation of the right atrium and right ventricle with an estimated pulmonary artery systolic pressure of 84mmHg. She died of progressive respiratory failure on day 4 of hospitalization. Autopsy revealed massive thrombi that completely obstructed the bilateral pulmonary trunks. Histological examination revealed that these thrombi were organized, and her interstitial lung disease (ILD) was specified as fibrotic NSIP with microscopic honeycomb. Massive pulmonary thromboembolism is extremely rare in patients with polymyositis-associated NSIP, but it should be considered as a possible cause of acute exacerbation of respiratory failure. This case provides valuable information on the clinical and pathological course of polymyositis-associated NSIP. PMID:19044031

  11. Histopathology of Pneumocystis carinii pneumonia in immunocompetent laboratory rats

    PubMed Central

    KIM, HYUN-SOO; DO, SUNG-IM; KIM, YOUN WHA

    2014-01-01

    The occurrence of idiopathic pulmonary lesions in laboratory rats, characterized by lymphohistiocytic interstitial pneumonia with dense perivascular lymphoid cuffs, has been reported over the past decade. Although the term rat respiratory virus (RRV) was adopted to confer a putative viral etiology to the idiopathic pulmonary lesions, the etiology of this disease remains to be elucidated. Recently, inflammatory lesions have been observed in the lungs of immunocompetent laboratory rats similar to those previously described. Based on the latest evidence indicating that Pneumocystis carinii (P. carinii), and not putative RRV, causes infectious interstitial pneumonia in laboratory rats, the present study investigated whether the pulmonary lesions observed were caused by P. carinii infection. Male Sprague-Dawley rats, free of known pathogens, were introduced into a rat colony positive for RRV-type lesions. Routine histopathological examinations were performed on the rat lung tissues following exposure. The presence of Pneumocystis organisms was confirmed using Grocott’s methenamine silver (GMS) staining. At week 3 following introduction, a few small lymphoid aggregates were located adjacent to the edematous vascular sheath. By week 5, foci of dense perivascular lymphoid cuffing were observed. Multifocal lymphohistiocytic interstitial pneumonia and prominent lymphoid perivascular cuffs were observed between week 7 and 10. GMS staining confirmed the presence of Pneumocystis cysts. Thus, the results of the present study demonstrated that P. carinii caused lymphohistiocytic interstitial pneumonia in a group of laboratory rats. The observations strongly support the conclusion that P. carinii infection in immunocompetent laboratory rats causes the lung lesions that were previously attributed to RRV. PMID:25009598

  12. Inherited interstitial lung disease.

    PubMed

    Garcia, Christine Kim; Raghu, Ganesh

    2004-09-01

    This article focuses on recent advances in the identification of genes and genetic polymorphisms that have been implicated in the development of human interstitial lung diseases. It focuses on the inherited mendelian diseases in which pulmonary fibrosis is part of the clinical phenotype and the genetics of familial idiopathic pulmonary fibrosis and other rare inherited interstitial lung diseases. The article also reviews the association studies that have been published to date regarding the genetics of sporadic idiopathic pulmonary fibrosis. The reader is directed to recent reviews on human genetic predisposition of sarcoidosis, environmental-related, drug-related, connective tissue related pulmonary fibrosis, and genetic predisposition of fibrosis in animal models. PMID:15331184

  13. Interstitial Lung Disease with ANCA-associated Vasculitis

    PubMed Central

    Katsumata, Yasuhiro; Kawaguchi, Yasushi; Yamanaka, Hisashi

    2015-01-01

    The association between interstitial lung disease (ILD) and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), particularly microscopic polyangiitis (MPA), has been described in a number of case reports and case series reports in the last 2 decades. In addition, patients with pulmonary fibrosis and ANCA positivity but without other manifestations of systemic vasculitis have also been reported. Pulmonary fibrosis was clinically manifested at the time of diagnosis in the majority of AAV patients that developed this condition. Moreover, ANCA-positive conversion occurs in patients initially diagnosed with idiopathic pulmonary fibrosis, and as a result, other manifestations of systemic vasculitis develop in some of these patients. There is significant predominance of myeloperoxidase (MPO)-ANCA and MPA in patients with AAV and ILD. Radiological and pathological findings generally demonstrate usual interstitial pneumonia (pattern) in the lungs of these patients. In most studies, AAV patients with ILD have a worse prognosis than those without it. PMID:26448696

  14. 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

    2015-01-01

    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. PMID:26422573

  15. [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

  16. 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

  17. 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

  18. 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. PMID:25894436

  19. Increased Serum LIGHT Levels Correlate with Disease Progression and Severity of Interstitial Pneumonia in Patients with Dermatomyositis: A Case Control Study

    PubMed Central

    Kotani, Takuya; Takeuchi, Tohru; Ishida, Takaaki; Masutani, Ryota; Isoda, Kentaro; Hata, Kenichiro; Yoshida, Shuzo; Makino, Shigeki; Hanafusa, Toshiaki

    2015-01-01

    Background Activated CD8+ T cells play an important role in the pathogenesis of dermatomyositis (DM) with interstitial pneumonia (IP). Serum CD8+ T-cell activator, LIGHT, and Th1/Th2/Th17 cytokines were measured in DM-IP patients and compared with clinical parameters to investigate their usefulness. Methods The correlations between the clinical findings and serum LIGHT and Th1/Th2/Th17 cytokine levels were investigated in 21 patients with DM-IP (14 with rapidly progressive IP [RPIP] and 7 with chronic IP [CIP], including 4 fatal cases of IP). Results The median serum LIGHT level was 119 (16–335.4) pg/ml, which was higher than that in healthy control subjects and DM patients without IP. The median serum IL–6 level was 14.7 (2.4–154.5) pg/ml (n = 13). The other cytokines were detected in only a few patients. The median serum LIGHT level in DM-RPIP patients (156 [49.6–335.4] pg/ml) was significantly higher than that in DM-CIP patients (94.3 [16–164.2] pg/ml) (P = 0.02). The serum IL–6 level did not correlate with either progression or outcome of DM-IP. ROC curve analysis determined a serum LIGHT level of ≥120 pg/ml to be the cut-off value for the rapid progression of DM-IP. Serum LIGHT levels correlated significantly with %DLco (R = 0.55, P = 0.04) and total ground-glass opacity scores (R = 0.72, P = 0.0002). The serum LIGHT level significantly decreased to 100.5 (12.4–259.3) pg/ml 4 weeks after treatment initiation (P = 0.04). Conclusions The serum LIGHT level may be a promising marker of disease progression and severity in patients with DM-IP. PMID:26448572

  20. IDIOPATHIC PULMONARY FIBROSIS: NEW CONCEPTS IN PATHOGENESIS AND IMPLICATIONS FOR DRUG THERAPY

    PubMed Central

    Horowitz, Jeffrey C.; Thannickal, Victor J.

    2008-01-01

    Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and usually fatal pulmonary disease for which there are no proven or approved drug therapies. Anti-inflammatory and immunosuppressive agents have been largely ineffective. The precise relationship of IPF to other idiopathic interstitial pneumonias (IIPs) is not known, despite the observation that different histopathological patterns of IIP may co-exist in the same patient. We propose that these different histopathological “reaction” patterns may be determined by complex interactions between host and environmental factors that alter the local alveolar milieu. Recent paradigms in IPF pathogenesis have focused on dysregulated epithelial-mesenchymal interactions, an imbalance in TH1/TH2 cytokines and potential roles for aberrant angiogenesis. In this review, we discuss these evolving concepts in disease pathogenesis and emerging therapies designed to target pro-fibrogenic pathways in IPF. PMID:16928146

  1. [Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD)].

    PubMed

    Goeckenjan, G

    2003-05-01

    Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) designates interstitial lung changes in smokers, characterized histologically by bronchiolocentric accumulation of pigmented alveolar macrophages and fibrotic or cellular inflammatory changes of pulmonary interstitium. The definition is nearly identical to that of condensate pneumopathy, smoker's pneumopathy or smoker's lung, defined by accumulation of pigmented alveolar macrophages with bland alveoloseptal or peribronchial fibrosis and cellular inflammation of the bronchial wall. In addition to respiratory bronchiolitis, which is found in nearly all smokers, RB-ILD comprises a broad spectrum of varying degrees of the interstitial reaction to the exogenous injury of inhalation smoking with gradual transition to desquamative interstitial pneumonia (DIP). In most cases RB-ILD manifestations are subclinical and detected coincidentally. Radiographic features are reticulonodular and ground glass opacities of the lung. The high resolution computed tomography reveals centrilobular nodules, ground glass opacities, thickening of bronchial walls, and in some cases a reticular pattern. Mild emphysema is frequent. Lung function analysis reveals only minor restrictive or obstructive defects in most cases, often combined with hyperinflation. CO diffusing capacity is slightly to moderately impaired. Pronounced interstitial lung diseases with serious restrictive defects and arterial hypoxemia have been reported infrequently. In differential diagnosis smoking related interstitial lung diseases (DIP, Langerhans cell histiocytosis, idiopathic pulmonary fibrosis) and other interstitial lung diseases have to be excluded. In most cases diagnosis can be achieved by bronchoalveolar lavage and transbronchial lung biopsy. In cases of pronounced interstitial lung disease or assumption of an additional interstitial lung disease besides RB-ILD a thoracoscopic or open lung biopsy can be necessary. RB-ILD has a favourable

  2. Atypical pneumonia

    MedlinePlus

    ... that cause typical pneumonia. These include Legionella pneumophila , Mycoplasma pneumoniae , and Chlamydophila pneumoniae . Atypical pneumonia also tends to have milder symptoms than typical pneumonia. Causes Mycoplasma pneumonia is a type of atypical pneumonia. It ...

  3. 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

  4. 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. PMID:27279757

  5. A combined therapeutic approach in progressive idiopathic pulmonary fibrosis-pirfenidone as bridge therapy for ex vivo lung transplantation: a case report.

    PubMed

    Paone, G; Sebastiani, A; Ialleni, E; Diso, D; Rose, D; Quagliarini, F; Ialongo, P; Peruzzi, M; Venuta, F; Frati, G

    2015-04-01

    Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial pneumonia of unknown etiology, which is associated with the histopathologic pattern of usual interstitial pneumonia (UIP) and leads to a progressive decrease of respiratory function. The present article describes a case of a 62-year-old ex-smoker referred to our hospital because of IPF. After 2 years of follow-up, the subject experienced a significant worsening of pulmonary function and was enrolled in a lung transplantation program. Afterward, a pharmacological treatment with pirfenidone was started, achieving a stabilization of respiratory function. The patient underwent a single lung transplantation by means of a normothermic ex vivo lung perfusion (EVLP) approach according to the Toronto model. At 20-month evaluation the subject's respiratory function was significantly improved, and quality of life was considerably ameliorated. We believe that an integrated multidisciplinary approach should be considered a key option for the treatment of individuals with IPF. PMID:25891747

  6. Idiopathic Pulmonary Fibrosis: Epidemiology, Clinical Features, Prognosis, and Management.

    PubMed

    Lynch, Joseph P; Huynh, Richard H; Fishbein, Michael C; Saggar, Rajan; Belperio, John A; Weigt, S Sam

    2016-06-01

    Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic interstitial lung pneumonia associated with the histologic pattern of usual interstitial pneumonia (UIP). Although UIP is a distinct histologic lesion, this histologic pattern is not specific for IPF and can also be found in other diseases (e.g., connective tissue disease and asbestosis). Clinical features of IPF include progressive cough, dyspnea, restrictive ventilatory defect, and progressive fibrosis and destruction of the lung parenchyma. IPF is rare (13-42 cases/100,000), and primarily affects older adults (>50 years of age). The diagnosis of IPF often requires surgical lung biopsy, but the diagnosis can be affirmed with confidence in some patients provided the results of computed tomographic (CT) scans and clinical features are consistent. The clinical course is variable, but inexorable progression (typically over months to years) is typical. Mean survival from the onset of symptoms approximates 3 to 5 years. Medical treatment is only modestly effective, primarily by slowing the rate of disease progression. Lung transplantation is the best therapeutic option. PMID:27231859

  7. Practical management of Idiopathic Pulmonary Fibrosis.

    PubMed

    Kishaba, Tomoo

    2015-01-01

    Idiopathic Pulmonary Fibrosis (IPF) is relentless progressive interstitial lung disease (ILD) of unknown etiology. Main pathogenesis is aberrant recovery of epithelial injury and collagen deposition. Majority of IPF patients have been elderly men with smokers. However, there are important differential diagnosis such as fibrotic non-specific interstitial pneumonia (NSIP), Connective Tissue Disease (CTD) associated ILD, chronic hypersensitivity pneumonia (CHP). Clinical point of view, non-productive cough and progressive exertional dyspnea are main symptoms. In addition, scalene muscle hypertrophy, fine crackles and finger clubbing are key findings. Serum marker such as lactate deydrogenase (LDH), Krebs von den Lungeng-6 (KL-6) are sensitive for ILD detection and activity. Pulmonary function test and 6 minute walk test (6MWT) are quite meaningful physiological examination. Serial change of forced vital capacity 6MWT distance predict mortality of IPF. International IPF guideline published recently and highlighted on the importance of high resolution computed tomography (HRCT) findings. Key findings of IPF are honeycombing, traction bronchiectasis and subpleural reticular opacity. IPF is chronic progressive disease. Therefore, tracing disease behavior is crucial and unifying clinical, physiological, imaging information over time provide useful information for physicians.In management, many candidate agent failed to have positive result. Pirfenidone which is anti-fibrotic agent showed to slow the decline of vital capacity and prevent of acute exacerbation. Molecular agent such as nintedanib is promising agent for prevention of progression of IPF. In this review, we review the clinical information of IPF and IPF guideline. Lastly, we show the clinical algorithm of this devastated disease. PMID:26278687

  8. 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

  9. 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

  10. 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. PMID:22797205

  11. Pneumonia in the immunocompetent patient

    PubMed Central

    Reynolds, J H; Mcdonald, G; Alton, H; Gordon, S B

    2010-01-01

    Pneumonia is an acute inflammation of the lower respiratory tract. Lower respiratory tract infection is a major cause of mortality worldwide. Pneumonia is most common at the extremes of life. Predisposing factors in children include an under-developed immune system together with other factors, such as malnutrition and over-crowding. In adults, tobacco smoking is the single most important preventable risk factor. The commonest infecting organisms in children are respiratory viruses and Streptoccocus pneumoniae. In adults, pneumonia can be broadly classified, on the basis of chest radiographic appearance, into lobar pneumonia, bronchopneumonia and pneumonia producing an interstitial pattern. Lobar pneumonia is most commonly associated with community acquired pneumonia, bronchopneumonia with hospital acquired infection and an interstitial pattern with the so called atypical pneumonias, which can be caused by viruses or organisms such as Mycoplasma pneumoniae. Most cases of pneumonia can be managed with chest radiographs as the only form of imaging, but CT can detect pneumonia not visible on the chest radiograph and may be of value, particularly in the hospital setting. Complications of pneumonia include pleural effusion, empyema and lung abscess. The chest radiograph may initially indicate an effusion but ultrasound is more sensitive, allows characterisation in some cases and can guide catheter placement for drainage. CT can also be used to characterise and estimate the extent of pleural disease. Most lung abscesses respond to medical therapy, with surgery and image guided catheter drainage serving as options for those cases who do not respond. PMID:21088086

  12. Pneumonia in the immunocompetent patient.

    PubMed

    Reynolds, J H; McDonald, G; Alton, H; Gordon, S B

    2010-12-01

    Pneumonia is an acute inflammation of the lower respiratory tract. Lower respiratory tract infection is a major cause of mortality worldwide. Pneumonia is most common at the extremes of life. Predisposing factors in children include an under-developed immune system together with other factors, such as malnutrition and over-crowding. In adults, tobacco smoking is the single most important preventable risk factor. The commonest infecting organisms in children are respiratory viruses and Streptoccocus pneumoniae. In adults, pneumonia can be broadly classified, on the basis of chest radiographic appearance, into lobar pneumonia, bronchopneumonia and pneumonia producing an interstitial pattern. Lobar pneumonia is most commonly associated with community acquired pneumonia, bronchopneumonia with hospital acquired infection and an interstitial pattern with the so called atypical pneumonias, which can be caused by viruses or organisms such as Mycoplasma pneumoniae. Most cases of pneumonia can be managed with chest radiographs as the only form of imaging, but CT can detect pneumonia not visible on the chest radiograph and may be of value, particularly in the hospital setting. Complications of pneumonia include pleural effusion, empyema and lung abscess. The chest radiograph may initially indicate an effusion but ultrasound is more sensitive, allows characterisation in some cases and can guide catheter placement for drainage. CT can also be used to characterise and estimate the extent of pleural disease. Most lung abscesses respond to medical therapy, with surgery and image guided catheter drainage serving as options for those cases who do not respond. PMID:21088086

  13. 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 Central

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

    1987-01-01

    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. Images PMID:3037104

  14. 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. PMID:26906287

  15. [Interstitial lung diseases associated with smoking].

    PubMed

    Nová, Markéta; Hornychová, Helena; Matěj, Radoslav

    2016-01-01

    There are many different interstitial lung diseases associated with smoking. This short review describes officially recognized disorders (desquamative interstitial pneumonia, respiratory bronchiolitis and pulmonary Langerhans´cells histiocytosis) and entities with uncertain relationship to smoking, which have recently been published in the literature. Histopathological pictures and differential diagnosis of smoking-related diseases of the lungs are discussed. PMID:27223588

  16. Interstitial Nephritis

    MedlinePlus

    ... rye-tus) is a kidney disorder. The kidneys filter waste and extra fluid from the body. Interstitial nephritis reduces the kidneys’ ability to filter properly. Interstitial nephritis is a serious condition, but ...

  17. Idiopathic pulmonary fibrosis: Recent advances on pharmacological therapy.

    PubMed

    Spagnolo, P; Maher, T M; Richeldi, L

    2015-08-01

    Idiopathic pulmonary fibrosis (IPF) is the most common and lethal of the idiopathic interstitial pneumonias with an estimated 5-year survival of approximately 20%. In the last two decades our understanding of disease pathogenesis has substantially evolved and novel compounds have been developed consequent to the increasing knowledge of the mechanisms underlying disease pathobiology. The disease appears to be driven - following chronic injury - by abnormal/dysfunctional alveolar epithelial cells that promote fibroblast recruitment and proliferation, resulting in scarring of the lung and irreversible loss of function. With very few exceptions, clinical trials evaluating novel potential therapies have provided disappointing results. More recently, pirfenidone and nintedanib, two compounds with pleiotropic mechanisms of action, have proven effective in slowing functional decline and disease progression in IPF patients with mild to moderate functional impairment, highlighting the importance of timely diagnosis and administration of treatment in early stages of disease. However, due to the complexity and uncertainties intrinsic to IPF, it is essential that each therapeutic strategy be tailored to the individual patient, after evaluation of potential benefits and risks. This article provides an overview of the most recent clinical trials in IPF and discusses how their results are going to change the clinical and clinical research landscape in IPF. A number of agents with high potential are currently being tested and many more are ready for clinical trials. Their completion is critical for achieving the ultimate goal of curing patients with IPF. PMID:25946646

  18. 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

  19. Transbronchial Lung Cryobiopsy in the Diagnosis of Fibrotic Interstitial Lung Diseases

    PubMed Central

    Cavazza, Alberto; Colby, Thomas V.; Dubini, Alessandra; Ryu, Jay H.; Carretta, Elisa; Tantalocco, Paola; Piciucchi, Sara; Ravaglia, Claudia; Gurioli, Christian; Romagnoli, Micaela; Gurioli, Carlo; Chilosi, Marco; Poletti, Venerino

    2014-01-01

    Background Histology is a key element for the multidisciplinary diagnosis of fibrotic diffuse parenchymal lung diseases (f-DPLD) when the clinical-radiological picture is nondiagnostic. Transbronchial lung cryobiopsy (TBLC) have been shown to be useful for obtaining large and well-preserved biopsies of lung parenchyma, but experience with TBLC in f-DPLD is limited. Objectives To evaluate safety, feasibility and diagnostic yield of TBLC in f-DPLD. Method Prospective study of 69 cases of TBLC using flexible cryoprobe in the clinical-radiological setting of f-DPLD with nondiagnostic high resolution computed tomography (HRCT) features. Results Safety: pneumothorax occurred in 19 patients (28%). One patient (1.4%) died of acute exacerbation. Feasibility: adequate cryobiopsies were obtained in 68 cases (99%). The median size of cryobiopsies was 43.11 mm2 (range, 11.94–76.25). Diagnostic yield: among adequate TBLC the pathologists were confident (“high confidence”) that histopathologic criteria sufficient to define a specific pattern in 52 patients (76%), including 36 of 47 with UIP (77%) and 9 nonspecific interstitial pneumonia (6 fibrosing and 3 cellular), 2 desquamative interstitial pneumonia/respiratory bronchiolitis–interstitial lung disease, 1 organizing pneumonia, 1 eosinophilic pneumonia, 1 diffuse alveolar damage, 1 hypersensitivity pneumonitis and 1 follicular bronchiolitis. In 11 diagnoses of UIP the pathologists were less confident (“low confidence”). Agreement between pathologists in the detection of UIP was very good with a Kappa coefficient of 0.83 (95% CI, 0.69–0.97). Using the current consensus guidelines for clinical-radiologic-pathologic correlation 32% (20/63) of cases were classified as Idiopathic Pulmonary Fibrosis (IPF), 30% (19/63) as possible IPF, 25% (16/63) as other f-DPLDs and 13% (8/63) were unclassifiable. Conclusions TBLC in the diagnosis of f-DPLD appears safe and feasible. TBLC has a good diagnostic yield in the clinical

  20. 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 ...

  1. 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

  2. Genetic studies provide clues on the pathogenesis of idiopathic pulmonary fibrosis.

    PubMed

    Kropski, Jonathan A; Lawson, William E; Young, Lisa R; Blackwell, Timothy S

    2013-01-01

    Idiopathic pulmonary fibrosis (IPF) is a progressive and often fatal lung disease for which there is no known treatment. Although the traditional paradigm of IPF pathogenesis emphasized chronic inflammation as the primary driver of fibrotic remodeling, more recent insights have challenged this view. Linkage analysis and candidate gene approaches have identified four genes that cause the inherited form of IPF, familial interstitial pneumonia (FIP). These four genes encode two surfactant proteins, surfactant protein C (encoded by SFTPC) and surfactant protein A2 (SFTPA2), and two components of the telomerase complex, telomerase reverse transcriptase (TERT) and the RNA component of telomerase (TERC). In this review, we discuss how investigating these mutations, as well as genetic variants identified in other inherited disorders associated with pulmonary fibrosis, are providing new insights into the pathogenesis of common idiopathic interstitial lung diseases, particularly IPF. Studies in this area have highlighted key roles for epithelial cell injury and dysfunction in the development of lung fibrosis. In addition, genetic approaches have uncovered the importance of several processes - including endoplasmic reticulum stress and the unfolded protein response, DNA-damage and -repair pathways, and cellular senescence - that might provide new therapeutic targets in fibrotic lung diseases. PMID:23268535

  3. Idiopathic anaphylaxis.

    PubMed

    Greenberger, Paul A

    2007-05-01

    Idiopathic anaphylaxis is a prednisone-responsive condition without external cause, but it can coexist with food-, medication-, or exercise-induced anaphylaxis. Mast cell activation may occur at night or after foods that have been eaten with impunity many times previously. Idiopathic anaphylaxis can be classified into frequent (if there are six or more episodes per year or two episodes in the last 2 months) or infrequent (if episodes occur less often). Idiopathic anaphylaxis-generalized consists of urticaria or angioedema associated with severe respiratory distress, syncope or hypotension, and gastrointestinal symptoms. Idiopathic anaphylaxis-angioedema consists of massive tongue enlargement or severe pharyngeal or laryngeal swelling with urticaria or peripheral angioedema. The differential diagnosis of idiopathic anaphylaxis is reviewed, and treatment approaches are presented. PMID:17493503

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

    PubMed Central

    Chung, Min Jae; Lee, Jae Hee

    2015-01-01

    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. PMID:26770905

  5. The multidisciplinary approach in the diagnosis of idiopathic pulmonary fibrosis: a patient case-based review.

    PubMed

    Tomassetti, Sara; Piciucchi, Sara; Tantalocco, Paola; Dubini, Alessandra; Poletti, Venerino

    2015-03-01

    Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressively fibrosing interstitial pneumonia that is associated with a significantly worse prognosis than other forms of chronic interstitial pneumonia. An early and accurate diagnosis of IPF is important to enable the initiation of disease-specific therapies, which have the potential to reduce disease progression, and the avoidance of inappropriate and potentially harmful drugs. Establishing an accurate diagnosis of IPF can be challenging. Recent studies and international guidelines advocate the importance of a multidisciplinary team (MDT) in the initial diagnostic assessment of patients with suspected IPF. Typical MDT members include a pulmonologist, a radiologist and a pathologist, with further input from a thoracic surgeon, a rheumatologist, a specialist nurse and an occupational physician where appropriate. Multidisciplinary diagnosis is considered the gold standard because it can improve the accuracy of diagnosis of IPF, avoid unnecessary testing (e.g. lung biopsy), and optimise patient management. Here we highlight the strengths and limitations of the multidisciplinary approach to IPF diagnosis through MDT discussion of two patient cases. PMID:25726558

  6. Idiopathic hypersomnia

    MedlinePlus

    ... page, please enable JavaScript. Idiopathic hypersomnia is a sleep disorder in which a person is excessively sleepy ( hypersomnia ) ... other potential causes of excessive daytime sleepiness. Other sleep disorders that may cause daytime sleepiness include: Narcolepsy Obstructive ...

  7. Increased fibroblast telomerase expression precedes myofibroblast α-smooth muscle actin expression in idiopathic pulmonary fibrosis

    PubMed Central

    Waisberg, Daniel Reis; Parra, Edwin Roger; Barbas-Filho, João Valente; Fernezlian, Sandra; Capelozzi, Vera Luiza

    2012-01-01

    OBJECTIVE: This study sought to identify the relationship between fibroblast telomerase expression, myofibroblasts, and telomerase-mediated regulatory signals in idiopathic pulmonary fibrosis. METHODS: Thirty-four surgical lung biopsies, which had been obtained from patients with idiopathic pulmonary fibrosis and histologically classified as usual interstitial pneumonia, were examined. Immunohistochemistry was used to evaluate fibroblast telomerase expression, myofibroblast α-smooth muscle actin expression and the tissue expression of interleukin-4, transforming growth factor-β, and basic fibroblast growth factor. The point-counting technique was used to quantify the expression of these markers in unaffected, collapsed, mural fibrosis, and honeycombing areas. The results were correlated to patient survival. RESULTS: Fibroblast telomerase expression and basic fibroblast growth factor tissue expression were higher in collapsed areas, whereas myofibroblast expression and interleukine-4 tissue expression were higher in areas of mural fibrosis. Transforming growth factor-β expression was higher in collapsed, mural fibrosis and honeycombing areas in comparison to unaffected areas. Positive correlations were found between basic fibroblast growth factor tissue expression and fibroblast telomerase expression and between interleukin-4 tissue expression and myofibroblast α-smooth muscle actin expression. Negative correlations were observed between interleukin-4 expression and basic fibroblast growth factor tissue expression in areas of mural fibrosis. Myofibroblast α-smooth muscle actin expression and interleukin-4 tissue expression in areas of mural fibrosis were negatively associated with patient survival. CONCLUSION: Fibroblast telomerase expression is higher in areas of early remodeling in lung tissues demonstrating typical interstitial pneumonia, whereas myofibroblast α-smooth muscle actin expression predominates in areas of late remodeling. These events seem to be

  8. 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

  9. 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 ...

  10. 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

  11. Mineral Oil Aspiration Related Juvenile Idiopathic Arthritis.

    PubMed

    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

  12. Soluble intercellular adhesion molecule-1 for stable and acute phases of idiopathic pulmonary fibrosis.

    PubMed

    Okuda, Ryo; Matsushima, Hidekazu; Aoshiba, Kazutetsu; Oba, Tomohiro; Kawabe, Rie; Honda, Koujiro; Amano, Masako

    2015-01-01

    The levels of soluble intercellular adhesion molecule-1 (sICAM-1) have been reported to increase in patients with idiopathic pulmonary fibrosis. However, the utility of sICAM-1 has not been reported in detail. The aim of this study was to investigate whether sICAM-1 was a useful biomarker for stable idiopathic pulmonary fibrosis (IPF) and early phase of acute exacerbation of IPF. The patients who were diagnosed with IPF between 2013 and 2015 were enrolled. The levels of sICAM-1 and other interstitial pneumonia markers were measured. In this study, 30 patients with stable IPF and 11 patients with acute exacerbation of IPF were collected. Mean sICAM-1 levels were 434 ± 139 ng/mL for the stable phase of IPF, 645 ± 247 ng/mL for early phase of acute exacerbation of IPF, 534 ± 223 ng/mL for connective tissue disease-associated interstitial pneumonia, 221 ± 42 for chronic obstructive pulmonary disease, and 150 ± 32 ng/mL in healthy volunteers. For the stable phase of IPF, sICAM-1 levels correlated with Krebs von den Lungen-6 (KL-6) (r value: 0.41; p value: 0.036). Mean sICAM-1 levels were significantly higher in patients with early phase of acute exacerbation of IPF than with stable phase of IPF (p = 0.0199). Multiple logistic analyses indicated that the predictors for early phase of acute exacerbation of IPF were only sICAM-1 and C-reactive protein (odds ratio: 1.0093; 1.6069). In patients with stable IPF, sICAM-1 levels correlated with KL-6; sICAM-1 might be a predictive indicator for prognosis. In the early phase of acute exacerbation of IPF, sICAM-1 might be more useful for diagnosis than other interstitial pneumonia markers. PMID:26543791

  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. Pneumonia - weakened immune system

    MedlinePlus

    ... immunocompromised host." Related conditions include: Hospital-acquired pneumonia Pneumocystis jirovecii (previously called Pneumocystis carinii) pneumonia Pneumonia - cytomegalovirus Pneumonia ...

  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. [Chest radiograph of atypical pneumonia: comparison among Chlamydia pneumoniae. Pneumonia, ornithosis, and Mycoplasma pneumoniae pneumonia].

    PubMed

    Itoh, I; Ishida, T; Hashimoto, T; Arita, M; Osawa, M; Tachibana, H; Nishiyama, H; Takakura, S; Bando, K; Nishizawa, Y; Amitani, R; Onishi, H; Taguchi, Y

    2000-11-01

    No report has been found comparing Chlamydia pneumoniae (C. pneumoniae) pneumonia radiographically with other atypical pneumonias, Chlamydia psittaci (C. psittaci) pneumonia and Mycoplasma pneumoniae (M. pneumoniae) pneumonia. We described the chest radiographs of three kinds of pneumonia cases: 46 cases of C. pneumoniae pneumonia, 39 cases of C. psittaci pneumonia, and 131 cases of M. pneumoniae pneumonia. Radiographic shadows were categorized into main shadows and sub-shadows. The main shadows are classified from the viewpoint of the characteristics; air space consolidation(AS), ground-glass opacity(GG), reticular shadow(RS), bronchopneumonia(BP), and small nodular shadows (SN). The size, the site, and the number of the main shadows were also analyzed. In comparison among the three pneumonias, BP was the most frequent in M. pneumoniae pneumonia (0.40/case). AS predominated in C. pneumoniae pneumonia (0.67/case), and GG in C. psittaci pneumonia (0.62/case). The number of main shadows was equal, about 1.4/case in three pneumonias. Large shadows were less frequent in M. pneumoniae pneumonia than C. pneumoniae pneumonia (p = 0.02) and C. psittaci pneumonia (p = 0.01). Main shadows were more frequent in the outer zone in M. pneumoniae pneumonia than C. psittaci pneumonia (p = 0.01), and in the middle zone in C. psittaci pneumonia than in M. pneumoniae pneumonia (p = 0.02). Cases with bilateral main shadows were less common in M. pneumoniae pneumonia (9%) than C. pneumoniae pneumonia(33%, p = 0.001) and C. psittaci pneumonia(30%, p = 0.005). Thickening of bronchovascular bundles as a sub-shadow was most frequently noted in M. pneumoniae pneumonia. Some differences among the three atypical pneumonias were seen in the chest radiograph. However, no specific findings of C. pneumoniae pneumonia were shown radiographically in this study. PMID:11140079

  17. 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.

  18. Biomarkers in connective tissue disease-associated interstitial lung disease.

    PubMed

    Bonella, Francesco; Costabel, Ulrich

    2014-04-01

    This article reviews major biomarkers in serum and bronchoalveolar lavage fluid (BALF) with respect to their diagnostic and prognostic value in connective tissue disease-associated interstitial lung disease (CTD-ILD). In some CTD such as systemic sclerosis (SSc), the incidence of ILD is up to two-third of patients, and currently ILD represents the leading cause of death in SSc. Because of the extremely variable incidence and outcome of ILD in CTD, progress in the discovery and validation of biomarkers for diagnosis, prognosis, patients' subtyping, response to treatment, or as surrogate endpoints in clinical trials is extremely important. In contrast to idiopathic interstitial pneumonias, autoantibodies play a crucial role as biomarkers in CTD-ILD because their presence is strictly linked to the pathogenesis and tissue damage. Patterns of autoantibodies, for instance, anticitrullinated peptide antibodies in rheumatoid arthritis or aminoacyl-tRNA synthetases (ARS) in polymyositis/dermatomyositis, have been found to correlate with the presence and occasionally with the course of ILD in CTD. Besides autoantibodies, an increase in serum or BALF of a biomarker of pulmonary origin may be able to predict or reflect the development of fibrosis, the impairment of lung function, and ideally also the prognosis. Promising biomarkers are lung epithelium-derived proteins such as KL-6 (Krebs von den Lungen-6), SP-D (surfactant protein-D), SP-A (surfactant protein-A), YKL-40 (chitinase-3-like protein 1 [CHI3L1] or cytokines such as CCL18 [chemokine (C-C) motif ligand 18]). In the future, genetic/epigenetic markers, such as human leukocyte antigen (HLA) haplotypes, single nucleotide polymorphisms, and micro-RNA, may help to identify subtypes of patients with different needs of management and treatment strategies. PMID:24668534

  19. Idiopathic hypersomnia.

    PubMed

    Billiard, Michel; Sonka, Karel

    2016-10-01

    Idiopathic hypersomnia continues to evolve from the concept of "sleep drunkenness" introduced by Bedrich Roth in Prague in 1956 and the description of idiopathic hypersomnia with two forms, polysymptomatic and monosymptomatic, by the same Bedrich Roth in 1976. The diagnostic criteria of idiopathic hypersomnia have varied with the successive revisions of the International classifications of sleep disorders, including the recent 3rd edition. No epidemiological studies have been conducted so far. Disease onset occurs most often during adolescence or young adulthood. A familial background is often present but rigorous studies are still lacking. The key manifestation is hypersomnolence. It is often accompanied by sleep of long duration and debilitating sleep inertia. Polysomnography (PSG) followed by a multiple sleep latency test (MSLT) is mandatory, as well as a 24 h PSG or a 2-wk actigraphy in association with a sleep log to ensure a total 24-h sleep time longer than or equal to 66O minutes, when the mean sleep latency on the MSLT is longer than 8 min. Yet, MSLT is neither sensitive nor specific and the polysomnographic diagnostic criteria require continuous readjustment and biologic markers are still lacking. Idiopathic hypersomnia is most often a chronic condition though spontaneous remission may occur. The condition is disabling, sometimes even more so than narcolepsy type 1 or 2. Based on neurochemical, genetic and immunological analyses as well as on exploration of the homeostatic and circadian processes of sleep, various pathophysiological hypotheses have been proposed. Differential diagnosis involves a number of diseases and it is not yet clear whether idiopathic hypersomnia and narcolepsy type 2 are not the same condition. Until now, the treatment of idiopathic hypersomnia has mirrored that of the sleepiness of narcolepsy type 1 or 2. The first randomized, double-blind, placebo-controlled trials of modafinil have just been published, as well as a double

  20. Idiopathic pulmonary fibrosis: Diagnosis, epidemiology and natural history.

    PubMed

    Sgalla, Giacomo; Biffi, Alice; Richeldi, Luca

    2016-04-01

    Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing lung disorder of unknown aetiology whose diagnosis involves the careful exclusion of secondary causes for pulmonary fibrosis and the presence of a pattern of usual interstitial pneumonia (UIP) at either high-resolution computed tomography (HRCT) scan or surgical lung biopsy. Despite great efforts made in establishing precise, universally acknowledged diagnostic criteria for IPF, its ascertainment remains a challenge, especially in those individuals presenting with atypical HRCT patterns. With new drugs emerging, establishing a precise diagnosis is becoming a clinically relevant issue. Although regarded as a rare disease, IPF epidemiology is controversial due to studies relying on old data and adopting mixed, incomparable methodologies for cases definition. Overall, the prevalence and incidence appear to be increasing over the last decades, suggesting that in earlier studies they might have been underestimated because of diagnostic uncertainty. IPF is invariably progressive, although its clinical course might greatly vary on an individual basis, with episodes of severe acute respiratory deterioration (acute exacerbations) being unpredictable. A deeper understanding of the mechanisms responsible for an accelerated course of the disease and the identification of biomarkers of progression would lead to a better stratification of the disease, essential for delivering individualized therapeutic strategies. PMID:26595062

  1. 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

  2. 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

  3. NLRP3 inflammasome expression in idiopathic pulmonary fibrosis and rheumatoid lung.

    PubMed

    Lasithiotaki, Ismini; Giannarakis, Ioannis; Tsitoura, Eliza; Samara, Katerina D; Margaritopoulos, George A; Choulaki, Christiana; Vasarmidi, Eirini; Tzanakis, Nikolaos; Voloudaki, Argyro; Sidiropoulos, Prodromos; Siafakas, Nikolaos M; Antoniou, Katerina M

    2016-03-01

    In this study we investigated the implication of NLRP3 inflammasomes in the pathogenesis of idiopathic pulmonary fibrosis (IPF) and rheumatoid arthritis-usual interstitial pneumonia (RA-UIP).NLRP3 inflammasome activation at baseline and following stimulation with lipopolysaccharide/ATP was evaluated by measuring interleukin (IL)-1β and IL-18 levels released in the bronchoalveolar lavage fluid (BALF) fluid and by cultures of BALF cells. IL-1β and IL-18 levels were significantly elevated in the BALF and BALF macrophage cultures from RA-UIP patients, consistent with pre-existing inflammasome activation in these patients. In contrast, in IPF, BALF levels of IL-1β were significantly less elevated relative to RA-UIP and IL-18 was lower than controls. Furthermore, upon inflammasome stimulation, IPF BALF macrophage cultures failed to upregulate IL-1β and partly IL-18 secretion, in contrast to controls, which showed robust IL-1β and IL-18 upregulation. Interestingly, RA-UIP BALF cell cultures treated with lipopolysaccharide/ATP showed a potent stimulation of IL-18 secretion but not IL-1β, the latter being already elevated in the unstimulated cultures, while examination of the intracellular IL-1β levels in RA-UIP BALF cells upon NLRP3 inflammasome stimulation showed a significant upregulation of IL-1β suggesting the NLRP3 pathway could be further activated.Taken together, our results suggest distinct inflammasome activation profiles between autoimmune and idiopathic lung fibrosis. PMID:26743485

  4. [Romanian national registry for interstitial lung diseases and sarcoidosis (REGIS): rationale and methods].

    PubMed

    Strâmbu, Irina; Ioniţă, Diana; Belaconi, Ionela; Stoia, Irina; Tudorache, Voicu

    2012-01-01

    Interstitial lung diseases (ILD) are a group of extremely heterogeneous conditions (over 200), with low prevalence, but in most cases with severe impact on the quality of life and survival of the patients. The ILD group comprises: idiopathic pulmonary fibrosis, sarcoidosis, hypersensitivity pneumonitis, colagen diseases, vasculitis, eosinophilic pneumonia etc. The prevalence of these diseases is unknown in Romania; the accurate diagnosis needs access to special investigations and expertise with this group of diseases. The authors propose the initiation of a National Registry for Interstitial Lung Diseases and Sarcoidosis (REGIS), based on the lessons learned from other national ILD) registries, which should allow building-up an extended clinical expertise in ILD, evaluating epidemiological data, creating an educational platform for young physicians, better understanding of the outcome and prognosis of these diseases, shaping Romanian Guidelines for the diagnosis and management of ILD, using the data base for clinical research. The registry is started as a pilot in two Romanian centres: "Marius Nasta" Institute of Pneumology in Bucharest and Pneumology Clinic, "Victor Babey" Infectious Diseases Hospital Timişoara. After refining the inclusion criteria for the data base, editing guidelines for ILD diagnosis and launching the registry website, other Romanian centres with interest in ILD will also be able to feed information. PMID:22545484

  5. Pulmonary Hemodynamics and Six-Minute Walk Test Outcomes in Patients with Interstitial Lung Disease

    PubMed Central

    Nishiyama, Osamu; Yamazaki, Ryo; Sano, Hiroyuki; Iwanaga, Takashi; Higashimoto, Yuji; Kume, Hiroaki; Tohda, Yuji

    2016-01-01

    Background. Six-minute walk test (6MWT) has 3 measurement outcomes, which are walk distance, desaturation, and symptom. The aim of this study was to examine whether routinely measured right-heart catheter (RHC) data correlate with 6MWT outcomes in patients with interstitial lung disease (ILD). Methods. Between June 2010 and December 2012, consecutive patients with ILD who underwent evaluation, including pulmonary function test, hemodynamic studies with right-heart catheter, and 6MWT as routine general practice, were recruited. Correlates of 3 outcomes of 6MWT were examined to reveal significant predictors. Results. Forty-six patients consisting of 20 with idiopathic pulmonary fibrosis, 14 with collagen vascular disease associated ILD, and 12 with other idiopathic interstitial pneumonia were recruited (mean % predicted FVC: 76.7 ± 17.1%). Several physiological variables, including mean pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR), were correlated with each 6MWT outcome. Stepwise multivariate regression analyses showed that % predicted FVC and % predicted DLco were independent predictors of walk distance (r2 = 0.35, p = 0.0002). For SpO2 at the end of 6MWT, % predicted DLco and PVR were selected as independent predictors (r2 = 0.46, p < 0.0001). For dyspnea at the end of 6MWT, % predicted DLco was only one predictor (r2 = 0.18, p = 0.005). Conclusion. Mean PAP had little impact on 6MWT outcomes in ILD patients who were nonselectively recruited, although PVR was one of predictors of desaturation.

  6. Lipoid pneumonia: an overview.

    PubMed

    Hadda, Vijay; Khilnani, Gopi C

    2010-12-01

    Lipoid pneumonia is an uncommon disease caused by the presence of lipid in the alveoli. It is classified into two major groups, depending on whether the lipid/oil in the respiratory tract is from an exogenous (exogenous lipoid pneumonia) or endogenous/idiopathic (endogenous lipoid pneumonia) source. The usual presentation occurs with insidious onset and nonspecific respiratory symptoms such as dyspnea and/or cough. The main radiological findings include airspace consolidations, ground-glass attenuation, airspace nodules and 'crazy-paving' pattern. However, the radiological appearance of the disorder can mimic many other lung diseases, including carcinoma. Owing to the nonspecific clinical presentation and radiological features, the diagnosis is often missed or delayed. Pathologically, lipoid pneumonia is a chronic foreign body reaction to fat, characterized by lipid-laden macrophages. Diagnosis of this disease requires a high index of suspicion and can be confirmed by demonstration of lipid-laden macrophages in respiratory samples such as sputum, bronchoalveolar lavage fluid or fine-needle aspiration cytology/biopsy from lung lesions. Treatment protocols for this illness are poorly defined. PMID:21128754

  7. Radiological diagnosis of pneumonia in children.

    PubMed

    Kiekara, O; Korppi, M; Tanska, S; Soimakallio, S

    1996-02-01

    During 12 months in 1981-82, 201 children were hospitalized due to radiologically verified definite or probable pneumonia. In 1985, 194 chest radiographs (anteroposterior views) were re-evaluated jointly by two radiologists, and classified into three categories: alveolar, interstitial and probable pneumonia. In 127 cases definite pneumonia was diagnosed on both occasions, alveolar in 48 cases and interstitial in 79 cases. Variation between the two evaluations 3 years apart was observed in 46 (24%) of the 194 cases; the adjusted kappa (0.47) was in the modest region. Factors contributing to this variation were young age, less than 12 months, and the presence of interstitial infiltration, bronchial obstruction and low C-reactive protein. Factors associated with less marked variation were the presence of alveolar infiltration, auscultatory fine rates and elevated C-reactive protein. The microbial aetiology of infection, assessed by viral and bacterial antigen and antibody assays, showed no association with diagnostic variation. A lateral view of the chest radiograph was obtained from 158 patients; it was positive in 99 (91%) of the 109 cases with definite pneumonia. In only three cases the diagnosis was based on the lateral view alone. Our results show that the radiological diagnosis of pneumonia is difficult in children, especially in young children with interstitial pneumonia. PMID:8932509

  8. Smoking and interstitial lung diseases.

    PubMed

    Margaritopoulos, George A; Vasarmidi, Eirini; Jacob, Joseph; Wells, Athol U; Antoniou, Katerina M

    2015-09-01

    For many years has been well known that smoking could cause lung damage. Chronic obstructive pulmonary disease and lung cancer have been the two most common smoking-related lung diseases. In the recent years, attention has also focused on the role of smoking in the development of interstitial lung diseases (ILDs). Indeed, there are three diseases, namely respiratory bronchiolitis-associated ILD, desquamative interstitial pneumonia and pulmonary Langerhans cell histiocytosis, that are currently considered aetiologically linked to smoking and a few others which are more likely to develop in smokers. Here, we aim to focus on the most recent findings regarding the role of smoking in the pathogenesis and clinical behaviour of ILDs. PMID:26324804

  9. 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 ...

  10. Mycoplasma pneumonia

    MedlinePlus

    ... page: //medlineplus.gov/ency/article/000082.htm Mycoplasma pneumonia To use the sharing features on this page, please enable JavaScript. Mycoplasma pneumonia is an infection of the lungs by the ...

  11. 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. ...

  12. Childhood Interstitial Lung Disease

    MedlinePlus

    ... from the NHLBI on Twitter. What Is Childhood Interstitial Lung Disease? Childhood interstitial (in-ter-STISH-al) lung disease, ... with similar symptoms—it's not a precise diagnosis. Interstitial lung disease (ILD) also occurs in adults. However, the cause ...

  13. Pemetrexed for advanced non-small cell lung cancer patients with interstitial lung disease

    PubMed Central

    2014-01-01

    Background Non-small cell lung cancer (NSCLC) patients with interstitial lung disease (ILD) need to be approached carefully given the high incidence of pulmonary toxicity. Pemetrexed (PEM) is the key drug for the treatment of NSCLC. However, its safety, especially with respect to the exacerbation of ILD, and efficacy in NSCLC patients with ILD have yet to be established. Method We investigated the safety and efficacy of PEM monotherapy in NSCLC patients with or without idiopathic interstitial pneumonia (IIPs). The medical charts of these patients were retrospectively reviewed. Results Twenty-five patients diagnosed as having IIPs (IIPs group) and 88 patients without ILD (non-ILD group) were treated with PEM monotherapy at Juntendo University Hospital between 2009 and 2013. In the IIPs group, 12 patients were found to have usual interstitial pneumonitis (UIP) on chest computed tomography (CT) (UIP group) and the other 13 patients showed a non-UIP pattern on chest CT (non-UIP IIPs group). Three patients in the IIPs group (2 in the UIP group and 1 in the non-UIP IIPs group) and 1 in the non-ILD group developed pulmonary toxicity during treatment (3.5% overall, 12.0% in the IIPs group versus 1.1% in the non-ILD group). Moreover, all 3 patients in the IIPs group died of pulmonary toxicity. Overall survival tended to be longer in the non-ILD group than in the IIPs group (p = 0.08). Multivariate analyses demonstrated that IIPs was the only significant independent risk factor for PEM-related pulmonary toxicity. Conclusion We found that the incidence of PEM-related pulmonary toxicity was significantly higher amongst NSCLC patients with IIPs than among those without IIPs. Particular care must be taken when administering PEM to treat NSCLC patients with IIPs. PMID:25012241

  14. 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

  15. Juvenile Idiopathic Arthritis

    MedlinePlus

    ... Is Juvenile Idiopathic Arthritis the same as Juvenile Rheumatoid Arthritis? Yes, Juvenile Idiopathic Arthritis (JIA) is a new ... of chronic inflammatory diseases that affect children. Juvenile Rheumatoid Arthritis (JRA) is the older term that was used ...

  16. Idiopathic cardiomegaly*

    PubMed Central

    1968-01-01

    Cardiomyopathies are certain heart diseases of unknown etiology and pathogenesis, occurring mostly in tropical and subtropical areas, where they constitute a major clinical problem and sometimes a public health problem. The need for international co-operation in the study of such forms of heart disease has long been recognized and WHO convened informal meetings of investigators on various aspects of the subject in 1964, 1965 and 1966. Out of these have arisen co-operative studies co-ordinated by WHO. In November 1967 a fourth informal meeting was held in Kingston, Jamaica, to review the following topics: the progress reports from all co-operating laboratories; the different types of cardiomyopathies; past experience with cardiac registries, and the diagnostic importance of coronary angiography. Steps were taken towards the formulation of a standard terminology, since too many confusing names are currently employed to mean “cardiomegaly of unknown origin”. A common name, “idiopathic cardiomegaly”, was therefore suggested for future use. The account presented here was prepared by Dr Z. Fejfar, Chief Medical Officer, Cardiovascular Diseases, World Health Organization, Geneva, on behalf of the other participants and is a précis of some of the information that was exchanged, some of the views that were expressed and of the suggestions that were made. PMID:4235740

  17. The inter-connection between fibrosis and microvascular remodeling in idiopathic pulmonary fibrosis: Reality or just a phenomenon

    PubMed Central

    Mlika, Mona; Bacha, Saoussen; Braham, Emna; El Mezni, Faouzi

    2015-01-01

    Background Idiopathic pulmonary fibrosis is the most frequent interstitial disease with the worst prognosis. It is characterized by an uncontrolled fibrosis which is difficult to manage. The pathogenesis of this disease remains unclear with many theories resulting in multiple target therapies. The relation between fibrosis and vascular remodeling has been debated in the literature with different results that may seem contradictory. Aim We target to evaluate the connection between fibrosis and vascular remodeling in usual interstitial pneumonia. Material and methods 26 cases of idiopathic pulmonary fibrosis were reviewed by 2 pathologists and the diagnosis of UIP was retained according to the American Thoracic Society's criteria. Fibrotic changes and vascular remodeling were evaluated blindly. The fibrotic changes were classified as severe, intermediate and mild. Vascular occlusion was graded in 4 grades extending from medial hypertrophy (grade 1) to plexiform lesions of the vascular wall (grade 4). Results We noticed that severe degrees of fibrosis were correlated with severe grades of vascular obstruction. In fact, our 26 cases were classified as severe fibrosis in 11 cases with grade IV vascular lesions in 6 cases, intermediate fibrosis in 12 cases with grade II vascular lesions in 8 cases and mild fibrosis in 3 cases with grade I vascular lesions in all cases. Conclusion Many theories have been reported concerning the UIP's pathogenesis. Recently, many authors reported that the primum movens of these lesions was an epithelial/endothelial injury which induces uncontrolled fibrosis and microvascular remodeling using different pathways. This puts emphasis on the necessity of multi-target therapies in order to improve the management of this fatal disease. PMID:27222781

  18. 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: ...

  19. 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

  20. Sildenafil Induced Acute Interstitial Nephritis

    PubMed Central

    Burkhart, Ryan; Shah, Nina; Lewin, Matthew

    2015-01-01

    Acute interstitial nephritis (AIN) is characterized by inflammation of the renal interstitium and usually occurs in a temporal relationship with the medication. We present a case of an Asian male who had nephrotic range proteinuria and presented with acute kidney injury. The patient reported an acute change in physical appearance and symptomatology after the ingestion of a single dose of sildenafil. Renal biopsy was notable for minimal change disease (MCD) with acute and chronic interstitial nephritis. Renal replacement and glucocorticoid therapy were initiated. Renal recovery within six weeks permitted discontinuation of dialysis. AIN superimposed on MCD is a known association of NSAID induced nephropathy. The temporal association and the absence of any new drugs suggest that the AIN was most likely due to the sildenafil. NSAIDs are less likely to have caused the AIN given their remote use. The ease of steroid responsiveness would also suggest another cause as NSAID induced AIN is often steroid resistant. The MCD was most likely idiopathic given the lack of temporal association with a secondary cause. As the number of sildenafil prescriptions increases, more cases of AIN may be identified and physician awareness for this potential drug disease association is necessary. PMID:26491581

  1. What We Know About the Pathogenesis of Idiopathic Pulmonary Fibrosis.

    PubMed

    Puglisi, S; Torrisi, S E; Giuliano, R; Vindigni, V; Vancheri, C

    2016-06-01

    Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease of unknown cause, occurring in adults, limited to the lungs and associated with the pathologic and radiologic pattern of usual interstitial pneumonia. Prognosis is poor, and most patients die of respiratory failure within 3 to 6 years from the onset of symptoms. Although our understanding of the pathogenesis of IPF has improved over the past two decades, the mechanisms responsible for this disorder have not been clearly defined. Aging is the single most important risk factor, but genetic, environmental, and diverse exogenous factors such as smoking, viral infections, chronic tissue injury (i.e., gastroesophageal reflux disease, traction injury) play contributory roles. In this review, we focus on pathogenetic mechanisms that we think are crucial for the initiation of the fibrotic process and for its progressive evolution. In the early stage of the disease, in the context of the permissive genetic background combined with the presence of specific risk factors, alveolar epithelial cells play a leading role. Subsequent evolution of the fibrotic process and its lethal progression is likely due to the abnormal tissue repair process that takes place in the lung and to the inability to counteract this process. In this phase of the disease, fibroblasts assume a crucial role. Current pharmacological treatment strategies for IPF have only modest value, principally by slowing the course of disease progression. Unfortunately, improvement or cure has not yet been achieved with pharmacological agents. The challenge for the future is to improve the comprehension of the mechanisms involved in the inception and evolution of IPF and their articulated interactions. This is fundamental not only to conceive and develop new drugs against this dreadful disease but also to apply different therapeutic approaches such as drug repositioning and personalized therapies in the management of IPF. PMID:27231860

  2. 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 ... is responsible for some types of interstitial lung diseases. Specific types include Black lung disease among coal ...

  3. Comparison of radiological findings and microbial aetiology of childhood pneumonia.

    PubMed

    Korppi, M; Kiekara, O; Heiskanen-Kosma, T; Soimakallio, S

    1993-04-01

    Sixty-one children were treated in hospital from 1981 to 1982 because of both radiologically and microbiologically verified viral or bacterial pneumonia. The chest radiographs were interpreted by two radiologists, not familiar with the clinical data, on two occasions three years apart, and only those patients with a definite alveolar (n = 27) or interstitial (n = 34) pneumonia at both evaluations were included in the present analysis. In addition, all patients had viral (n = 20), mixed viral-bacterial (n = 21) or bacterial (n = 20) infections diagnosed by viral or bacterial antibody or antigen assays. Viral infection alone was seen in 7 (26%), mixed viral-bacterial infection in 8 (30%) and bacterial infection alone in 12 (44%) of the 27 patients with alveolar pneumonia. The respective figures were 13 (38%), 13 (38%) and 8 (24%) for the 34 patients with interstitial pneumonia. C-reactive protein concentration was greater than 40 mg/l (a screening limit for viral and bacterial infections) in 15 (56%) of the patients with alveolar and in 11 (32%) of the patients with interstitial pneumonia. Thus 74% of the patients with alveolar and 62% with interstitial pneumonia had bacterial infection, either alone or as a mixed viral-bacterial infection. Our results suggest that the presence of an alveolar infiltrate in a chest radiograph is a specific but insensitive indicator of bacterial pneumonia. We conclude that patients with alveolar pneumonia should be treated with antibiotics. In patients with interstitial pneumonia, however, both viral and bacterial aetiology are possible. In those, the decision concerning antibiotic treatment should be based on clinical and laboratory findings. PMID:8318803

  4. 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 ...

  5. Acute Pneumonia.

    PubMed

    Arshad, Hammad; Fasanya, Adebayo; Cheema, Tariq; Singh, Anil C

    2016-01-01

    Acute pneumonia is an active infection of the lungs that results when an individual at risk gets exposed to a particular microbiological pathogen. Acute pneumonia is the leading cause of death in the United States that is attributable to an infection. The risk factors, pathogenesis, and microbiological organisms involved differ if the pneumonia develops in the community versus health care-associated environment. The development of concise and comprehensive guidelines has led to an improvement in the management of the problem. However, the emergence of multidrug-resistant organisms and the increase in the percentage of elderly population keep mortality risk very substantial. PMID:26919676

  6. Clinical characteristics of idiopathic pulmonary fibrosis patients according to their smoking status

    PubMed Central

    Nagano, Hiroaki; Nei, Yuichiro; Yamashiro, Shin

    2016-01-01

    Background Idiopathic pulmonary fibrosis (IPF) is the most common form of idiopathic interstitial pneumonias (IIPs) of unknown etiology that often affects male, elderly smokers. However, it is sometimes observed in never smokers. This study aimed to clarify the clinical characteristics of IPF in never-smoking patients compared with those in smoking patients. Methods We retrospectively reviewed medical records, pulmonary function tests, and chest high-resolution computed tomography (HRCT) scan of never-smoking and smoking IPF patients from July 1, 2008 to June 30, 2013 at our hospital. Results We identified 32 never-smoking IPF patients and 66 smoking IPF patients. Never-smoking IPF patients developed more acute exacerbation (AE) than smoking IPF patients (50% vs. 18.2%, P<0.0001). The strongest predictor of AE in never-smoking IPF was modified Medical Research Council (mMRC) breathlessness scale [Hazards ratio (HR), 2.84, P=0.006]. The median survival time of never-smoking and smoking were 18.5 (0.1–138) and 26.3 (0.1–98.4) months, P<0.0001, respectively. The Cox proportional hazard model showed that 1-year mMRC breathlessness scale (HR, 3.24, P=0.001) and gender, age, and physiology (GAP) score (HR, 1.59, P=0.029) were strong predictors of mortality in never-smoking IPF patients at our hospital. Conclusions In conclusion, never-smoking IPF patients developed AE more often and showed poor prognosis compared with smoking IPF patients. The 1-year mMRC breathlessness scale was an important predictor of mortality at our hospital. PMID:27293827

  7. 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.

  8. Viral pneumonia

    MedlinePlus

    More serious infections can result in respiratory failure, liver failure, and heart failure. Sometimes, bacterial infections occur during or just after viral pneumonia, which may lead to more serious forms ...

  9. 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 ...

  10. Renal involvement in idiopathic hypereosinophic syndrome

    PubMed Central

    Shehwaro, Nathalie; Langlois, Anne Lyse; Gueutin, Victor; Izzedine, Hassane

    2013-01-01

    The hypereosinophilic syndromes (HESs) are a group of disorders marked by the sustained overproduction of eosinophils, in which eosinophilic infiltration and mediator release cause damage to multiple organs. In idiopathic HES, the underlying cause of hypereosinophilia (HE) remains unknown despite thorough aetiological work-up. Kidney disease is thought to be rare in HES. Renal manifestations described include eosinophilic interstitial nephritis, various types of glomerulopathies, thrombotic microangiopathy (TMA) and electrolyte disturbances. The diagnosis must be made in time, because a recovery of renal function can be obtained if treatment is initiated promptly. PMID:26064485

  11. 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.

  12. [The influence of intraoperative oxygen inhalation on patients with idiopathic pulmonary fibrosis].

    PubMed

    Sekine, Yasuo; Ko, Eitetsu

    2011-03-01

    Idiopathic pulmonary fibrosis (IPF) is a high risk factor for acute exacerbation of interstitial pneumonia (IP) after pulmonary resection. Other risk factors for inducing IP exacerbation are thought to be intraoperative inhalation of high concentration of oxygen, high pressure mechanical ventilation, major thoracic surgery, massive blood transfusion and preoperative chemotherapy and irradiation. The prophylactic strategy for this phenomenon has not been established, although mechanical ventilation by low pressure and low oxygen concentration, minimum invasive surgery and prophylactic administration of steroid, ulinastatin and sivelestat sodium hydride are performed. Acute exacerbation of IP is the same concept with acute lung injury (ALI)/acute respiratory distress syndrome (ARDS). This pulmonary injury is closely associated with reactive oxygen species (ROS). In particular, high concentration of oxygen induces excessive production of ROS. ROS stimulates alveolar macrophages and neutrophils to release inflammatory cytokines, such as TNF-alpha, IL-8, IFN-gamma, IL-6 and IL-1beta. These cytokines injure pulmonary endothelium and alveolus, and atelectasis, pulmonary hemorrhage, lung edema, hyalinization and alveolar thickness occur, and this is a manifestation of ALL Therefore, although there is no evidence, high pressure ventilation and inhalation of high oxygen concentration during anesthesia should be avoided. PMID:21485100

  13. 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. ...

  14. Idiopathic pulmonary hemosiderosis complicated by Down syndrome.

    PubMed

    Watanabe, Hirofumi; Ayusawa, Mamoru; Kato, Masataka; Chou, Ami; Komori, Akiko; Abe, Yuriko; Matsumura, Masaharu; Kamiyama, Hiroshi; Izumi, Hiroyuki; Takahashi, Shori

    2015-10-01

    We report the case of a 9-year-old girl with Down syndrome (DS) diagnosed with idiopathic pulmonary hemosiderosis (IPH). Although acute pneumonia complicated by hemolytic anemia was suspected, IPH was finally diagnosed on bronchoscopy. Treatment with prednisolone achieved good clinical response. An association between IPH and DS was not able to be identified, but immunological issues in DS may contribute to the onset of IPH. Recurrent and intractable respiratory symptoms with marked infiltrative shadows in the bilateral lungs and complicated by severe anemia in patients with DS should suggest IPH. PMID:26508184

  15. 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

  16. The use of auto-antibody testing in the evaluation of interstitial lung disease (ILD) - A practical approach for the pulmonologist.

    PubMed

    Bahmer, Thomas; Romagnoli, Micaela; Girelli, Francesco; Claussen, Martin; Rabe, Klaus F

    2016-04-01

    Interstitial lung diseases (ILD), also defined as diffuse parenchymal lung diseases (DPLD) include a heterogeneous group of pulmonary disorders. They may be caused by an underlying connective tissue disease (CTD), Rheumatoid Arthritis (RA) or ANCA-associated Vasculitis (AAV). Pulmonary manifestations of these conditions may also precede systemic onset and therefore, pulmonologists may be confronted with diagnosing a systemic rheumatic disease. For the discrimination of CTD-related ILD and idiopathic interstitial pneumonia (IIP), serological testing is recommended. After careful reviewing the available literature, we suggest a serologic diagnostic algorithm for pulmonologists dealing with ILD-patients. This algorithm depicts the consensus for antibody testing that was reached amongst authors. Obviously this consensus approach requires further validation in everyday practice and leaves room for local adaption of the diagnostic strategy depending on the availability of diagnostic capacity and cost. It is our hope, however, that the rational and stepwise approach of serological testing for ILD will ultimately save unnecessary expenses associated with general laboratory screening. Finally a broader consensus on the strategy for laboratory testing in ILD in general might also improve the detection level of these relatively rare diseases and this will ultimately improve management and care of patients suffering from these complex disorders. PMID:26921132

  17. Chronic eosinophilic pneumonia.

    PubMed Central

    Fox, B; Seed, W A

    1980-01-01

    We described three cases of eosinophilic pneumonia of unknown aetiology investigated clinically and by lung biopsy. The illnesses lasted between six and 20 weeks and consisted of cough, dyspnoea, malaise, and in two cases prolonged pyrexia. All had blood eosinophilia and chest radiographs showing widespread bilateral shadowing; in two cases this had a characteristic peripheral distribution. One patient recovered spontaneously and the other two responded to steroids, with disappearance of pyrexia within 12 hours and radiological clearing within 14 days. Lung function tests during the acute illness showed volume restriction or gas transfer defects or both in two cases. After remission all three showed abnormalities if small airways function. Lung biopsies performed during the acute illness were examined histologically and by transmission electron microscopy, and in two cases by immunofluorescence. There was both intra-alveolar and interstitial eosinophilic pneumonia with bronchiolitis obliterans, microgranulomata, and a vasculitis. Electron microscopy showed numerous eosinophils, many degranulated, and macrophages with phagocytosed eosinophilic granules and intracytoplasmic inclusions. In one case IgM, IgG, and IgA were demonstrated in the bronchial walls and interstitium. No IgE or complement was present. We believe that eosinophil granules are responsible for the tissue damage and fever and suggest mechanisms for this and for the response to steroid therapy. Images PMID:7003796

  18. Meningococcal pneumonia.

    PubMed

    Vossen, Matthias; Mitteregger, Dieter; Steininger, Christoph

    2016-08-17

    Neisseria meningitidis remains the most important cause of bacterial meningitis worldwide, particularly in children and young adults. The second most common and a potentially severe end-organ manifestation of invasive meningococcal disease (excluding systemic sepsis) is meningococcal pneumonia. It occurs in between 5% and 15% of all patients with invasive meningococcal disease and is thus the second most common non-systemic end-organ manifestation. To establish the diagnosis requires a high level of clinical awareness - the incidence is therefore very likely underreported and underestimated. This review of 344 meningococcal pneumonia cases reported in the Americas, Europe, Australia, and Asia between 1906 and 2015 presents risk factors, pathogenesis, clinical manifestations, diagnostic approaches, treatment, and prognosis of meningococcal pneumonia. PMID:27443594

  19. Lipoid pneumonia: an uncommon entity.

    PubMed

    Khilnani, G C; Hadda, V

    2009-10-01

    Lipoid pneumonia is a rare form of pneumonia caused by inhalation or aspiration of fat-containing substances like petroleum jelly, mineral oils, certain laxatives, etc. It usually presents as an insidious onset, chronic respiratory illness simulating interstitial lung diseases. Rarely, it may present as an acute respiratory illness, especially when the exposure to fatty substance(s) is massive. Radiological findings are diverse and can mimic many other diseases including carcinoma, acute or chronic pneumonia, ARDS, or a localized granuloma. Pathologically it is a chronic foreign body reaction characterized by lipid-laden macrophages. Diagnosis of this disease is often missed as it is usually not considered in the differential diagnoses of community-acquired pneumonia; it requires a high degree of suspicion. In suspected cases, diagnosis may be confirmed by demonstrating the presence of lipid-laden macrophages in sputum, bronchoalveolar lavage fluid, or fine needle aspiration cytology/biopsy from the lung lesion. Treatment of this illness is poorly defined and constitutes supportive therapy, repeated bronchoalveolar lavage, and corticosteroids. PMID:19901490

  20. Statin Use Is Associated with Reduced Mortality in Patients with Interstitial Lung Disease

    PubMed Central

    2015-01-01

    Introduction We hypothesized that statin use begun before the diagnosis of interstitial lung disease is associated with reduced mortality. Methods We studied all patients diagnosed with interstitial lung disease in the entire Danish population from 1995 through 2009, comparing statin use versus no statin use in a nested 1:2 matched study. Results The cumulative survival as a function of follow-up time from the date of diagnosis of interstitial lung disease (n = 1,786+3,572) and idiopathic lung fibrosis (n = 261+522) was higher for statin users versus never users (log-rank: P = 7·10−9 and P = 0.05). The median survival time in patients with interstitial lung disease was 3.3 years in statin users and 2.1 years in never users. Corresponding values in patients with idiopathic lung fibrosis were 3.4 versus 2.4 years. After multivariable adjustment, the hazard ratio for all-cause mortality for statin users versus never users was 0.73 (95% confidence interval, 0.68 to 0.79) in patients with interstitial lung disease and 0.76 (0.62 to 0.93) in patients with idiopathic lung fibrosis. Results were robust in all sensitivity analyses. Conclusion Among patients with interstitial lung disease statin use was associated with reduced all-cause mortality. PMID:26473476

  1. Idiopathic headshaking: is it still idiopathic?

    PubMed

    Pickles, Kirstie; Madigan, John; Aleman, Monica

    2014-07-01

    The clinical syndrome of equine idiopathic headshaking (HSK) was first described in the veterinary literature over 100 years ago, and the disorder continues to be a cause of substantial distress for the horse, frustration for the owner and therapeutic challenge for the veterinarian. This review presents a summary of the current knowledge of clinical signs, signalment, aetiopathogenesis, anatomy, diagnosis and treatment of idiopathic HSK. Recent advances in understanding the pathogenesis of the disease will be discussed with reference to human trigeminal neuralgia, along with the implications this may have for potential therapies. PMID:24821361

  2. latrogenic lipoid pneumonia in an adult horse.

    PubMed

    Metcalfe, Lucy; Cummins, Carolyn; Maischberger, Eva; Katz, Lisa

    2010-01-01

    A 20-year-old gelding presented with a history of acute respiratory distress which began immediately after administration of a mineral oil and water mix, via nasogastric intubation, for treatment of suspected gastrointestinal dysfunction. An initial presumptive diagnosis of acute lipoid pneumonia was made; this was further supported by evidence of arterial hypoxaemia and oxygen desaturation on arterial blood gas analysis, ultrasonographic signs of bilateral ventral lung consolidation and a mixed bronchoalveolar-interstitial lung pattern seen on thoracic radiographs. Despite intensive supportive therapy the horse's condition continued to deteriorate and the decision was made for humane euthanasia. Gross necropsy findings supported the clinical diagnosis of lipoid pneumonia. PMID:21851746

  3. [Aspiration pneumonia].

    PubMed

    Almirall, Jordi; Cabré, Mateu; Clavé, Pere

    2007-09-29

    The incidence and the prevalence of aspiration pneumonia (AP) in the community is poorly defined. It increases in direct relation with age and underlying diseases. The pathogenesis of AP presumes the contribution of risk factors that alter swallowing funtion and predispose the orofaringe and gastric region to bacterial colonization. The microbial etiology of AP involves Staphylococcus aureus, Haemophilus influenzae and Streptococcus pneumoniae for community-acquired aspiration pneumonia and Gram-negative aerobic bacilli in nosocomial pneumonia. It is worth bearing in mind the relative unimportance of anaerobic bacterias in AP. When we choose the empirical antibiotic treatmentant we have to consider some pathogens identified in orofaríngea flora. Empirical treatment with antianaerobics should only be used in certain patients. Videofluoroscopic swallowing studies should be used to determine the nature and extent of any swallow disorder and to rule out silent aspiration. Assessment of swallowing disorders is cost-effective and results in a significant reduction in overall morbidity and mortality. PMID:17927938

  4. CMV pneumonia

    MedlinePlus

    ... help prevent CMV pneumonia in certain people: Using organ transplant donors who don't have CMV Using CMV-negative blood products for transfusion Using CMV-immune globulin in certain ... that can occur in people who have a weakened immune system.

  5. 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 ...

  6. 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

  7. [Idiopathic pulmonary trunk aneurysm].

    PubMed

    Uehara, Mayuko; Kuroda, Yosuke; Ohori, Syunsuke; Mawatari, Toru; Morishita, Kiyofumi

    2010-07-01

    Pulmonary trunk aneurysm is generally associated with congenital cardiac defects, pulmonary hypertension, or infection. Idiopathic pulmonary trunk aneurysm without any associated diseases is a rare lesion and has seldom been reported. Here, we report a case of a 68-year-old woman with idiopathic pulmonary trunk aneurysm. The maximum diameter of the aneurysm was 53 mm while she was 142 cm in height. We successfully performed aneurysmorrhaphy and her postoperative course was uneventful. Aneurysmorrhaphy was an effective technique for idiopathic pulmonary trunk aneurysm without pulmonary hypertention. PMID:20662238

  8. [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

  9. 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' ...

  10. 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 ... illness . This means the body attacks and destroys healthy body ...

  11. Idiopathic cardiomegaly in Africa.

    PubMed

    Ikeme, A C

    1976-01-01

    Idiopathic cardiomegaly is probably the commonest single diagnosis other than hypertension made in tropical and subtropical African cardiovascular practice. Understanding of the nature of this disease has been hampered by failure to recognize the possibility that the term "idiopathic cardiomegaly" may embrace several disease entities. Evidence suggests that many factors, sometimes acting singly, but often acting in combination, may be responsible for the genesis of so-called idiopathic myocardial failure. The future attitude to research should not be one of excluding well-defined forms from the concept of idiopathic cardiomegaly, but one of clinicopathological classification, which should be a prelude to the search, within each moiety of this group of disorders, for a specific or dominant etiological factor. PMID:829042

  12. Idiopathic Scrotal Calcinosis.

    PubMed

    Killedar, Madhura Milind; Shivani, Aslam A; Shinde, Usha

    2016-08-01

    Idiopathic scrotal calcinosis (ISC) is a rare benign condition which presents with multiple, asymptomatic, and painless nodules on the scrotal skin wall. The lesions have been attributed as sebaceous cysts, calcified steatocystoma, fibroma, atheroma, and xanthoma. Shapiro et al. reviewed the histologic data and found no evidence of an epithelial lining, residual cysts, and lipid or organisms, and concluded that the calcification was idiopathic introducing the term "idiopathic scrotal calcinosis." We have studied four cases of idiopathic scrotal calcinosis, one of which had scrotal calcinosis involving the whole of the scrotum. He presented with painless multiple nodules over the scrotum. He was subjected for surgery with SOS skin grafting, but as the scrotal skin is so lax, primary closure is easily possible. In all our four cases, primary closure was easily possible. PMID:27574356

  13. Idiopathic calcified myocardial mass

    PubMed Central

    Patterson, David; Gibson, Derek; Gomes, Ricardo; McDonald, Lawson; Olsen, Eckhardt; Parker, John; Ross, Donald

    1974-01-01

    Patterson, D., Gibson, D., Gomes, R., McDonald, L., Olsen, E., Parker, J., and Ross, D. (1974).Thorax,29, 589-594. Idiopathic calcified myocardial mass. Myocardial calcification can be subdivided into three groups—metastatic, dystrophic or an extension inwards from the pericardium. This case in which the calcified myocardial mass was initially delineated by radiography and by echocardiography and subsequently removed does not fit into any subdivision and has been termed idiopathic. Images PMID:4279467

  14. 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 ...

  15. 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, ...

  16. 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 ...

  17. Investigating significant health trends in idiopathic pulmonary fibrosis (INSIGHTS-IPF): rationale, aims and design of a nationwide prospective registry

    PubMed Central

    Behr, Juergen; Hoeper, Marius M; Kreuter, Michael; Klotsche, Jens; Wirtz, Hubert; Pittrow, David

    2014-01-01

    Background Guidelines on the diagnosis and management of idiopathic pulmonary fibrosis (IPF), a rare manifestation of chronic progressive fibrosing interstitial pneumonia, have been updated by ATS/ERS/JRS/ALAT in 2011. In Europe, data are limited on the characteristics and management of such patients. Methods/design Investigating significant health trends (INSIGHTS)-IPF is a prospective observational longitudinal registry designed to describe the characteristics and management of newly diagnosed (incident) and prevalent patients with IPF on the long term. The registry uses a non-probability sampling approach to collect data on characteristics, therapeutic interventions, health-related quality of life and health economic parameters. At least 500 patients in ambulatory care will be included consecutively in about 30 centres. The study has been initiated in November 2012, and currently (December 2013) follows 344 patients. ClinTrials.gov identifier is NCT01695408. Discussion INSIGHTS-IPF documents one of the largest IPF cohorts in Europe. The registry is expected to provide much-needed data on the characteristics and management situation of patients with IPF in Germany. It will allow comparisons with other countries. Gap analyses based on current guidelines for management of these patients will be possible. PMID:25478169

  18. Idiopathic pulmonary fibrosis: the role of the pharmacy benefit manager in providing access to effective, high-value care.

    PubMed

    Morrow, Thomas J

    2015-10-01

    Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing interstitial pneumonia of unknown cause that primarily affects individuals aged 60 and older. The economic costs of the disease are significant, with patients twice as likely to be hospitalized and twice as likely to require outpatient medical care as compared with those without IPF, resulting in an additional annual cost to the Medicare system of $1 billion. The first pharmacologic treatments for IPF, nintedanib and pirfenidone, were approved in 2014 for conditional use. Their use is expected to significantly increase the cost of care for this population, given that patients will likely continue to take the medication until their death. The use of these medications requires that payers implement innovative opportunities to manage their utilization and cost, as well as other medical costs related to the disease. Pharmacy benefit managers have an important role to play in managing the cost and appropriate utilization of these new treatments through disease management programs, negotiated discounts and rebates, improved adherence to treatment recommendations, and benefit design to optimize patient care. PMID:26788616

  19. Acute eosinophilic pneumonia as a complication of influenza A (H1N1) pulmonary infection.

    PubMed

    Larranaga, Jose Maria; Marcos, Pedro J; Pombo, Francisco; Otero-Gonzalez, Isabel

    2016-01-01

    Acute eosinophilic pneumonia (AEP) is a rare disease characterized by its acute onset and a clinical presentation simulating a bacterial pneumonia. Although it can be idiopathic, it has been described related to drugs, toxic agents and infections, mostly parasitic. We describe the case of influenza A (H1N1) severe pneumonia complicated by an acute eosinophilic pneumonia. Patient presented with respiratory failure and diffuse ground-glass opacities at chest-computed tomography. Clinical suspicion for this complication and bronchoalveolar lavage with cellular count analysis is crucial. PMID:27055842

  20. 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 ...

  1. Healthcare-associated Pneumonia and Aspiration Pneumonia

    PubMed Central

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

    2015-01-01

    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. PMID:25657850

  2. Interstitial Lung Disease due to Siderosis in a Lathe Machine Worker.

    PubMed

    Gothi, D; Satija, B; Kumar, S; Kaur, Omkar

    2015-01-01

    Since its first description in 1936, siderosis of lung has been considered a benign pneumoconiosis due to absence of significant clinical symptoms or respiratory impairment. Subsequently, authors have questioned the non-fibrogenic property of iron. However, siderosis causing interstitial lung disease with usual interstitial pneumonia (UIP) pattern has not been described in the past. We report a case of UIP on high resolution computed tomography, proven to be siderosis on transbronchial lung biopsy in a lathe machine worker. PMID:26410982

  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. Successful alectinib treatment after crizotinib‐induced interstitial lung disease

    PubMed Central

    Fujita, Yuka; Sasaki, Takaaki; Ohsaki, Yoshinobu

    2016-01-01

    Abstract 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

  5. A Newfoundland cohort of familial and sporadic idiopathic pulmonary fibrosis patients: clinical and genetic features

    PubMed Central

    2012-01-01

    Background Idiopathic pulmonary fibrosis (IPF) is an adult-onset Idiopathic Interstitial Pneumonia (IIP) usually diagnosed between age 50 to 70 years. Individuals with Familial Pulmonary Fibrosis (FPF) have at least one affected first or second-degree relative and account for 0.5-20% of cases. Methods We ascertained and collected DNA samples from a large population-based cohort of IPF patients from Newfoundland, Canada. For each proband, a family history was documented and medical records were reviewed. Each proband was classified as familial (28 patients) or sporadic (50 patients) and all 78 probands were screened for variants in four highly penetrant, adult-onset PF genes (SFTPC, SFTPA2, TERT,TERC). Results Seventy-eight IPF probands were enrolled of whom 28 (35.9%) had a positive family history. These 28 familial patients led to the recruitment of an additional 49 affected relatives (total of 77 FPF patients). By age 60 years, 42% of the familial cohort had been diagnosed with PF compared with only 16% of the sporadic patient collection (χ2 = 8.77, p = 0.003). Mean age of diagnosis in the familial group was significantly younger than the sporadic group (61.4 years vs. 66.6 yrs, p = 0.012) with a wider age range of diagnosis (19–92 years compared with 47–82 years). Thirty-three of 77 (42.8%) FPF patients had a tissue diagnosis and all but five had usual interstitial pneumonia histology. Compared with other published case series, the familial IIP histologies were more homogeneous. Three of 28 familial probands (10.7%) and none of the 50 sporadic probands had pathogenic variants in the four genes tested. All three familial probands had mutations in TERT. Other phenotypes associated with telomerase deficiency were present in these families including cirrhosis, bone marrow hypoplasia and premature graying. Telomere length assays were performed on mutation carriers from two families and confirmed telomere-related deficiency. Conclusion The

  6. AB017. Interstitial lung disease patient diagnostic journey (intensity)

    PubMed Central

    Lederer, David J.; Bianchi, Pauline; Loboda, Jeanne; Danese, Sherry; Cosgrove, Gregory P.

    2016-01-01

    Background Interstitial lung disease (ILD) is complicated to diagnose because its signs and symptoms mimic a wide range of common medical conditions. The objective of the INTENSITY survey was to advance the understanding of respondents’ diagnostic experiences with ILDs, including idiopathic pulmonary fibrosis (IPF). The survey was designed to trace the path that respondents took to diagnosis, identify obstacles they faced, and gauge the emotional impact of their journey. Results from the INTENSITY survey will help us more fully understand respondents’ diagnostic experiences so we can further our efforts to improve timely diagnosis and treatment of ILDs. Methods We administered a 25-minute quantitative online survey from August 14–26, 2015. Of the 1,152 respondents screened to ensure they were US residents, and had been diagnosed with an interstitial lung disease, 600 met criteria and completed the survey. Recruitment was accomplished through two channels: email invitations sent to 16,000 Pulmonary Fibrosis Foundation (PFF) members and an open invitation to participate through the pulmonary fibrosis community website Results Shortness of breath (77%) and cough (53%) were the most common early symptoms in ILD patients. Twenty-five percent of respondents saw their primary care physician once before being referred to a specialist, however 61% of respondents saw their primary care physician >2 times before referral to a pulmonologist. Misdiagnosis occurred in 55% of respondents. The median time from symptom onset to correct diagnosis was 11 months, however 49% of respondents carried an incorrect diagnosis for 1 to 10 years. Thirty-eight percent were misdiagnosed more than twice, most frequently with asthma, pneumonia, bronchitis, and allergies. An accurate diagnosis was achieved after a median of 6 months and a median of 3 physician visits. The majority underwent an invasive diagnostic procedure. Fourteen percent of respondents saw more than 6 physicians before

  7. Pulmonary interstitial emphysema.

    PubMed Central

    Greenough, A; Dixon, A K; Roberton, N R

    1984-01-01

    Forty one of 210 preterm infants ventilated for respiratory distress syndrome in a three year period had radiological evidence of pulmonary interstitial emphysema. The development of this condition was significantly associated with malpositioning of the endotracheal tube in a main bronchus and the use of high peak pressure ventilation. Pulmonary interstitial emphysema was associated with a significant increase in the number of pneumothoraces, intraventricular haemorrhages, and the need for prolonged respiratory support, but did not increase mortality. Although in 12 infants in whom fast rate ventilation was used there was a significant reduction in the number of pneumothoraces, outcome was not altered in any other way. Fast rate ventilation may be of greater benefit if initiated before the development of pulmonary interstitial emphysema. Images Fig. 1 Fig. 2 Fig. 3 Fig. 4 PMID:6508339

  8. Relevance analysis of clinical and lung function parameters changing and prognosis of idiopathic pulmonary fibrosis

    PubMed Central

    Li, Xueren; Peng, Shouchun; Wei, Luqing; Li, Zhenhua

    2014-01-01

    Objective: Idiopathic pulmonary fibrosis (IPF) is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia with unknown cause. We analyzed the changed rate of pulmonary function and arterial blood gas in IPF patients, and evaluated their influence of changed rate to IPF prognosis. Methods: 81 patients with IPF were recruited successfully, they were followed-up at 6 and 12 months. Dyspnea score and respiratory assessment parameters including FVC, FEV1, TLC, SaO2, PA-aO2, and DLCO were evaluated at their 6 and 12 months follow-up. The changed value and changed rate of above parameters were calculated, and their treatment effects were divided into 3 subgroup: improved, stable and deteriorated group. Statistical analysis was performed between groups for survival and hazards regression analysis. Results: 55 of 81 patients were follow-up at 12 months. Dyspnea score and its changed rate, the changed value of FEV1%, FVC%, TLC%, DLCO%, and PaO2, SaO2, PA-aO2 were prognosis effect factors in IPF patients in 6 and 12 months group. The survival analysis of dyspnea scores, FVC%, TLC%, DLCO%, PaO2, SaO2 and PA-aO2 at K-M were all statistical significant (P < 0.05) in improved, stable and deteriorated group. Conclusion: FVC% changed rate, dyspnea score changed rate and PaO2 changed rate were IPF patient prognosis associated factors in 6 months group; and FVC% changed rate, DLCO% changed rate and TLC% changed rate were prognosis associated factors for IPF patient in 12 months group. PMID:25663972

  9. Not your typical pneumonia: a case of exogenous lipoid pneumonia.

    PubMed

    Simmons, Ashley; Rouf, Emran; Whittle, Jeff

    2007-11-01

    The constellation of chronic cough, dyspnea, and hemoptysis can include a broad range of differential diagnoses. Although uncommon, exogenous lipoid pneumonia (ELP) should be considered when patients present with this symptom complex. We report a case of a 72-year-old female who presented with hemoptysis, cough, and dyspnea. The admission computed tomography scan of the chest revealed progressive interstitial infiltrates. Bronchoscopy revealed diffuse erythema without bleeding. Culture and cytology of lavage fluid were negative. Open-lung biopsy revealed numerous lipid-laden macrophages and multinucleated foreign-body giant cells. On further questioning, the patient admitted to the daily use of mineral oil for constipation. The diagnosis of ELP was made. The literature review revealed that many cases typically present with chronic cough with or without dyspnea. Our case illustrates an unusual presenting symptom of hemoptysis and the need to identify patients who can be at risk of developing this rare condition. PMID:17846847

  10. 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