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Sample records for corticosteroid-naive sarcoidosis patients

  1. Sarcoidosis

    MedlinePlus

    ... page from the NHLBI on Twitter. What Is Sarcoidosis? Español Sarcoidosis (sar-koy-DO-sis) is a ... Content: NEXT >> Featured Video Living With and Managing Sarcoidosis 05/18/2011 This video—presented by the ...

  2. Sarcoidosis

    MedlinePlus

    ... Favorite Name: Category: Share: Yes No, Keep Private Sarcoidosis Share | Sarcoidosis is characterized by granulomas (a mass of red, ... kidney), and endocrine systems. Signs and symptoms of sarcoidosis are variable because of its ability to affect ...

  3. Subcutaneous sarcoidosis: a clinical analysis of nine patients.

    PubMed

    Ando, Masaru; Miyazaki, Eishi; Hatano, Yutaka; Nishio, Suehiro; Torigoe, Chihiro; Yamasue, Mari; Mukai, Yutaka; Nureki, Shin-Ichi; Kadota, Jun-Ichi

    2016-09-01

    Subcutaneous sarcoidosis is the specific subset of cutaneous sarcoidosis frequently associated with systemic disease. However, the disease activity, severity, and prognosis have not yet been elucidated due to the limited number of reported cases. The purpose of this study was to identify the clinical, laboratory, and prognostic differences between subcutaneous sarcoidosis and other type of cutaneous sarcoidosis. All patients with sarcoidosis diagnosed histopathologically from 2000 to 2012 at our institution were enrolled. The clinical, laboratory, chest X-ray, and pulmonary function test results were retrospectively evaluated in the patients with cutaneous sarcoidosis. In the 130 patients with sarcoidosis, cutaneous sarcoidosis was diagnosed in 37 patients (28.4 %), and 9 (6.9 %) of these patients had subcutaneous sarcoidosis. The serum levels of soluble interleukin-2 receptor (sIL-2R) were significantly elevated in the group of patients with subcutaneous sarcoidosis in comparison to the patients with other types of cutaneous sarcoidosis, whereas there was no significant difference in the severity score between the two groups. Following a 2-year observation period, three patients were in remission, five patients demonstrated stable disease, and one patient had progressive disease. Subcutaneous sarcoidosis may be associated with the disease activity, although it was not found to be associated with the disease severity and it was not a predictive factor for the prognosis. Furthermore, the prevalence of subcutaneous sarcoidosis may be higher than that in previously reported series. PMID:27449952

  4. Sarcoidosis in Melanoma Patients: Case Report and Literature Review

    PubMed Central

    Beutler, Bryce D.; Cohen, Philip R.

    2015-01-01

    Sarcoidosis is a systemic inflammatory disease characterized by the development of noncaseating granulomas in multiple organ systems. Many hematologic malignancies and solid tumors, including melanoma, have been associated with sarcoidosis. We describe the clinical and pathologic findings of a 54-year-old man with melanoma-associated sarcoidosis. In addition, we not only review the literature describing characteristics of other melanoma patients with sarcoidosis, but also the features of melanoma patients with antineoplastic therapy-associated sarcoidosis. Sarcoidosis has been described in 80 melanoma patients; sufficient information for analysis was provided in 39 of these individuals. In 43.6% of individuals (17 out of 39), sarcoidosis was directly associated with melanoma; in 56.4% of oncologic patients (22 out of 39), sarcoidosis was induced by antineoplastic therapy that had been administered for the treatment of their metastatic melanoma. The discovery of melanoma preceded the development of sarcoidosis in 12 of the 17 (70.5%) individuals who did not receive systemic treatment. Pulmonary and/or cutaneous manifestations of sarcoidosis were common among both groups of patients. Most patients did not require treatment for sarcoidosis. Melanoma patients—either following antineoplastic therapy or without systemic treatment—may be at an increased risk to develop sarcoidosis. In antineoplastic therapy naive melanoma patients, a common etiologic factor—such as exposure to ultraviolet light—may play a role in their developing melanoma and sarcoidosis. PMID:26083934

  5. Sarcoidosis

    PubMed Central

    Nunes, Hilario; Bouvry, Diane; Soler, Paul; Valeyre, Dominique

    2007-01-01

    Sarcoidosis is a multisystemic disorder of unknown cause characterized by the formation of immune granulomas in involved organs. It is an ubiquitous disease with incidence (varying according to age, sex, race and geographic origin) estimated at around 16.5/100,000 in men and 19/100,000 in women. The lung and the lymphatic system are predominantly affected but virtually every organ may be involved. Other severe manifestations result from cardiac, neurological, ocular, kidney or laryngeal localizations. In most cases, sarcoidosis is revealed by persistent dry cough, eye or skin manifestations, peripheral lymph nodes, fatigue, weight loss, fever or night sweats, and erythema nodosum. Abnormal metabolism of vitamin D3 within granulomatous lesions and hypercalcemia are possible. Chest radiography is abnormal in about 90% of cases and shows lymphadenopathy and/or pulmonary infiltrates (without or with fibrosis), defining sarcoidosis stages from I to IV. The etiology remains unknown but the prevailing hypothesis is that various unidentified, likely poorly degradable antigens of either infectious or environmental origin could trigger an exaggerated immune reaction in genetically susceptible hosts. Diagnosis relies on compatible clinical and radiographic manifestations, evidence of non-caseating granulomas obtained by biopsy through tracheobronchial endoscopy or at other sites, and exclusion of all other granulomatous diseases. The evolution and severity of sarcoidosis are highly variable. Mortality is estimated at between 0.5–5%. In most benign cases (spontaneous resolution within 24–36 months), no treatment is required but a regular follow-up until recovery is necessary. In more serious cases, a medical treatment has to be prescribed either initially or at some point during follow-up according to clinical manifestations and their evolution. Systemic corticosteroids are the mainstay of treatment of sarcoidosis. The minimal duration of treatment is 12 months. Some

  6. Right Heart Involvement in Patients with Sarcoidosis.

    PubMed

    Patel, Mita B; Mor-Avi, Victor; Murtagh, Gillian; Bonham, Catherine A; Laffin, Luke J; Hogarth, Douglas Kyle; Medvedofsky, Diego; Lang, Roberto M; Patel, Amit R

    2016-05-01

    The left ventricle (LV) is affected in 20-25% of patients with sarcoidosis and its involvement is associated with morbidity and mortality. However, effects of sarcoidosis on the right ventricle (RV) are not well documented. Our aims were to investigate the prevalence of RV dysfunction in patients with sarcoidosis and determine whether it is predominantly associated with direct cardiac involvement, severity of lung disease, or pulmonary hypertension (PH). We identified 50 patients with biopsy-proven extra-cardiac sarcoidosis and preserved LV function, who underwent echocardiography, pulmonary function (PF) testing, and cardiovascular magnetic resonance. RV function was quantified by free wall longitudinal strain. Tricuspid valve Doppler and estimated right atrial pressure were used to estimate systolic pulmonary artery pressure. Myocardial late gadolinium enhancement was considered diagnostic for cardiac sarcoidosis and assumed to involve both ventricles. Of the 50 patients, 28 (56%) had RV dysfunction, 4 with poorly defined PF status. Of the remaining 24 patients, 16 (67%) had lung disease, 8 (33%) had PH, and 10 (42%) had LV involvement. Ten patients had greater than one of these findings, and 4 had all 3. In contrast, in 4/24 patients (17%), RV dysfunction could not be explained by these mechanisms, despite severely reduced RV strain. In conclusion, RV dysfunction is common in patients with sarcoidosis and is usually associated with either direct LV involvement, lung disease, or PH, but may occur in the absence of these mechanisms, suggesting the possibility of isolated RV involvement and underscoring the need for imaging protocols that would include RV strain analysis.

  7. Sarcoidosis

    MedlinePlus

    Sarcoidosis is a disease that leads to inflammation, usually in your lungs, skin, or lymph nodes. It starts as tiny, grain-like lumps, called granulomas. Sarcoidosis can affect any organ in your body. No ...

  8. Sarcoidosis and lymphoma in the same patient.

    PubMed Central

    Brennan, N. J.; Fennelly, J. J.; Towers, R. P.; FitzGerald, M. X.

    1983-01-01

    A case of lymphocytic lymphoma in a patient with coincidental sarcoidosis is described, and the possible relationship of the two conditions is discussed. A revised set of diagnostic criteria is proposed to overcome previous difficulties encountered in validating such dual pathology. Images Fig. 1 Fig. 2 PMID:6688876

  9. Sarcoidosis

    MedlinePlus

    ... the elbow Sarcoidosis on the nose and forehead Respiratory system References Judson MA, Morgenthau AS, Baughman RP. Sarcoidosis. In: Broaddus VC, Mason RJ, Ernst JD, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine . 6th ed. Philadelphia, PA: Elsevier Saunders; 2016: ...

  10. [Sarcoidosis].

    PubMed

    Frye, B C; Schupp, J C; Köhler, T C; Voll, R E; Müller-Quernheim, J

    2016-05-01

    Sarcoidosis is a rare granulomatous disease mainly affecting lymph nodes and the lungs but joints, bones, muscles and other organs can also be affected. Sarcoidosis therefore represents an important differential diagnosis to various rheumatic diseases. For the diagnosis and differential diagnostic clarification, bronchoscopy including endobronchial ultrasound-guided fine needle aspiration of mediastinal and hilar lymph nodes represent the main procedures. Because of the high spontaneous remission rate initiating a therapy requires a therapeutic goal defined by sarcoidosis-associated functional organ impairment, especially for acute sarcoidosis. Cortisone represents the most commonly administered medication whereas methotrexate and azathioprine are well-established second-line medications. Antibodies which neutralize tumor necrosis factors (TNF) are a potential third-line therapy. PMID:27146405

  11. Sarcoidosis.

    PubMed

    Sanchez, Miguel; Haimovic, Adele; Prystowsky, Steve

    2015-07-01

    Sarcoidosis is a disease characterized by noncaseating granulomatous infiltration of 1 or more organs. In North America, after the lungs and thoracic lymph nodes, the skin is the next most commonly involved organ. Data from multiple studies indicate a coaction between genetic and environmental factors in immunologically susceptible hosts. The disease's many clinical manifestations and course vary greatly and are influenced by race, ethnicity, and gender. In the skin, the lesions of sarcoidosis are classified as specific when noncaseating granulomas are present, and nonspecific when there is an inflammatory reaction pattern devoid of granulomas.

  12. Characteristics of patients presenting with erythema nodosum and sarcoidosis.

    PubMed

    O'Connor, T M; Cagney, D; Jahangir, A; Brady, A; Fitzgibbon, J; Lee, G; El-Gammal, A; Brennan, N J

    2009-06-01

    We explored the relationship between erythema nodosum (EN) and sex, age, serum angiotensin converting enzyme (ACE), bronchoalveolar lavage lymphocytosis (BAL-I), interstitial granulomas and radiological stage in patients presenting with pulmonary sarcoidosis in Ireland. Sixty-nine patients diagnosed with sarcoidosis between 2003 and 2006 were studied. Forty one patients (59%) were male. Sixteen patients (23%) presented with EN. Forty one patients of 65 (63%) had transbronchial biopsies demonstrating non-caseating granulomas. Patients with sarcoidosis presenting with EN were more likely to be female (p=0.042), younger (p=0.012) and have earlier stage pulmonary disease (p=0.02). There were no correlations between serum ACE, interstitial granulomas and disease stage. BAL-I did however predict increasing disease radiological stage (p=0.042). In this study, one quarter of patients with sarcoidosis presented with EN among their presenting features. These patients were more likely to be young females with early stage radiological disease.

  13. Propionibacterium acnes isolated from lymph nodes of patients with sarcoidosis.

    PubMed

    de Brouwer, Bart; Veltkamp, Marcel; Wauters, Carla A; Grutters, Jan C; Janssen, Rob

    2015-01-01

    Propionibacterium acnes has been repeatedly suggested as a candidate causative agent of sarcoidosis. It is the only microorganism that has been isolated from sarcoid lesions by bacterial culture so far and this has been described in Japanese patients only. We report two non-Japanese patients in whom mediastinoscopy was performed in order to obtain lymph node tissue for histopathology, which was suggestive for sarcoidosis. Bacterial culture of these uncontaminated mediastinal lymph nodes revealed P. acnes in both patients. As shown in these two cases, P. acnes can be isolated from sterile biopsied sarcoid lymph nodes of non-Japanese patients and supports the belief that there is an etiologic link between P. acnes sarcoidosis. Further elucidation could provide an opening to novel strategies using antibiotics for treating sarcoidosis.

  14. Propionibacterium acnes isolated from lymph nodes of patients with sarcoidosis.

    PubMed

    de Brouwer, Bart; Veltkamp, Marcel; Wauters, Carla A; Grutters, Jan C; Janssen, Rob

    2015-01-01

    Propionibacterium acnes has been repeatedly suggested as a candidate causative agent of sarcoidosis. It is the only microorganism that has been isolated from sarcoid lesions by bacterial culture so far and this has been described in Japanese patients only. We report two non-Japanese patients in whom mediastinoscopy was performed in order to obtain lymph node tissue for histopathology, which was suggestive for sarcoidosis. Bacterial culture of these uncontaminated mediastinal lymph nodes revealed P. acnes in both patients. As shown in these two cases, P. acnes can be isolated from sterile biopsied sarcoid lymph nodes of non-Japanese patients and supports the belief that there is an etiologic link between P. acnes sarcoidosis. Further elucidation could provide an opening to novel strategies using antibiotics for treating sarcoidosis. PMID:26422574

  15. Tracheo-oesophageal fistula in a patient with chronic sarcoidosis.

    PubMed

    Darr, A; Mohamed, S; Eaton, D; Kalkat, M S

    2015-10-01

    Sarcoidosis is a common multisystem granulomatous condition of unknown aetiology, predominantly involving the respiratory system. Tracheal stenosis has been described but we believe that we present the first case of a tracheo-oesophageal fistula secondary to chronic sarcoidosis. A 57-year-old woman with sarcoidosis, a known tracheal stricture and a Polyflex(®) stent in situ presented with stridor. Bronchoscopy confirmed in-stent stenosis, by exuberant granulation tissue. The stent was removed and the granulation tissue was resected accordingly. Postoperatively, the patient was noticed to have an incessant cough and video fluoroscopy raised the suspicion of a tracheo-oesophageal fistula. A repeat bronchoscopy demonstrated marked granulation tissue, accompanied by a fistulous connection with the oesophagus at the mid-lower [middle of the lower] third of the trachea. Three Polyflex(®) stents were sited across the entire length of the trachea. Sarcoidosis presents with varying clinical manifestations and disease progression. Tracheal involvement appears to be a rare phenomenon and usually results in stenosis. To date, there has been little or no documented literature describing the formation of a tracheo-oesophageal fistula resulting from sarcoidosis. Early reports documented the presence of sarcoidosis induced weakening in the tracheal wall, a process termed tracheal dystonia. Weaknesses are more apparent in the membranous aspect of the trachea. Despite the rare nature of such pathology, this case report highlights the need to consider the presence of a tracheo-oesophageal fistula in sarcoidosis patients presenting with repeat aspiration in the absence of an alternate pathology.

  16. Multifactorial Etiology Pulmonary Hypertension in a Patient with Sarcoidosis

    PubMed Central

    Lucas Vinícius da Fonseca, Barreto; Felipe Naze Rodrigues, Cavalcante; Joselina Luzia Menezes, Oliveira; Marcos Antônio, Almeida-Santos; José Augusto Soares, Barreto-Filho; Antônio Carlos Sobral, Sousa

    2016-01-01

    Differential diagnosis between pre- and postcapillary pulmonary hypertension (PH) in patients with diastolic heart failure (DHF) is a challenge in clinical practice. The presence of PH is implicated in worse prognosis in patients with this disease. This case report approaches the process of investigation of pulmonary hypertension in adult patient with DHF, double mitral lesion, and sarcoidosis with poor clinical outcome. PMID:27800190

  17. Psoriasiform Sarcoidosis

    PubMed Central

    Vega, Miriam L.; Abrahams, Jennifer

    2016-01-01

    Background: Psoriasiform lesions are an established, but rare, manifestation of sarcoidosis. Only 0.9 percent of patients with cutaneous sarcoidosis develop this form of the disease. Observation: The authors present a case of a 61-year-old woman with a history of pulmonary sarcoidosis who presented to their dermatology clinic with thick plaques resembling psoriasis. Biopsy of one of the lesions revealed sarcoidal granulomas in association with psoriasiform changes. Conclusion: Psoriasiform lesions are a rare manifestation of sarcoidosis. The authors theorize that the co-expression of TNF-α in both entities is a possible explanation of the psoriasiform expression of sarcoidosis. PMID:27462388

  18. Sarcoidosis occurring after lymphoma: report of 14 patients and review of the literature.

    PubMed

    London, Jonathan; Grados, Aurélie; Fermé, Christophe; Charmillon, Alexandre; Maurier, François; Deau, Bénédicte; Crickx, Etienne; Brice, Pauline; Chapelon-Abric, Catherine; Haioun, Corinne; Burroni, Barbara; Alifano, Marco; Le Jeunne, Claire; Guillevin, Loïc; Costedoat-Chalumeau, Nathalie; Schleinitz, Nicolas; Mouthon, Luc; Terrier, Benjamin

    2014-11-01

    Sarcoidosis is a granulomatous disease that most frequently affects the lungs with pulmonary infiltrates and/or bilateral hilar and mediastinal lymphadenopathy. An association of sarcoidosis and lymphoproliferative disease has previously been reported as the sarcoidosis-lymphoma syndrome. Although this syndrome is characterized by sarcoidosis preceding lymphoma, very few cases of sarcoidosis following lymphoma have been reported. We describe the clinical, biological, and radiological characteristics and outcome of 39 patients presenting with sarcoidosis following lymphoproliferative disease, including 14 previously unreported cases and 25 additional patients, after performing a literature review. Hodgkin lymphoma and non-Hodgkin lymphoma were equally represented. The median delay between lymphoma and sarcoidosis was 18 months. Only 16 patients (41%) required treatment. Sarcoidosis was of mild intensity or self-healing in most cases, and overall clinical response to sarcoidosis was excellent with complete clinical response in 91% of patients. Sarcoidosis was identified after a follow-up computerized tomography scan (CT-scan) or fluorodeoxyglucose-positron emission tomography/computerized tomography (FDG-PET/CT) evaluation in 18/34 patients (53%). Sarcoidosis is therefore a differential diagnosis to consider when lymphoma relapse is suspected on a CT-scan or FDG-PET/CT, emphasizing the necessity to rely on histological confirmation of lymphoma relapse. PMID:25380084

  19. Cutaneous Sarcoidosis Clinically Mimicking Necrobiosis Lipoidica in a Patient with Systemic Sarcoidosis

    PubMed Central

    Takahara, Masakazu; Nakahara, Takeshi; Fukagawa, Shuji; Takei, Kenjiro; Shono, Asuka; Kiryu, Hiromaro; Moroi, Yoichi; Furue, Masutaka

    2012-01-01

    A 70-year-old woman with an 8-year history of systemic sarcoidosis developed round, red-brown eruptions, with central atrophic lesions on her lower legs. The features of the biopsy specimen resembled those of necrobiosis lipoidica (NL), but although necrobiosis was present there were well-formed non-necrotizing granulomas in the dermis. The histological diagnosis was cutaneous sarcoidosis. Systemic sarcoidosis presenting with NL has rarely been reported. The histological features of cutaneous sarcoidosis sometimes mimic those of other granulomatous diseases, including NL and granuloma annulare, which are difficult to distinguish. We discuss the novel association between sarcoidosis and other granulomatous diseases. PMID:22363160

  20. Serum ACE Level in Sarcoidosis Patients with Typical and Atypical HRCT Manifestation

    PubMed Central

    Kahkouee, Shahram; Samadi, Katayoon; Alai, Ali; Abedini, Atefeh; Rezaiian, Lida

    2016-01-01

    Summary Background Sarcoidosis is an inflammatory disease that affects multiple organs. Before widespread use of computed tomography (CT), the severity of sarcoidosis was assessed based on chest X-ray abnormalities. HRCT can distinguish between active inflammatory changes and irreversible fibrosis. In this study, we analyzed different ACE levels in 148 patients diagnosed with sarcoidosis. Material/Methods We categorized these patients based on their HRCT results into four groups: 1) patients diagnosed with chronic disease; 2) patients diagnosed with non-chronic disease; 3) patients who exhibited typical HRCT changes; and 4) patients who exhibited atypical HRCT changes. Afterward the mean ACE level of each group was calculated and compared. Result The HRCT scans of chronic sarcoidosis patients tended to show more atypical sarcoidosis patterns. Moreover, there was a reverse correlation between chronicity and ACE level (P-value <0.05). Conclusions HRCT is another modality which would be useful when the diagnosis of sarcoidosis is not definite. PMID:27733890

  1. Radioaerosol lung clearance in patients with active pulmonary sarcoidosis

    SciTech Connect

    Jacobs, M.P.; Baughman, R.P.; Hughes, J.; Fernandez-Ulloa, M.

    1985-05-01

    Pulmonary radioaerosol clearance rate of /sup 99m/Tc diethylenetriamine pentacetate (DTPA) in 14 patients with untreated sarcoidosis was compared with /sup 67/Ga lung scan and increased lymphocytes in the bronchoalveolar lavage (BAL) fluid. Nine healthy nonsmoking subjects had a mean DTPA clearance rate of 1.18%/min (range, 0.54 to 1.60%/min). Eight of 14 patients with sarcoidosis had clearance rates greater than 1.60%/min. Of those 8 patients with abnormal DTPA clearance, 4 had positive gallium scans, 4 had more than 17% lymphocytes in the BAL fluid, and 3 had both tests positive. To study the cause of abnormal DTPA clearance, 23 subjects (including 3 normal controls, all 14 patients with sarcoidosis, and 6 patients with localized disease on chest roentgenogram) underwent both DTPA clearance studies and BAL for quantitation of the amount of albumin in lung fluid. There was a positive correlation between the rate of DTPA clearance and the albumin concentration in lung fluid (r = 0.87, p less than 0.01).

  2. Limited role of auxiliary endobronchial biopsy in the diagnosis of Japanese patients with sarcoidosis.

    PubMed

    Ishii, Hiroshi; Otani, Satoshi; Iwata, Atsuko; Oka, Hiroaki; Komiya, Kosaku; Yoshioka, Daisuke; Hashinaga, Kazuhiko; Kadota, Jun-ichi

    2011-01-01

    The diagnosis of sarcoidosis, a multisystem granulomatous disease of unknown etiology, is established when clinicoradiological findings are supported by histological evidence of non-caseating epithelioid cell granulomas. For pathological diagnosis, an endobronchial biopsy of normal-appearing bronchial mucosa in combination with transbronchial lung biopsy (TBLB) has been reported to be useful for sarcoidosis patients in Europe or the U.S. This is the first report assessing the utility of endobronchial biopsy for diagnosis of Japanese patients with sarcoidosis. Eighteen consecutive patients with strongly suspected sarcoidosis were evaluated by endobronchial biopsy of normal-appearing bronchial mucosa, together with TBLB and bronchoalveolar lavage. The TBLB specimens demonstrated non-caseating epithelioid cell granulomas in the lungs of 11 patients (61.1%), but not any specific findings in those of other 7 patients. In contrast, endobronchial biopsy specimens confirmed a diagnosis of sarcoidosis in only one patient that required steroid therapy for deterioration of pulmonary sarcoidosis. All 18 patients of this study, including 5 patients with pathological findings obtained from extrapulmonary sites, met the pathological or clinical diagnostic criteria. In conclusion, endobronchial biopsy of normal-appearing bronchial mucosa in combination with TBLB does not improve the diagnostic capacity for detecting sarcoidosis in Japanese patients, despite earlier reports. Thus, this method is of limited usefulness as a conventional diagnostic modality for Japanese patients with suspicious sarcoidosis. The present study also suggests the racial difference in the endobronchial involvement in pulmonary sarcoidosis.

  3. Detection of Myocardial Damage in Patients with Sarcoidosis

    PubMed Central

    Patel, Manesh R.; Cawley, Peter J.; Heitner, John F.; Klem, Igor; Parker, Michele A.; Jaroudi, Wael A.; Meine, Trip J.; White, James B.; Elliott, Michael D.; Kim, Han W.; Judd, Robert M.; Kim, Raymond J.

    2009-01-01

    Background In patients with sarcoidosis, sudden death is a leading cause of mortality, which may represent unrecognized cardiac involvement. Delayed-enhancement cardiovascular magnetic resonance (DE-CMR) can detect minute amounts of myocardial damage. We sought to compare DE-CMR with standard clinical evaluation for the identification of cardiac involvement. Methods and Results Eighty-one consecutive patients with biopsy proven extra-cardiac sarcoidosis were prospectively recruited for a parallel and masked comparison of cardiac involvement between: (1) DE-CMR, and (2) standard clinical evaluation using consensus criteria (modified Japanese Ministry of Health [JMH] guidelines). Standard evaluation included 12-lead electrocardiography and at least one dedicated non-CMR cardiac study (echocardiography, radionuclide scintigraphy, or cardiac catheterization). Patients were followed 21±8 months for major adverse events (death, defibrillator shock, or pacemaker requirement). Patients were predominantly middle-aged (46±11 years), female (62%), African-American (73%), had chronic sarcoidosis (median, 7 years), and preserved LVEF (median, 56%). DE-CMR identified cardiac involvement in 21 patients (26%) and JMH criteria in 10 (12%, 8 overlapping), a more than two-fold higher rate for DE-CMR (p=0.005). All patients with myocardial damage on DE-CMR had coronary disease excluded by x-ray angiography. Pathology evaluation in 15 patients (19%) identified 4 with cardiac sarcoidosis; all 4 were positive by DE-CMR whereas 2 were JMH positive. On follow-up, 8 had adverse events including 5 cardiac deaths. Patients with myocardial damage on DE-CMR had a 9-fold higher rate of adverse events and a 11.5-fold higher rate of cardiac death than patients without damage. Conclusion In patients with sarcoidosis, DE-CMR is more than twice as sensitive for cardiac involvement than current consensus criteria. Myocardial damage detected by DE-CMR appears to be associated with future adverse

  4. Cutaneous sarcoidosis.

    PubMed

    Wilson, N J; King, C M

    1998-11-01

    Sarcoidosis is a multi-organ granulomatous disorder of unknown cause. Skin sarcoidosis occurs in about 25% of patients with systemic disease and may also arise in isolation. A wide range of clinical presentations of cutaneous sarcoidosis is recognised. The diagnosis rests on the presence of non-caseating granulomas on skin biopsy and the exclusion of other granulomatous skin disease. The treatment and overall prognosis of cutaneous sarcoidosis is primarily dependent on the degree of systemic involvement. In patients with aggressive disease limited to the skin immunosuppressive therapy may be indicated.

  5. Assessment of Mycobacterial, Propionibacterial, and Human Herpesvirus 8 DNA in Tissues of Greek Patients with Sarcoidosis

    PubMed Central

    Gazouli, M.; Ikonomopoulos, J.; Trigidou, R.; Foteinou, M.; Kittas, C.; Gorgoulis, V.

    2002-01-01

    The causes of sarcoidosis are unknown. In this study, we report the presence of Mycobacterium tuberculosis complex and Propionibacterium granulosum DNA in a significant proportion of Greek patients with sarcoidosis. Human herpesvirus 8 DNA was not detected in sarcoid tissues from Greek patients. Our findings are discussed. PMID:12149380

  6. Hepatic Sarcoidosis.

    PubMed

    Tadros, Micheal; Forouhar, Faripour; Wu, George Y

    2013-12-01

    Sarcoidosis is a multisystem disease characterized by the presence of non-caseating granulomas in affected organs. Pulmonary involvement is the most common site of disease activity. However, hepatic involvement is also common in sarcoidosis, occurring in up to 70% of patients. Most patients with liver involvement are asymptomatic. Therefore, the majority of cases are discovered incidentally, frequently by the finding of elevated liver enzymes. Pain in the right upper quadrant of the abdomen, fatigue, pruritus, and jaundice may be associated with liver involvement. Portal hypertension and cirrhosis are complications linked to long-standing hepatic sarcoidosis. Liver biopsy is usually required to confirm the diagnosis. It is important to differentiate hepatic sarcoidosis from other autoimmune and granulomatous liver diseases. Not all cases of hepatic sarcoidosis require treatment. For symptomatic patients, the first line treatment includes corticosteroids or ursodeoxycholic acid. Various immunosuppressant agents can be used as second line agents. Rarely, severe cases require liver transplantation.

  7. A toll-like receptor 3 single nucleotide polymorphism in Japanese patients with sarcoidosis.

    PubMed

    Ikezoe, K; Handa, T; Tanizawa, K; Kubo, T; Ito, I; Sokai, A; Nakatsuka, Y; Nagai, S; Izumi, T; Mishima, M

    2015-03-01

    Toll-like receptor 3 (TLR3) may be associated with T helper 1 immune response. This study aimed to investigate the role of a functional TLR3 single nucleotide polymorphism (SNP) in sarcoidosis. We genotyped 220 Japanese patients with sarcoidosis and 140 controls for TLR3 SNP rs3775291 to analyze its association with susceptibility to sarcoidosis and assessed its relationship to clinical features in 172 patients over 2 years. The TLR3 rs3775291 genotype was not significantly associated with disease susceptibility. However, patients with cardiac sarcoidosis (CS) significantly more frequently had the TT genotype (p < 0.01) or the T allele (p < 0.05) than those patients without CS. We conclude that TLR3 SNP rs3775291 may affect cardiac involvement in Japanese patients with sarcoidosis.

  8. Cardiac Sarcoidosis.

    PubMed

    Birnie, David; Ha, Andrew C T; Gula, Lorne J; Chakrabarti, Santabhanu; Beanlands, Rob S B; Nery, Pablo

    2015-12-01

    Studies suggest clinically manifest cardiac involvement occurs in 5% of patients with pulmonary/systemic sarcoidosis. The principal manifestations of cardiac sarcoidosis (CS) are conduction abnormalities, ventricular arrhythmias, and heart failure. Data indicate that an 20% to 25% of patients with pulmonary/systemic sarcoidosis have asymptomatic (clinically silent) cardiac involvement. An international guideline for the diagnosis and management of CS recommends that patients be screened for cardiac involvement. Most studies suggest a benign prognosis for patients with clinically silent CS. Immunosuppression therapy is advocated for clinically manifest CS. Device therapy, with implantable cardioverter defibrillators, is recommended for some patients.

  9. Hypercalciuria and renal stones in a sarcoidosis patient treated by extracorporeal shockwave lithotripsy.

    PubMed

    Sharma, O P; Alfaro, C

    1986-03-01

    A case of chronic pulmonary sarcoidosis and hypercalciuria complicated by bilateral renal stones is reported. Urinary stones were pulverized by extracorporeal shockwave lithotripsy (ESWL) as the patient had declined any surgical procedure. The use of ESWL in conjunction with corticosteroids appears to be the treatment of choice in the management of renal stones secondary to abnormalities of calcium metabolism in sarcoidosis.

  10. Large pituitary incidentaloma in a patient with sarcoidosis

    PubMed Central

    Jalota, Leena; Abaroa-Salvatierra, Ana; Alweis, Richard

    2014-01-01

    A 60 year old male with a medical history of pulmonary sarcoidosis and chronic low testosterone presented to his allergist for excessive lacrimation. Computed tomography (CT) scan of sinuses ordered for possible blocked nasolacrimal duct revealed an abnormal expansion of the sella turcica. Magnetic resonance imaging suggested a homogeneously enhancing 4 cm soft tissue mass enveloping the internal carotid and abutting the optic nerves. Since the patient indicated no symptoms, it was felt to be consistent with a pituitary incidentaloma. Laboratory investigation showed only minimally elevated prolactin. Visual field testing at the office was normal but computed campimetry was suggestive of few minimally depressed points in the supra-temporal quadrant on the right. Even with high suspicion of neurosarcoidosis, the patient had a surgical indication so he underwent transsphenoidal excision of the mass with no complications. Pathology was consistent with a null-cell pituitary adenoma. PMID:25147632

  11. Severe Sarcoidosis.

    PubMed

    Kouranos, Vasileios; Jacob, Joe; Wells, Athol U

    2015-12-01

    In sarcoidosis, reduction in mortality and the prevention of disability due to major organ involvement are treatment goals. Thus, it is important to recognize severe disease and identify patients at higher risk of progression to severe disease. In this article, fibrotic lung disease and cardiac sarcoidosis are reviewed as the major contributors to sarcoidosis mortality and morbidity. In the absence of a standardized definition of severe pulmonary disease, a multidisciplinary approach to clinical staging is suggested, based on symptoms, pulmonary function tests, and imaging findings at presentation, integrated with the duration of disease and longitudinal disease behavior during early follow-up.

  12. Ocular Sarcoidosis.

    PubMed

    Pasadhika, Sirichai; Rosenbaum, James T

    2015-12-01

    Sarcoidosis is one of the leading causes of inflammatory eye disease. Ocular sarcoidosis can involve any part of the eye and its adnexal tissues and may cause uveitis, episcleritis/scleritis, eyelid abnormalities, conjunctival granuloma, optic neuropathy, lacrimal gland enlargement, and orbital inflammation. Glaucoma and cataract can be complications from inflammation itself or adverse effects from therapy. Ophthalmic manifestations can be isolated or associated with other organ involvement. Patients with ocular sarcoidosis can present with a wide range of clinical presentations and severity. Multidisciplinary approaches are required to achieve the best treatment outcomes for both ocular and systemic manifestations.

  13. [Sarcoidosis of the skin].

    PubMed

    Suga, Y; Ogawa, H

    1994-06-01

    Sarcoidosis is characterized by formation of epithelioid-cell tubercules, without caseation, of the affected organ systems. The mediastinum, peripheral lymph nodes and eyes, in addition to the skin, are most frequently affected. Between 10% and 30% of patients with systemic sarcoidosis in Japan have skin lesions. Skin sarcoidosis is morphologically classified into three basic groups, erythema nodosum, scar sarcoidosis and skin sarcoid. Skin sarcoid is characterized by specific cutaneous lesions of sarcoidosis, and may take nodular, plaque, angiolupoid, subcutaneous and some other forms. Clinical manifestations of the cutaneous lesions are usually asymptomatic and polymorphous. Skin biopsy is, however, often highly useful for confirming a diagnosis of sarcoidosis.

  14. Cutaneous sarcoidosis.

    PubMed Central

    Wilson, N. J.; King, C. M.

    1998-01-01

    Sarcoidosis is a multi-organ granulomatous disorder of unknown cause. Skin sarcoidosis occurs in about 25% of patients with systemic disease and may also arise in isolation. A wide range of clinical presentations of cutaneous sarcoidosis is recognised. The diagnosis rests on the presence of non-caseating granulomas on skin biopsy and the exclusion of other granulomatous skin disease. The treatment and overall prognosis of cutaneous sarcoidosis is primarily dependent on the degree of systemic involvement. In patients with aggressive disease limited to the skin immunosuppressive therapy may be indicated. Images Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 Figure 7 Figure 8 Figure 9 Figure 10 PMID:10197194

  15. [Clinical diagnostic and organizational aspects of providing care to patients with pulmonary sarcoidosis in the Armed Forces].

    PubMed

    Ovchinnikov, Iu V; Kriukov, E V; Zaĭtsev, A A; Antipushina, D N

    2014-11-01

    The data on the epidemiology and aetiology of sarcoidosis, the current classifications are presented. The basic provisions of the legal framework of medical management of patients suffering from sarcoidosis are given. The authors provided an analysis of the characteristics of diagnosis and treatment of sarcoidosis in the military, based on which we propose an algorithm of examination of patients with respiratory sarcoidosis in military health care facilities the Russian Defence Ministry, the recommended treatment regimens and order dynamic observation of patients. Invited to provide skilled care to patients with respiratory sarcoidosis selection based on the Main Military Clinical Burdenko Hospital specialized centre (department with bunks for the treatment of patients with sarcoidosis). PMID:25816680

  16. Disseminated scar sarcoidosis may predict pulmonary involvement in sarcoidosis.

    PubMed

    Su, Ozlem; Onsun, Nahide; Topukçu, Buğçe; Ozçelik, Hatice Kutbay; Cakıter, Alkım Unal; Büyükpınarbaşılı, Nur

    2013-09-01

    Sarcoidosis is a chronic, inflammatory, multi-organ disease of unknown origin that is characterized by non-caseating granuloma formation in affected organs. Cutaneous involvement is reported in 25% of patients with sarcoidosis. Scar sarcoidosis is rare but is clinically specific for skin sarcoidosis. Systemic involvement is seen in most patients with scar sarcoidosis. We present a case of scar sarcoidosis in a 30-year-old male that developed infiltrated nodules on old scars, including on his penile shaft, which is rare, and that also had pulmonary involvement. Scar sarcoidosis should be considered in the differential diagnosis of changes in all scar areas and should be investigated for systemic involvement.

  17. Cumulative Incidence and Predictors of Progression in Corticosteroid-Naïve Patients with Sarcoidosis

    PubMed Central

    Hashimoto, Dai; Enomoto, Noriyuki; Fujisawa, Tomoyuki; Nakamura, Yutaro; Suda, Takafumi

    2015-01-01

    Background Assessment of the clinical course of sarcoidosis requires long-term observation. However, the appropriate period of follow-up for sarcoidosis remains unclear, especially in patients without indication of corticosteroid therapy at the time of diagnosis. Objective This study aimed to clarify the cumulative incidence and identify risk factors for disease progression in corticosteroid-naïve sarcoidosis patients. Methods The clinical courses of 150 Japanese patients with sarcoidosis, who were followed for more than 2 years and had no indication for corticosteroid therapy at diagnosis, were retrospectively reviewed. Disease progression was defined as worsening of pulmonary sarcoidosis, development of new organ involvement, or extrapulmonary organ damage. The cumulative incidence of progression was estimated by generating a cumulative incidence curve with the Fine and Gray method. Results The median follow-up duration was 7.7 years (interquartile range, 4.7–13.6 years). Thirty-two (21%) patients experienced disease progression. New organ involvement appeared in 16 patients (11%). The 6-month, and 1-, 5-, 10-, and 15-year cumulative incidence of progression was 2%, 5%, 15%, 28%, and 31%, respectively. The number of organs involved at diagnosis was an independent predictor for progression with a multifactorial adjusted hazard ratio of 1.71 (95% confidence interval, 1.11–2.62). The optimal cut-off of the number of organs involved at diagnosis to identify future progression was three. Conclusions In corticosteroid-naïve sarcoidosis patients, the risks of disease progression are comparable from 0–5 years and 5–10 years after diagnosis. The number of organs involved at diagnosis is a useful predictor for progression of sarcoidosis. PMID:26575272

  18. Sarcoidosis Occurring After Lymphoma

    PubMed Central

    London, Jonathan; Grados, Aurélie; Fermé, Christophe; Charmillon, Alexandre; Maurier, François; Deau, Bénédicte; Crickx, Etienne; Brice, Pauline; Chapelon-Abric, Catherine; Haioun, Corinne; Burroni, Barbara; Alifano, Marco; Le Jeunne, Claire; Guillevin, Loïc; Costedoat-Chalumeau, Nathalie; Schleinitz, Nicolas; Mouthon, Luc; Terrier, Benjamin

    2014-01-01

    Abstract Sarcoidosis is a granulomatous disease that most frequently affects the lungs with pulmonary infiltrates and/or bilateral hilar and mediastinal lymphadenopathy. An association of sarcoidosis and lymphoproliferative disease has previously been reported as the sarcoidosis-lymphoma syndrome. Although this syndrome is characterized by sarcoidosis preceding lymphoma, very few cases of sarcoidosis following lymphoma have been reported. We describe the clinical, biological, and radiological characteristics and outcome of 39 patients presenting with sarcoidosis following lymphoproliferative disease, including 14 previously unreported cases and 25 additional patients, after performing a literature review. Hodgkin lymphoma and non-Hodgkin lymphoma were equally represented. The median delay between lymphoma and sarcoidosis was 18 months. Only 16 patients (41%) required treatment. Sarcoidosis was of mild intensity or self-healing in most cases, and overall clinical response to sarcoidosis was excellent with complete clinical response in 91% of patients. Sarcoidosis was identified after a follow-up computerized tomography scan (CT-scan) or 18fluorodeoxyglucose-positron emission tomography/computerized tomography (18FDG-PET/CT) evaluation in 18/34 patients (53%). Sarcoidosis is therefore a differential diagnosis to consider when lymphoma relapse is suspected on a CT-scan or 18FDG-PET/CT, emphasizing the necessity to rely on histological confirmation of lymphoma relapse. PMID:25380084

  19. Possible role of L-selectin in T lymphocyte alveolitis in patients with active pulmonary sarcoidosis.

    PubMed

    Kaseda, M; Kadota, J; Mukae, H; Kawamoto, S; Shukuwa, T; Iwashita, T; Matsubara, Y; Ishimatsu, Y; Yoshinaga, M; Abe, K; Kohno, S

    2000-07-01

    A number of adhesion molecules participate in the recruitment of inflammatory cells to the site of inflammation, and selectins together with their ligands are important in the early transient adhesion phase. In this study, we evaluated the role of L-selectin in T lymphocyte alveolitis in patients with active pulmonary sarcoidosis. We measured serum and bronchoalveolar lavage fluid (BALF) concentrations of soluble (s)L-selectin using an ELISA. Serum and BALF concentrations of sL-selectin were significantly elevated in patients with sarcoidosis compared with control healthy subjects and idiopathic pulmonary fibrosis (IPF) patients (P < 0.05 and P < 0. 01, respectively). The lymphocyte surface marker was also examined in peripheral blood and BALF by flow cytometric analysis. The percentage of CD3+CD62L+ cells (L-selectin-bearing T lymphocytes) was significantly lower in peripheral blood of sarcoidosis than in that of healthy subjects (P < 0.01). In contrast, the percentage of CD3+CD62L- cells (L-selectin-negative T lymphocytes) in BALF of patients with sarcoidosis was significantly higher than in healthy subjects (P < 0.05) and IPF patients (P < 0.01). Furthermore, there was a significant correlation between serum concentrations of sL-selectin and the number of L-selectin-negative T lymphocytes in BALF (r = 0.535, P < 0.01). Our results suggest that L-selectin may be involved in T lymphocyte alveolitis in patients with active pulmonary sarcoidosis. PMID:10886252

  20. Possible role of l-selectin in T lymphocyte alveolitis in patients with active pulmonary sarcoidosis

    PubMed Central

    Kaseda, M; Kadota, J; Mukae, H; Kawamoto, S; Shukuwa, T; Iwashita, T; Matsubara, Y; Ishimatsu, Y; Yoshinaga, M; Abe, K; Kohno, S

    2000-01-01

    A number of adhesion molecules participate in the recruitment of inflammatory cells to the site of inflammation, and selectins together with their ligands are important in the early transient adhesion phase. In this study, we evaluated the role of l-selectin in T lymphocyte alveolitis in patients with active pulmonary sarcoidosis. We measured serum and bronchoalveolar lavage fluid (BALF) concentrations of soluble (s)l-selectin using an ELISA. Serum and BALF concentrations of sl-selectin were significantly elevated in patients with sarcoidosis compared with control healthy subjects and idiopathic pulmonary fibrosis (IPF) patients (P < 0·05 and P < 0·01, respectively). The lymphocyte surface marker was also examined in peripheral blood and BALF by flow cytometric analysis. The percentage of CD3+CD62L+ cells (l-selectin-bearing T lymphocytes) was significantly lower in peripheral blood of sarcoidosis than in that of healthy subjects (P < 0·01). In contrast, the percentage of CD3+CD62L− cells (l-selectin-negative T lymphocytes) in BALF of patients with sarcoidosis was significantly higher than in healthy subjects (P < 0·05) and IPF patients (P < 0·01). Furthermore, there was a significant correlation between serum concentrations of sl-selectin and the number of l-selectin-negative T lymphocytes in BALF (r = 0·535, P < 0·01). Our results suggest that l-selectin may be involved in T lymphocyte alveolitis in patients with active pulmonary sarcoidosis. PMID:10886252

  1. Comorbid illnesses and chest radiographic severity in African-American sarcoidosis patients.

    PubMed

    Westney, Gloria E; Habib, Sadia; Quarshie, Alexander

    2007-01-01

    Sarcoidosis disease expression differs along racial/ethnic lines and black race has been cited as a poor prognostic factor. Besides genetic, healthcare, and socioeconomic factors, comorbid illnesses may influence sarcoidosis disease expression. We set out to investigate the association between comorbid illnesses and chest radiographic severity in a population of African-American sarcoidosis patients. The study was designed as a retrospective database analysis. The hospital and outpatient databases of the Grady Health System were searched to capture adult patients between November 1999 and December 2003 with the ICD-9 codes of 135 or 519.8, along with all associated secondary and tertiary diagnostic codes. Patient electronic pathology and radiographic reports were reviewed for tissue biopsies showing noncaseating granulomas and for chest radiographic Scadding stage. A total of 165 African-American patients were identified (64% female, 43 +/- 10 years old). Ninety percent (149/165) had comorbid illnesses. The most frequent chronic comorbid illnesses were hypertension (39%), diabetes mellitus (19%), anemia (19%), asthma (15%), gastroesophageal reflux disease (15%), depression (13%), and heart failure (10%). Females had increased frequency and clustering of chronic illnesses. Chest radiographic stages were more severe in patients with anemia, depression, and those less than 40 years old. Males, within each chronic illnesses category, had more severe CXR stages compared to females; however, significance was not achieved. We concluded that most adult patients with sarcoidosis have comorbid illnesses and these, in addition to gender differences, may influence sarcoidosis disease expression. Screening for comorbid illnesses should be an important aspect of sarcoidosis patient management.

  2. [Multicenter retrospective analysis of the patients with sarcoidosis with a 10 year interval in observations].

    PubMed

    Vizel', I Iu; Shmelev, E I; Baranova, O P; Barlamov, P N; Borodina, G L; Denisova, O A; Dobin, V L; Kulbaisov, A M; Kupaev, V I; Listopadova, M V; Ovsiannikov, N V; Os'kin, D N; Petrov, D V; Solov'ev, K I; Shul'zhenko, L V; Vizel', A A

    2014-01-01

    Comparison of the state of 83 patients with histologically confirmed sarcoidosis observed with a 10 year interval revealed remission in 47% of the cases. The main factors having negative effect on prognosis of the disease included extrapulmonary symptoms, the use ofcorticosteroids (at all stages, especially at stage I and in Lofgren syndrome) and antituberculosis drugs, positive TB test. Risk factors of relapses were stage II sarcoidosis, the use of systemic corticosteroids in patients with Lofgren syndrome and antituberculosis drugs, initially low FEV1/FVLC ratio and the number of lymphocytes in peripheral blood. PMID:25799827

  3. Sarcoidosis Quiz

    MedlinePlus

    ... Share this page from the NHLBI on Twitter. Sarcoidosis Quiz Sarcoidosis is a disease of unknown cause that leads ... various organs in the body. The outcome of sarcoidosis varies. The disease leads to organ damage in ...

  4. Bone histomorphometry in a long-term hemodialysis patient with hypoparathyroidism and sarcoidosis.

    PubMed

    Sumida, K; Ubara, Y; Hoshino, J; Hayami, N; Suwabe, T; Hiramatsu, R; Hasegawa, E; Yamanouchi, M; Sawa, N; Fujii, T; Takaichi, K

    2015-04-01

    A bone biopsy specimen in a long-term hemodialysis patient with sarcoidosis coexisting with severe hypoparathyroidism has demonstrated that a persistent near physiological level of 1,25-dihydroxyvitamin D3 contributes to the preservation of bone remodeling and has the potential to retard the development of vascular calcification and atherosclerosis. Sarcoidosis-related hypercalcemia and hypoparathyroidism, which is characterized by 1,25-dihydroxyvitamin D3 (1,25(OH)2D3) overproduction, is rarely seen in hemodialysis patients. Herein, we describe a 60-year-old Japanese woman on hemodialysis for 35 years who presented with malaise and hypercalcemia. Severe hypoparathyroidism without parathyroidectomy and a preserved 1,25(OH)2D3 level were detected. Computed tomography showed bilateral axillary lymphadenopathy and minimal aortic and soft tissue calcification. The axillary node biopsy led to a definite diagnosis of sarcoidosis. A bone biopsy specimen obtained from the right iliac crest showed remodeling of normal lamellar bone with scalloped cement lines and clear double labeling by tetracycline on fluorescence microscopy. Histomorphometric analysis revealed that the bone formation rate was preserved (30.0 %/year), together with a decrease of osteoid volume (5.75 %) and fibrous volume (0 %), indicating that the patient did not have adynamic bone disease and only showed mild disease. This is the first documented case of sarcoidosis-related hypercalcemia associated with severe hypoparathyroidism in a long-term hemodialysis patient who underwent bone histomorphometry. Our findings suggest that, in hemodialysis patients with sarcoidosis coexisting with severe hypoparathyroidism, a persistent near physiological level of 1,25(OH)2D3 contributes to the preservation of bone remodeling and has the potential to retard the development of vascular calcification and atherosclerosis.

  5. Rheumatologic manifestations of sarcoidosis.

    PubMed

    Sweiss, Nadera J; Patterson, Karen; Sawaqed, Ray; Jabbar, Umair; Korsten, Peter; Hogarth, Kyle; Wollman, Robert; Garcia, Joe G N; Niewold, Timothy B; Baughman, Robert P

    2010-08-01

    Sarcoidosis is a systemic, clinically heterogeneous disease characterized by the development of granulomas. Any organ system can be involved, and patients may present with any number of rheumatologic symptoms. There are no U.S. Food and Drug Administration-approved therapies for the treatment of sarcoidosis. Diagnosing sarcoidosis becomes challenging, particularly when its complications cause patients' symptoms to mimic other conditions, including polymyositis, Sjögren syndrome, or vasculitis. This review presents an overview of the etiology of and biomarkers associated with sarcoidosis. We then provide a detailed description of the rheumatologic manifestations of sarcoidosis and present a treatment algorithm based on current clinical evidence for patients with sarcoid arthritis. The discussion will focus on characteristic findings in patients with sarcoid arthritis, osseous involvement in sarcoidosis, and sarcoid myopathy. Arthritic conditions that sometimes coexist with sarcoidosis are described as well. We present two cases of sarcoidosis with rheumatologic manifestations. Our intent is to encourage a multidisciplinary, translational approach to meet the challenges and difficulties in understanding and treating sarcoidosis.

  6. Diagnosis of sarcoidosis

    SciTech Connect

    Sharma, O.P.

    1983-07-01

    During the last decade, many biochemical and immunologic advances have been made in the treatment and understanding of sarcoidosis. These studies have helped us to understand the basic mechanisms involved in granuloma formation, and many clinicians have used the information to diagnose and assess the activity of sarcoidosis. Further studies are needed to clearly establish the role of these advances in the everyday management of patients with sarcoidosis.

  7. Fungal Exposure and Low Levels of IL-10 in Patients with Sarcoidosis

    PubMed Central

    Terčelj, Marjeta; Stopinšek, Sanja; Ihan, Alojz; Salobir, Barbara; Simčič, Saša; Rylander, Ragnar

    2014-01-01

    Background and Objectives. Sarcoidosis is an inflammatory disease with increased levels of inflammatory cytokines. Previous studies have shown a relation between the degree of granuloma infiltration and serum cytokine levels, except for interleukin- (IL-) 10. The aim of the study was to further investigate the serum levels of IL-10 in patients with sarcoidosis and relate them to fungal exposure in terms of the amount of fungi in the air of their homes and β-glucan in bronchoalveolar lavage (BAL) fluid. Methods. Patients with sarcoidosis (n = 71) and healthy controls (n = 27) were enrolled. IL-10 was determined in serum. BAL was performed and the amount of β-glucan was measured. Domestic exposure to fungi was determined by measuring airborne β-N-acetylhexosaminidase (NAHA) in the bedrooms. Results. At high levels of fungal exposure (domestic fungal exposure and β-glucan in BAL), serum IL-10 values were lower than at low and intermediate exposure levels. Conclusion. The low serum IL-10 values at high fungal exposure suggest that fungal cell wall agents play a role in granuloma formation in sarcoidosis by inhibiting the secretion of the anti-inflammatory cytokine IL-10. PMID:25180094

  8. Rheumatologic Manifestations of Sarcoidosis

    PubMed Central

    Sweiss, Nadera J.; Patterson, Karen; Sawaqed, Ray; Jabbar, Umair; Korsten, Peter; Hogarth, Kyle; Wollman, Robert; Garcia, Joe G.N.; Niewold, Timothy B.; Baughman, Robert P.

    2012-01-01

    Sarcoidosis is a systemic, clinically heterogeneous disease characterized by the development of granulomas. Any organ system can be involved, and patients may present with any number of rheumatologic symptoms. There are no U.S. Food and Drug Administration–approved therapies for the treatment of sarcoidosis. Diagnosing sarcoidosis becomes challenging, particularly when its complications cause patients’ symptoms to mimic other conditions, including polymyositis, Sjögren syndrome, or vasculitis. This review presents an overview of the etiology of and biomarkers associated with sarcoidosis. We then provide a detailed description of the rheumatologic manifestations of sarcoidosis and present a treatment algorithm based on current clinical evidence for patients with sarcoid arthritis. The discussion will focus on characteristic findings in patients with sarcoid arthritis, osseous involvement in sarcoidosis, and sarcoid myopathy. Arthritic conditions that sometimes coexist with sarcoidosis are described as well. We present two cases of sarcoidosis with rheumatologic manifestations. Our intent is to encourage a multidisciplinary, translational approach to meet the challenges and difficulties in understanding and treating sarcoidosis. PMID:20665396

  9. Novel pharmacotherapy of sarcoidosis.

    PubMed

    Sah, Birendra P; Goyal, Shraddha; Iannuzzi, Michael C

    2016-01-01

    Sarcoidosis is a multisystem granulomatous disease of unknown etiology that most commonly affects the lungs. Treatment of sarcoidosis can be challenging as it is often difficult to measure disease activity and distinguish active inflammation from fibrosis. Identifying the inflammatory mediators in sarcoidosis has led to the development and use of novel therapeutic agents. The goal of pharmacotherapy is to decrease granuloma accumulation, ameliorate symptoms and improve organ function. Systemic corticosteroids remain the first line treatment. Other immunosuppressive agents may be considered for the patients who respond poorly to corticosteroids or who experience significant adverse effects. An overview of pharmacotherapy of sarcoidosis is provided here.

  10. Recent advances in sarcoidosis.

    PubMed

    Morgenthau, Adam S; Iannuzzi, Michael C

    2011-01-01

    Sarcoidosis, a systemic granulomatous disease of undetermined etiology, is characterized by a variable clinical presentation and course. During the past decade, advances have been made in the study of sarcoidosis. The multicenter ACCESS (A Case Control Etiologic Study of Sarcoidosis) trial recruited > 700 subjects with newly diagnosed sarcoidosis and matched control subjects. Investigators were unable to identify a single cause of sarcoidosis, but ACCESS paved the way for subsequent etiologic studies. The Mycobacterium tuberculosis catalase-peroxidase protein has been identified as a potential sarcoidosis antigen. Genetic aspects of the disease have been elucidated further. Genome-wide scans have identified candidate genes. Gene expression analyses have defined cytokine dysregulation in sarcoidosis more clearly. Although the criteria for diagnosis have not changed, sarcoidosis remains a diagnosis of exclusion best supported by a tissue biopsy specimen that demonstrates noncaseating granulomas in a patient with compatible clinical and radiologic features of the disease. Endobronchial ultrasound-guided transbronchial needle aspiration of mediastinal lymph nodes has facilitated diagnosis, often eliminating the need for more invasive procedures, such as mediastinoscopy. PET scanning has proven valuable in locating occult sites of active disease. Currently, no reliable prognostic biomarkers have been identified. The tumor necrosis factor inhibitors, a relatively new class of agents, have been used in patients with refractory disease. It is unclear whether phosphodiesterase-5 inhibitors, prostaglandin analogs, or endothelin antagonists should be used for the treatment of sarcoidosis-associated pulmonary hypertension. PMID:21208877

  11. A case of chylothorax in a patient with sarcoidosis: a rare and potentially fatal complication

    PubMed Central

    Bhattarai, Bikash; Schmidt, Frances; Devkota, Ashok; Policard, Geraldine; Manhas, Saveena; Oke, Vikram; Agu, Chidozie Charles; Basunia, Md Rawshan; Enriquez, Danilo; Quist, Joseph; Kharel, Prakash

    2015-01-01

    Obstruction of the thoracic duct may lead to accumulation of a lymphatic fluid rich in triglycerides named chyle. When chyle accumulates in the pleural cavity, it becomes a chylothorax. Malignancy, particularly lymphoma, is the most common cause of chylothorax; however, any pathology leading to obstruction or destruction of the thoracic duct can lead to a chylothorax. This particular case investigates an incidence of chylothorax in sarcoidosis. A 54-year-old African American woman with a medical history of sarcoidosis, congestive heart failure, and smoking presented to the emergency department with complaints of bilateral foot swelling and exertional shortness of breath 3 days in duration. Physical examination was positive for bilateral crepitations with decreased air entry, abdominal ascites, and bilateral 2+ pitting edema. Both chest X-ray and chest CT were positive for stable bilateral pleural effusions (when compared to imaging done 3 years previously), and thoracocentesis and paracentesis were positive for chylous fluid accumulation. Chylothorax was diagnosed, and based on the previous medical history, the lymphadenopathy of sarcoidosis was determined to cause the occlusion of the thoracic duct. Lymphoscintigraphy and surgical intervention were advised; however, the family decided on conservative management and the patient expired intubated in the ICU. Chylothorax is a rare manifestation of sarcoidosis and high index of suspicion should be there to diagnose this, as there is high morbidity and mortality associated with it. PMID:26333861

  12. Spontaneous production of various cytokines except IL-4 from CD4+ T cells in the affected organs of sarcoidosis patients.

    PubMed

    Hoshino, T; Itoh, K; Gouhara, R; Yamada, A; Tanaka, Y; Ichikawa, Y; Azuma, M; Mochizuki, M; Oizumi, K

    1995-11-01

    We investigated surface antigens and spontaneous cytokine production of T cells from bronchoalveolar lavage fluid (BALF) and aqueous humor (AH) from pulmonary sarcoidosis patients for a better understanding of the role of T cells in granuloma formation. The levels of CD3, CD11b, and CD28 antigen expression on freshly isolated T cells in the BALF of patients were significantly lower than those in peripheral blood lymphocytes (PBL) of either sarcoidosis patients or healthy donors (HD). In contrast, the levels of CD80 (B7/B7-1) and CD86 (B70/B7-2) antigen expression were significantly higher on these T cells and alveolar macrophages in the BALF of patients. Fifty-three T cell clones (TCC) established from the BALF and AH of the three sarcoidosis patients displayed primarily either CD4+ CD11b+ CD28+ or CD4+ CD11b- CD28- phenotypes. Most (61-90%) of these TCC spontaneously produced greater amounts of IL-1 alpha, IL-10, tumour necrosis factor (TNF), and granulocyte-macrophage colony-stimulating factor (GM-CSF) than did TCC from the PBL from sarcoidosis patients or HD (P < 0.05). Interferon-gamma (IFN-gamma), IL-6, and IL-2, but not IL-4, were also produced by 40-48% of these TCC. These results suggest that CD4+ T cells of the affected organs of sarcoidosis patients are activated and involved in the immunopathogenesis of sarcoidosis through production of various cytokines.

  13. Etiologies of Sarcoidosis.

    PubMed

    Chen, Edward S; Moller, David R

    2015-08-01

    Since sarcoidosis was first described more than a century ago, the etiologic determinants causing this disease remain uncertain. Studies suggest that genetic, host immunologic, and environmental factors interact together to cause sarcoidosis. Immunologic characteristics of sarcoidosis include non-caseating granulomas, enhanced local expression of T helper-1 (and often Th17) cytokines and chemokines, dysfunctional regulatory T-cell responses, dysregulated Toll-like receptor signaling, and oligoclonal expansion of CD4+ T cells consistent with chronic antigenic stimulation. Multiple environmental agents have been suggested to cause sarcoidosis. Studies from several groups implicate mycobacterial or propionibacterial organisms in the etiology of sarcoidosis based on tissue analyses and immunologic responses in sarcoidosis patients. Despite these studies, there is no consensus on the nature of a microbial pathogenesis of sarcoidosis. Some groups postulate sarcoidosis is caused by an active viable replicating infection while other groups contend there is no clinical, pathologic, or microbiologic evidence for such a pathogenic mechanism. The authors posit a novel hypothesis that proposes that sarcoidosis is triggered by a hyperimmune Th1 response to pathogenic microbial and tissue antigens associated with the aberrant aggregation of serum amyloid A within granulomas, which promotes progressive chronic granulomatous inflammation in the absence of ongoing infection. PMID:25771769

  14. Etiologies of Sarcoidosis.

    PubMed

    Chen, Edward S; Moller, David R

    2015-08-01

    Since sarcoidosis was first described more than a century ago, the etiologic determinants causing this disease remain uncertain. Studies suggest that genetic, host immunologic, and environmental factors interact together to cause sarcoidosis. Immunologic characteristics of sarcoidosis include non-caseating granulomas, enhanced local expression of T helper-1 (and often Th17) cytokines and chemokines, dysfunctional regulatory T-cell responses, dysregulated Toll-like receptor signaling, and oligoclonal expansion of CD4+ T cells consistent with chronic antigenic stimulation. Multiple environmental agents have been suggested to cause sarcoidosis. Studies from several groups implicate mycobacterial or propionibacterial organisms in the etiology of sarcoidosis based on tissue analyses and immunologic responses in sarcoidosis patients. Despite these studies, there is no consensus on the nature of a microbial pathogenesis of sarcoidosis. Some groups postulate sarcoidosis is caused by an active viable replicating infection while other groups contend there is no clinical, pathologic, or microbiologic evidence for such a pathogenic mechanism. The authors posit a novel hypothesis that proposes that sarcoidosis is triggered by a hyperimmune Th1 response to pathogenic microbial and tissue antigens associated with the aberrant aggregation of serum amyloid A within granulomas, which promotes progressive chronic granulomatous inflammation in the absence of ongoing infection.

  15. Polarizable Material from a Conjunctival Biopsy in a Patient with Sarcoidosis

    PubMed Central

    Ali, Omair B.; Deroque, Ryan; Morshedi, R. Grant; Brown, Harry

    2016-01-01

    Purpose Conjunctival biopsies may contain polarizable material in patients with sarcoidosis despite no history of prior trauma or eye surgeries. Procedures A 39-year-old male with uveitic glaucoma presented with decreased vision and throbbing pain in his right eye. His intraocular pressure was elevated, and his vision was reduced to hand motion. Due to persistently elevated intraocular pressure refractory to medical treatment, the patient underwent a glaucoma drainage device procedure. During the procedure, a yellow, nodular conjunctival growth was noted and biopsied. Results Histopathological examination revealed multiple nonnecrotizing granulomata, some of which contained polarizable material. Conclusions Biopsies of patients with sarcoidosis may contain polarizable material without evidence of foreign body inoculation. PMID:27239467

  16. Bone and bone marrow involvement in sarcoidosis.

    PubMed

    Yachoui, Ralph; Parker, Brian J; Nguyen, Thanhcuong T

    2015-11-01

    Bone and bone marrow involvement in sarcoidosis have been infrequently reported. We aimed to describe the clinical features, radiological descriptions, pathological examinations, and outcomes of three patients with osseous sarcoidosis and one patient with bone marrow sarcoidosis seen at our institution. Our case series included fluorodeoxyglucose positron emission tomography descriptions in assessing the whole-body extent of sarcoidosis. In the era of advanced imaging, large bone and axial skeleton sarcoidosis lesions are more common than previously reported.

  17. [Cardiac sarcoidosis - clinical manifestation and diagnosis].

    PubMed

    Błaut-Jurkowska, Justyna; Podolec, Piotr; Olszowska, Maria

    2016-08-01

    Sarcoidosis is a multisystem inflammatory disease defined histologically by the formation of noncaseating granulomas. The etiology of sarcoidosis remains unknown. Heart involvement in the course of sarcoidosis concerns about 5% of patients. The most common manifestation of cardiac sarcoidosis are conduction abnormalities, arrhythmias and heart failure. The diagnostic algorithm includes performing a clinical history, a 12-lead electrocardiogram (ECG) and an echocardiogram. If any of the initial screening investigations yields an abnormality, diagnostics should be continue using advanced imaging techniques: cardiovascular magnetic resonance (CMR) or fluorodeoxyglucose positron emission tomography (FDG-PET). Nowadays endomyocardial biopsy is not performed routinely.The clinical picture of cardiac sarcoidosis is highly variable. Screening for cardiac sarcoidosis should be performed in all patients diagnosed with extracardiac sarcoidosis. Cardiac sarcoidosis should also be suspected in young patients without a diagnosis of sarcoidosis who present with conduction abnormalities of unknown etiology, because cardiac sarcoidosis may be the first or the only manifestation of the disease. PMID:27591449

  18. [Cardiac sarcoidosis - clinical manifestation and diagnosis].

    PubMed

    Błaut-Jurkowska, Justyna; Podolec, Piotr; Olszowska, Maria

    2016-07-01

    Sarcoidosis is a multisystem inflammatory disease defined histologically by the formation of noncaseating granulomas. The etiology of sarcoidosis remains unknown. Heart involvement in the course of sarcoidosis concerns about 5% of patients. The most common manifestation of cardiac sarcoidosis are conduction abnormalities, arrhythmias and heart failure. The diagnostic algorithm includes performing a clinical history, a 12-lead electrocardiogram (ECG) and an echocardiogram. If any of the initial screening investigations yields an abnormality, diagnostics should be continue using advanced imaging techniques: cardiovascular magnetic resonance (CMR) or fluorodeoxyglucose positron emission tomography (FDG-PET). Nowadays endomyocardial biopsy is not performed routinely.The clinical picture of cardiac sarcoidosis is highly variable. Screening for cardiac sarcoidosis should be performed in all patients diagnosed with extracardiac sarcoidosis. Cardiac sarcoidosis should also be suspected in young patients without a diagnosis of sarcoidosis who present with conduction abnormalities of unknown etiology, because cardiac sarcoidosis may be the first or the only manifestation of the disease. PMID:27590654

  19. Gallium-67 scintigraphy, bronchoalveolar lavage, and pathologic changes in patients with pulmonary sarcoidosis

    SciTech Connect

    Abe, S.; Munakata, M.; Nishimura, M.; Tsuneta, Y.; Terai, T.; Nakano, I.; Ohsaki, Y.; Kawakami, Y.

    1984-05-01

    The intensity of gallium-67 scintiscans, lymphocyte counts in bronchoalveolar lavage fluid, and pathologic changes were studied in 26 patients with untreated pulmonary sarcoidosis. Noncaseating granulomas were recognized with significantly greater frequency in stage 2 (80 percent; 8/10 cases) than in stage 1 (43 percent; 6/14 cases). Alveolitis showed little relation to the roentgenographic stage. There was a strong correlation between the intensity of gallium uptake in pulmonary parenchyma and the detection rate of granuloma; however, the detection rate of alveolitis was not statistically different from the intensity of gallium uptake. A highly significant correlation was revealed between the lymphocyte counts in bronchoalveolar lavage fluid and the intensity of alveolitis. These observations suggest that the gallium uptake reflects mainly the presence of granuloma, and the lymphocyte count in bronchoalveolar lavage fluid reflects the intensity of alveolitis in patients with pulmonary sarcoidosis.

  20. Reversibility by dipyridamole of thallium-201 myocardial scan defects in patients with sarcoidosis

    SciTech Connect

    Tellier, P.; Paycha, F.; Antony, I.; Nitenberg, A.; Valeyre, D.; Foult, J.M.; Battesti, J.P.

    1988-08-01

    In order to clarify the significance of anginal pain and myocardial thallium-201 scan defects in cardiac sarcoidosis, the pharmacologic effect of dipyridamole on myocardial perfusion was assessed by planar thallium-201 myocardial scintigraphy in patients with sarcoidosis. Thallium-201 myocardial scintigraphy was performed at rest and after 0.56 mg/kg intravenous dipyridamole during four minutes in 16 patients with sarcoidosis. The myocardial scan (45-degree and 70-degree left anterior oblique, and anterior views) was divided into 15 segments. Results were evaluated by the number of segmental defects and with a global perfusion score (from 0 to 60) by a semi-quantitative index depending on the size and severity of myocardial thallium-201 defects. Thirteen of the 16 patients showed partial or total reversion of their thallium-201 defects on redistribution scanning either at rest or after dipyridamole. The mean (+/- SD) number of myocardial perfusion defects that were present in all the patients decreased from 5.31 +/- 1.78 at rest to 3.25 +/- 2.52 after redistribution (p less than 0.001) and to 2.19 +/- 2.10 after dipyridamole (p less than 0.001). The mean global perfusion score increased from 53.2 +/- 3.0 at rest to 56.2 +/- 2.9 after redistribution (p less than 0.001) and to 57.2 +/- 2.7 after dipyridamole (p less than 0.001). A significant correlation (r = 0.82, p less than 0.001) was found between the increase of global perfusion score on redistribution and after dipyridamole. The reversibility of myocardial scan defects is a common finding in sarcoidosis. It makes unlikely the role of scar fibrosis or extensive confluent granulomas as a mechanism for such defects. The effect of dipyridamole suggests the presence of reversible disorders lying at the coronary microvascular level.

  1. Search for chronic beryllium disease among sarcoidosis patients in Ontario, Canada.

    PubMed

    Ribeiro, Marcos; Fritscher, Leandro G; Al-Musaed, Ahmed M; Balter, Meyer S; Hoffstein, Victor; Mazer, Bruce D; Maier, Lisa A; Liss, Gary M; Tarlo, Susan M

    2011-06-01

    Chronic beryllium disease (CBD) is clinically similar to other granulomatous diseases such as sarcoidosis. It is often misdiagnosed if a thorough occupational history is not taken. When appropriate, a beryllium lymphocyte proliferation tests (BeLPT) need to be performed. We aimed to search for CBD among currently diagnosed pulmonary sarcoidosis patients and to identify the occupations and exposures in Ontario leading to CBD. Questionnaire items included work history and details of possible exposure to beryllium. Participants who provided a history of previous work with metals underwent BeLPTs and an ELISPOT on the basis of having a higher pretest probability of CBD. Among 121 sarcoid patients enrolled, 87 (72%) reported no known previous metal dust or fume exposure, while 34 (28%) had metal exposure, including 17 (14%) with beryllium exposure at work or home. However, none of these 34 who underwent testing had positive test results. Self-reported exposure to beryllium or metals was relatively common in these patients with clinical sarcoidosis, but CBD was not confirmed using blood assays in this population.

  2. Gastric involvement of sarcoidosis in a patient with multiple lung nodules

    PubMed Central

    Ceylan, Emel; Şen, Serdar; Coşkun, Adil; Meteoğlu, İbrahim; Demirtaş, Nimet; Çildağ, Orhan

    2015-01-01

    Sarcoidosis is a granulomatous disorder mostly could involve intrathoracic structures. The gastric involvement is rare and the symptoms may be non-specific. We herein report a case of a 56-year-old female patient who was admitted due to chest tightness and discomfort. Computed tomography (CT) of the thorax revealed bilaterally nodular lesions in the lower lobes of the lung and pleural effusion on the left side. Positron emission tomography/CT showed lung nodules and gastric involvement with mesenteric lymphadenomegalies with pathological uptake of 18F-fluoro-2-deoxy-d-glucose. Pathological examination of the lung biopsy taken by thoracotomy demonstrated non-caseating granulomas. The gastric biopsies taken by endoscopy also showed non-caseating granulomas consistent with a diagnosis of sarcoidosis. PMID:26487882

  3. Safety and efficacy of ustekinumab or golimumab in patients with chronic sarcoidosis.

    PubMed

    Judson, Marc A; Baughman, Robert P; Costabel, Ulrich; Drent, Marjolein; Gibson, Kevin F; Raghu, Ganesh; Shigemitsu, Hidenobu; Barney, Joseph B; Culver, Daniel A; Hamzeh, Nabeel Y; Wijsenbeek, Marlies S; Albera, Carlo; Huizar, Isham; Agarwal, Prasheen; Brodmerkel, Carrie; Watt, Rosemary; Barnathan, Elliot S

    2014-11-01

    Sarcoidosis is characterised by non-caseating granulomas that secrete pro-inflammatory cytokines, including interleukin (IL)-12, IL-23, and tumour necrosis factor (TNF)-α. Ustekinumab and golimumab are monoclonal antibodies that specifically inhibit IL-12/IL-23 and TNF-α, respectively. Patients with chronic pulmonary sarcoidosis (lung group) and/or skin sarcoidosis (skin group) received either 180 mg ustekinumab at week 0 followed by 90 mg every 8 weeks, 200 mg golimumab at week 0 followed by 100 mg every 4 weeks, or placebo. Patients underwent corticosteroid tapering between weeks 16 and 28. The primary end-point was week 16 change in percentage predicted forced vital capacity (ΔFVC % pred) in the lung group. Major secondary end-points were: week 28 for ΔFVC % pred, 6-min walking distance, St George's Respiratory Questionnaire (lung group), and Skin Physician Global Assessment response (skin group). At week 16, no significant differences were observed in ΔFVC % pred with ustekinumab (-0.15, p = 0.13) or golimumab (1.15, p = 0.54) compared with placebo (2.02). At week 28, there were no significant improvements in the major secondary end-points, although a nonsignificant numerically greater Skin Physician Global Assessment response was observed following golimumab treatment (53%) when compared with the placebo (30%). Serious adverse events were similar in all treatment groups. Although treatment was well tolerated, neither ustekinumab nor golimumab demonstrated efficacy in pulmonary sarcoidosis. However, trends towards improvement were observed with golimumab in some dermatological end-points.

  4. Pathogenesis of Sarcoidosis

    PubMed Central

    Gundy, Karl Van; Sharma, Om P.

    1987-01-01

    Sarcoidosis is a multisystem granulomatous disease of unknown etiology. The organs that are involved by sarcoidosis include the lungs in which the granuloma is seen in more than 90% of patients to the pituitary, which is only rarely affected. There are many hypotheses as to the cause of sarcoidosis. Some of them rely on the similarities seen between sarcoidosis and the other granuloma-forming diseases such as tuberculosis, berylliosis, pine pollen inhalation and acute and chronic bacterial and viral infections, while others find similarities between sarcoidosis and immune reactions observed in autoimmune disorders. Still other explanations implicate a genetic predisposition or a still-unknown agent as the underlying cause of the granuloma formation. Images PMID:3310401

  5. [Calcified deep vein thrombosis in a patient with recurrent deep vein thrombosis and sarcoidosis].

    PubMed

    Krmek, Dubravka Zupanić; Brajković, Ivana; Bekić, Dinko; Krnić, Antun; Jurković, Petar; Pavlović, Tomislav

    2013-12-01

    In this article, we present a rare case of calcified deep vein thrombosis in a 42-year-old female patient with frequent relapses of pulmonary sarcoidosis since 1995, for which she was on maintenance therapy with corticosteroids and with consequential secondary diabetes. Recent femoral vein thrombosis was diagnosed with color Doppler in 2012. At the same time, calcified occlusive thrombus in vena cava inferior from the level of renal vein to the confluence of hepatic veins was diagnosed on abdominal multi-slice computed tomography (MSCT). Digital subtraction venography (DSV) revealed a well-developed collateral circulation through the paravertebral veins, azygos and hemiazygos vein. There were no risk factors for thrombosis other than sarcoidosis and diabetes. Deep vein thrombosis is rarely described with sarcoidosis, but according to literature reports, it usually appears as a recurrence and simultaneously at multiple locations. According to the current knowledge, we cannot say for sure whether it is a disease with an increased risk of deep vein thrombosis or there is a combination of multiple risk factors present simultaneously.

  6. Cardiac Sarcoidosis

    MedlinePlus

    ... is Cardiac Sarcoidosis? Sarcoidosis is a poorly understood disease that commonly affects the lungs. It can also involve the lymph nodes, liver, spleen, eyes, skin, bones, salivary glands and heart. ...

  7. Imaging of Sarcoidosis.

    PubMed

    Silva, Mario; Nunes, Hilario; Valeyre, Dominique; Sverzellati, Nicola

    2015-08-01

    The diagnostic imaging contributes significantly to the diagnosis and management of sarcoidosis. Imaging techniques are widely employed in the assessment of thoracic and extra-thoracic involvement from sarcoidosis. For the diagnosis of sarcoidosis, chest radiograph has been the cornerstone of sarcoidosis since 1961, when Scadding proposed a standardized staging system. Currently, computed tomography (CT) represents the reference standard for the assessment of both mediastinal lymph nodes and pulmonary findings. In particular, high-resolution computed tomography (HRCT) is more accurate compared to chest radiography for the detection of subtle parenchymal involvement, and provides comprehensive overview of anatomical detail and abnormalities of lung structures. Notably, HRCT allows for accurate differentiation between reversible and irreversible lung disease, which is cornerstone of prognostication. Radionuclide imaging (gallium-67 and (18)F-fluorodeoxyglucose) provides information about activity of the disease and is also useful for diagnostic workup of patients with unexplained persistent disabling symptoms. Magnetic resonance is sensitive for the detection of sarcoidosis granulomata within myocardium, thus providing detailed roadmap for biopsy. For the management of sarcoidosis, CT is of paramount importance in the detection and differential of most common complications, such as vascular disease and suspicious nodular lesions. Conversely, the role of CT in the follow-up of asymptomatic subjects is still under debate. This review focuses on the role of diagnostic imaging in the diagnosis and follow-up of sarcoidosis.

  8. Musculoskeletal involvement in sarcoidosis*, **

    PubMed Central

    Nessrine, Akasbi; Zahra, Abourazzak Fatima; Taoufik, Harzy

    2014-01-01

    Sarcoidosis is a multisystem inflammatory disorder of unknown cause. It most commonly affects the pulmonary system but can also affect the musculoskeletal system, albeit less frequently. In patients with sarcoidosis, rheumatic involvement is polymorphic. It can be the presenting symptom of the disease or can appear during its progression. Articular involvement is dominated by nonspecific arthralgia, polyarthritis, and Löfgren's syndrome, which is defined as the presence of lung adenopathy, arthralgia (or arthritis), and erythema nodosum. Skeletal manifestations, especially dactylitis, appear mainly as complications of chronic, multiorgan sarcoidosis. Muscle involvement in sarcoidosis is rare and usually asymptomatic. The diagnosis of rheumatic sarcoidosis is based on X-ray findings and magnetic resonance imaging findings, although the definitive diagnosis is made by anatomopathological study of biopsy samples. Musculoskeletal involvement in sarcoidosis is generally relieved with nonsteroidal anti-inflammatory drugs or corticosteroids. In corticosteroid-resistant or -dependent forms of the disease, immunosuppressive therapy, such as treatment with methotrexate or anti-TNF-α, is employed. The aim of this review was to present an overview of the various types of osteoarticular and muscle involvement in sarcoidosis, focusing on their diagnosis and management. PMID:24831403

  9. Tertiary syphilis and Kaposi sarcoma mistaken for systemic sarcoidosis in an HIV-negative patient.

    PubMed

    Anyasodor, M C; Bewley, A

    2015-12-01

    We describe a case of an HIV-negative man who was mistakenly diagnosed as having systemic sarcoidosis, which led to a delay in diagnosing tertiary syphilis and Kaposi sarcoma (KS). The patient presented initially with scrotal swelling and leg oedema. Initial blood tests were unremarkable and HIV testing was negative. The patient then developed unilateral limb weakness. Computed tomography showed lung lesions and hilar lymphadenopathy, while magnetic resonance imaging showed an increased signal in the cervical cord. Serum angiotensin-converting enzyme was raised, and a diagnosis of sarcoidosis was made and the patient started on steroids. Subsequently, his clinical symptoms and radiological abnormalities improved. However, he then developed progressive neurological deficits over several weeks, together with uveitis and cutaneous lesions. A uveitis screen showed a raised venereal disease research laboratory test titre and the cause of his multisystemic symptoms was revisited. He was diagnosed with tertiary syphilis and treated with antibiotics. Dermatologists reviewed the skin lesions and diagnosed KS, which was confirmed with biopsies. The patient's neurological deficit remains. Syphilis should be considered in the differential diagnosis of any patient presenting with neurological problems, skin lesions or symptoms affecting multiple systems. Co-existing KS presented an extra therapeutic challenge in this case.

  10. [Life quality in patients with sarcoidosis and isolated skin syndrome in chronic dermatoses].

    PubMed

    Guryleva, M E

    2003-01-01

    Seventeen patients, including 3 (17.6%) males and 14 (82.4%) females, who had respiratory sarcoidosis with the skin syndrome as erythema nodosum, were examined; their mean age was 41.65 +/- 2.63 years. The histological verification of the diagnosis was 94%. Among 77 patients with chronic dermatoses with the isolated skin syndrome (psoriasis, acne vulgaris, atopic dermatitis, neurodermitis, lichen ruber planus, seborrheal pemphigns, eczema), there were 33 (42.9%) males and 44 (57.1%) females, whose mean age was 36.64 +/- 1.3 years. Comparative analysis of life quality (LQ) in these two groups by using the WHOQOL-100 questionnaire revealed differences in two indices that reflect the level of independence and social relations (p < or = 0.05). In terms of independence, the impact of the disease on LQ was more pronounced in patients with sarcoidosis, its negative effect being in 75% of the criteria rated. Thus, poorer LQ may be stated in patients with skin lesion than in those with the isolated skin syndrome in chronic dermatoses. PMID:14524097

  11. [Bilateral sarcoidosis of parotid glands].

    PubMed

    Hahn, Pernille; Krogdahl, Annelise; Godballe, Christian

    2012-04-23

    We describe an unusual case of sarcoidosis in which the patient presented with a bilateral swelling of the parotid salivary glands and no other manifestation of the disease. Sarcoidosis is a multisystem granulomatous disorder of unknown cause in which there may be multiple exocrine involvement, including the salivary glands. This case emphasises the importance of including sarcoidosis in the differential diagnosis of bilateral parotid swelling. PMID:22533935

  12. Multimodality Imaging in Cardiac Sarcoidosis: Is There a Winner?

    PubMed

    Perez, Irving E; Garcia, Mario J; Taub, Cynthia C

    2016-01-01

    Sarcoidosis is a multisystem granulomatous disease of unknown cause that can affect the heart. Cardiac sarcoidosis may be present in as many as 25% of patients with systemic sarcoidosis, and it is frequently underdiagnosed. The early and accurate diagnosis of myocardial involvement is challenging. Advanced imaging techniques play important roles in the diagnosis and management of patients with cardiac sarcoidosis.

  13. The Circulating Treg/Th17 Cell Ratio Is Correlated with Relapse and Treatment Response in Pulmonary Sarcoidosis Patients after Corticosteroid Withdrawal

    PubMed Central

    Liu, Yongzhe; Qiu, Lan; Wang, Yanxun; Aimurola, Halimulati; Zhao, Yuyue; Li, Shan; Xu, Zuojun

    2016-01-01

    Objectives Pulmonary sarcoidosis is an immune-mediated disease, and some patients can be effectively treated with corticosteroids. However, nearly half of all sarcoidosis patients relapse after corticosteroid withdrawal. Different subsets of CD4+ helper T cells participate in the immunopathogenesis of sarcoidosis. Thus, the aims of our study were to investigate whether the circulating subsets of CD4+ helper T cells were associated with sarcoidosis relapse and with its remission after retreatment. Additionally, we identified a useful biomarker for predicting the relapse and remission of sarcoidosis patients. Methods Forty-two patients were enrolled in the present study who had previously been diagnosed with pulmonary sarcoidosis and treated with corticosteroids. The patients were allocated into either a stable group if they exhibited sustained remission (n = 22) or a relapse group if they experienced clinical or radiological recurrence after treatment withdrawal (n = 20). Peripheral blood cells were collected from these patients and analyzed to determine the frequencies of subsets of circulating CD4+ helper T cells by flow cytometry. The patients in the relapse group were retreated with corticosteroids and immunosuppressive agents and were then reevaluated to determine the frequencies of dynamic subsets of circulating CD4+ helper T cells after remission. Results The frequencies of circulating Tregs were significantly increased concomitant with a decrease in the circulating Th17 cell frequency in the relapsed patients compared with the stable patients. The Treg/Th17 ratio was negatively correlated with sarcoidosis activity and was sensitive to retreatment. In addition, the percentage of isolated CD45RO+Ki67+ Tregs was higher in the patients who were stable and in those who recovered after retreatment than in those who relapsed. Conclusions An imbalance between Tregs and Th17 cells is associated with pulmonary sarcoidosis relapse after corticosteroid withdrawal. The

  14. Diagnosis of Sarcoidosis.

    PubMed

    Wessendorf, Thomas E; Bonella, Francesco; Costabel, Ulrich

    2015-08-01

    The diagnosis of sarcoidosis, a systemic granulomatous disease, is based on a compatible clinical-radiological picture and the histological evidence of noncaseating granulomas. Other diseases mimicking sarcoidosis, mostly infections and other granulomatoses, have to be excluded. There is no single test for sarcoidosis, and the presence of granulomas alone does not establish the diagnosis. Symptoms of sarcoidosis are not specific and can be markedly different according to organ involvement and disease course. Respiratory symptoms and fatigue are the most common symptoms at any stage of disease. Histological confirmation is not needed for Löfgren's or Heerfordt's syndrome and asymptomatic bihilar lymphadenopathy. The radiological staging system is still based on chest radiography, and computed tomography is not mandatory for routine follow-up. (18)F-fluorodeoxyglucose positron emission tomography may be of value in special cases. For assessment of lung involvement and follow-up, pulmonary function tests are necessary with vital capacity being the most important single parameter and diffusion capacity the most sensitive. Bronchoscopy with biopsy is the most common procedure for detection of granulomas, when there is no easier biopsy site like skin or peripheral lymph nodes. Endobronchial ultrasonography-guided transbronchial needle aspiration has replaced mediastinoscopy for evaluation of mediastinal and hilar lymph nodes with a high diagnostic yield. Despite numerous studies, no single biomarker can be reliably used for correct diagnosis or exclusion of sarcoidosis. Genetic testing, despite promising advances, has still not been included in routine care for sarcoidosis patients. The long-term prognosis of sarcoidosis depends on the different organ manifestations: Cardiac or central nervous involvement, together with respiratory complications, is critical. A multidisciplinary approach is necessary for comprehensive care of the sarcoidosis patient.

  15. [Kidney involvement in sarcoidosis].

    PubMed

    Stehlé, T; Boffa, J-J; Lang, P; Desvaux, D; Sahali, D; Audard, V

    2013-09-01

    Sarcoidosis is a chronic multisystemic inflammatory disorder of unknown etiology, characterized by the presence of non-necrotizing epithelioid and giant cell granulomas. Various renal manifestations have been reported in patients with sarcoidosis. Disorders of bone and mineral metabolism related to the overexpression of 25-hydroxyvitamin-D1α-hydroxylase by alveolar and granuloma macrophages are frequently associated with sarcoidosis. Hypercalcemia and hypercalciuria are a major cause of renal injury predisposing to pre renal azotemia, acute tubular necrosis, nephrolithiasis and nephrocalcinosis. Therapeutic management of hypercalcemia includes preventive measures (limited sunlight exposure, limited vitamin D and calcium intakes, and adequate hydration) and specific treatment in cases of severe hypercalcemia (corticosteroid therapy, chloroquine or ketoconazole). Granulomatous tubulointerstitial nephritis is the most common renal lesion associated with sarcoidosis leading to end stage renal disease in some patients. In these cases, interstitial fibrosis seems to appear early in the course of sarcoidosis and is a major prognostic factor requiring rapid corticosteroid therapy to reduce the risk of severe renal impairment. Membranous nephropathy seems to be the most frequent glomerular disease that may occur in association with sarcoidosis. Among kidney allograft recipients, the risk of recurrence of granulomatous tubulointerstitial nephritis is high and may have a negative impact on the graft survival.

  16. [Impact of various millimeter-range electromagnetic radiation schedules on immunological parameters in patients with respiratory sarcoidosis].

    PubMed

    Borisov, S B; Shpykov, A S; Terent'eva, N A

    2007-01-01

    The paper analyzes the impact of various millimeter-range electromagnetic radiation schedules on immunological parameters in 152 patients with new-onset respiratory sarcoidosis. It shows that the immunomodulatory effect of millimeter-range therapy depends on the treatment regimen chosen. There is evidence for the advantages of millimeter-range noise electromagnetic radiation.

  17. FDG PET imaging in sarcoidosis.

    PubMed

    Sobic-Saranovic, Dragana; Artiko, Vera; Obradovic, Vladimir

    2013-11-01

    The objective of this review is to highlight the clinical utility of FDG-PET/CT for evaluation of patients with chronic sarcoidosis. The emphasis was on the potential advantages and disadvantages of this technique in these patients based on which recommendations were made. The advantage of FDG-PET/CT technique is that it can visualize FDG accumulation in activated inflammatory cells and simultaneously provide PET and CT images. Of particular interest is the use of FDG-PET/CT for the staging and identification of occult sites and sites suitable for biopsy and for the assessment of inflammatory active sarcoidosis in patients with prolonged symptoms, especially when other markers of the disease are within normal values. FDG-PET/CT also provides a better visualization of extrathoracic sites of active sarcoidosis, such as in the bones, liver, spleen, and retroperitoneal lymph nodes. The use of FDG-PET/CT is of special interest in cardiac sarcoidosis because this potentially life-threatening disease is sometimes present in asymptomatic patients. FDG-PET/CT also has a role in the clinical management of patients with chronic persistent sarcoidosis, such as for planning treatment, monitoring response, and long-term follow-up. The limitations of FDG-PET/CT in patients with sarcoidosis are discussed in the context of a "sarcoidosis-lymphoma syndrome" and potentially excessive radiation exposure. Further prospective multicentre studies are needed to refine the clinical applications of FDG-PET/CT in patients with sarcoidosis and drive the field forward.

  18. A natural herbal remedy modulates angiogenic activity of bronchoalveolar lavage cells from sarcoidosis patients

    PubMed Central

    Radomska-Leśniewska, Dorota M.; Skopińska-Różewska, Ewa; Demkow, Urszula; Jóźwiak, Jarosław; Sobiecka, Małgorzata

    2016-01-01

    Sarcoidosis is a systemic inflammatory disease with abnormally high angiogenic activity of inflammatory cells. Reumaherb preparation consisting of three herbs: Echinacea purpurea, Harpagophytum procumbens, and Filipendula ulmaria, and it exerts anti-inflammatory, antioxidant, and analgesic activity and stimulates regenerative and immunological processes. The aim of this paper was to estimate the effect of Reumaherb on immunological angiogenesis induced by bronchoalveolar lavage (BAL) cells collected from six patients with sarcoidosis and grafted into Balb/c mice skin. After grafting, the animals were fed for three days with 0.6 or 1.2 mg of Reumaherb (calculated from recommended human daily dose) daily, suspended in 40 µl of water, or 40 µl of water alone (control group). A significant reduction of newly formed blood vessels was obtained in four cases for 1.2 mg and in three cases for 0.6 mg daily dose of this remedy. Thus, we hypothesise that Reumaherb promotes anti-angiogenic activity and may potentially be used in diseases associated with excessive blood vessel formation. PMID:27095919

  19. A natural herbal remedy modulates angiogenic activity of bronchoalveolar lavage cells from sarcoidosis patients.

    PubMed

    Radomska-Leśniewska, Dorota M; Skopińska-Różewska, Ewa; Demkow, Urszula; Jóźwiak, Jarosław; Sobiecka, Małgorzata; Bałan, Barbara Joanna

    2016-01-01

    Sarcoidosis is a systemic inflammatory disease with abnormally high angiogenic activity of inflammatory cells. Reumaherb preparation consisting of three herbs: Echinacea purpurea, Harpagophytum procumbens, and Filipendula ulmaria, and it exerts anti-inflammatory, antioxidant, and analgesic activity and stimulates regenerative and immunological processes. The aim of this paper was to estimate the effect of Reumaherb on immunological angiogenesis induced by bronchoalveolar lavage (BAL) cells collected from six patients with sarcoidosis and grafted into Balb/c mice skin. After grafting, the animals were fed for three days with 0.6 or 1.2 mg of Reumaherb (calculated from recommended human daily dose) daily, suspended in 40 µl of water, or 40 µl of water alone (control group). A significant reduction of newly formed blood vessels was obtained in four cases for 1.2 mg and in three cases for 0.6 mg daily dose of this remedy. Thus, we hypothesise that Reumaherb promotes anti-angiogenic activity and may potentially be used in diseases associated with excessive blood vessel formation. PMID:27095919

  20. New brain lesions in a patient with sarcoidosis: is it neurosarcoidosis?

    PubMed

    Pandey, Subodh; Mukhopadhyay, Sanjay; Iannuzzi, Michael C; Sah, Birendra P

    2014-01-01

    A 45-year-old woman with pulmonary sarcoidosis diagnosed 5 years previously, who was on treatment with prednisone and methotrexate for 1year, developed partial seizure with secondary generalization. MRI showed three non-cavitary enhancing lesions in the cerebello-occipital region. These lesions were presumed to be neurosarcoidosis. Methotrexate was discontinued, prednisone dose was increased and azathiopurine and levetiracetam were added. While on treatment, follow up imaging showed enlarging brain lesions. Biopsy of the lesions showed Epstein Barr virus (EBV) positive diffuse B cell lymphoma. Immunosuppressants were tapered off and she was begun on Rituximab. Because of lack of improvement after 4 cycles of Rituximab, she was then treated with high dose Methotrexate and Temozolamide. We present this case as a diagnostic challenge. New enhancing brain lesions occurring in a patient with long standing sarcoidosis, while likely to be neurosarcoidosis, may be due to a complication of immunosuppressant therapy. The need for early biopsy, if the lesions do not improve, should be considered. PMID:24751455

  1. [Sarcoidosis--a diagnostic and therapeutic problem].

    PubMed

    Wojas-Pelc, A; Setkowicz, M; Lipko-Godlewska, S

    2000-01-01

    Sarcoidosis is a multi-organ granulomatous disorder of an unknown cause. Skin sarcoidosis occurs in about 20-35% of patients with systemic disease and may also arise in isolation. A wide range of clinical presentations of cutaneous sarcoidosis is recognised. The diagnosis rests on the presence of non-caseating granulomas on skin biopsy. Treatment and overall prognosis of cutaneous sarcoidosis is primarily dependent on the degree of systemic involvement.

  2. Exacerbation of pulmonary sarcoidosis after liver transplantation.

    PubMed

    Shibolet, Oren; Kalish, Yossi; Wolf, Dana; Pappo, Orit; Laxer, Uri; Berkman, Neville; Shaham, Dorit; Ashur, Yaffa; Ilan, Yaron

    2002-10-01

    Patients with hepatic sarcoidosis rarely require orthotopic liver transplantation (OLT). Progression of granulomatous activity involving other organs after OLT has rarely been described. We describe a 32-year-old woman who underwent liver transplantation for sarcoidosis-associated end-stage liver disease. She presented 4 years later with shortness of breath, hilar lymphadenopathy, and interstitial lung abnormalities. Liver functions were normal. Open lung biopsy results revealed granulomata compatible with sarcoidosis. The patient made a complete recovery after corticosteroid treatment. To the best of our knowledge, this is a first description of severe exacerbation of pulmonary sarcoidosis in an immunosuppressed patient who underwent liver transplantation for sarcoidosis-associated liver disease.

  3. Cryptococcal meningitis complicating sarcoidosis

    PubMed Central

    Leonhard, Sonja E.; Fritz, Daan; van de Beek, Diederik; Brouwer, Matthijs C.

    2016-01-01

    Abstract Background: Cryptococcal meningitis is an uncommon but severe complication of sarcoidosis. Methods: We present 2 patients with cryptococcal meningitis complicating sarcoidosis and compared findings with 38 cases reported in the literature. Results: When analyzing our patients and 38 cases reported in the literature, we found that median age of sarcoidosis patients with cryptococcal meningitis was 39 years (range 30–48); 27 of 33 reported cases (82%) had a history of sarcoidosis. Only 16 of 40 patients (40%) received immunomodulating therapy at the time of diagnosis of cryptococcal meningitis. The diagnosis of cryptococcal meningitis was delayed in 17 of 40 patients (43%), mainly because of the initial suspicion of neurosarcoidosis. Cerebrospinal fluid (CSF) examination showed mildly elevated white blood cell count (range 23–129/mm3). Twenty-nine of 32 cases (91%) had a positive CSF culture for Cryptococcus neoformans and 25 of 27 cases (93%) had a positive CSF C neoformans antigen test. CD4 counts were low in all patients in whom counts were performed (84–228/mL). Twelve patients had an unfavorable outcome (32%), of which 7 died (19%) and 24 patients (65%) had a favorable outcome. The rate of unfavorable outcome in patients with a delayed diagnosis was 7 of 17 (41%) compared to 5 of 28 (21%) in patients in whom diagnosis was not delayed. Conclusion: Cryptococcal meningitis is a rare but life-threatening complication of sarcoidosis. Patients were often initially misdiagnosed as neurosarcoidosis, which resulted in considerable treatment delay and worse outcome. CSF cryptococcal antigen tests are advised in patients with sarcoidosis and meningitis. PMID:27583871

  4. Bone fragility in sarcoidosis and relationships with calcium metabolism disorders: a cross sectional study on 142 patients

    PubMed Central

    2014-01-01

    Introduction The prevention of fragility fractures in patients with sarcoidosis is a serious concern and the potential risk of hypercalcemia limits vitamin D and calcium supplementation. The objective of this study was to evaluate the risk factors for low bone mineral density (BMD) and fractures in sarcoidosis. In particular, we aimed to determine the link among bone fragility and calcium and vitamin D metabolism in this population. Methods We performed a cross-sectional analysis on 142 consecutive patients with histologically proven sarcoidosis. BMD and prevalence of vertebral fractures on X-rays were assessed and the association with potential risk factors was studied by regression analysis. Results Fragility fractures occurred in 23.5% of patients, despite a normal mean BMD in the study population. In a multivariate analysis, low dietary calcium, fracture, age, gender and menopause were associated with increased risk of low BMD. Low dietary calcium, high current corticosteroid dose and low creatinine clearance were associated with increased risk of fracture. Serum 25(OH)D between 10 and 20 ng/ml was significantly associated with higher BMD. Conversely, values greater than 20 ng/ml were associated with increased risk of fracture. Serum 25(OH)D level was inversely correlated with disease activity. Of note, vitamin D supplements increased serum 25(OH)D in a dose-dependent manner but had no effect on serum calcium level. Conclusions Sarcoidosis patients have a high risk of fracture despite not having a lowered BMD suggesting that other independent factors are involved. Current corticosteroid dose, low dietary calcium and serum 25(OH)D levels are associated with bone fragility. In sarcoidosis, calcium and vitamin D supplementation might be warranted, but desirable 25(OH)D serum levels might be lower than those advised for the general population. PMID:24655357

  5. Risk stratification for major adverse cardiac events and ventricular tachyarrhythmias by cardiac MRI in patients with cardiac sarcoidosis

    PubMed Central

    Yasuda, Masakazu; Iwanaga, Yoshitaka; Kato, Takao; Izumi, Toshiaki; Inuzuka, Yasutaka; Nakamura, Takashi; Miyaji, Yuki; Kawamura, Takayuki; Ikeguchi, Shigeru; Inoko, Moriaki; Kurita, Takashi; Miyazaki, Shunichi

    2016-01-01

    Background The presence of myocardial fibrosis by cardiac MRI has prognostic value in cardiac sarcoidosis, and localisation may be equally relevant to clinical outcomes. Objective We aimed to analyse cardiac damage and function in detail and explore the relationship with clinical outcomes in patients with cardiac sarcoidosis using cardiac MRI. Methods We included 81 consecutive patients with cardiac sarcoidosis undergoing cardiac MR. Left ventricular mass and fibrosis mass were calculated, and localisation was analysed using a 17-segment model. Participants underwent follow-up through 2015, and the development of major adverse cardiac events including ventricular tachyarrhythmias was recorded. Results Increased left ventricular fibrosis mass was associated with increased prevalence of ventricular tachyarrhythmias (p<0.001). When localisation was defined as the sum of late gadolinium enhancement in the left ventricular basal anterior and basal anteroseptal areas, or the right ventricular area, it was associated with ventricular tachyarrhythmias (p<0.001). Kaplan-Meier analysis during a median follow-up of 22.1 months showed that both the mass and localisation groupings for fibrosis were significantly associated with major adverse cardiac events or ventricular tachyarrhythmias and that when combined, the risk stratification was better than for each variable alone (p<0.001, respectively). By Cox-proportional hazard risk analysis, the localisation grouping was an independent predictor for the both. Conclusions In patients with cardiac sarcoidosis, both fibrosis mass and its localisation to the basal anterior/anteroseptal left ventricle, or right ventricle was associated with the development of major adverse cardiac events or ventricular tachyarrhythmias. Cardiac MR with late gadolinium enhancement may be useful for improving risk stratification in patients with cardiac sarcoidosis. PMID:27547432

  6. [Skin sarcoidosis simulating necrobiosis lipoidica].

    PubMed

    Brüngger, A

    1987-04-01

    A patient who had had pulmonary sarcoidosis for 11 years developed multiple symmetrical lesions with ulcerations on the lower legs. The lesions showed the typical clinical picture of necrobiosis lipoidica and could not be differentiated histologically with certainty. Three years later persistent reddish plaques appeared on both arms, which allowed clinical and histological diagnosis of cutaneous sarcoidosis.

  7. Fatigue syndrome in sarcoidosis.

    PubMed

    Górski, Witold; Piotrowski, Wojciech J

    2016-01-01

    Sarcoidosis is an inflammatory disease of unknown etiology. Most commonly it results in the formation of non-caseating granulomas in intrathoracic lymph nodes and lung parenchyma, but the clinical course and picture may be complicated by extrapulmonary involvement and many non-respiratory signs and symptoms which are directly related to the disease. In addition, sarcoidosis patients may suffer from a plethora of symptoms of uncertain or unknown origin. Fatigue is one of these symptoms, and according to some authors it is reported by the majority of patients with active sarcoidosis, but also by a smaller proportion of patients with inactive sarcoidosis, or even with complete clinical and radiological remission. Therefore the term fatigue syndrome is frequently used to name this clinical problem. The definition of fatigue syndrome in sarcoidosis is imprecise and the syndrome is usually recognized by use of validated questionnaires. In this review the uptodate knowledge in this field was presented and different challenges connected with this syndrome were described.

  8. Gastric sarcoidosis: rare presentation of a rare disease.

    PubMed

    Vanderhulst, J

    2015-02-01

    Gastrointestinal sarcoidosis is a rare form of extrapulmonary sarcoidosis. Most of the cases are represented by gastric involvement. We describe a patient with previous systemic sarcoidosis who presented with non-specific abdominal complaints. The workup showed the unusual combination of isolated active gastric sarcoidosis and quiescent activity of the disease elsewhere. We briefly review the clinical, diagnostic and therapeutic aspects of gastric sarcoidosis. We hope to increase awareness about this rare disease.

  9. Consequences of Sarcoidosis.

    PubMed

    Drent, Marjolein; Strookappe, Bert; Hoitsma, Elske; De Vries, Jolanda

    2015-12-01

    Sarcoidosis is a multisystem disorder of unknown cause(s). Less specific disabling symptoms, including fatigue and physical impairments, may have a major influence on the daily activities and the social and professional lives of the patients, resulting in a reduced quality of life. A multidisciplinary approach focusing on somatic and psychosocial aspects is recommended. Patients self-perceived knowledge about the importance of exercise and lifestyle should be improved. Developing the most appropriate therapeutic approach for sarcoidosis requires careful consideration of the possible impact of fatigue, small fiber neuropathy related symptoms, pain, cognitive functioning, and coping strategies. Personalized medicine and appropriate communication are beneficial. PMID:26593145

  10. Granuloma Formation in Pulmonary Sarcoidosis

    PubMed Central

    Broos, Caroline E.; van Nimwegen, Menno; Hoogsteden, Henk C.; Hendriks, Rudi W.; Kool, Mirjam; van den Blink, Bernt

    2013-01-01

    Sarcoidosis is a granulomatous disorder of unknown cause, affecting multiple organs, but mainly the lungs. The exact order of immunological events remains obscure. Reviewing current literature, combined with careful clinical observations, we propose a model for granuloma formation in pulmonary sarcoidosis. A tight collaboration between macrophages, dendritic cells, and lymphocyte subsets, initiates the first steps toward granuloma formation, orchestrated by cytokines and chemokines. In a substantial part of pulmonary sarcoidosis patients, granuloma formation becomes an on-going process, leading to debilitating disease, and sometimes death. The immunological response, determining granuloma sustainment is not well understood. An impaired immunosuppressive function of regulatory T cells has been suggested to contribute to the exaggerated response. Interestingly, therapeutical agents commonly used in sarcoidosis, such as glucocorticosteroids and anti-TNF agents, interfere with granuloma integrity and restore the immune homeostasis in autoimmune disorders. Increasing insight into their mechanisms of action may contribute to the search for new therapeutical targets in pulmonary sarcoidosis. PMID:24339826

  11. Granulomatous Lithiasic Cholecystitis in Sarcoidosis

    PubMed Central

    Handra-Luca, Adriana

    2016-01-01

    Gallbladder granulomas are exceedingly rare, reported in association with tuberculosis or sarcoidosis. Here we report a case of gallbladder granulomatous cholecystitis occurring in the context of sarcoidosis. A 70-years old man presented with abdominal pain, nausea and vomiting. The medical history revealed sarcoidosis diagnosed more than 20-years previously. 2-years previously the patient showed renal lithiasis, hypercalcemia and, increased angiotensin converting enzyme. The imaging features suggested thoraco-abdominal sarcoidosis. Prednisone was given at 1.2 mg/kg/day initially, than decreased, being at 2.5 mg/day at present. The ultrasound examination showed gallbladder lithiasis. A cholecystectomy was performed. Microscopy showed subacute and chronic cholecystitis with several epithelioid and giant cell granulomas some of them perineural. In conclusion, we report a case of granulomatous cholecystitis occurring in the course of treated sarcoidosis. The perineural location of granulomas may give further insights into the pathogenesis of gallbladder dysmotility. PMID:27162601

  12. Successful treatment of refractory midgut bleeding with ocreotide and corticosteroids in a dialysis patient with suspected sarcoidosis.

    PubMed

    Velasco, Nestor; Imtiaz, Toufeeq; Shah, Amir Ali; Koulaouzidis, Anastasios

    2016-01-01

    We present a case of severe and recurrent small-bowel bleeding, due to multiple intestinal angiodysplasias, in a female patient with chronic renal failure due to suspected sarcoidosis. Over the years, she required numerous admissions and >200 units of blood for symptomatic anaemia. However, following a small-bowel capsule endoscopy that revealed several small-bowel angiectasis, she was treated successfully with octreotide and corticosteroids. Her transfusion requirements and hospital admissions were reduced drastically. Moreover, hypercalcaemia and liver function tests also normalised after treatment and double-balloon enteroscopy confirmed the complete resolution of these angiodysplasias. This case presentation confirms the usefulness of octreotide in the management of small-bowel angiodysplasias in dialysis patients and highlights the additional benefit of corticosteroids in portal hypertension due to suspected sarcoidosis. PMID:27417989

  13. Mathematical model of sarcoidosis

    PubMed Central

    Hao, Wenrui; Crouser, Elliott D.; Friedman, Avner

    2014-01-01

    Sarcoidosis is a disease involving abnormal collection of inflammatory cells forming nodules, called granulomas. Such granulomas occur in the lung and the mediastinal lymph nodes, in the heart, and in other vital and nonvital organs. The origin of the disease is unknown, and there are only limited clinical data on lung tissue of patients. No current model of sarcoidosis exists. In this paper we develop a mathematical model on the dynamics of the disease in the lung and use patients’ lung tissue data to validate the model. The model is used to explore potential treatments. PMID:25349384

  14. Interrelationship between sleep-disordered breathing and sarcoidosis.

    PubMed

    Lal, Chitra; Medarov, Boris I; Judson, Marc A

    2015-10-01

    Sleep-disordered breathing (SDB) has a high prevalence in sarcoidosis. This high prevalence may be the result of increased upper airways resistance from sarcoidosis of the upper respiratory tract, corticosteroid-induced obesity, or parenchymal lung involvement from sarcoidosis. OSA is a form of SDB that is particularly common in patients with sarcoidosis. Sarcoidosis and SDB share many similar symptoms and clinical findings, including fatigue, gas exchange abnormalities, and pulmonary hypertension (PH). Sarcoidosis-associated fatigue is a common entity for which stimulants may be beneficial. Sarcoidosis-associated fatigue is a diagnosis of exclusion that requires an evaluation for the possibility of OSA. Hypercapnia is unusual in a patient with sarcoidosis without severe pulmonary dysfunction and, in this situation, should prompt evaluation for alternative causes of hypercapnia, such as SDB. PH is usually mild when associated with OSA, whereas the severity of sarcoidosis-associated PH is related to the severity of sarcoidosis. PH caused by OSA usually responds to CPAP, whereas sarcoidosis-associated PH commonly requires the use of vasodilators. Management of OSA in sarcoidosis is problematic because corticosteroid treatment of sarcoidosis may worsen OSA. Aggressive efforts should be made to place the patient on the lowest effective dose of corticosteroids, which involves early consideration of corticosteroid-sparing agents. Because of the significant morbidity associated with SDB, early recognition and treatment of SDB in patients with sarcoidosis may improve their overall quality of life.

  15. [Pulmonary involvements of sarcoidosis].

    PubMed

    Ohmichi, M; Hiraga, Y; Hirasawa, M

    1990-01-01

    We reported about intrathoracic changes and prognosis of 686 patients with sarcoidosis diagnosed in our hospital between 1963 and 1988. We evaluated CT findings in 135 patients with sarcoidosis and found pulmonary involvements in 81. We analyzed CT findings according to the classification by Tuengerthal which classified radiographic findings combining ILO classification of pneumoconiosis and characteristic findings of bronchovascular sheath with sarcoidosis. The CT findings were as follows: small opacities (44 out of 81 cases, 54.3%), large opacities (37 cases, 46.7%). Additional findings were as follows: peribronchial marking (42 cases, 51.9%), contraction (17 cases, 21.0%), pleural involvement (9 cases, 11.1%), bulla (5 cases, 6.2%). The characteristic CT findings of serious sarcoidosis were extasis of bronchus, thickening of the bronchial wall, unclearness of vascular shadow, atelectasis and thickening of pleura. Concerning the prognosis of pulmonary involvement, according to age, patients younger than 30 years old at initial diagnosis were better than those of 30 years and over in terms of disappearance of pulmonary involvements. According to stage, patients of stage I and stage II were better than those of stage III. Among the patients we were able to observe chest X-ray findings during five years according to the character of shadow, ill-defined shadow of small opacities and rounded shadows of large opacities had a higher disappearance rate of pulmonary involvements than irregular shadows of large opacities, atelectasis and contraction.

  16. Mediastinal Abscess Following Endobronchial Ultrasound Transbronchial Needle Aspiration in a Patient With Sarcoidosis.

    PubMed

    McGovern Murphy, Frederic; Grondin-Beaudoin, Brian; Poulin, Yannick; Boileau, Robert; Dumoulin, Elaine

    2015-10-01

    Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a minimally invasive procedure with a low rate of complications. It is used in the diagnosis of malignant and benign disease such as sarcoidosis. We report a case a 42-year-old man who had undergone EBUS-TBNA for diagnosis of mediastinal and hilar lymph node enlargement. Sarcoidosis was diagnosed on cytologic examination. Three weeks after the procedure, he developed a mediastinal abscess secondary to EBUS-TBNA. Sarcoidosis may be a risk factor for mediastinal infection complication. A local immune defect related to sarcoidosis may explain this risk. Our case underlines the importance of considering and recognizing this complication, and its possibility should be taken into account when undertaking the procedure for benign disease.

  17. Real-time quantitative reverse transcription-polymerase chain reaction to detect propionibacterial ribosomal RNA in the lymph nodes of Chinese patients with sarcoidosis.

    PubMed

    Zhou, Y; Wei, Y-R; Zhang, Y; Du, S-S; Baughman, R P; Li, H-P

    2015-09-01

    The aim of this study was to investigate the diagnostic value of using the copy number of propionibacterial rRNA as a biomarker for sarcoidosis. Ribosomal RNA of Propionibacterium acnes and P. granulosum was measured by real-time quantitative reverse transcription-polymerase chain reaction (RT-PCR) using formalin-fixed and paraffin-embedded tissue of lymph node biopsy from 65 Chinese patients with sarcoidosis, 45 with tuberculosis and 50 controls with other diseases (23 with non-specific lymphadenitis and 27 with mediastinal lymph node metastasis from lung cancer). The receiver operating characteristic (ROC) curve was analysed to determine an optimal cut-off value for diagnosis, and the diagnostic accuracy of the cut-off value was evaluated in additional tissue samples [24 patients with sarcoidosis and 22 with tuberculosis (TB)]. P. acnes or P. granulosum rRNA was detected in 48 of the 65 sarcoidosis samples but only in four of the 45 TB samples and three of the 50 control samples. Analysis of the ROC curve revealed that an optimal cut-off value of the copy number of propionibacterial rRNA for diagnosis of sarcoidosis was 50·5 copies/ml with a sensitivity and specificity of 73·8 and 92·6%, respectively. Based on the cut-off value, 19 of the 24 additional sarcoidosis samples exhibited positive P. acnes or P. granulosum, whereas only one of the 22 additional TB samples was positive, resulting in a sensitivity and specificity of 79·2 and 95·5%, respectively. These findings suggest that propionibacteria might be associated with sarcoidosis granulomatous inflammation. Detection of propionibacterial rRNA by RT-PCR might possibly distinguish sarcoidosis from TB. PMID:25959360

  18. Real-time quantitative reverse transcription-polymerase chain reaction to detect propionibacterial ribosomal RNA in the lymph nodes of Chinese patients with sarcoidosis.

    PubMed

    Zhou, Y; Wei, Y-R; Zhang, Y; Du, S-S; Baughman, R P; Li, H-P

    2015-09-01

    The aim of this study was to investigate the diagnostic value of using the copy number of propionibacterial rRNA as a biomarker for sarcoidosis. Ribosomal RNA of Propionibacterium acnes and P. granulosum was measured by real-time quantitative reverse transcription-polymerase chain reaction (RT-PCR) using formalin-fixed and paraffin-embedded tissue of lymph node biopsy from 65 Chinese patients with sarcoidosis, 45 with tuberculosis and 50 controls with other diseases (23 with non-specific lymphadenitis and 27 with mediastinal lymph node metastasis from lung cancer). The receiver operating characteristic (ROC) curve was analysed to determine an optimal cut-off value for diagnosis, and the diagnostic accuracy of the cut-off value was evaluated in additional tissue samples [24 patients with sarcoidosis and 22 with tuberculosis (TB)]. P. acnes or P. granulosum rRNA was detected in 48 of the 65 sarcoidosis samples but only in four of the 45 TB samples and three of the 50 control samples. Analysis of the ROC curve revealed that an optimal cut-off value of the copy number of propionibacterial rRNA for diagnosis of sarcoidosis was 50·5 copies/ml with a sensitivity and specificity of 73·8 and 92·6%, respectively. Based on the cut-off value, 19 of the 24 additional sarcoidosis samples exhibited positive P. acnes or P. granulosum, whereas only one of the 22 additional TB samples was positive, resulting in a sensitivity and specificity of 79·2 and 95·5%, respectively. These findings suggest that propionibacteria might be associated with sarcoidosis granulomatous inflammation. Detection of propionibacterial rRNA by RT-PCR might possibly distinguish sarcoidosis from TB.

  19. Real-time quantitative reverse transcription–polymerase chain reaction to detect propionibacterial ribosomal RNA in the lymph nodes of Chinese patients with sarcoidosis

    PubMed Central

    Zhou, Y; Wei, Y-R; Zhang, Y; Du, S-S; Baughman, R P; Li, H-P

    2015-01-01

    The aim of this study was to investigate the diagnostic value of using the copy number of propionibacterial rRNA as a biomarker for sarcoidosis. Ribosomal RNA of Propionibacterium acnes and P. granulosum was measured by real-time quantitative reverse transcription–polymerase chain reaction (RT–PCR) using formalin-fixed and paraffin-embedded tissue of lymph node biopsy from 65 Chinese patients with sarcoidosis, 45 with tuberculosis and 50 controls with other diseases (23 with non-specific lymphadenitis and 27 with mediastinal lymph node metastasis from lung cancer). The receiver operating characteristic (ROC) curve was analysed to determine an optimal cut-off value for diagnosis, and the diagnostic accuracy of the cut-off value was evaluated in additional tissue samples [24 patients with sarcoidosis and 22 with tuberculosis (TB)]. P. acnes or P. granulosum rRNA was detected in 48 of the 65 sarcoidosis samples but only in four of the 45 TB samples and three of the 50 control samples. Analysis of the ROC curve revealed that an optimal cut-off value of the copy number of propionibacterial rRNA for diagnosis of sarcoidosis was 50·5 copies/ml with a sensitivity and specificity of 73·8 and 92·6%, respectively. Based on the cut-off value, 19 of the 24 additional sarcoidosis samples exhibited positive P. acnes or P. granulosum, whereas only one of the 22 additional TB samples was positive, resulting in a sensitivity and specificity of 79·2 and 95·5%, respectively. These findings suggest that propionibacteria might be associated with sarcoidosis granulomatous inflammation. Detection of propionibacterial rRNA by RT–PCR might possibly distinguish sarcoidosis from TB. PMID:25959360

  20. Osseous sarcoidosis: a case series.

    PubMed

    Kuzyshyn, Halyna; Feinstein, David; Kolasinski, Sharon L; Eid, Hala

    2015-05-01

    Sarcoidosis is an inflammatory disorder characterized by noncaseating granulomas infiltrating affected organs. Any organ system can be involved, and more than 90 % of patients have a primary pulmonary manifestation. The incidence of radiographically evident bone involvement has been reported over a wide range: from 3 to 39 % depending on the population studied and imaging modalities used. Any bone may be affected in osseous sarcoidosis. Bilateral involvement of the phalanges of the hand and feet is most common. However, reports of long bone, skull, rib and vertebral involvement have appeared. To date, there are no specific tools for the assessment of skeletal disease activity in sarcoidosis. Plain radiograph picks lesions in the small bones of hands and feet greater than does in other bones. Bone scan is useful for defining the extent of the skeletal disease and bone marrow involvement. Magnetic resonance imaging usually demonstrates multifocal lesions within the vertebrae that are hypointense on T1-weighted images and hyperintense on T2-weighted images. In cases of multifocal bone lesions and an established diagnosis of sarcoidosis, a diagnosis of bone sarcoidosis should be considered in the differential diagnosis. Optimal treatment of osseous sarcoidosis remains controversial. We present five cases of multisystem sarcoidosis with skeletal involvement including long bones and vertebrae and a description of immunosuppressive therapies used in our patients. A literature review highlighting the diagnostic approach using radiographic imaging, as well as treatment strategies, is provided.

  1. Pulmonary Sarcoidosis: Diagnosis and Treatment.

    PubMed

    Carmona, Eva M; Kalra, Sanjay; Ryu, Jay H

    2016-07-01

    Sarcoidosis is a chronic granulomatous disease of unknown cause that is seen worldwide and occurs mainly in patients between the ages of 20 and 60 years. It can be difficult to diagnose because it can mimic many other diseases including lymphoproliferative disorders and granulomatous infections and because there is no specific test for diagnosis, which depends on correlation of clinicoradiologic and histopathologic features. This review will focus on recent discoveries regarding the pathogenesis of sarcoidosis, common clinical presentations, diagnostic evaluation, and indications for treatment. This review is aimed largely at general practitioners and emphasizes the importance of differentiating pulmonary sarcoidosis from its common imitators. PMID:27378039

  2. Radioisotope scanning in osseous sarcoidosis

    SciTech Connect

    Rohatgi, P.K.

    1980-01-01

    Technetium-99m (/sup 99m/Tc)-labeled pyrophosphate or diphosphonate compounds and gallium-67 citrate (/sup 67/Ga) are two radionuclide scanning agents that are in widespread use in clinical practice. Technetium-99m pyrophosphate is used extensively for bone scanning to detect metastatic bone disease, benign bone tumors, osteomyelitis, benign hypertrophic osteoarthropathy, and Paget's disease. Only two reports describe abnormal /sup 99m/Tc/ pyrophosphate bone scans in four patients with osseous sarcoidosis. Gallium-67 scans are used primarily to localize neoplastic or inflammatory lesions anywhere in the body. In recent years /sup 67/Ga scans have also been used to detect the presence of both pulmonary and extrapulmonary sarcoidosis, but there are no reports describing abnormal uptake of gallium in patients with osseous sarcoidosis. This report describes experience with radioisotope scanning in two patients with osseous sarcoidosis.

  3. Multisystemic Sarcoidosis Presenting as Pretibial Leg Ulcers.

    PubMed

    Wollina, Uwe; Baunacke, Anja; Hansel, Gesina

    2016-09-01

    Sarcoidosis is a multisystemic disease of unknown etiology. Up to 30% of patients develop cutaneous manifestations, either specific or nonspecific. Ulcerating sarcoidosis leading to leg ulcers is a rare observation that may lead to confusions with other, more common types of chronic leg ulcers. We report the case of a 45-year-old female patient with chronic multisystemic sarcoidosis presenting with pretibial leg ulcers. Other etiology could be excluded. Histology revealed nonspecific findings. Therefore, the diagnosis of nonspecific leg ulcers in sarcoidosis was confirmed. Treatment consisted of oral prednisolone and good ulcer care. Complete healing was achieved within 6 months. Sarcoidosis is a rare cause of leg ulcers and usually sarcoid granulomas can be found. Our patient illustrates that even in the absence of sarcoid granulomas, leg ulcers can be due to sarcoidosis. PMID:27272316

  4. Higher levels of interleukin IL-17 and antigen-specific IL-17 responses in pulmonary sarcoidosis patients with Löfgren's syndrome.

    PubMed

    Ostadkarampour, M; Eklund, A; Moller, D; Glader, P; Olgart Höglund, C; Lindén, A; Grunewald, J; Wahlström, J

    2014-11-01

    Sarcoidosis is a granulomatous disorder of unknown aetiology. The presence of Mycobacterium tuberculosis catalase-peroxidase (mKatG) in sarcoidosis tissue has been reported. T helper type 1 (Th1) responses against mKatG have previously been observed. However, little is known about interleukin (IL)-17 and Th17 responses in sarcoidosis. Here, we investigated the levels of IL-17 and frequencies of IL-17-producing cells responding to mKatG in sarcoidosis patients with different prognosis. Peripheral blood and bronchoalveolar lavage (BAL) cells were obtained from sarcoidosis patients with or without Löfgren's syndrome (often associated with spontaneous recovery), and also stratified according to human leucocyte antigen (HLA) type. Cells producing IL-17 and interferon (IFN)-γ after stimulation with mKatG were enumerated by enzyme-linked immunospot (ELISPOT). The level of IL-17 in the BAL fluid of sarcoidosis patients and healthy controls was measured by quantitative immuno-polymerase chain reaction (qIPCR). We also performed flow cytometry and immunohistochemistry for further characterization of IL-17 expression. Patients with Löfgren's syndrome had a higher frequency of IL-17-producing cells responding to mKatG in BAL fluid compared to patients without Löfgren's syndrome (P < 0·05). The HLA-DR3(+) sarcoidosis patients with Löfgren's syndrome (known to have a particularly good prognosis) also had a clearly higher level of IL-17 in BAL fluid compared to healthy controls and sarcoidosis patients without Löfgren's syndrome (P < 0·01) and (P < 0·05), respectively. No such difference between patient groups was observed with regard to IFN-γ and not with regard to either cytokine in peripheral blood. These findings suggest that IL-17-producing cells may be a useful biomarker for the prognosis of sarcoidosis and play a role in the spontaneous recovery typical of patients with Löfgren's syndrome.

  5. Hepatic sarcoidosis.

    PubMed

    Karagiannidis, Alexandros; Karavalaki, Maria; Koulaouzidis, Anastasios

    2006-01-01

    Sarcoidosis is a multisystem disease of unknown aetiology. Histological evidence of non-caseating granulomas represents the main finding. It affects mostly young people, targeting primary the lung and hilar lymph nodes although liver involvement is often encountered. Hepatic sarcoidosis covers a broad spectrum from asymptomatic hepatic granulomas formation and slightly deranged liver function tests to clinically evident disease with cholestasis or, in advanced cases, cirrhosis and portal hypertension. Other granulomatous diseases (mainly systemic infections like tuberculosis) should be excluded prior to treatment, as longstanding corticosteroid administration is the main stem of therapy. In advanced cases, liver transplantation represents the ultimate therapeutic option.

  6. Progressive kidney failure as the sole manifestation of extrapulmonary sarcoidosis.

    PubMed

    Sethi, Supreet; Relia, Nitin; Syal, Gaurav; Kaushik, Chhavi; Gokden, Neriman; Malik, Ahmad B

    2013-09-01

    Sarcoidosis is a chronic multisystem disorder characterized by an accumulation of T lymphocytes and mononuclear phagocytes, non-caseating epitheliod granulomas and derangement of normal tissue architecture in affected organs. Sarcoidosis can affect any organ system, however approximately 90% of patients with sarcoidosis have pulmonary, lymph node, cutaneous or ocular manifestations. Renal involvement in sarcoidosis is rare and clinically significant renal dysfunction even less common. We present a case of isolated renal sarcoidosis which manifested with progressively worsening renal function and hypercalcemia. A systematic diagnostic approach with pertinent laboratory studies, imaging and renal biopsy elucidated the diagnosis of renal sarcoidosis without any evidence of systemic involvement.

  7. How Is Sarcoidosis Treated?

    MedlinePlus

    ... page from the NHLBI on Twitter. How Is Sarcoidosis Treated? Not everyone who has sarcoidosis needs treatment. ... Content: NEXT >> Featured Video Living With and Managing Sarcoidosis 05/18/2011 This video—presented by the ...

  8. What Causes Sarcoidosis?

    MedlinePlus

    ... page from the NHLBI on Twitter. What Causes Sarcoidosis? The cause of sarcoidosis isn't known. More ... Content: NEXT >> Featured Video Living With and Managing Sarcoidosis 05/18/2011 This video—presented by the ...

  9. Living with Sarcoidosis

    MedlinePlus

    ... page from the NHLBI on Twitter. Living With Sarcoidosis Sarcoidosis has no cure, but you can take ... Content: NEXT >> Featured Video Living With and Managing Sarcoidosis 05/18/2011 This video—presented by the ...

  10. How Is Sarcoidosis Diagnosed?

    MedlinePlus

    ... page from the NHLBI on Twitter. How Is Sarcoidosis Diagnosed? Your doctor will diagnose sarcoidosis based on ... Content: NEXT >> Featured Video Living With and Managing Sarcoidosis 05/18/2011 This video—presented by the ...

  11. Cavitary alveolar sarcoidosis complicated by an aspergilloma

    PubMed Central

    Gera, Kamal; Panjabi, Chandramani; Dash, Devijyoti; Shah, Ashok

    2014-01-01

    Cavitation in pulmonary sarcoidosis is rather unusual and even rarer in the alveolar form of the disease. A review of the literature revealed only 13 documented patients with cavitating alveolar sarcoidosis. Of the 13 patients, clinical information was available in only eight cases. Aspergilloma complicating cavitary alveolar sarcoidosis has been documented only once before. A 38-year-old woman with progressive dyspnoea for 3 years presented owing to a bout of haemoptysis. Imaging prior to presentation demonstrated mediastinal lymphadenopathy and coalescent parenchymal alveolar opacities along with air bronchograms, suggestive of alveolar sarcoidosis. On presentation, cavitations were visible in both lungs within the consolidation along with an intracavitary mass in the left upper lobe, which on evaluation was recognised as an aspergilloma. Bronchoscopy disclosed non-caseating granulomas on histopathology confirming pulmonary sarcoidosis. Cavitary alveolar sarcoidosis with aspergilloma formation can result in a diagnostic dilemma, especially in endemic tuberculous areas. PMID:25326564

  12. Lung function declines in patients with pulmonary sarcoidosis and increased respiratory epithelial permeability to sup 99m Tc-DTPA

    SciTech Connect

    Chinet, T.; Dusser, D.; Labrune, S.; Collignon, M.A.; Chretien, J.; Huchon, G.J. )

    1990-02-01

    Respiratory epithelial clearance of {sup 99m}Tc-DTPA (RC-Tc-DTPA) and pulmonary function tests (PFT) were determined at intervals of 6 or 12 months in 37 untreated, nonsmoking patients with sarcoidosis over a period of 6 to 36 months. PFT included the measurements of total lung capacity (TLC), vital capacity (VC), FEV1, and diffusing capacity for carbon monoxide. No difference was found between the respiratory clearance of {sup 113m}In-DTPA (2.25 +/- 1.00%/min) and RC-Tc-DTPA (2.29 +/- 1.11%/min) in eight patients with pulmonary sarcoidosis. Pulmonary function decreased 15% or more in at least 2 function tests during 11 follow-up periods, but it remained stable during 47 follow-up periods. In patients whose lung function deteriorated, RC-Tc-DTPA increased to 3.51 +/- 1.55%/min; in contrast, in patients whose lung function remained stable, regardless of the initial values, RC-Tc-DTPA was normal (1.00 +/- 0.50%/min; p less than 0.001). In eight patients who were treated with corticosteroids, RC-Tc-DTPA decreased from 3.48 +/- 1.31%/min to 1.56 +/- 0.64%/min (p less than 0.001), and PFT improved. We conclude that in nonsmokers with pulmonary sarcoidosis, increased RC-Tc-DTPA is not related to dissociation of 99mTc from DTPA, RC-Tc-DTPA is increased when pulmonary function decreases, and, when increased, RC-Tc-DTPA decreases with corticosteroid therapy.

  13. Activation of peripheral blood mononuclear cells in bronchoalveolar lavage fluid from patients with sarcoidosis: visualisation of single cell activation products.

    PubMed Central

    Pantelidis, P.; Southcott, A. M.; Cambrey, A. D.; Laurent, G. J.; du Bois, R. M.

    1994-01-01

    BACKGROUND--Interstitial lung diseases are characterised by the recruitment of mononuclear cells to disease sites where maturation occurs and activation products, including lysozyme (LZM), are released. Analysis of in vitro cell culture supernatants for activation products masks the functional heterogeneity of cell populations. It is therefore necessary to examine the secretion of activation products by single cells to assess whether the activation of newly recruited mononuclear phagocytes at the sites of disease in the lung is uniform and controlled by the local microenvironment. METHODS--The reverse haemolytic plaque assay was used to evaluate, at a single cell level, the ability of bronchoalveolar lavage (BAL) fluid from seven patients with sarcoidosis to activate Ficoll-Hypaque-separated peripheral blood mononuclear cells by comparison with BAL fluid from six normal volunteers and nine patients with systemic sclerosis. Monolayers of peripheral blood mononuclear cells and sheep red blood cells were cultured either alone or in the presence of 20% (v/v) BAL fluid with a polyclonal anti-LZM antibody. LZM/anti-LZM complexes bound to red blood cells surrounding the secreting cells were disclosed following complement lysis of red blood cells and quantification of plaque dimensions using microscopy and image analysis. RESULTS--Bronchoalveolar lavage fluid from all the patients with sarcoidosis increased LZM secretion by peripheral blood mononuclear cells compared with unstimulated mononuclear cells. By contrast, BAL fluid from the other individuals had no effect on LZM secretion. CONCLUSIONS--Single cells activated by BAL fluid can be evaluated by the reverse haemolytic plaque assay. BAL fluid from patients with sarcoidosis, but not from patients with systemic sclerosis or normal individuals, contains components capable of activating mononuclear phagocytes to secrete lysozyme. Images PMID:7831632

  14. Expanded lung T-bet+RORγT+ CD4+ T-cells in sarcoidosis patients with a favourable disease phenotype.

    PubMed

    Kaiser, Ylva; Lepzien, Rico; Kullberg, Susanna; Eklund, Anders; Smed-Sörensen, Anna; Grunewald, Johan

    2016-08-01

    Disease phenotypes of pulmonary sarcoidosis are distinguished by clinical rather than immunological criteria. We aimed to characterise patterns of CD4(+) T-cell lineage plasticity underlying the differences in clinical presentation and disease course between the acute form, Löfgren's syndrome, and the heterogeneous, potentially progressive "non-Löfgren" form.33 pulmonary sarcoidosis patients and nine controls underwent bronchoscopy with bronchoalveolar lavage. CD4(+) T-cell transcription factor, chemokine receptor and T-cell receptor expression, proliferation and cytokine production were assessed in the lavage fluid and peripheral blood using flow cytometry and multicolour FluoroSpot.CD4(+) T-cells simultaneously expressing the T-helper cell (Th)1 and Th17 transcriptional regulators T-bet and RORγT (T-bet(+)RORγT(+)) were identified in the lavage, but not blood, of all subjects, and to a significantly higher degree in Löfgren's patients. T-bet(+)RORγT(+) cells proliferated actively, produced interferon (IFN)γ and interleukin (IL)-17A, co-expressed the chemokine receptors CXCR3 and CCR6, and correlated with nonchronic disease. T-cell receptor-restricted Vα2.3(+)Vβ22(+) T-cells strongly co-expressed T-bet/RORγT and CXCR3/CCR6. Cytokine production was more heterogeneous in Löfgren's patients, with significantly higher IL-17A, IL-10, IL-22 and IL-2, but lower IFNγ.Here we demonstrate the presence of lung T-bet(+)RORγT(+)CXCR3(+)CCR6(+) CD4(+) T-cells and Th17-associated cytokines especially in sarcoidosis patients with a favourable prognosis, suggesting a Th1/Th17-permissive environment in the lung with implications for disease resolution. PMID:27230441

  15. Exhaled nitric oxide in sarcoidosis

    PubMed Central

    Wilsher, M; Fergusson, W; Milne, D; Wells, A

    2005-01-01

    Background: Increased production of nitric oxide (NO) by the lower respiratory tract is viewed as a marker of airway inflammation in asthma and bronchiectasis. NO is a potentially important immune modulator, inhibiting the release of several key pro-inflammatory cytokines. As sarcoidosis is characterised by granulomatous airway inflammation, we hypothesised that exhaled NO levels might be raised in sarcoidosis and correlate with the morphological extent and functional severity of disease. Methods: Fifty two patients with sarcoidosis (29 men) of mean age 42 years underwent thin section computed tomography (CT), pulmonary function tests, and measurement of exhaled NO. Results: Exhaled NO levels (median 6.8 ppb, range 2.4–21.8) did not differ significantly from values in 44 control subjects, and were not related to the extent of individual CT abnormalities or the level of pulmonary function impairment. Conclusion: Exhaled NO levels are not increased in pulmonary sarcoidosis. PMID:16244094

  16. Abdominal Sarcoidosis May Mimic Peritoneal Carcinomatosis

    PubMed Central

    Gorkem, Umit; Gungor, Tayfun; Bas, Yılmaz; Togrul, Cihan

    2015-01-01

    Sarcoidosis is a multisystem inflammatory disorder of unknown etiology. It shows a great variety of clinical presentation, organ involvement, and disease progression. Lungs and lymphoid system are the most common sites involved with a frequency of 90% and 30%, respectively. Extrapulmonary involvement of sarcoidosis is reported in 30% of patients and abdomen is the most frequent site. Furthermore, peritoneal involvement is extremely rare in sarcoidosis. The case presented here described peritoneal manifestations of sarcoidosis without involvement of lungs. A 78-year-old woman possessing signs of malignancy on blood test and abdominal magnetic resonance imaging underwent laparatomy with a suspicion of ovarian malignancy. The macroscopic interpretation during surgery was peritoneal carcinomatosis. Total abdominal hysterectomy, bilateral salpingo-oophorectomy, peritoneal biopsies, total omentectomy, and appendectomy were performed. Final histopathological result revealed the diagnosis of sarcoidosis. Clinicians must keep in mind that peritoneal sarcoidosis can mimic intra-abdominal malignancies. PMID:26558122

  17. Sarcoidosis of the upper and lower airways.

    PubMed

    Morgenthau, Adam S; Teirstein, Alvin S

    2011-12-01

    Sarcoidosis is a systemic granulomatous disease of undetermined etiology characterized by a variable clinical presentation and disease course. Although clinical granulomatous inflammation may occur within any organ system, more than 90% of sarcoidosis patients have lung disease. Sarcoidosis is considered an interstitial lung disease that is frequently characterized by restrictive physiologic dysfunction on pulmonary function tests. However, sarcoidosis also involves the airways (large and small), causing obstructive airways disease. It is one of a few interstitial lung diseases that affects the entire length of the respiratory tract - from the nose to the terminal bronchioles - and causes a broad spectrum of airways dysfunction. This article examines airway dysfunction in sarcoidosis. The anatomical structure of the airways is the organizational framework for our discussion. We discuss sarcoidosis involving the nose, sinuses, nasal passages, larynx, trachea, bronchi and small airways. Common complications of airways disease, such as, atelectasis, fibrosis, bullous leions, bronchiectasis, cavitary lesions and mycetomas, are also reviewed. PMID:22082167

  18. Skin changes in sarcoidosis

    PubMed Central

    Cronin, Etain

    1970-01-01

    The skin changes which occur in sarcoidosis are erythema nodosum and specific granulomata. The incidence of erythema nodosum and its frequency in England and Scandinavia is contrasted with its comparative rarity among Negro patients in the United States. The various types of granulomata are described and classified. In contrast with erythema nodosum, granulomata are more common in Negro than in Caucasian patients. ImagesPlate 2 PMID:5481102

  19. A rare clinical presentation of sarcoidosis; gingivitis.

    PubMed

    Güzel, Aygül; Köksal, Nurhan; Aydın, Davut; Aslan, Kerim; Gören, Fikret; Karagöz, Filiz

    2013-10-01

    Gingivitis due to sarcoidosis is a relatively rare condition. Gingivitis or isolated gingival involvement may be the first sign of systemic sarcoidosis. We report the case of a 37 year-old woman with isolated gingivitis due to sarcoidosis confirmed by biopsy. Following treatment with a systemic corticosteroid (prednisolone 40 mg/day), all clinical and radiologic findings were completely improved. In cases of chronic and intractable gingivitis, systemic sarcoidosis should be suspected. It should be confirmed with a biopsy, and the patient should be referred to a chest disease clinic to exclude other organ involvement.

  20. Sarcoidosis in developing countries.

    PubMed

    Jindal, S K; Gupta, D; Aggarwal, A N

    2000-09-01

    Sarcoidosis is seen in different parts of India and other developing countries with almost similar frequency as in the West. It was largely due to lack of awareness and non-availability of investigations for diagnosis that the disease was reported to be rare in the past. A combination of clinical, radiologic, and histologic criteria are used to diagnose sarcoidosis. A confident exclusion of other causes of granuloma formation, especially tuberculosis, is required. Absence of mycobacteria and of caseation in the histologic specimens and presence of skin anergy to tuberculin help make a diagnosis. Transbronchial lung biopsy obtained with the help of fiberoptic bronchoscopy is positive in about 80% of patients. Corticosteroids are used to treat patients with symptoms and those showing active organ involvement. Aggressive treatment is required for patients with acute and severe pulmonary, cardiac, ocular, or neurologic involvements.

  1. Pulmonary Embolism in a Sarcoidosis Patient Double Heterozygous for Methylenetetrahydrofolate Reductase Gene Polymorphisms and Factor V Leiden and Homozygous for the D-Allele of Angiotensin Converting Enzyme Gene

    PubMed Central

    El-Majzoub, Nadim; Mahfouz, Rami; Kanj, Nadim

    2015-01-01

    Sarcoidosis is a multisystem granulomatous disease of unknown etiology and pathogenesis. It presents in patients younger than 40 years of age. The lungs are the most commonly affected organ. Till the present day, there is no single specific test that will accurately diagnose sarcoidosis; as a result, the diagnosis of sarcoidosis relies on a combination of clinical, radiologic, and histologic findings. Patients with sarcoidosis have been found to have an increased risk of pulmonary embolism compared to the normal population. MTHFR and factor V Leiden mutations have been reported to increase the risk of thrombosis in patients. We hereby present a case of a middle aged man with sarcoidosis who developed a right main pulmonary embolism and was found to be double heterozygous for methylenetetrahydrofolate reductase gene polymorphisms and factor V Leiden and homozygous for the D-allele of the angiotensin converting enzyme gene. PMID:26347783

  2. Pulmonary sarcoidosis associated with etanercept therapy.

    PubMed

    Farah, Raymond E; Shay, Moshe D

    2007-10-01

    We present a case of probable pulmonary sarcoidosis associated with the use of etanercept for psoriatic arthritis. Other cases of etanercept-induced granulomas and skin sarcoidosis were recently published in the medical literature, but we found only one case that involved lung sarcoidosis during etanercept therapy. We describe a 40-year-old man who was receiving etanercept for severe psoriatic arthritis and was admitted to the hospital with dyspnea and subfebrile illness several months after the start of treatment. His diagnosis was consistent exclusively with sarcoidosis. The patient's symptoms improved when etanercept was discontinued, but they did not resolve completely. Treatment with prednisone 40 mg led to complete improvement of his pulmonary disease. Etanercept therapy can induce or exacerbate sarcoidosis. The disease disappears when etanercept is discontinued, although treatment with corticosteroids is sometimes required, as in our patient. Use of the Naranjo adverse drug reaction probability scale revealed a probable likelihood (score of 6) that the adverse reaction was related to etanercept. The association of etanercept with sarcoidosis is still a rare finding. This case highlights the importance of monitoring and possibly discontinuing the drug when sarcoidosis is suspected. Patients should be monitored during and after etanercept therapy for manifestations suggesting sarcoidosis, and we recommend patients receive baseline chest radiography at the start of therapy with follow-up of respiratory symptoms.

  3. The Clinical Features of Sarcoidosis: A Comprehensive Review.

    PubMed

    Judson, Marc A

    2015-08-01

    Sarcoidosis has innumerable clinical manifestations, as the disease may affect every body organ. Furthermore, the severity of sarcoidosis involvement may range from an asymptomatic state to a life-threatening condition. This manuscript reviews a wide variety of common and less common clinical characteristics of sarcoidosis. These manifestations are presented organ by organ, although additional sections describe systemic and multiorgan presentations of sarcoidosis. The lung is the organ most commonly involved with sarcoidosis with at least 90 % of sarcoidosis patients demonstrating lung involvement in most series. The skin, eye, liver, and peripheral lymph node are the next most commonly clinically involved organs in most series, with the frequency of involvement ranging from 10 to 30 %. The actual frequency of sarcoidosis organ involvement is probably much higher as it is frequently asymptomatic and may avoid detection. This is particularly common with lung, liver, cardiac, and bone involvement. Cardiac sarcoidosis is present in 25 % of all sarcoidosis but only causes clinical problems in 5 % of them. Nevertheless, unlike sarcoidosis involvement of most other organs, it may be suddenly fatal. Therefore, it is important to screen for cardiac sarcoidosis in all sarcoidosis patients. All sarcoidosis patients should also be screened for eye involvement as asymptomatic patients may have eye involvement that may cause permanent vision impairment. Pulmonary fibrosis from sarcoidosis is usually slowly progressive but may be life-threatening because of the development of respiratory failure, pulmonary hypertension, or hemoptysis related to a mycetoma or bronchiectasis. Some manifestations of sarcoidosis are not organ-specific and probably are the result of a release of mediators from the sarcoid granuloma. Two such manifestations include small fiber neuropathy and fatigue syndromes, and they are observed in a large percentage of patients.

  4. The Clinical Features of Sarcoidosis: A Comprehensive Review.

    PubMed

    Judson, Marc A

    2015-08-01

    Sarcoidosis has innumerable clinical manifestations, as the disease may affect every body organ. Furthermore, the severity of sarcoidosis involvement may range from an asymptomatic state to a life-threatening condition. This manuscript reviews a wide variety of common and less common clinical characteristics of sarcoidosis. These manifestations are presented organ by organ, although additional sections describe systemic and multiorgan presentations of sarcoidosis. The lung is the organ most commonly involved with sarcoidosis with at least 90 % of sarcoidosis patients demonstrating lung involvement in most series. The skin, eye, liver, and peripheral lymph node are the next most commonly clinically involved organs in most series, with the frequency of involvement ranging from 10 to 30 %. The actual frequency of sarcoidosis organ involvement is probably much higher as it is frequently asymptomatic and may avoid detection. This is particularly common with lung, liver, cardiac, and bone involvement. Cardiac sarcoidosis is present in 25 % of all sarcoidosis but only causes clinical problems in 5 % of them. Nevertheless, unlike sarcoidosis involvement of most other organs, it may be suddenly fatal. Therefore, it is important to screen for cardiac sarcoidosis in all sarcoidosis patients. All sarcoidosis patients should also be screened for eye involvement as asymptomatic patients may have eye involvement that may cause permanent vision impairment. Pulmonary fibrosis from sarcoidosis is usually slowly progressive but may be life-threatening because of the development of respiratory failure, pulmonary hypertension, or hemoptysis related to a mycetoma or bronchiectasis. Some manifestations of sarcoidosis are not organ-specific and probably are the result of a release of mediators from the sarcoid granuloma. Two such manifestations include small fiber neuropathy and fatigue syndromes, and they are observed in a large percentage of patients. PMID:25274450

  5. Tumor necrosis factor-alpha antagonist-induced sarcoidosis.

    PubMed

    Clementine, Rochelle Robicheaux; Lyman, Justin; Zakem, Jerald; Mallepalli, Jyothi; Lindsey, Stephen; Quinet, Robert

    2010-09-01

    Sarcoidosis is a multisystem granulomatous disease of unknown etiology. Tumor necrosis factor (TNF)-alpha is an important player in granuloma formation, and recent clinical trials have investigated the efficacy of TNF-alpha inhibitors in sarcoidosis. Paradoxically, there are several case reports in the medical literature describing the development of sarcoidosis in patients treated with TNF-alpha inhibitors. We describe 3 cases of TNF-alpha antagonist-induced sarcoidosis: 1 case of pulmonary, ocular and cutaneous sarcoidosis developing in a patient receiving infliximab for erosive rheumatoid arthritis, 1 case of etanercept-induced sarcoidosis in a patient with seronegative rheumatoid arthritis, and 1 case of sarcoidosis developing in a patient receiving etanercept for erosive rheumatoid arthritis. We also provide a brief discussion on the role of TNF alpha in granuloma formation and implications in the use of TNF-alpha antagonists in autoimmune disease.

  6. [Eye and sarcoidosis].

    PubMed

    Weber, M

    1999-11-15

    Sarcoidosis is a multisystem granulomatous disease, of unknown cause, characterised by non-caseating granulomas. The most common organs involved are the lung, thoracic lymph nodes, skin and eyes. Ophthalmic involvement occurred in approximately 30% of the patients and could be the first manifestation of the disease and preceding pulmonary involvement for many years. If anterior uveitis is the most common ocular manifestation, the posterior segment involvement (intermediate uveitis, vasculitis, choroidal granuloma, macular oedema...) is less common but more visually disabling. The non specific ocular manifestations of the disease make it as a difficult diagnosis. The "suspected sarcoidosis" diagnosis is made with regards to clinical, radiological and (or) biological criteria and the "definitive diagnosis" require the demonstration of a non-caseating, granulomatous, non-infectious inflammation process on biopsy.

  7. Evaluation of Known or Suspected Cardiac Sarcoidosis.

    PubMed

    Blankstein, Ron; Waller, Alfonso H

    2016-03-01

    Sarcoidosis is a multisystem disorder of unknown cause, and cardiac sarcoidosis affects at least 25% of patients and accounts for substantial mortality and morbidity from this disease. Cardiac sarcoidosis may present with heart failure, left ventricular systolic dysfunction, AV block, atrial or ventricular arrhythmias, and sudden cardiac death. Cardiac involvement can be challenging to detect and diagnose because of the focal nature of the disease, as well as the fact that clinical criteria have limited diagnostic accuracy. Nevertheless, the diagnosis of cardiac sarcoidosis can be enhanced by integrating both clinical and imaging findings. This article reviews the various roles that different imaging modalities provide in the evaluation and management of patients with known or suspected cardiac sarcoidosis.

  8. Evaluation of Known or Suspected Cardiac Sarcoidosis.

    PubMed

    Blankstein, Ron; Waller, Alfonso H

    2016-03-01

    Sarcoidosis is a multisystem disorder of unknown cause, and cardiac sarcoidosis affects at least 25% of patients and accounts for substantial mortality and morbidity from this disease. Cardiac sarcoidosis may present with heart failure, left ventricular systolic dysfunction, AV block, atrial or ventricular arrhythmias, and sudden cardiac death. Cardiac involvement can be challenging to detect and diagnose because of the focal nature of the disease, as well as the fact that clinical criteria have limited diagnostic accuracy. Nevertheless, the diagnosis of cardiac sarcoidosis can be enhanced by integrating both clinical and imaging findings. This article reviews the various roles that different imaging modalities provide in the evaluation and management of patients with known or suspected cardiac sarcoidosis. PMID:26926267

  9. Hepatic sarcoidosis: a case series

    PubMed Central

    Ennaifer, Rym; Ayadi, Shema; Romdhane, Hayfa; Cheikh, Myriam; Nejma, Houda Ben; Bougassas, Wassila; Hadj, Najet Bel

    2016-01-01

    Sarcoidosis is a systemic non caseous granulomas disease. Liver is a common location but usually asymptomatic. Evidence based guidelines for this location treatment is lacking and the effect of corticosteroids may be inadequate. The aim of our study was to describe the clinical, biochemical, radiological and therapeutic features of seven patients with systemic sarcoidosis and liver involvement. A retrospective and descriptive monocentric study, over 3 years, including seven patients with systemic sarcoidosis and liver involvement. We included 5 women and 2 men with an average age of 43 years. Hepatic localization revealed sarcoidosis in 5 cases. Hepatomegaly was observed in all patients as well as abnormal serum liver function test reflected by anicteric cholestasis. Liver biopsy, showed in all granulomatous lesions consistent with sarcoidosis and severe fibrosis in 2 cases. Extra-hepatic manifestations were present in all patients represented mainly by pulmonary location. All patients were treated, five by corticosteroid and two with ursodeoxycholic acid (UDCA). Complete response was observed in one case, partial response in another case and corticosteroid refractoriness in one case. In two cases, corticosteroid therapy was introduced for less than 1 month, not allowing assessment of response. Antimalarials in combination with UDCA were used successfully in a patient with steroid-resistant liver disease. Liver involvement can reveal systemic sarcoidois. Given the risk of progression to severe liver disease, it must be screened in all patients with systemic sarcoidosis. Treatment is not systematic, and still based on corticosteroid therapy. In the absence of prospective randomized controlled trials, the efficacy of UDCA need to be proven. PMID:27795804

  10. Advances in the diagnosis and treatment of sarcoidosis

    PubMed Central

    2014-01-01

    This manuscript outlines recent advances in the diagnosis and treatment of sarcoidosis. The diagnosis of sarcoidosis can occasionally be made on clinical grounds without a confirmatory biopsy when very specific clinical findings are present. Otherwise, the diagnosis requires histologic evidence of granulomatous inflammation, exclusion of alternative causes, and evidence of systemic disease. Because there is no available diagnostic test for sarcoidosis, the diagnosis is never completely secure. Instruments have been developed to establish the presence of sarcoidosis in a second organ and hence establish the systemic nature of the disease. Corticosteroids remain the drug of choice for the treatment of sarcoidosis. Additional sarcoidosis medications are most commonly used as corticosteroid-sparing agents. Recent clinical sarcoidosis drug trials have exposed important issues that may confound trial results, including selecting patients with active disease, identifying study drug effects in patients receiving concomitant corticosteroids, and establishing proper study endpoints. PMID:25374667

  11. Foreign body granulomatous reaction to silica, silicone, and hyaluronic acid in a patient with interferon-induced sarcoidosis.

    PubMed

    Novoa, R; Barnadas, M A; Torras, X; Curell, R; Alomar, A

    2013-12-01

    We report the case of a patient who developed sarcoid granulomas 11 months after starting treatment with pegylated interferon alfa and ribavirin for chronic hepatitis C. The sites of the lesions were related to 3 different foreign bodies: silica in old scars on the skin, hyaluronic acid that had been injected into facial tissues, and silicone in an axillary lymph node draining the area of a breast implant. Systemic sarcoidosis was diagnosed on the basis of a history of dry cough and fever and blood tests that revealed elevated angiotensin converting enzyme and liver enzymes. Interruption of the antiviral therapy led to normalization of liver function tests and disappearance of the skin lesions and lymphadenopathies. Dermatologists and cosmetic surgeons should be aware of the risk of sarcoid lesions related to cosmetic implants in patients who may require treatment with interferon in the future.

  12. Gallium scan in intracerebral sarcoidosis

    SciTech Connect

    Makhija, M.C.; Anayiotos, C.P.

    1981-07-01

    Sarcoidosis involving the nervous system probably occurs in about 4% of patients. The usefulness of brain scintigraphy in these cases has been suggested. In this case of cerebral sarcoid granuloma, gallium imaging demonstrated the lesion before treatment and showed disappearance of the lesion after corticosteroid treatment, which correlated with the patient's clinical improvement.

  13. Mycobacterial spindle cell pseudotumour of the brain in a patient with sarcoidosis.

    PubMed

    Ismail, Iyad; Carey, Martyn; Trotter, Simon; Kunst, Heinke

    2015-01-01

    Mycobacterial spindle cell pseudotumours (MSP) are benign lesions characterised by local proliferation of spindle-shaped histiocytes caused by mycobacterial infections. Cerebral MSP due to Mycobacterium avium intracellulare (MAI) infection is rare, and is often misdiagnosed clinically and radiologically as a brain tumour. We present a case with underlying sarcoidosis and known pulmonary MAI infection presenting with partial seizures and headaches. Imaging of the brain revealed a solitary extra axial tumour within the right temporal area. Biopsy of the tumour showed evidence of MPS due to MAI infection. Prolonged treatment with antituberculous therapy showed complete resolution of the cerebral lesion.

  14. Lupus pernio. Radiogallium imaging in a patient with chronic cutaneous sarcoidosis.

    PubMed

    Sulavik, S B; Whitaker, D L; Spencer, R P

    1994-08-01

    A 37-year-old man with a history of chronic skin sarcoidosis had the classic triad of lupus pernio. Each of the lesions (nose, ears, and hands) revealed avid radiogallium accumulation. In addition, both the panda sign and the lambda sign of hilar-mediastinal lymph node uptake of radiogallium were present, despite normal results of chest radiography. Several other lymph node chains were also involved. The case can serve as a baseline to determine if other instances of lupus pernio have such a wide systemic distribution of radiogallium uptake.

  15. Cardiac Involvement in Sarcoidosis: Evolving Concepts in Diagnosis and Treatment

    PubMed Central

    Lynch, Joseph P.; Hwang, Jennifer; Bradfield, Jason; Fishbein, Michael; Shivkumar, Kalyanam; Tung, Roderick

    2014-01-01

    Clinically evident sarcoidosis involving the heart has been noted in at least 2 to 7% of patients with sarcoidosis, but occult involvement is much higher (> 20%). Cardiac sarcoidosis is often not recognized antemortem, as sudden death may be the presenting feature. Cardiac involvement may occur at any point during the course of sarcoidosis and may occur in the absence of pulmonary or systemic involvement. Sarcoidosis can involve any part of the heart, with protean manifestations. Prognosis of cardiac sarcoidosis is related to extent and site(s) of involvement. Most deaths due to cardiac sarcoidosis are due to arrhythmias or conduction defects, but granulomatous infiltration of the myocardium may be lethal. The definitive diagnosis of isolated cardiac sarcoidosis is difficult. The yield of endomyocardial biopsies is low; treatment of cardiac sarcoidosis is often warranted even in the absence of histologic proof. Radionuclide scans are integral to the diagnosis. Currently, 18F-fluorodeoxyglucose positron emission tomography/computed tomography and gadolinium-enhanced magnetic resonance imaging scans are the key imaging modalities to diagnose cardiac sarcoidosis. The prognosis of cardiac sarcoidosis is variable, but mortality rates of untreated cardiac sarcoidosis are high. Although randomized therapeutic trials have not been done, corticosteroids (alone or combined with additional immunosuppressive medications) remain the mainstay of treatment. Because of the potential for sudden cardiac death, implantable cardioverter-defibrillators should be placed in any patient with cardiac sarcoidosis and serious ventricular arrhythmias or heart block, and should be considered for cardiomyopathy. Cardiac transplantation is a viable option for patients with end-stage cardiac sarcoidosis refractory to medical therapy. PMID:25007089

  16. Sarcoidosis and the heart: A review of the literature

    PubMed Central

    Ipek, Emrah; Demirelli, Selami; Ermis, Emrah; Inci, Sinan

    2015-01-01

    Summary Sarcoidosis is a chronic multisystem disorder without any defined etiology. Cardiac sarcoidosis (CS) is detected in 2–7% of patients with sarcoidosis and more than 20% of the cases of sarcoidosis are clinically silent. Cardiac involvement in systemic sarcoidosis (SS) and isolated cardiac sarcoidosis (iCS) are associated with arrhythmia and severe heart failure (HF) and have a poor prognosis. Early diagnosis of CS and prompt initiation of corticosteroid therapy with or without other immunosuppressants is crucial. Electrocardiography, Holter monitoring, and Doppler echocardiography with speckle tracking imaging can serve as the initial steps to diagnosis of CS. Cardiac magnetic resonance (CMR) imaging and positron emission tomography (PET) are promising techniques for both diagnosis and follow-up of CS. This review discusses the main aspects of cardiac involvement in sarcoidosis. PMID:26668777

  17. A case of childhood sarcoidosis.

    PubMed

    Hunt, S J; O'toole, E; Philips, W; Hardman, C; Wakelin, S H; Walters, S

    2002-09-01

    Cutaneous sarcoidosis is rare in children. We report a case of a 5-year-old Bangladeshi girl who presented with fever, a papular eruption on the lower limbs and trunk, malaise, anorexia and weight loss. There was multisystem involvement with marked hepatosplenomegaly, generalized lymphadenopathy, parotid fullness and chronic uveitis. Pulmonary infiltrates were seen on the chest X-ray. Histology of a skin biopsy showed naked noncaseating granulomata and PCR for Mycobacterium tuberculosis was negative. A clinical diagnosis of sarcoidosis was made. The patient was treated with oral prednisolone (2 mg/kg per day). An excellent clinical response with resolution of the rash and improvement of extracutaneous signs was noted within 3 months and she remains well on low-dose prednisolone on alternate days. We discuss the presentation and management of sarcoidosis in children, and highlight the potential difficulty in differentiating this from disseminated tuberculosis.

  18. Sarcoidosis associated with neuromyelitis optica.

    PubMed

    Sawaya, Raja; Radwan, Wael

    2013-08-01

    Neuromyelitis optica (NMO) is an autoimmune disorder diagnosed by an elongated spinal cord lesion associated with unilateral or bilateral optic neuritis and anti-aquaporin 4 (AQP4) antibodies in the serum. It is triggered by or associated with several autoimmune diseases, but not with sarcoidosis. It responds to immunomodulators better than to steroid treatment. Sarcoidosis is an autoimmune disorder which manifests as non-caseating granulomas, usually in the lung parenchyma, but also in other tissues, including the brain. The involvement of the central nervous system in neurosarcoidosis differs considerably from that in neuromyelitis optica and the association of these two diseases concurrently in the same patient is unusual.

  19. Sarcoidosis of the head and neck.

    PubMed

    Badhey, Arvind K; Kadakia, Sameep; Carrau, Ricardo L; Iacob, Codrin; Khorsandi, Azita

    2015-06-01

    Sarcoidosis is a complex disorder that often times involves the head and neck. Despite the presence of strong clinical evidence, tissue diagnosis and imaging is needed for confirmation of the disease. Although typically managed medically, when found in the sinonasal tract or intracranially, it may necessitate the intervention of a rhinologist-skull base surgeon. This article seeks to provide a comprehensive review of head and neck sarcoidosis, as this fascinating disorder often poses a diagnostic and therapeutic challenge. A brief discussion of surgical treatment for pituitary lesions is also provided. Articles from 1997 to 2013 were selected and reviewed by three researchers utilizing the most recent literature regarding sarcoidosis in the head and neck. PubMed searches were conducted using search terms such as "sarcoidosis", "neurosarcoid", and "extra-pulmonary sarcoid", among many others. A large collection of articles was generated and reviewed by the team of authors, and appropriate information was extracted to compose a thorough and expansive review of the subject. 10-15 % of patients with sarcoidosis have head and neck manifestations. Sinonasal and pituitary sarcoidosis presents a diagnostic challenge owing to its non-specific symptoms. Although systemic steroid therapy is often the first time treatment, endoscopic surgery is commonly used to treat advanced pituitary sarcoidosis refractory to medical management. As tissue diagnosis and imaging is key, a multi-disciplinary team approach is advantageous. Our study collates the available literature on head and neck sarcoidosis to provide a comprehensive review of the subject. This provides helpful information to guide all practitioners involved in the care of these challenging patients, namely pathologists, radiologists, otolaryngologists, and skull base surgeons, in the workup and management of head and neck sarcoidosis.

  20. TREM-2 Receptor Expression Increases with 25(OH)D Vitamin Serum Levels in Patients with Pulmonary Sarcoidosis.

    PubMed

    Bucova, Maria; Suchankova, Magda; Tibenska, Elena; Tedlova, Eva; Demian, Juraj; Majer, Ivan; Novosadova, Helena; Tedla, Miroslav

    2015-01-01

    TREM-1 and TREM-2 molecules are members of the TREM transmembrane glycoproteins. In our previous study we identified increased expressions of TREM-1 and TREM-2 receptors in pulmonary sarcoidosis (PS). Only a few studies concerning the association between vitamin D and TREM receptor expression can be found. The aim of our current study was to determine the association between the levels of an inactive form of 25(OH)D vitamin and TREM-1 and TREM-2 receptor expressions. We have detected low levels of 25(OH)D vitamin in 79% of PS patients. Only 21% of patients had normal serum level of 25(OH)D vitamin with values clustered within the low-normal range. The most striking findings were the increased TREM-2 expressions on myeloid cells surfaces in BALF of PS patients with normal 25(OH)D vitamin serum levels compared with those with its decreased levels. The total number of TREM-2 positive cells was 5.7 times higher and the percentage of TREM-2 positive cells was also significantly increased in BALF of PS patients with normal compared to PS patients with low 25(OH)D vitamin serum levels. A significant correlation between total TREM-2 expression and vitamin D levels has been detected too. However, we have not detected similar differences in TREM-1expression and 25(OH)D vitamin serum levels.

  1. TREM-2 Receptor Expression Increases with 25(OH)D Vitamin Serum Levels in Patients with Pulmonary Sarcoidosis

    PubMed Central

    Bucova, Maria; Suchankova, Magda; Tibenska, Elena; Tedlova, Eva; Demian, Juraj; Majer, Ivan; Novosadova, Helena; Tedla, Miroslav

    2015-01-01

    TREM-1 and TREM-2 molecules are members of the TREM transmembrane glycoproteins. In our previous study we identified increased expressions of TREM-1 and TREM-2 receptors in pulmonary sarcoidosis (PS). Only a few studies concerning the association between vitamin D and TREM receptor expression can be found. The aim of our current study was to determine the association between the levels of an inactive form of 25(OH)D vitamin and TREM-1 and TREM-2 receptor expressions. We have detected low levels of 25(OH)D vitamin in 79% of PS patients. Only 21% of patients had normal serum level of 25(OH)D vitamin with values clustered within the low-normal range. The most striking findings were the increased TREM-2 expressions on myeloid cells surfaces in BALF of PS patients with normal 25(OH)D vitamin serum levels compared with those with its decreased levels. The total number of TREM-2 positive cells was 5.7 times higher and the percentage of TREM-2 positive cells was also significantly increased in BALF of PS patients with normal compared to PS patients with low 25(OH)D vitamin serum levels. A significant correlation between total TREM-2 expression and vitamin D levels has been detected too. However, we have not detected similar differences in TREM-1expression and 25(OH)D vitamin serum levels. PMID:26166951

  2. TREM-2 Receptor Expression Increases with 25(OH)D Vitamin Serum Levels in Patients with Pulmonary Sarcoidosis.

    PubMed

    Bucova, Maria; Suchankova, Magda; Tibenska, Elena; Tedlova, Eva; Demian, Juraj; Majer, Ivan; Novosadova, Helena; Tedla, Miroslav

    2015-01-01

    TREM-1 and TREM-2 molecules are members of the TREM transmembrane glycoproteins. In our previous study we identified increased expressions of TREM-1 and TREM-2 receptors in pulmonary sarcoidosis (PS). Only a few studies concerning the association between vitamin D and TREM receptor expression can be found. The aim of our current study was to determine the association between the levels of an inactive form of 25(OH)D vitamin and TREM-1 and TREM-2 receptor expressions. We have detected low levels of 25(OH)D vitamin in 79% of PS patients. Only 21% of patients had normal serum level of 25(OH)D vitamin with values clustered within the low-normal range. The most striking findings were the increased TREM-2 expressions on myeloid cells surfaces in BALF of PS patients with normal 25(OH)D vitamin serum levels compared with those with its decreased levels. The total number of TREM-2 positive cells was 5.7 times higher and the percentage of TREM-2 positive cells was also significantly increased in BALF of PS patients with normal compared to PS patients with low 25(OH)D vitamin serum levels. A significant correlation between total TREM-2 expression and vitamin D levels has been detected too. However, we have not detected similar differences in TREM-1expression and 25(OH)D vitamin serum levels. PMID:26166951

  3. Pathophysiology and clinical management of cardiac sarcoidosis.

    PubMed

    Hamzeh, Nabeel; Steckman, David A; Sauer, William H; Judson, Marc A

    2015-05-01

    Cardiac sarcoidosis is a potentially life-threatening condition characterized by formation of granulomas in the heart, resulting in conduction disturbances, atrial and ventricular arrhythmias, and ventricular dysfunction. The presentation of cardiac sarcoidosis ranges from asymptomatic with an abnormal imaging scan, to palpitations, syncope, symptoms of congestive heart failure, and sudden cardiac death. Screening for cardiac sarcoidosis has not been standardized, but the presence of cardiac symptoms on medical history and physical examination, and an abnormal electrocardiogram (ECG), Holter monitoring, or echocardiogram has been shown to be highly sensitive for detecting cardiac sarcoidosis. A signal-averaged ECG might also have a role in screening for cardiac sarcoidosis in asymptomatic patients. Although endomyocardial biopsies are highly specific for the diagnosis of cardiac sarcoidosis, procedural yield is very low and appropriate findings on cardiac MRI or PET are, therefore, often used as diagnostic surrogates. Treatment for cardiac sarcoidosis usually involves immunosuppressive therapy, particularly corticosteroids. Additional therapy might be required, depending on the clinical presentation, including implantation of an internal defibrillator, antiarrhythmic agents, and catheter ablation.

  4. Abdominal sarcoidosis: cross-sectional imaging findings

    PubMed Central

    Gezer, Naciye Sinem; Başara, Işıl; Altay, Canan; Harman, Mustafa; Rocher, Laurence; Karabulut, Nevzat; Seçil, Mustafa

    2015-01-01

    Sarcoidosis is a multisystem inflammatory disease of unknown etiology. The lungs and the lymphoid system are the most commonly involved organs. Extrapulmonary involvement is reported in 30% of patients, and the abdomen is the most common extrapulmonary site with a frequency of 50%–70%. Although intra-abdominal sarcoidosis is usually asymptomatic, its presence may affect the prognosis and treatment options. The lesions are less characteristic and may mimick neoplastic or infectious diseases such as lymphoma, diffuse metastasis, and granulomatous inflammation. The liver and spleen are the most common abdominal sites of involvement. Sarcoidosis of the gastrointestinal system, pancreas, and kidneys are extremely rare. Adenopathy which is most commonly found in the porta hepatis, exudative ascites, and multiple granulomatous nodules studding the peritoneum are the reported manifestations of abdominal sarcoidosis. Since abdominal sarcoidosis is less common and long-standing, unrecognized disease can result in significant morbidity and mortality. Imaging contributes to diagnosis and management of intra-abdominal sarcoidosis. In this report we reviewed the cross-sectional imaging findings of hepatobiliary, gastrointestinal, and genitourinary sarcoidosis. PMID:25512071

  5. [Subcutaneous sarcoidosis as the first manifestation of systemic disease].

    PubMed

    Calzado, Leticia; Galera, Carmen María; Arrue, Itziar; Rodríguez-Peralto, José L; López, Sara; Guerra, Aurora; Vanaclocha, Francisco

    2005-01-01

    There are dermatological symptoms in up to 25% of patients with sarcoidosis, and the appearance of specific subcutaneous nodules as a manifestation of this entity is rare. They may even predate other manifestations of sarcoidosis. We present the case of a 38-year-old woman with asymptomatic subcutaneous nodules in the limbs, which corresponded to deep sarcoid granulomas in the histological study. She did not present with any extracutaneous indications. The imaging tests performed revealed right paratracheal adenopathies. This led to the diagnosis of sarcoidosis, in both its subcutaneous and pulmonary forms (stage I). Subcutaneous sarcoidosis is probably an underdiagnosed entity, as fewer than 40 cases are reflected in literature. Its value lies in the fact that it may be the first manifestation of extracutaneous or systemic sarcoidosis, which means that this form of sarcoidosis must be considered in the differential diagnosis of subcutaneous nodular lesions; close follow-up of these patients is also necessary.

  6. Improved myocardial strain measured by strain-encoded magnetic resonance imaging in a patient with cardiac sarcoidosis.

    PubMed

    Nakano, Shintaro; Kimura, Fumiko; Osman, Nael; Sugi, Keiki; Tanno, Jun; Uchida, Yoshitaka; Shiono, Ayako; Senbonmatsu, Takaaki; Nishimura, Shigeyuki

    2013-11-01

    A woman aged 64 years with cardiac sarcoidosis responded favourably to corticosteroid therapy in terms of recovered longitudinal myocardial strain, as evaluated by strain-encoded magnetic resonance imaging (SENC-MRI). In contrast, circumferential myocardial strain and late gadolinium enhancement demonstrated minimal improvement, suggesting relatively advanced pathology of the myocardial middle layer. We propose SENC-MRI as a marker of disease at an early stage of cardiac sarcoidosis.

  7. Phospholipase A2 receptor and sarcoidosis-associated membranous nephropathy.

    PubMed

    Stehlé, Thomas; Audard, Vincent; Ronco, Pierre; Debiec, Hanna

    2015-06-01

    Of the glomerulonephritis associated with sarcoidosis, membranous nephropathy (MN) is the most prevalent. Coincidence or a causal relationship between these two diseases is unclear. Here, we present for the first time a high prevalence of PLA2R-related MN among patients with MN associated with active sarcoidosis. Our results suggest some causal link between sarcoidosis and PLA2R-related MN. Detection of anti-PLA2R antibodies in serum or PLA2R antigen in biopsy should not be taken as evidence against a secondary cause, particularly sarcoidosis. This important observation can affect treatment decision in these patients.

  8. sTREM-1 in bronchoalveolar lavage fluid in patients with pulmonary sarcoidosis, effect of smoking and inflammation.

    PubMed

    Suchankova, M; Bucova, M; E, Tibenska; Demian, J; Majer, I; Novosadova, H; Tedlova, E; Durmanova, V; Paulovicova, E

    2013-01-01

    Soluble TREM-1 (sTREM-1; Triggering receptor expressed on myelocytes) is a new inflammatory marker indicating the intensity of myeloid cells activation and the presence of infection caused by extracellular bacteria and mould.The aim of our work was to detect and compare the levels of sTREM-1 in bronchoalveolar lavage fluid (BALF) in patients with pulmonary sarcoidosis (PS) and other ILD of non-infectious origin. The sTREM-1 levels were assessed by ELISA in 46 patients suffering from ILD, out of them 22 with PS. The levels of BALF sTREM-1 in PS patients were higher than in control group of ILD patients of non-infectious origin, however, the difference was not statistically significant. Since all PS patients except one were non-smokers we compared non-smokers PS with non-smokers ILD patients and found four times higher levels of BALF sTREM-1 in PS patients (P = 0.001). We also recorded the effect of smoking, ILD smokers had higher sTREM-1 levels than non-smokers (P = 0.0019). Higher concentrations of sTREM-1 were detected in BALF of patients with lymphadenopathy and with elevated inflammatory markers in BALF. Our results show that BALF sTREM-1 could be a good inflammatory marker and could help in diagnosis and PS monitoring. Detection of sTREM-1 in BALF indirectly points to myeloid cells activation in the lungs and helps to complete the information about the number of myeloid cells commonly determined in BALF with additional information concerning the intensity of their activation. This is the first study that analyses BALF sTREM-1 levels in patients with PS (Tab. 8, Ref. 28). Text in PDF www.elis.sk.

  9. sTREM-1 in bronchoalveolar lavage fluid in patients with pulmonary sarcoidosis, effect of smoking and inflammation.

    PubMed

    Suchankova, M; Bucova, M; E, Tibenska; Demian, J; Majer, I; Novosadova, H; Tedlova, E; Durmanova, V; Paulovicova, E

    2013-01-01

    Soluble TREM-1 (sTREM-1; Triggering receptor expressed on myelocytes) is a new inflammatory marker indicating the intensity of myeloid cells activation and the presence of infection caused by extracellular bacteria and mould.The aim of our work was to detect and compare the levels of sTREM-1 in bronchoalveolar lavage fluid (BALF) in patients with pulmonary sarcoidosis (PS) and other ILD of non-infectious origin. The sTREM-1 levels were assessed by ELISA in 46 patients suffering from ILD, out of them 22 with PS. The levels of BALF sTREM-1 in PS patients were higher than in control group of ILD patients of non-infectious origin, however, the difference was not statistically significant. Since all PS patients except one were non-smokers we compared non-smokers PS with non-smokers ILD patients and found four times higher levels of BALF sTREM-1 in PS patients (P = 0.001). We also recorded the effect of smoking, ILD smokers had higher sTREM-1 levels than non-smokers (P = 0.0019). Higher concentrations of sTREM-1 were detected in BALF of patients with lymphadenopathy and with elevated inflammatory markers in BALF. Our results show that BALF sTREM-1 could be a good inflammatory marker and could help in diagnosis and PS monitoring. Detection of sTREM-1 in BALF indirectly points to myeloid cells activation in the lungs and helps to complete the information about the number of myeloid cells commonly determined in BALF with additional information concerning the intensity of their activation. This is the first study that analyses BALF sTREM-1 levels in patients with PS (Tab. 8, Ref. 28). Text in PDF www.elis.sk. PMID:24329508

  10. Sarcoidosis Occurring After Solid Cancer: A Nonfortuitous Association

    PubMed Central

    Grados, Aurélie; Ebbo, Mikael; Bernit, Emmanuelle; Veit, Véronique; Mazodier, Karin; Jean, Rodolphe; Coso, Diane; Aurran-Schleinitz, Thérèse; Broussais, Florence; Bouabdallah, Reda; Gravis, Gwenaelle; Goncalves, Anthony; Giovaninni, Marc; Sève, Pascal; Chetaille, Bruno; Gavet-Bongo, Florence; Weitten, Thierry; Pavic, Michel; Harlé, Jean-Robert; Schleinitz, Nicolas

    2015-01-01

    Abstract The association between cancer and sarcoidosis is controversial. Some epidemiological studies show an increase of the incidence of cancer in patients with sarcoidosis but only few cases of sarcoidosis following cancer treatment have been reported. We conducted a retrospective case study from internal medicine and oncology departments for patients presenting sarcoidosis after solid cancer treatment. We also performed a literature review to search for patients who developed sarcoidosis after solid cancer. We describe the clinical, biological, and radiological characteristics and outcome of these patients. Twelve patients were included in our study. Various cancers were observed with a predominance of breast cancer. Development of sarcoidosis appeared in the 3 years following cancer and was asymptomatic in half of the patients. The disease was frequently identified after a follow-up positron emission tomography computerized tomography evaluation. Various manifestations were observed but all patients presented lymph node involvement. Half of the patients required systemic therapy. With a median follow-up of 73 months, no patient developed cancer relapse. Review of the literature identified 61 other patients for which the characteristics of both solid cancer and sarcoidosis were similar to those observed in our series. This report demonstrates that sarcoidosis must be considered in the differential diagnosis of patients with a history of malignancy who have developed lymphadenopathy or other lesions on positron emission tomography computerized tomography. Histological confirmation of cancer relapse is mandatory in order to avoid unjustified treatments. This association should be consider as a protective factor against cancer relapse. PMID:26181571

  11. Perineural granulomas in cutaneous sarcoidosis may be associated with sarcoidosis small-fiber neuropathy.

    PubMed

    Munday, William R; McNiff, Jennifer; Watsky, Kalman; DiCapua, Daniel; Galan, Anjela

    2015-07-01

    Perineural granulomas in cutaneous sarcoidosis have been rarely reported and their clinical significance has yet to be evaluated. Recently, a 27-year-old male presented with multiple pink papules on the flank and lower back, accompanied by a painful, burning sensation. Biopsies revealed well-defined granulomas, consistent with sarcoidosis, in the dermis and involving small cutaneous nerves. We hypothesized that perineural granulomas may be an under-recognized feature of cutaneous sarcoidosis and may be responsible for sensory disturbances. We reviewed cases from 29 consecutive patients with cutaneous sarcoidosis. Perineural granulomas were identified in 18/29 (62%) patients and in 22/40 (55%) biopsies. Perineural granulomas were identified in 7/9 biopsies from the proximal upper extremity, 1/3 from the distal upper extremity, 7/12 from the head and the neck, including 4/4 from the nose, 5/9 from the back, 1/2 from the flank and 1/1 from the proximal lower extremity and 0/4 from the distal lower extremity. The anatomical distribution is similar to sarcoidosis small-fiber neuropathy (SSFN), in which sarcoidosis patients without evident skin lesions experience sensory disturbances of unknown etiology involving the face, the proximal extremities and the trunk. Our results indicate perineural granulomas in cutaneous sarcoidosis are more common than previously appreciated, primarily involve the head, the proximal upper extremities and the back, and may be responsible for neurological manifestations.

  12. Circulating immune complexes in sarcoidosis.

    PubMed Central

    Johnson, N M; NcNicol, M W; Burton-Kee, J E; Mowbray, J F

    1980-01-01

    The sera of 50 patients with sarcoidosis were tested for the presence of circulating immune complexes using polyethylene glycol precipitation, followed by single radial immuno diffusion for the amounts of Clg, IgG, IgM, and double diffusion for the presence of IgA. Complexes were detected in 29 (58%) patients. No correlation could be found between the presence of these complexes and the length of history stage, or activity of disease, nor to steroid therapy. Rheumatoid factor was detected in 14 patients (28%), 13 of whom had circulating immune complexes, and 12 of whom had active disease. Total serum C3, CH50, and Clq were normal, as were immunoglobulin levels. In patients with extrathoracic sarcoidosis, especially skin or joint involvement, complexes were commonly found. The aetiological significance of these complexes remains uncertain. PMID:7434270

  13. Disseminated sporotrichosis mimicking sarcoidosis.

    PubMed

    Yang, Deborah J; Krishnan, Ravi S; Guillen, David R; Schmiege, Lorenz M; Leis, Paula F; Hsu, Sylvia

    2006-04-01

    A 40-year-old Caucasian man presented to the dermatology clinic at Baylor College of Medicine, Houston, Texas, in February 2003, for the evaluation of three nonhealing ulcers. The patient's past medical history was significant for hypothyroidism and pulmonary sarcoidosis, the diagnosis of which was made in June 2000. In March 2000, the patient had complained of cough and shortness of breath. A purified protein derivative (PPD) (Mantoux text) was negative. Computed tomography (CT) scans of the chest revealed diffuse hilar and mediastinal adenopathy and bilateral interstitial and alveolar infiltrates. Although consistent with sarcoidosis, these findings were insufficient to exclude other etiologies, including disseminated fungal infection. Cultures and stains of subsequent bronchoscopy specimens failed to reveal any organisms, and histopathologic evaluation of the specimens was nondiagnostic. Based on the imaging studies and the negative cultures, a diagnosis of sarcoidosis was made, and the patient was started on therapy with prednisone. Before coming to our clinic, the patient had been on several courses of prednisone. In May 2002, the patient had presented to a private dermatologist with a 1-year history of a nonhealing 2.4 cm x 2.0 cm ulcer on the left medial forearm. Two biopsies were reported as nondiagnostic. The patient's presentation was interpreted as most consistent with Mycobacterium marinum infection, and so he was empirically treated with minocycline. This treatment was continued for almost 3 months without improvement in the ulcer. A few months after the minocycline had been discontinued, the patient was treated empirically for 2 months with ciprofloxacin. This treatment was also unsuccessful in ameliorating the ulcer. In between the two courses of antibiotics, specimens from the lesion were sent for bacterial and fungal cultures, which revealed normal skin flora. In January 2003, the patient returned to his private dermatologist with three ulcerations

  14. Cutaneous localization of Ga-67 in systemic sarcoidosis

    SciTech Connect

    Rohatgi, P.K.

    1981-03-01

    /sup 67/Ga scans have been used in patients with sarcoidosis to assess noninvasively the intensity of alveolitis and to detect areas of extrathoracic involvement. To the author's knowledge, this is the first report of the localization of gallium in cutaneous lesions of a patient with sarcoidosis.

  15. Genetics of Sarcoidosis.

    PubMed

    Fingerlin, Tasha E; Hamzeh, Nabeel; Maier, Lisa A

    2015-12-01

    Sarcoidosis is a disease with highly variable presentation and progression; although it is hypothesized that disease phenotype is related to genetic variation, how much of this variability is driven by genetic factors is not known. The HLA region is the most strongly and consistently associated genetic risk factor for sarcoidosis, supporting the notion that sarcoidosis is an exposure-mediated immunologic disease. Most of the genetic etiology of sarcoidosis remains unknown in terms of the specific variants that increase risk in various populations, their biologic functions, and how they interact with environmental exposures.

  16. Corticosteroids in Sarcoidosis.

    PubMed

    Judson, Marc A

    2016-02-01

    Corticosteroids are the drug of choice for the treatment of sarcoidosis. Because the natural course of sarcoidosis may be self-limiting and/or cause no long-term harm, treatment is not mandatory. Corticosteroids are usually effective for all forms of sarcoidosis, and they work quickly. However, because of the potential toxicities of corticosteroids, alternative medications often need to be considered. Efforts should be made to minimize the corticosteroid dose while keeping the risk of toxicity as low as possible. This article outlines the indications for corticosteroid therapy for sarcoidosis, discusses various dosing regimens, and suggests when alternative corticosteroid agents should be considered.

  17. Current and emerging pharmacological treatments for sarcoidosis: a review

    PubMed Central

    Beegle, Scott H; Barba, Kerry; Gobunsuy, Romel; Judson, Marc A

    2013-01-01

    The treatment of sarcoidosis is not standardized. Because sarcoidosis may never cause significant symptoms or organ dysfunction, treatment is not mandatory. When treatment is indicated, oral corticosteroids are usually recommended because they are highly likely to be effective in a relative short period of time. However, because sarcoidosis is often a chronic condition, long-term treatment with corticosteroids may cause significant toxicity. Therefore, corticosteroid sparing agents are often indicated in patients requiring chronic therapy. This review outlines the indications for treatment, corticosteroid treatment, and corticosteroid sparing treatments for sarcoidosis. PMID:23596348

  18. Talc granulomatosis mimicking sarcoidosis.

    PubMed

    Iqbal, A; Aggarwal, B; Menon, B; Kulshreshtha, R

    2008-07-01

    Pulmonary disease due to talc, a group of hydrous magnesium silicates, is almost exclusively encountered secondary to occupational exposure or intravenous drug abuse. Talcosis or talc pneumoconiosis is one of the rarer forms of silicate-induced lung disease. It is seen in workers exposed during its production, and occasionally, in users of cosmetic talc and in intravenous drug addicts. Very often, the history of exposure is not recognised by the patient, and it is only the finding of granulomatous cellular interstitial lesions containing birefringent crystals which indicates considerable talc exposure. We report a 38-year-old woman who was initially diagnosed with sarcoidosis, until a bronchoscopic biopsy revealed the presence of numerous foreign body giant cells and birefringent particles forming non-caseating granulomas. There was no history of occupational exposure to talc or intravenous drug abuse. The patient responded to oral corticosteroid treatment. Talcosis is generally considered to be relatively benign.

  19. Sarcoidosis and chronic hepatitis C: a case report.

    PubMed

    Brjalin, Vadim; Salupere, Riina; Tefanova, Valentina; Prikk, Kaiu; Lapidus, Natalia; Jõeste, Enn

    2012-10-28

    Several case reports deal with the relationship between hepatitis C virus (HCV) infection and pulmonary or hepatic sarcoidosis. Most publications describe interferon α-induced sarcoidosis. However, HCV infection per se is also suggested to cause sarcoidosis. The present case report describes a case of biopsy-verified lung and liver sarcoidosis and HCV infection, and the outcome of antiviral therapy. In March 2009, a 25-year-old man presented with moderately elevated liver enzymes without any clinical symptoms. The patient was positive for HCV antibodies and HCV RNA of genotype 1b. Four months later the patient became dyspnoic and pulmonary sarcoidosis was diagnosed by lung biopsy and radiography. A short course of corticosteroid treatment relieved symptoms. Three months later, liver biopsy showed noncaseating granulomas consisting of epithelioid histiocytes and giant cells with a small amount of peripheral lymphocyte infiltration, without any signs of fibrosis. Chronic HCV infection with coexistence of pulmonary and hepatic sarcoidosis was diagnosed. Antiviral therapy with peginterferon α and ribavirin at standard doses was started, which lasted 48 wk, and sustained viral response was achieved. A second liver biopsy showed disappearance of granulomas and chest radiography revealed normalization of mediastinal and perihilar glands. The hypothesis that HCV infection per se may have triggered systemic sarcoidosis was proposed. Successful treatment of HCV infection led to continuous remission of pulmonary and hepatic sarcoidosis. Further studies are required to understand the relationship between systemic sarcoidosis and HCV infection. PMID:23155326

  20. Comorbidity of Leishmania Major with Cutaneous Sarcoidosis

    PubMed Central

    Moravvej, Hamideh; Vesal, Parvaneh; Abolhasani, Ehsan; Nahidi, Shizar; Mahboudi, Fereidoun

    2014-01-01

    Background: leishmaniasis infection might manifest as sarcoidosis; on the other hand, some evidences propose an association between sarcoidosis and leishmaniasis. Most of the times, it is impossible to discriminate idiopathic sarcoidosis from leishmaniasis by conventional histopathologic exam. Aim: We performed a cross-sectional study to examine the association of sarcoidosis with leishmaniasis in histopathologically diagnosed sarcoidal granuloma biopsy samples by polymerase chain reaction (PCR). Materials and Methods: We examined paraffin-embedded skin biopsy samples obtained from patients with clinical and histopathological diagnosis as naked sarcoidal granuloma, referred to Skin Research Center of Shaheed Beheshti Medical University from January 2001 to March 2010, in order to isolate Leishmania parasite. The samples were reassessed by an independent dermatopathologist. DNA extracted from all specimens was analyzed by the commercially available PCR kits (DNPTM Kit, CinnaGen, Tehran, Iran) to detect endemic Leishmania species, namely leishmania major (L. major). Results: L. major was positive in PCR of Eight out of twenty-five examined samples. Conclusion: Cutaneous leishmaniasis may be misinterpreted as sarcoidosis; in endemic areas, when conventional methods fail to detect Leishmania parasite, PCR should be utilized in any granulomatous skin disease compatible with sarcoidosis, regardless of the clinical presentation or histopathological interpretation. PMID:24891680

  1. Diagnosis of sarcoidosis by lip biopsy of minor salivary glands

    PubMed Central

    Tannenbaum, Hyman; Anderson, Larry G.; Rosenberg, Earle H.; Sheffer, Albert L.

    1974-01-01

    In two patients with bilateral parotid gland swelling of unknown etiology the diagnosis of sarcoidosis was established by lip biopsy of the minor salivary glands. This simple, innocuous biopsy procedure may prove useful in tissue documentation of sarcoidosis. ImagesFIG. 1 PMID:4442017

  2. Recognition of distinctive patterns of gallium-67 distribution in sarcoidosis

    SciTech Connect

    Sulavik, S.B.; Spencer, R.P.; Weed, D.A.; Shapiro, H.R.; Shiue, S.T.; Castriotta, R.J. )

    1990-12-01

    Assessment of gallium-67 ({sup 67}Ga) uptake in the salivary and lacrimal glands and intrathoracic lymph nodes was made in 605 consecutive patients including 65 with sarcoidosis. A distinctive intrathoracic lymph node {sup 67}Ga uptake pattern, resembling the Greek letter lambda, was observed only in sarcoidosis (72%). Symmetrical lacrimal gland and parotid gland {sup 67}Ga uptake (panda appearance) was noted in 79% of sarcoidosis patients. A simultaneous lambda and panda pattern (62%) or a panda appearance with radiographic bilateral, symmetrical, hilar lymphadenopathy (6%) was present only in sarcoidosis patients. The presence of either of these patterns was particularly prevalent in roentgen Stages I (80%) or II (74%). We conclude that simultaneous (a) lambda and panda images, or (b) a panda image with bilateral symmetrical hilar lymphadenopathy on chest X-ray represent distinctive patterns which are highly specific for sarcoidosis, and may obviate the need for invasive diagnostic procedures.

  3. What type of different clinical manifestations can cardiac sarcoidosis present?

    PubMed

    Şentürk, Ayşegül; Maraş, Yüksel; Argüder, Emine; Karalezli, Ayşegül; Hasanoğlu, H Canan; Öğüt, Tuba; Baştuğ, Serdal; Karabekir, Ercan

    2015-06-01

    Cardiac sarcoidosis is an infiltrative, granulomatous inflammatory disease of the myocardium. Generally, it can be difficult to diagnose cardiac sarcoidosis clinically because of the non-specific nature of its clinical manifestations. This property can be based on the presence of any clinical evidence of sarcoidosis in the other organs. We present two cases of cardiac sarcoidosis so as to demonstrate its different clinical manifestations. The first patient displayed no cardiac symptoms; the electrocardiogram showed an incidental right bundle branch block. Her cardiac magnetic resonance imaging (CMRI) revealed late-phase opaque material enhancement involving the inferior and inferoseptal segment of the left ventricle. The second patient was severely symptomatic in terms of cardiac involvement, and a transthoracic echocardiogram revealed global hypokinesia and septal brightness; his ejection fraction decreased to 45%. These cases highlighted the challenges encountered in the diagnosis and treatment of cardiac sarcoidosis. CMRI should be considered in all patients who have suspected findings for cardiac involvement.

  4. Sarcoidosis at onset of Psoriasis: a common immunopathogenesis. Review and case report.

    PubMed

    Petroianni, A; Halili, I; Lagalla, M; Mougkaraki, E; Terzano, C

    2015-05-01

    Sarcoidosis is an inflammatory systemic disease that may present in many different ways. The pathophysiological mechanisms are not still well known, although sarcoidosis results from an exaggerated Th1 immune response. About 30% of sarcoidosis patients may suffer from skin lesions during the course of the disease and, occasionally, psoriasiform lesions have been observed. Sarcoidosis may present associated with other diseases and psoriasis is actually one of them, even though not particularly frequent. Few cases of patients who showed clinical and histological features compatible with both pulmonary sarcoidosis and psoriasis vulgaris have been reported. We report an interesting case of a patient affected by sarcoidosis at the onset of psoriasis and discuss immunopathogenetic mechanisms that can be associated with these conditions. Recent data confirm that sarcoidosis is a Th1/Th17 multisystem disorder. These clarifications may be helpful in the management of the diseases and in identifying patients at risk.

  5. Comparison of flexible fiberoptic bronchoscopy and scalene lymph node biopsy in the diagnosis of sarcoidosis.

    PubMed

    Stjernberg, N; Thunell, M; Lundgren, R

    1983-09-01

    The diagnostic accuracy achieved by taking bronchial mucosal and lung biopsies through flexible fiberoptic bronchoscopes was compared with scalene lymph node biopsy in 55 patients with sarcoidosis. The diagnostic yield with flexible fiberoptic bronchoscopy was 62 per cent in the whole material and increased to 76 per cent in patients with stage II sarcoidosis. Scalene lymph node biopsy was positive in 80 per cent. Flexible fiberoptic bronchoscopy is a useful method for obtaining biopsy material in sarcoidosis, especially in stage II sarcoidosis. We consider flexible fiberoptic bronchoscopy and scalene lymph node biopsy the methods of choice in the diagnosis of sarcoidosis. PMID:6628338

  6. Uptake of fluorine-18-fluorodeoxyglucose in sarcoidosis

    SciTech Connect

    Lewis, P.J.; Salama, A.

    1994-10-01

    Whole-body PET scanning was performed using {sup 18}F-fluorodeoxyglucose (FDG) in two patients with hilar lymphadenopathy in whom the clinical differential diagnosis was between sarcoidosis and lymphoma. Both patients were later proven to have sarcoidosis. Uptake of {sup 18}FDG was seen in both intra- and extrathoracic lesions as well as in associated erythema nodosum. One patient underwent a repeat scan after steroid therapy where a marked decrease in hilar uptake was seen. Fluorine-18-fluorodeoxyglucose uptake is observed in lymph nodes with sarcoid involvement. Further investigation is necessary to assess if quantitative differences exist between sarcoid and malignant lymphadenopathy. 30 refs., 3 figs.

  7. [Deafness and sarcoidosis].

    PubMed

    Moine, A; Frachet, B; Van Den Abbeele, T; Tison, P; Battesti, J P

    1990-01-01

    The cochleovestibular tract is seldom involved by sarcoidosis (about 50 cases have been described since 1948). As a clinical expression of sarcoidosis, deafness is fluctuant in 50% of all cases, bilateral, and most often associated with facial palsy and uveitis, the vestibular reflexes being reduced. The histological studies demonstrate lesions at all levels from the cochlea to be brain stem, but the main mechanism is an infiltration of the arachnoid vessels. The prognosis of sarcoidosis deafness is usually poor in spite of corticosteroid therapy. This paper is illustrated by 3 cases observed in Avicenne Hospital.

  8. Granuloma genes in sarcoidosis – what's new?

    PubMed Central

    Fischer, Annegret; Rybicki, Benjamin A.

    2015-01-01

    Purpose of review Non-necrotizing granulomas in the affected organ are the hallmark of sarcoidosis. This review summarizes most recent genetic findings in sarcoidosis with a focus on genes that might influence granuloma formation or resolution. Specific results in multiple ethnic groups and certain clinical subphenotypes, such as extra-pulmonary organ involvement, are discussed. Recent findings Associations of genetic variants in antigen-presenting molecules (HLA-DRB1) were shown to confer risk to sarcoidosis and certain disease phenotypes in populations of different ethnic origin. Specific DRB1 alleles, such as *0301 and *0302, appear to confer protection against chronic disease, but in an ethnic-specific manner illustrating the extensive genetic heterogeneity and complexity at this locus. Mechanistic studies of putative sarcoid antigens lend further credence to a role of HLA-DRB1 in disease pathogenesis. With relevance to granuloma formation, genes involved in apoptotic processes and immune cell activation were further confirmed (ANXA11 and BTNL2) in multiple ethnicities; others were newly identified (XAF1). Linking mechanism to clinical application, a TNF variant was shown to correlate with anti-TNF response in sarcoidosis patients. Summary The investigation of known and novel risk variants for sarcoidosis and specific clinical phenotypes in various ethnicities highlights the genetic complexity of the disease. Detailed subanalysis of disease phenotypes revealed the potential for prediction of extra-pulmonary organ involvement and therapy response based on the patient's genotype. PMID:26164297

  9. Cutaneous sarcoidosis masquerading as psoriatic plaques.

    PubMed

    Argraves, Melissa; Sloan, Steven B; Dadras, Soheil S

    2015-04-16

    Sarcoidosis is a multisystem disease characterized by non-caseating granulomas present in the involved organ systems. The disease is believed to result from an interaction among genetic factors, antigens, and the immune response. Environmental exposures and infectious agents have been implicated as potential causes. Cutaneous sarcoidosis presents clinically in many forms and the lesions are classified as either specific or non-specific. Non-specific lesions show a nondescript inflammatory process whereas specific lesions display typical, non-caseating granulomas. There are many different forms of specific lesions with some being more common than others. Psoriasiform lesions are uncommon. The literature suggests that as few as 0.9% of patients display this type of cutaneous sarcoidosis. Some of these patients present solely with cutaneous sarcoidosis, but others have systemic involvement with pulmonary involvement being the most common concomitant presentation. Plaques appear as round or oval, brownish, red infiltrated lesions, frequently involving the extensor surface of the extremities, face, scalp, back, and buttocks. Multiple configurations, including discrete, confluent, annular, and polycyclic, have been reported. Despite the clinical resemblance to psoriasis, on histological examination, only non-caseating granulomas are seen in the dermis. In rare cases both psoriasiform sarcoidosis and psoriasis were present.

  10. Primary care paradigm for management of sarcoidosis, Part 2.

    PubMed Central

    Young, R. C.; Rachal, R. E.; Nelson-Knuckles, B.; Arthur, C. N.; Nevels, H. V.

    1997-01-01

    Sarcoidosis is a systemic granulomatous disease of undetermined etiology in which the immune system is overstimulated. Management of the patient with sarcoidosis entails continuity of patient care far beyond disease, even into remission. Care is comprehensive, including all involved organ systems, coordination of specialty consultations and services, and includes diagnostic tests. Therapeutic decisions are the responsibility of the primary care practitioner. Prognosis of sarcoidosis is not uniformly good. Patients can die. Subacute sarcoidosis patients usually do not require pharmacologic therapy. Chronic sarcoidosis may require long-term treatment for years to indefinitely. Corticosteroids are still the drugs of choice and other therapy is now available. Health organizations and private nonprofit support groups are available for patient education and assistance. Close support must still come from the family unit. Sarcoidosis may occur in family members in different cohorts at all ages in life and in any racial or ethnic group. The second in a two-part series on management of the patient with sarcoidosis, this article describes coordination of care and considers community, prevention, and family aspects of the disease. Images Figure 1 Figure 2 Figure 3 PMID:9145629

  11. TNF-α: a treatment target or cause of sarcoidosis?

    PubMed

    Amber, K T; Bloom, R; Mrowietz, U; Hertl, M

    2015-11-01

    Sarcoidosis is a systemic granulomatous disease that affects numerous organs, commonly manifesting at the lungs and skin. While corticosteroids remain the first line of treatment, tumour necrosis factor alpha (TNF-α) inhibitors have been investigated as one potential steroid sparing treatment for sarcoidosis. TNF-α is one of many components involved in the formation of granulomas in sarcoidosis. While there have been larger scale studies of biologic TNF-α inhibition in systemic sarcoidosis, studies in cutaneous disease are limited. Paradoxically, in some patients treated with biologic TNF-α inhibitors for other diseases, treatment can induce the development of sarcoidosis. In the light of this complexity, we discuss the role of TNF-α in granuloma formation, the therapeutic role of TNF-α inhibition and immunologic abnormalities following treatment with these TNF-α inhibitors including drug-specific alterations involving interferon-γ, lymphotoxin-α, TNF receptor 2 (TNFR2) and T-regulatory cells.

  12. Foundation for Sarcoidosis Research

    MedlinePlus

    ... have caused a flare up or even the development of their sarcoidosis. The post generated an overwhelming number of responses, as well as productive discussion on genetics, environment, and… 2017 K.I.S.S. 5K in Nashville FSR ...

  13. Interferon-induced sarcoidosis

    PubMed Central

    Cardoso, Cláudia; Freire, Ricardo; Alves, Ana; Oliveira, Ana

    2011-01-01

    Sarcoidosis is a rare side effect of interferon therapy that has been reported over the last years. We present the case of a 43-year-old man presenting with systemic sarcoidosis during treatment with pegylated interferon and ribavirin. Skin lesions, which were found to be associated with asymptomatic bilateral hilar lymph nodes and pulmonary nodules, were the sole clinical manifestation of the disease. Stopping therapy resulted in clinical and radiological improvement. PMID:22696628

  14. Imaging of cardiac sarcoidosis.

    PubMed

    Erthal, Fernanda; Juneau, Daniel; Lim, Siok P; Dwivedi, Girish; Nery, Pablo B; Birnie, David; Beanlands, Rob S

    2016-09-01

    Sarcoidosis is a multisystem inflammatory disease. Cardiac involvement is described in up to 50% of the cases. The disease spectrum is wide and cardiac manifestations ranges from being asymptomatic to heart failure, arrhythmias and sudden cardiac death. The diagnosis of cardiac sarcoidosis can be challenging due to its non-specific nature and the focal involvement of the heart. In this review, we discuss the utility of a stepwise approach with multimodality cardiac imaging in the diagnosis and management of CS. PMID:27225318

  15. Sarcoidosis mimicking metastatic papillary thyroid cancer

    PubMed Central

    Salih, Abdulwahid M.; Fatih, Salah M.; Kakamad, F.H.

    2015-01-01

    Introduction Sarcoidosis is a multisystemic, idiopathic disease. It has a highly variable clinical course. It has been reported to present in association with malignancy. Coexistence of sarcoidosis and thyroid cancer is rarely reported in the literature. Presentatioin of the case We present a case with neck swelling for 3 months, and symmetrical painless thyroid enlargement without fixation to deep tissues of the neck. Multiple nodules on the both thyroid lobes, hard irregular, grade two goiter with lymphadenopathy all over anterior neck compartments. Fine needle aspiration cytology was done under ultrasound guide from right thyroid nodule and showed papillary thyroid carcinoma. Excisional biopsy of the neck lymphnode showed picture typical for sarcoidosis. Discussion Most researchers believe that patients with pulmonary sarcoidosis are predisposed to develop malignancies, less than a dozen of cases are reported in the literature to be associated with PTC with a very wide range of presentations and clincal coarses. An interesting finding of our case is that in contrast to what is reported, both diseases were not known by the physician until the time of presentation. Conclusion Cervical lymphadenopathy in association with goiter could be metastasis, sarcoidosis or mixed, therefore should be seperately biopsied. PMID:26432997

  16. Computed tomography in pulmonary sarcoidosis

    SciTech Connect

    Lynch, D.A.; Webb, W.R.; Gamsu, G.; Stulbarg, M.; Golden, J.

    1989-05-01

    We studied the high resolution CT (HRCT) scans of 15 patients with biopsy-proven sarcoidosis and correlated the findings with pulmonary function tests (12 patients), 67Ga scans (10 patients), bronchoalveolar lavage (five patients), recent transbronchial biopsy (six patients), and recent open lung biopsy (three patients). The HRCT features included small nodules, thickened interlobular septa, patchy focal increase in lung density, honeycombing, and central conglomeration of vessels and bronchi. Active alveolitis was present by gallium scanning criteria in 5 of 10 cases. By bronchoalveolar lavage criteria, activity was present in three of five cases. Patchy increase in density may correlate with active alveolitis as seen on /sup 67/Ga scanning. High resolution CT was better than chest X-radiography for demonstration of patchy increase in density and for distinguishing nodules from septal thickening. Both nodules and patchy density were partly reversible following therapy. Nodular densities seen on CT correlated with the presence of granulomata on histology. Resting pulmonary function tests correlated poorly with presence and extent of lung disease on HRCT. The presence on HRCT of focal fine nodules, patchy focal increase in lung density, and central crowding of bronchi and vessels should suggest the diagnosis of sarcoidosis. In some patients, HRCT can identify unsuspected parenchymal lung disease and document the reversible components of sarcoid lung disease.

  17. Metabolomics connects aberrant bioenergetic, transmethylation, and gut microbiota in sarcoidosis

    PubMed Central

    Geamanu, Andreea; Gupta, Smiti V.; Bauerfeld, Christian

    2016-01-01

    Sarcoidosis is a systemic granulomatous disease of unknown etiology. Granulomatous inflammation in sarcoidosis may affect multiple organs, including the lungs, skin, CNS, and the eyes, leading to severe morbidity and mortality. The underlying mechanisms for sustained inflammation in sarcoidosis are unknown. We hypothesized that metabolic changes play a critical role in perpetuation of inflammation in sarcoidosis. 1H nuclear magnetic resonance (NMR)-based untargeted metabolomic analysis was used to identify circulating molecules in serum to discriminate sarcoidosis patients from healthy controls. Principal component analyses (PCA) were performed to identify different metabolic markers and explore the changes of associated biochemical pathways. Using Chenomx 7.6 NMR Suite software, we identified and quantified metabolites responsible for such separation in the PCA models. Quantitative analysis showed that the levels of metabolites, such as 3-hydroxybutyrate, acetoacetate, carnitine, cystine, homocysteine, pyruvate, and trimethylamine N-oxide were significantly increased in sarcoidosis patients. Interestingly, succinate, a major intermediate metabolite involved in the tricyclic acid cycle was significantly decreased in sarcoidosis patients. Application of integrative pathway analyses identified deregulation of butanoate, ketone bodies, citric cycle metabolisms, and transmethylation. This may be used for development of new drugs or nutritional modification. PMID:27489531

  18. Extrapulmonary localization of gallium in sarcoidosis

    SciTech Connect

    Rohatgi, P.K.; Singh, R.; Vieras, F.

    1987-01-01

    This paper describes the spectrum of extrapulmonary localization of gallium in patients with sarcoidosis. The usefulness of Ga-67 scintiscans in detecting clinically occult lesions, in directing clinicians to accessible sites for biopsy, and in following the course of extrapulmonary sites of involvement with therapy is emphasized.

  19. Cutaneous Sarcoidosis in Blood Donation Venepuncture Sites

    PubMed Central

    Hancock, B. W.

    1972-01-01

    Six patients were studied in whom sarcoidosis first showed itself by the development of granulomata at the site of previous venepunctures. The diagnosis was histologically confirmed in all cases. The treatment of the lesions with triamcinolone did not seem to hasten resolution. ImagesFIG. 1FIG. 2FIG. 3FIG. 4p708-a PMID:4646848

  20. Sarcoidosis and chronic beryllium disease: similarities and differences.

    PubMed

    Mayer, Annyce S; Hamzeh, Nabeel; Maier, Lisa A

    2014-06-01

    Chronic beryllium disease (CBD) is a granulomatous lung disease that may be pathologically and clinically indistinguishable from pulmonary sarcoidosis, except through use of immunologic testing, such as the beryllium lymphocyte proliferation test (BeLPT). Similar to sarcoidosis, the pulmonary manifestations of CBD are variable and overlap with other respiratory diseases. Definitive diagnosis of CBD is established by evidence of immune sensitization to beryllium and diagnostic bronchoscopy with bronchoalveolar lavage and transbronchial biopsy. However, the diagnosis of CBD can also be established on a medically probable basis in beryllium-exposed patients with consistent radiographic imaging and clinical course. Beryllium workers exposed too much higher levels of beryllium in the past demonstrated a much more fulminant disease than is usually seen today. Some extrapulmonary manifestations similar to sarcoidosis were noted in these historic cohorts, although with a narrower spectrum. Extrapulmonary manifestations of CBD are rare today. Since lung-predominant sarcoidosis can very closely resemble CBD, CBD is still misdiagnosed as sarcoidosis when current or past exposure to beryllium is not recognized and no BeLPT is obtained. This article describes the similarities and differences between CBD and sarcoidosis, including clinical and diagnostic features that can help physicians consider CBD in patients with apparent lung-predominant sarcoidosis.

  1. Oligoclonal banding of IgG in CSF, blood-brain barrier function, and MRI findings in patients with sarcoidosis, systemic lupus erythematosus, and Behçet's disease involving the nervous system.

    PubMed Central

    McLean, B N; Miller, D; Thompson, E J

    1995-01-01

    A retrospective study of CSF and serum analysis from a total of 43 patients with sarcoidosis, 20 with systemic lupus erythematosus, and 12 with Behçet's disease with neurological involvement found local synthesis of oligoclonal IgG using isoelectric focusing and immunoblotting in 51%, 25%, and 8% respectively at some stage in their disease. Blood-brain barrier breakdown, when assessed with an albumin ratio found 47% of patients with sarcoidosis, 30% of those with systemic lupus erythematosus, and 42% of patients with Behçet's disease exhibiting abnormal barrier function at some time. Serial CSF analysis showed that clinical relapses were associated with worsening barrier function and in some patients the development of local oligoclonal IgG synthesis; conversely steroid treatment led to a statistically significant improvement in barrier function, and in two patients a loss of oligoclonal IgG bands. A higher proportion of patients had MRI abnormalities than oligoclonal IgG or blood-brain barrier breakdown, MRI being abnormal in 16 of 19 patients with sarcoidosis, three of four patients with systemic lupus erythematosus, and seven of nine patients with Behçet's disease, although this may have been due to temporal factors. In the differential diagnosis of chronic neurological disorders, locally synthesised oligoclonal IgG cannot distinguish between diseases, but the loss of bands seen in two patients contrasts with what is seen in multiple sclerosis, and thus may be a useful diagnostic clue. PMID:7745401

  2. Gallium-67 activity in bronchoalveolar lavage fluid in sarcoidosis

    SciTech Connect

    Trauth, H.A.; Heimes, K.; Schubotz, R.; von Wichert, P.

    1986-01-01

    Roentgenograms and gallium-67 scans and gallium-67 counts of BAL fluid samples, together with differential cell counts, have proved to be useful in assessing activity and lung involvement in sarcoidosis. In active pulmonary sarcoidosis gallium-67 scans are usually positive. Quantitation of gallium-67 uptake in lung scans, however, may be difficult. Because gallium-67 uptake and cell counts in BAL fluid may be correlated, we set out to investigate gallium-67 activity in BAL fluid recovered from patient of different groups. Sixteen patients with recently diagnosed and untreated sarcoidosis, nine patients with healthy lungs, and five patients with CFA were studied. Gallium-67 uptake of the lung, gallium-67 activity in the lavage fluid, SACE and LACE levels, and alpha 1-AT activity were measured. Significantly more gallium-67 activity was found in BAL fluid from sarcoidosis patients than in that from CFA patients (alpha = .001) or patients with healthy lungs (alpha = .001). Gallium-67 activity in BAL fluid could be well correlated with the number of lymphocytes in BAL fluid, but poorly with the number of macrophages. Subjects with increased levels of SACE or serum alpha 1-AT showed higher lavage gallium-67 activity than did normals, but no correlation could be established. High gallium-67 activity in lavage fluid may be correlated with acute sarcoidosis or physiological deterioration; low activity denotes change for the better. The results show that gallium-67 counts in BAL fluid reflects the intensity of gallium-67 uptake and thus of activity of pulmonary sarcoidosis.

  3. Sarcoidosis and chronic hepatitis C: treatment with prednisone and colchicine*

    PubMed Central

    Pereira, Eduardo Guimarães; Guimarães, Tais Ferreira; Bottino, Caroline Bertolini; D’Acri, Antonio Macedo; Lima, Ricardo Barbosa; Martins, Carlos José

    2016-01-01

    Sarcoidosis is a disease which still has uncertain etiology. Possible environmental causes are cited in the literature, like organic and inorganic particles and infectious agents. Recent studies have demonstrated the occurrence of sarcoidosis in patients with chronic C hepatitis; however, this association remains without statistical or causal evidence. In this report a case of sarcoidosis associated with chronic hepatitis C will be described, with subcutaneous lesions, considered rare, and good response to treatment with colchicine and prednisone. The hepatitis C virus was isolated in sarcoid tissue and the association between the two diseases will be discussed. PMID:27192527

  4. Sarcoidosis and chronic hepatitis C: treatment with prednisone and colchicine.

    PubMed

    Pereira, Eduardo Guimarães; Guimarães, Tais Ferreira; Bottino, Caroline Bertolini; D'Acri, Antonio Macedo; Lima, Ricardo Barbosa; Martins, Carlos José

    2016-04-01

    Sarcoidosis is a disease which still has uncertain etiology. Possible environmental causes are cited in the literature, like organic and inorganic particles and infectious agents. Recent studies have demonstrated the occurrence of sarcoidosis in patients with chronic C hepatitis; however, this association remains without statistical or causal evidence. In this report a case of sarcoidosis associated with chronic hepatitis C will be described, with subcutaneous lesions, considered rare, and good response to treatment with colchicine and prednisone. The hepatitis C virus was isolated in sarcoid tissue and the association between the two diseases will be discussed. PMID:27192527

  5. Two cases of interferon-alpha-induced sarcoidosis Koebnerized along venous drainage lines: new pathogenic insights and review of the literature of interferon-induced sarcoidosis.

    PubMed

    Buss, G; Cattin, V; Spring, P; Malinverni, R; Gilliet, M

    2013-01-01

    Sarcoidosis is a systemic granulomatous disorder of unknown origin commonly affecting the lung, the lymphoid system and the skin. We report here two cases of cutaneous sarcoidosis in two former intravenous drug users following interferon (IFN)-α and ribavirin therapy for chronic hepatitis C. Both patients developed skin sarcoidosis along venous drainage lines of both forearms, coinciding with the areas of prior drug injections. The unique distribution of the skin lesions suggests that tissue damage induced by repeated percutaneous drug injections represents a trigger for the local skin manifestation of sarcoidosis. Interestingly, skin damage was recently found to induce the local expression IFN-α, a well-known trigger of sarcoidosis in predisposed individuals. Here we review the literature on sarcoidosis elicited in the context of IFN-α therapy and propose a new link between the endogenous expression of IFN-α and the induction of disease manifestations in injured skin.

  6. Clinical presentation and protocol for management of hepatic sarcoidosis.

    PubMed

    Modaresi Esfeh, Jamak; Culver, Daniel; Plesec, Thomas; John, Binu

    2015-03-01

    The liver is one of the most commonly involved extrapulmonary sites in sarcoidosis. Hepatic sarcoidosis has a broad range of presentations from scattered, asymptomatic noncaseating granulomas with normal liver enzymes, which are very common in patients with known pulmonary sarcoidosis, to portal hypertension and cirrhosis, which are relatively uncommon. Diagnosis is based on a combination of clinical, laboratory and histological manifestations. The authors' protocol for management of patients with suspected sarcoidosis of the liver without focal lesions includes a transjugular liver biopsy with portal pressure measurements to confirm the diagnosis, rule out coexisting liver diseases and to identify select patients with fibrosis or portal hypertension for consideration of immunosuppression. Steroids and azathioprine are the preferred agents and methotrexate is not recommended.

  7. [Systemic sarcoidosis: a diagnostic challenge in pediatrics. Case report].

    PubMed

    Díaz Angarita, Tomás; Morales Camacho, William; Lozano Neira, Laura; Plata Ortiz, Jessica; Zárate Taborda, Linda

    2016-10-01

    Sarcoidosis is a granulomatous inflammatory disease of unknown etiology, evidenced most often in young adults, which can compromise several organs, mainly lung, lymph nodes, eyes and skin. The presence of the disease in pediatric population is rare. Denomination in pediatrics is made based on age and clinical manifestations of the patient. The aim of this report is to present a case of systemic sarcoidosis in a pediatric patient without lung involvement with severe extrapulmonary manifestations. Fifteen year old patient who debuted with nonspecific symptoms (emesis, weight loss). Radiographic studies without lung involvement but with findings suggestive of Langerhans cell histiocytosis; however, histopathological report (bone and lymph) showed the presence of chronic granulomatous nonnecrotizing disease, sarcoidosis type. Childhood sarcoidosis is a rare and difficult entity to diagnose, mainly by the lack of specific biomarkers for diagnosis. PMID:27606655

  8. [Systemic sarcoidosis: a diagnostic challenge in pediatrics. Case report].

    PubMed

    Díaz Angarita, Tomás; Morales Camacho, William; Lozano Neira, Laura; Plata Ortiz, Jessica; Zárate Taborda, Linda

    2016-10-01

    Sarcoidosis is a granulomatous inflammatory disease of unknown etiology, evidenced most often in young adults, which can compromise several organs, mainly lung, lymph nodes, eyes and skin. The presence of the disease in pediatric population is rare. Denomination in pediatrics is made based on age and clinical manifestations of the patient. The aim of this report is to present a case of systemic sarcoidosis in a pediatric patient without lung involvement with severe extrapulmonary manifestations. Fifteen year old patient who debuted with nonspecific symptoms (emesis, weight loss). Radiographic studies without lung involvement but with findings suggestive of Langerhans cell histiocytosis; however, histopathological report (bone and lymph) showed the presence of chronic granulomatous nonnecrotizing disease, sarcoidosis type. Childhood sarcoidosis is a rare and difficult entity to diagnose, mainly by the lack of specific biomarkers for diagnosis.

  9. Analysis of CD4-positive T cell subpopulation in sarcoidosis.

    PubMed Central

    Gerli, R; Darwish, S; Broccucci, L; Minotti, V; Spinozzi, F; Cernetti, C; Bertotto, A; Rambotti, P

    1988-01-01

    Double-labelling immunofluorescence analysis within the CD4+ cell subset was carried out in 27 bronchoalveolar lavage fluids and 11 peripheral blood samples of sarcoidosis patients with anti-TQ1, anti-2H4 and anti-4B4 monoclonal antibodies. Helper/inducer CD4+TQ1-/4B4+ cells were strongly increased in the lung and slightly, but significantly, decreased in the blood of sarcoidosis patients with respect to normal controls. No differences were found in the number of both lung and blood CD4+2H4+ cells between sarcoidosis patients and controls. The findings are further evidence for a compartmentalization of T cell subsets in sarcoidosis. PMID:3263230

  10. [Morpheiform sarcoidosis as atypical manifestation of sarcoidosis. Review of the literature and differential diagnosis].

    PubMed

    Castellanos-González, María; Picazo Talavera, María Remedios

    2016-09-16

    Sarcoidosis is an idiopathic multisystem granulomatous disease that commonly involves the skin in 25% of affected patients. Because lesions assume a vast array of morphologies, a classification dividing them into specific (with presence of typical granulomas in the biopsy) or nonspecific (not containing granulomas) has been proposed. In the first group the variant morpheaform is considered exceptional. We review the cases reported in the literature and describe the possible differential diagnosis. We highlight the importance of recognizing the very atypical presentation of sarcoidosis and its ability to mimic morpheaform or sclerosis diseases in our patients. PMID:27084558

  11. [Morpheiform sarcoidosis as atypical manifestation of sarcoidosis. Review of the literature and differential diagnosis].

    PubMed

    Castellanos-González, María; Picazo Talavera, María Remedios

    2016-09-16

    Sarcoidosis is an idiopathic multisystem granulomatous disease that commonly involves the skin in 25% of affected patients. Because lesions assume a vast array of morphologies, a classification dividing them into specific (with presence of typical granulomas in the biopsy) or nonspecific (not containing granulomas) has been proposed. In the first group the variant morpheaform is considered exceptional. We review the cases reported in the literature and describe the possible differential diagnosis. We highlight the importance of recognizing the very atypical presentation of sarcoidosis and its ability to mimic morpheaform or sclerosis diseases in our patients.

  12. Undiagnosed myocardial sarcoidosis presenting as life threatening cardiac dysrhythmia.

    PubMed

    Davis, Stacy A; Moore, Walter J; Oliver, Alyce M

    2011-04-13

    Sarcoidosis is a systemic disorder of unknown aetiology characterised by its pathological hallmark of non-caseating granuloma. Definitive diagnosis requires compatible clinical and imaging features as well as pathogenic identification of non-caseating granulomas in at least one organ. The disease has a wide variety of clinical and radiological manifestations but is associated with low mortality. However, cardiac involvement which is clinically only identified in 5% significantly worsens prognosis due to complications such as congestive heart failure, ventricular tachyarrhythmia, pulmonary hypertension or conduction disturbance leading to sudden death. Cardiac involvement is implicated in 77-85% of deaths directly related to sarcoidosis. Autopsy series in sarcoidosis patients show cardiac involvement with sarcoidosis in up to 79% cases. This case details the clinical course of a 56-year-old female who had experienced refractory cardiac dysrhythmias for many years in the context of also having biopsy proven pulmonary sarcoidosis. She had failed multiple antiarrhythmics as well as pacemaker and implantable cardioverter defibrillator placement. It was not until she presented as a potentially fatal ventricular tachycardia that the possibility of cardiac involvement from her sarcoidosis was entertained as the aetiology of her cardiac problems. Confirmation of myocardial sarcoidosis with PET CT imaging and subsequent treatment with prednisone resulted in her clinical improvement.

  13. Vanishing ovarian mass: Sarcoidosis.

    PubMed

    Turkay, Rustu; Bakir, Baris; Golabi, Uygar Cenik; Topuz, Samet; Ilhan, Huseyin Ridvan

    2012-01-01

    A woman was referred to our hospital with the working diagnosis of ovarian malignancy. While she was undergoing both clinical and radiological evaluation and monitoring, a decrease in the size of the ovarian mass was noted. After further evaluation via laboratory findings and tissue biopsy, we arrived at a final diagnosis of sarcoidosis, which is very unusual in the ovaries. Our case places emphasis on the importance of considering rare entities, such as ovarian sarcoidosis, and the importance of radiologic changes in solid ovarian mass dimensions over time.

  14. Gastric sarcoidosis: case report and literature review.

    PubMed

    Liang, David B; Price, Jennifer Cohen; Ahmed, Haitham; Farmer, Nicole; Montgomery, Elizabeth A; Giday, Samuel A

    2010-04-01

    Sarcoidosis involving the gastrointestinal tract is extremely rare. Clinically recognizable gastrointestinal system involvement occurs in 0.1% to 0.9% of patients with sarcoidosis. We encountered a 22-year-old African American female admitted to Johns Hopkins Hospital (Baltimore, Maryland) for a 2-week history of fever, chills, eye pain, and abdominal pain. Her abdominal CT scan showed multiple subcentimeter retroperitoneal lymph nodes. An upper endoscopy was performed and discovered an antral nodule that measured about 7 mm and antral gastritis in which biopsies showed active chronic necrotizing granulomatous gastritis. Biopsies of the antral polyp showed focal intestinal metaplasia and active chronic necrotizing granulomatous pattern. Stains for Helicobacter pylori, acid fast, and fungi were negative. A small-bowel series showed no abnormality. Ophthalmologic evaluation revealed panuveitis with bilateral optic disc edema. The patient was later prescribed 60 mg of prednisone by mouth once a day and subsequently her abdominal pain and fever resolved during follow-up 2 months later. This literature review demonstrates the importance in the diagnosis, pathophysiology, clinical manifestations, types of gastric sarcoidosis, major endoscopic findings, and management of gastric sarcoidosis. PMID:20437743

  15. Isolated cutaneous sarcoidosis: a new insight into the old entity.

    PubMed

    Kumar H K, Sharath; M N, Gayathri; M, Bharathi; T, Ravikumar

    2013-08-01

    Cutaneous lesions are a part of common manifestation in multi-systemic sarcoidosis and skin involvement occurs in 20% to 35% of the patients with systemic disease. An exclusive cutaneous involvement is rare and it is reported in about 4%-5% of the patients of sarcoidosis. These lesions have been classified into specific and non-specific, depending on the presence of non- caseating granulomas, on histopathologic studies. In the present study, we are reporting two cases of isolated cutaneous sarcoidosis without systemic manifestations, which is a rare presentation. Sarcoidosis will continue to challenge even the most experienced clinicians with respect to a high degree of suspicion and further investigations for diagnosing and treating this disorder.

  16. Who Is at Risk for Sarcoidosis?

    MedlinePlus

    ... NHLBI on Twitter. Who Is at Risk for Sarcoidosis? Sarcoidosis affects people of all ages and races. ... Content: NEXT >> Featured Video Living With and Managing Sarcoidosis 05/18/2011 This video—presented by the ...

  17. Unexpectedly high prevalence of sarcoidosis in a representative U.S. Metropolitan population.

    PubMed

    Erdal, Barbaros S; Clymer, Bradley D; Yildiz, Vedat O; Julian, Mark W; Crouser, Elliott D

    2012-06-01

    The prevalence of sarcoidosis in the United States is unknown, with estimates ranging widely from 1 to 40 per 100,000. We sought to determine the prevalence of sarcoidosis in our health system compared to other rare lung diseases and to further establish if the prevalence was changing over time. We interrogated the electronic medical records of all patients treated in our health system from 1995 to 2010 (1.48 million patients) using the common ICD9 codes for sarcoidosis (135), lung cancer (162), and several other lung diseases characterized, like sarcoidosis, as "rare lung diseases". The patient demographic information (race, gender, age) was further analyzed to identify signature data patterns. The prevalence of sarcoidosis in our health system increased steadily from 164/100,000 in 1995 to 330/100,000 in 2010, and this trend could not be ascribed simply to changes in patient demographics or patient referral patterns. We further estimate that the prevalence of sarcoidosis exceeds 48 per 100,000 in Franklin County, Ohio, the demographic profile of which is nearly identical to that of the U.S. Sarcoidosis prevalence increased over time relative to lung cancer, a benchmark disease with stable disease prevalence, and exceeded that of other rare lung diseases. We postulate that the observed 2-fold increase in sarcoidosis disease prevalence in our health system is primarily related to improved detection and diagnostic approaches, and we conclude that the actual prevalence of sarcoidosis in central Ohio greatly exceeds current U.S. estimates.

  18. Hepatic sarcoidosis: pathogenesis, clinical context, and treatment options.

    PubMed

    Syed, Umer; Alkhawam, Hassan; Bakhit, Mena; Companioni, Rafael A Ching; Walfish, Aron

    2016-09-01

    Sarcoidosis is typically characterized as a non-caseating granulomatous disease that has the ability to affect multiple different organ systems. Although extra-thoracic sarcoidosis can occur in the presence and also without lung involvement, isolated extra-pulmonary disease is rare. The liver is the third most commonly affected organ system after the lungs and lymph nodes. When discussing hepatic sarcoidosis it is important to keep in mind that many patients in this population may not present as one would typically expect since most of the patients are asymptomatic or have mild presentations. Therefore, the diagnosis can be difficult at times since no single laboratory or imaging study can definitively diagnose this systemic disease. In the rare case of some patients where there is difficulty in discerning between different pathologies, the use of image-guided tissue biopsy may be necessary to establish a diagnosis. At the current time, there are no clear guidelines for the management of hepatic sarcoidosis and are mostly dependent on a patient's clinical status at time of presentation. The current body of research in regard to treatment suggests steroids to be the mainstay of therapy. However, there is a role for additional immunosuppressive therapy in cases where the initial treatment is refractory to steroids. In this manuscript, we discussed the pathogenesis of liver sarcoidosis and context of its presentation. In addition, the differential diagnosis and imaging evaluation in this population is discussed. Finally, treatment options are reviewed in setting of previous studies for liver sarcoidosis. PMID:27175775

  19. [Roentgenological semiotics of sarcoidosis].

    PubMed

    Terpigorev, S A; Stashuk, G A; Dubrova, S E

    2008-01-01

    The aim of this review was to summarize semiotics of X-ray and CT-observable manifestations of intrathoracic sarcoidosis and clarify the role of conventional X-ray examination and CT (including high resolution CT) in the diagnosis of this disease and its complications. Also analysed are changes in pulmonary parenchyma compared with those detected in morphological studies.

  20. [Diagnosis and treatment of sarcoidosis. Current standards].

    PubMed

    Frye, B C; Schupp, J C; Köhler, T L; Müller-Quernheim, J

    2015-12-01

    Sarcoidosis is a granulomatous disease that mainly affects the lungs and intrathoracic lymph nodes; however, virtually any organ can be affected. As an orphan disease, recommendations are mainly based on observational or small randomized studies as well as experts' opinion. Diagnosing sarcoidosis requires proof of non-necrotizing granulomas in patients with a compatible symptomatic pattern and the exclusion of other granulomatous diseases. Granulomas can be detected best in the lungs or intrathoracic lymph nodes. Therefore, bronchoscopy and endobronchial ultrasound with biopsies of lymph nodes are the major tools to diagnose sarcoidosis. Frequently, close follow-up and symptomatic therapy are sufficient to allow for spontaneous resolution. In case of functional organ impairment, cardial or CNS involvement, or other complications, steroid therapy is necessary with a starting dose of 0.5 mg/kg body weight that should be tapered-off over 6-12 months. Steroid-refractory disease can be treated by adding methotrexate or azathioprine, two drugs long known in sarcoidosis treatment. Monoclonal antibodies against TNF and lung transplantation are further therapeutic options.

  1. A rare comorbidity: neurosarcoidosis and cutaneous sarcoidosis.

    PubMed

    Akyol, Lütfi; Aslan, Kerim; Özgen, Metin; Sayarlioglu, Mehmet

    2015-01-01

    We present a case of a neurosarcoidosis patient with skin lesions. A 50-year-old woman was admitted with a 1-year history of violaceous, smooth and shiny plaques on her face and right arm. These lesions were biopsied and the histological examination indicated sarcoidosis. The patient had a history of headache and syncope that lasted for about 1 h. Brain CT showed masses measuring 37×20 mm in both frontal lobes. Thoracic and abdominal CT showed many pathologically enlarged lymph nodes. The patient was diagnosed with cutaneous, lung and neuronal sarcoidosis, and treated with 20 mg/day methylprednisolone, 15 mg/week methotrexate, 10 mg/week folic acid, 400 mg/day hydroxychloroquine and 800 mg/day carbamazepine. One month later, the patient's neurological symptoms had improved and her skin lesions had decreased. At 6-month follow-up, the size of the cranial masses had markedly regressed.

  2. Sarcoidosis: Immunopathogenesis and Immunological Markers

    PubMed Central

    Loke, Wei Sheng Joshua; Herbert, Cristan; Thomas, Paul S.

    2013-01-01

    Sarcoidosis is a multisystem granulomatous disorder invariably affecting the lungs. It is a disease with noteworthy variations in clinical manifestation and disease outcome and has been described as an “immune paradox” with peripheral anergy despite exaggerated inflammation at disease sites. Despite extensive research, sarcoidosis remains a disease with undetermined aetiology. Current evidence supports the notion that the immune response in sarcoidosis is driven by a putative antigen in a genetically susceptible individual. Unfortunately, there currently exists no reliable biomarker to delineate the disease severity and prognosis. As such, the diagnosis of sarcoidosis remains a vexing clinical challenge. In this review, we outline the immunological features of sarcoidosis, discuss the evidence for and against various candidate etiological agents (infective and noninfective), describe the exhaled breath condensate, a novel method of identifying immunological biomarkers, and suggest other possible immunological biomarkers to better characterise the immunopathogenesis of sarcoidosis. PMID:26464848

  3. Sarcoidosis and Antiphospholipid Syndrome: A Systematic Review of Cases

    PubMed Central

    Pathak, Ranjan; Khanal, Raju; Aryal, Madan Raj; Giri, Smith; Karmacharya, Paras; Pathak, Basanta; Acharya, Upasana; Bhatt, Vijaya Raj

    2015-01-01

    Association between sarcoidosis and antiphospholipid syndrome (APS) is rare with few reported cases. We sought to systematically review the published cases of APS with sarcoidosis to better characterize the demographics, clinical characteristics, treatment, and the outcome of this association. Systematic electronic search for case report, case series, and related articles published until May 2014 was carried out and relevant data were extracted and analyzed. Four cases of APS with sarcoidosis were identified exclusively in females. These cases were seen in the sixth decade of life. Pulmonary embolism and central retinal artery occlusion were the presenting thrombotic events. All the patients were treated with lifelong anticoagulation with warfarin. During the median follow-up period of 5.5 months, additional thrombotic events were not observed. Although rare, sarcoidosis may be associated with APS. Further reporting of the cases will help to better establish this association, elucidate pathogenesis, and define clinical characteristics and outcomes. PMID:26605200

  4. Extensive upper respiratory tract sarcoidosis.

    PubMed

    Soares, Mafalda Trindade; Sousa, Carolina; Garanito, Luísa; Freire, Filipe

    2016-04-18

    Sarcoidosis is a chronic granulomatous disease of unknown aetiology. It can affect any part of the organism, although the lung is the most frequently affected organ. Upper airway involvement is rare, particularly if isolated. Sarcoidosis is a diagnosis of exclusion, established by histological evidence of non-caseating granulomas and the absence of other granulomatous diseases. The authors report a case of a man with sarcoidosis manifesting as a chronic inflammatory stenotic condition of the upper respiratory tract and trachea.

  5. Is micropapular sarcoidosis tuberculosis?1

    PubMed Central

    Ridgway, Hilary A; Ryan, Terence J

    1981-01-01

    Three cases of micropapular sarcoidosis are reported. Two of the cases have a history of tuberculosis and all three cases have atypical patterns of Kveim and Mantoux reactivity. The similarities between this form of sarcoidosis and lichen scrofulosorum or lichenoid tuberculid are observed and the evidence is presented for a tuberculoid aetiology in certain forms of sarcoidosis. ImagesFigure 1.Figure 2.Figure 3.Figure 4. PMID:7205849

  6. Sarcoid Myositis with Anti-Ku Antibody Consistent with both Sarcoidosis and Polymyositis.

    PubMed

    Awano, Nobuyasu; Fukuda, Kensuke; Sakayori, Masashi; Kondoh, Keisuke; Ono, Ryu; Moriya, Atsuko; Ando, Tsunehiro; Kumasaka, Toshio; Takemura, Tamiko; Ikushima, Soichiro

    2016-01-01

    We herein describe a case of sarcoid myositis with anti-Ku antibody positivity. Pathological findings of the muscle were compatible with sarcoidosis, but could not be completely distinguished from myositis diseases that arise from other causes. According to a physical examination, pathological findings, the detection of anti-Ku antibody and the human leukocyte antigen (HLA)-DPB1 allele, we strongly suspected that the patient developed both sarcoidosis and polymyositis. Sarcoidosis is often complicated by autoimmune diseases. This case suggests the possibility that sarcoidosis and other autoimmune diseases may have common causal genetic factors.

  7. Extrapulmonary sites of radiogallium accumulation in sarcoidosis

    SciTech Connect

    Sulavik, S.B.; Palestro, C.J.; Spencer, R.P.; Swyer, A.J.; Goldsmith, S.J.; Tierstein, A.S. )

    1990-12-01

    In an effort to detect extrapulmonary sites of radiogallium accumulation in cases of sarcoidosis, 145 separate Ga-67 citrate studies of 114 patients with biopsy-proven sarcoidosis were examined. The most characteristic extrapulmonary radiogallium uptake pattern was the panda sign in 47 patients (41%). The most common site of prominent extrapulmonary radiogallium uptake was the lacrimal glands in 101 patients (88%). Second most common was activity in one or more superficial lymph node regions such as the cervical, axillary, femoral, or inguinal in 19 patients (17%). Other extrapulmonary sites included breast uptake in 6 out of 80 women (8%), prominent splenic and nasal uptake in 9 (8%) patients, periportal accumulation in 7 (6%), and cutaneous/subcutaneous activity in 4 (4%). Because many of these individuals were receiving corticosteroids, the natural (untreated) prevalence of extrapulmonary findings may be even higher. Although the sensitivity and specificity of extrapulmonary radiogallium accumulation has still to be determined, many of the sites may be accessible to biopsy both for diagnostic purposes and to follow the effects of medications. It is therefore suggested that whole-body imaging be performed when radiogallium is administered to patients with suspected or known sarcoidosis.

  8. [The ulcerative form of skin sarcoidosis].

    PubMed

    Rodionov, A N; Samtsov, A V

    1990-01-01

    A female patient suffering from the ulcerative form of skin sarcoidosis is described and the literature dealing with this problem is reviewed. Peculiar features of this case are described: ulceration of the nodes, which is an extremely rare phenomenon; no involvement of other organs, lungs included, was detectable, which is not typical of ulcerative sarcoidosis; small-nodular elements are parallelled by nodes (Boeck's small-nodular sarcoid and Darier-Roussy's subcutaneous sarcoids) in this patient, this evidencing an uniform pathologic process in the skin and subcutaneous fat. Ulceration in this patient is explained by the development of allergic vasculitis of the immediate hypersensitivity type (leukocytoclastic vasculitis and manifest increase of the level of circulating immune complexes). Prednisolone therapy has resulted in an excellent clinical effect.

  9. [A clinical study on 8 cases of sarcoidosis with a cervical mass as a symptom].

    PubMed

    Mada, Yusuke; Ihara, Fumie; Ueki, Yuji; Konno, Akiyoshi

    2013-05-01

    Sarcoidosis is a multiorgan granulomatous disease of unknown origin, which frequently involves the lung and the eyes. It is rare that sarcoidosis causes cervical lymphadenopathy and a chronic continuous salivary gland swelling. In the present study, we examined how to diagnose sarcoidosis in patients complaining of a cervical mass and its clinical presentation according to 8 cases which we experienced. We undertook biopsy of the cervical lymph nodes in 7 cases, and biopsy of the parotid gland in 1 case. In cases non-caseating epithelioid cell granuloma was proved, diagnosed as sarcoidosis histopathlogically. In 6 patients, the sarcoidal lesion was in other organs, whereas in 2 patients the sarcoidal lesion was confined in the head and neck. Bilateral hilar lymphadenopathy and an elevated level of angiotensin-converting enzyme which are typical in sarcoidosis were confirmed in only one of our patients. Therefore, the diagnostic value of these tests is low for the diagnosis of sarcoidosis in the patients whose chief complaint is a cervical mass. Because 4 out of 7 cases were positive for the tuberculin test, it is imperative to distinguish between sarcoidosis and cervical tuberculous lymphadenitis by combining the culture of acid-bacilli and TB-PCR with histopathological evaluation of the biopsy specimen. We experienced one patient in whom the sarcoidal lesion developed in the skin and the eyes 20 years after the onset of cervical lymphadenopathy. It is important to follow the patients carefully, even if we diagnose the disease as sarcoidosis confined in the head and neck.

  10. Delayed diagnosis of sarcoidosis is common in Brazil*

    PubMed Central

    Rodrigues, Mauri Monteiro; Coletta, Ester Nei Aparecida Martins; Ferreira, Rimarcs Gomes; Pereira, Carlos Alberto de Castro

    2013-01-01

    OBJECTIVE: To determine the frequency of and the factors related to delayed diagnosis of sarcoidosis in Brazil. METHODS: We evaluated patients with a biopsy-proven diagnosis of sarcoidosis, using a questionnaire that addressed the following: time since symptom onset and since the first medical visit; and the number and specialty of the physicians visited. We divided the patients by the timeliness of the diagnosis-timely (< 6 months) and delayed (≥ 6 months)-comparing the two groups in terms of systemic and pulmonary symptoms; extrathoracic involvement; spirometric data; radiological staging; level of education; income; and tuberculosis (diagnosis and treatment). RESULTS: We evaluated 100 patients. The median number of physicians consulted was 3 (range, 1-14). In 11 cases, sarcoidosis was diagnosed at the first visit. In 54, the first physician seen was a general practitioner. The diagnosis of sarcoidosis was timely in 41 patients and delayed in 59. The groups did not differ in terms of gender; race; type of health insurance; level of education; income; respiratory/systemic symptoms; extrathoracic involvement; and radiological staging. In the delayed diagnosis group, FVC was lower (80.3 ± 20.4% vs. 90.5 ± 17.1%; p = 0.010), as was FEV1 (77.3 ± 19.9% vs. 86.4 ± 19.5%; p = 0.024), misdiagnosis with and treatment for tuberculosis (≥ 3 months) also being more common (24% vs. 7%, p = 0.032, and 20% vs. 0%; p = 0.002, respectively). CONCLUSIONS: The diagnosis of sarcoidosis is often delayed, even when the imaging is suggestive of sarcoidosis. Delayed diagnosis is associated with impaired lung function at the time of diagnosis. Many sarcoidosis patients are misdiagnosed with and treated for tuberculosis. PMID:24310626

  11. [Lung sarcoidosis: Clinical features and therapeutic issues].

    PubMed

    Uzunhan, Y; Jeny, F; Crockett, F; Piver, D; Kambouchner, M; Valeyre, D; Nunes, H

    2016-09-01

    Sarcoidosis is a granulomatous disease of unknown cause. This proteiform disease is characterized by an almost constant and often predominant lung involvement. The natural history of disease is difficult to predict at presentation. Diagnosis is based on a compatible clinical and radiological presentation and evidence of non-caseating granulomas. Exclusion of alternative diseases is also required according to clinical presentation. Biopsy samples of superficial lesions should be considered before other sites like per-endoscopic bronchial biopsies or endobronchial ultrasound-guided transbronchial needle aspiration. Therapeutic strategy for lung disease has to take into account the possible spontaneous resolution observed in newly diagnosed patients. Corticosteroids are the first choice when a treatment is decided, which concerns half of patients. Second and third line therapy are based respectively on immunosuppressive drugs and anti-TNFα drugs. Sarcoidosis mortality and morbidity are mainly linked to advanced pulmonary sarcoidosis - lung fibrosis, pulmonary hypertension, bronchial stenosis and chronic pulmonary aspergillosis. "Non anti-inflammatory" treatments have to be considered as well. Clinicians have an essential role in treatment indication, end-point targets and evaluation of response to treatment during follow-up and in finding the best benefice to risk balance. Progress made on pharmacogenetics may offer more personalized treatments for the patients. PMID:26897112

  12. [Lung sarcoidosis: Clinical features and therapeutic issues].

    PubMed

    Uzunhan, Y; Jeny, F; Crockett, F; Piver, D; Kambouchner, M; Valeyre, D; Nunes, H

    2016-09-01

    Sarcoidosis is a granulomatous disease of unknown cause. This proteiform disease is characterized by an almost constant and often predominant lung involvement. The natural history of disease is difficult to predict at presentation. Diagnosis is based on a compatible clinical and radiological presentation and evidence of non-caseating granulomas. Exclusion of alternative diseases is also required according to clinical presentation. Biopsy samples of superficial lesions should be considered before other sites like per-endoscopic bronchial biopsies or endobronchial ultrasound-guided transbronchial needle aspiration. Therapeutic strategy for lung disease has to take into account the possible spontaneous resolution observed in newly diagnosed patients. Corticosteroids are the first choice when a treatment is decided, which concerns half of patients. Second and third line therapy are based respectively on immunosuppressive drugs and anti-TNFα drugs. Sarcoidosis mortality and morbidity are mainly linked to advanced pulmonary sarcoidosis - lung fibrosis, pulmonary hypertension, bronchial stenosis and chronic pulmonary aspergillosis. "Non anti-inflammatory" treatments have to be considered as well. Clinicians have an essential role in treatment indication, end-point targets and evaluation of response to treatment during follow-up and in finding the best benefice to risk balance. Progress made on pharmacogenetics may offer more personalized treatments for the patients.

  13. Parallel Gene Expression Changes in Sarcoidosis Involving the Lacrimal Gland, Orbital Tissue, or Blood

    PubMed Central

    Rosenbaum, James T.; Choi, Dongseok; Wilson, David J.; Grossniklaus, Hans E.; Harrington, Christina A.; Sibley, Cailin H.; Dailey, Roger A.; Ng, John D.; Steele, Eric A.; Czyz, Craig N.; Foster, Jill A.; Tse, David; Alabiad, Chris; Dubovy, Sander; Parekh, Prashant; Harris, Gerald J.; Kazim, Michael; Patel, Payal; White, Valerie; Dolman, Peter; Korn, Bobby S.; Kikkawa, Don; Edward, Deepak P.; Alkatan, Hind; al-Hussain, Hailah; Yeatts, R. Patrick; Selva, Dinesh; Stauffer, Patrick; Planck, Stephen R.

    2016-01-01

    IMPORTANCE Sarcoidosis is a major cause of ocular or periocular inflammation. The pathogenesis of sarcoidosis is incompletely understood and diagnosis often requires a biopsy. OBJECTIVE To determine how gene expression in either orbital adipose tissue or the lacrimal gland affected by sarcoidosis compares with gene expression in other causes of orbital disease and how gene expression in tissue affected by sarcoidosis compares with gene expression in peripheral blood samples obtained from patients with sarcoidosis. DESIGN, SETTING, AND PARTICIPANTS In a multicenter, international, observational study, gene expression profiling of formalin-fixed biopsy specimens, using GeneChipp U133 Plus 2 microarrays (Affymetrix), was conducted between October 2012 and January 2014 on tissues biopsied from January 2000 through June 2013. Participants included 12 patients with orbital sarcoidosis (7 in adipose tissue; 5 affecting the lacrimal gland) as well as comparable tissue from 6 healthy individuals serving as controls or patients with thyroid eye disease, nonspecific orbital inflammation, or granulomatosis with polyangiitis. In addition, results were compared with gene expression in peripheral blood samples obtained from 12 historical individuals with sarcoidosis. MAIN OUTCOMES AND MEASURES Significantly differentially expressed transcripts defined as a minimum of a 1.5-fold increase or a comparable decrease and a false discovery rate of P < .05. RESULTS Signals from 2449 probe sets (transcripts from approximately 1522 genes) were significantly increased in the orbital adipose tissue from patients with sarcoidosis. Signals from 4050 probe sets (approximately 2619 genes) were significantly decreased. Signals from 3069 probe sets (approximately 2001 genes) were significantly higher and 3320 (approximately 2283 genes) were significantly lower in the lacrimal gland for patients with sarcoidosis. Ninety-two probe sets (approximately 69 genes) had significantly elevated signals and

  14. Granuloma Annulare Mimicking Sarcoidosis: Report of Patient With Localized Granuloma Annulare Whose Skin Lesions Show 3 Clinical Morphologies and 2 Histology Patterns.

    PubMed

    Cohen, Philip R; Carlos, Casey A

    2015-07-01

    Granuloma annulare, a benign dermatosis of undetermined etiology, typically presents in a localized or generalized form. It has 3 distinctive histologic patterns: an infiltrative (interstitial) pattern, a palisading granuloma pattern, and an epithelioid nodule (sarcoidal granuloma) pattern. A man whose granuloma annulare skin lesions mimicked sarcoidosis is described. His localized granuloma annulare presented with a total of 3 lesions that each had a distinctive clinical morphology: an annular lesion of individual papules, a dermal nodule, and a linear arrangement of 3 papules. Two of his lesions showed a palisading granuloma histology pattern of granuloma annulare; however, the linear papules on his posterior neck lesion demonstrated noncaseating granulomas consistent with either the epithelioid nodule histology pattern of granuloma annulare or sarcoidal granuloma compatible with sarcoidosis. A comprehensive evaluation excluded the diagnosis of systemic sarcoidosis. Using the PubMed database, an extensive literature search was performed on granuloma annulare, epithelioid nodule, sarcoidal granuloma, and sarcoidosis. The histology patterns of granuloma annulare-emphasizing the history and differentiating features of the epithelioid nodule pattern from cutaneous sarcoidosis-were reviewed. The epithelioid nodule (sarcoidal granuloma) histology pattern of granuloma annulare is uncommon and may mimic the histology changes observed in sarcoidosis skin lesions; the absence of asteroid or other giant cell inclusions and an increase in mucin deposition between the collagen bundles favor the diagnosis of granuloma annulare. In addition, the epithelioid nodule pattern of granuloma annulare can rarely also show other histologic patterns of granuloma annulare in the same biopsy specimen or concurrently present with other clinical lesions of granuloma annulare that demonstrate a palisading granuloma, or possibly an infiltrative, histology pattern. However, the presence of an

  15. Granuloma Annulare Mimicking Sarcoidosis: Report of Patient With Localized Granuloma Annulare Whose Skin Lesions Show 3 Clinical Morphologies and 2 Histology Patterns.

    PubMed

    Cohen, Philip R; Carlos, Casey A

    2015-07-01

    Granuloma annulare, a benign dermatosis of undetermined etiology, typically presents in a localized or generalized form. It has 3 distinctive histologic patterns: an infiltrative (interstitial) pattern, a palisading granuloma pattern, and an epithelioid nodule (sarcoidal granuloma) pattern. A man whose granuloma annulare skin lesions mimicked sarcoidosis is described. His localized granuloma annulare presented with a total of 3 lesions that each had a distinctive clinical morphology: an annular lesion of individual papules, a dermal nodule, and a linear arrangement of 3 papules. Two of his lesions showed a palisading granuloma histology pattern of granuloma annulare; however, the linear papules on his posterior neck lesion demonstrated noncaseating granulomas consistent with either the epithelioid nodule histology pattern of granuloma annulare or sarcoidal granuloma compatible with sarcoidosis. A comprehensive evaluation excluded the diagnosis of systemic sarcoidosis. Using the PubMed database, an extensive literature search was performed on granuloma annulare, epithelioid nodule, sarcoidal granuloma, and sarcoidosis. The histology patterns of granuloma annulare-emphasizing the history and differentiating features of the epithelioid nodule pattern from cutaneous sarcoidosis-were reviewed. The epithelioid nodule (sarcoidal granuloma) histology pattern of granuloma annulare is uncommon and may mimic the histology changes observed in sarcoidosis skin lesions; the absence of asteroid or other giant cell inclusions and an increase in mucin deposition between the collagen bundles favor the diagnosis of granuloma annulare. In addition, the epithelioid nodule pattern of granuloma annulare can rarely also show other histologic patterns of granuloma annulare in the same biopsy specimen or concurrently present with other clinical lesions of granuloma annulare that demonstrate a palisading granuloma, or possibly an infiltrative, histology pattern. However, the presence of an

  16. Pulmonary hyalinizing granuloma with associated elevation in serum and tissue IgG4 occurring in a patient with a history of sarcoidosis.

    PubMed

    Chapman, Erin M; Gown, Allen; Mazziotta, Robert; Churg, Andrew

    2012-05-01

    Pulmonary hyalinizing granulomas (PHGs) are unusual fibrosclerotic inflammatory lung lesions. The organ-based manifestations of the recently defined IgG4-related sclerosing disease typically show dense fibrosis and heavy lymphoplasmacytic infiltrates. IgG4-related sclerosing disease is also defined by increased serum IgG4 levels and increased tissue levels of IgG4-positive plasma cells. The morphologic features of PHG overlap with those seen in IgG4-related sclerosing disease, and this suggests that PHG may be a form of IgG4-related sclerosing disease. We present a case of a 51-year-old man with a history of sarcoidosis who presented with slowly enlarging pulmonary nodules. Histologic evaluation of one of the nodules yielded a diagnosis of PHG. Further investigation demonstrated both elevated serum IgG4 and elevated tissue IgG4-positive plasma cells in the PHG. In previous reports, lesions that are now considered part of IgG4-related sclerosing disease were documented in patients also diagnosed with PHG, although these reports date from before the description of IgG4 sclerosing disease. This case provides the first definitive evidence that PHG is part of the spectrum of IgG4-related sclerosing disease. PMID:22498827

  17. Direct quantitation of thoracic gallium-67 uptake in sarcoidosis

    SciTech Connect

    Bourguet, P.; Delaval, P.; Herry, J.Y.

    1986-10-01

    A method of direct quantitation of /sup 67/Ga uptake in the lung is described. The attenuation coefficient requires calculation and is obtained simply for each patient by transmission using a planar radionuclide source. The validity of the method was tested with a phantom (error less than 10%). Forty-three patients with pulmonary and/or mediastinal sarcoidosis were classified. The different groups of patients as defined clinically and radiographically (controls, nonactive, and active sarcoidosis) were well-differentiated (p less than 0.001).

  18. Unilateral Eyelid Edema as Initial Sign of Orbital Sarcoidosis

    PubMed Central

    Filho, Flávio David Haddad; Dedivitis, Rogério Aparecido; Petrarolha, Samuel Brunini

    2016-01-01

    Introduction. Sarcoidosis is a rare multisystemic granulomatous inflammatory disease of unknown etiology affecting the respiratory system, skin, and eyes. Sarcoidosis outside the lacrimal gland is rare. The case study concerns a patient with a final diagnosis of orbital sarcoidosis. Case Report. A 37-year-old male patient went to the ophthalmic emergency room complaining of pain in the left eye, diplopia, and decreased visual acuity. An external eye examination showed hard and cold edema of the lower eyelid, ocular motility with limitation of adduction, and discreet ipsilateral proptosis. Magnetic resonance of the orbit showed left eye proptosis and thickening and increase of soft tissues associated with heterogeneous impregnation of contrast in the infralateral region of the left eyelid. A biopsy of the lesion showed a chronic inflammatory process, with numerous compact nonnecrotizing granulomas surrounded by lamellar hyaline collagen, providing histological confirmation of sarcoidosis. Discussion. A biopsy of the orbital tumor is essential for the diagnosis of sarcoidosis, in addition to the search for systemic findings such as hilar adenopathy or parenchymal lung disease found in 90% of patients. PMID:27298746

  19. Predominant diffuse ground glass opacity in both lung fields: A case of sarcoidosis with atypical CT findings

    PubMed Central

    Ma, Chunmei; Zhao, Yadong; Wu, Taihua

    2016-01-01

    Sarcoidosis can cause fatal diffuse lung fibrosis in the end stage, so its early diagnosis and treatment can prevent the progression of fibrosis. Predominant ground glass opacity on high-resolution CT (HRCT) scans is a rare presentation of sarcoidosis. We report the case of a patient who presented with very few symptoms and signs of sarcoidosis; HRCT revealed large-scale ground glass opacity and minor lymphadenopathy. Bronchoalveolar lavage fluid contained turbid liquid. Sarcoidosis could be confirmed only based on pathological examination of the resected tissue. The patient was administrated prednisone at 40 mg/d orally with tapering of the dose. Lung HRCT scans taken 6 months after the prednisone treatment showed ablation of the ground glass opacity. This case report sheds light on an atypical HRCT presentation of sarcoidosis; the findings here will be useful for the early diagnosis of sarcoidosis and prevention of fatal complications. PMID:27222788

  20. Vascular function and ocular involvement in sarcoidosis.

    PubMed

    Siasos, Gerasimos; Paraskevopoulos, Theodoros; Gialafos, Elias; Rapti, Aggeliki; Oikonomou, Evangelos; Zaromitidou, Marina; Mourouzis, Konstantinos; Siasou, Georgia; Gouliopoulos, Nikolaos; Tsalamandris, Sotiris; Vlasis, Konstantinos; Stefanadis, Christodoulos; Papavassiliou, Athanasios G; Tousoulis, Dimitris

    2015-07-01

    Ocular involvement occurs in sarcoidosis (Sar) patients mainly in the form of uveitis. This study was designed to determine if uveitis in Sar patients is associated with vascular impairment. We enrolled 82 Sar patients and 77, age and sex matched, control subjects (Cl). Sar patients were divided into those with ocular sarcoidosis (OS) and those without ocular sarcoidosis (WOS). Endothelial function was evaluated by flow-mediated dilation (FMD). Pulse wave velocity (PWV) was measured as an index of aortic stiffness and augmentation index (AIx) as a measure of arterial wave reflections. Although there was no significant difference in sex, age and mean arterial pressure, patients with OS compared to WOS patients and Cl subjects had impaired FMD (p<0.001), increased AIx (p=0.02) and increased PWV (p=0.001). Interestingly, impaired FMD in Sar patients was independently, from possible covariates (age, sex, smoking habits, arterial hypertension, dyslipidemia), associated with increased odds of ocular involvement (odds ratio=1.69, p=0.001). More precisely ROC curve analysis revealed that FMD had a significant diagnostic ability for the detection of OS (AUC=0.77, p<0.001) with a sensitivity of 79% and a specificity of 68% for an FMD value below 6.00%. To conclude in the present study we have shown that ocular involvement in Sar patients is associated with impaired endothelial function and increased arterial stiffness. These results strengthen the vascular theory which considers uveitis a consequence of vascular dysfunction in Sar patients and reveals a possible clinical importance of the use of endothelial function tests.

  1. Cutaneous sarcoidosis: an intriguing model of immune dysregulation.

    PubMed

    Ruocco, Eleonora; Gambardella, Alessio; Langella, Giovanni Giuseppe; Lo Schiavo, Ada; Ruocco, Vincenzo

    2015-01-01

    Sarcoidosis is a systemic granulomatous disease characterized by the presence of non-caseating granulomas. Its etiology remains obscure. A plausible hypothesis suggests that a complex interplay of host factors, infectious processes, and non-infectious environmental factors, matched with a susceptible genetic background, results in a pathway that leads to systemic granulomatous inflammation. Although presentations of sarcoidosis vary enormously, multi-organ involvement is a common feature. Cutaneous involvement occurs in about 25% of patients with protean manifestations and variable prognoses. Skin manifestations are divided into specific lesions with histopathologically evident non-caseating granulomas and nonspecific lesions arising from a reactive process that does not form granulomas. A peculiar form of cutaneous sarcoidosis is represented by sarcoidal lesions at sites of trauma that has caused scarring. The pathogenesis of scar sarcoidosis remains unknown. Scar sarcoidosis is also associated with herpes zoster infection, surgery, and tattooing. Such heterogeneous events, along with those at the sites of chronic lymphedema, thermal burns, radiation dermatitis, and vaccinations, occur on areas of vulnerable skin labeled "immunocompromised districts". Numerous options are available for the treatment of cutaneous sarcoidosis. Although corticosteroids remain the treatment of choice for initial systemic therapy, other nonsteroidal agents have proven effective and therefore useful for long-term management. Tumor necrosis factor-α antagonists such as infliximab may have a role in the treatment of cutaneous sarcoidosis, especially in refractory cases that are resistant to standard regimens. Elucidation of the relationship of sarcoidal granulomas with malignancy and immunity may facilitate a better understanding of some pathomechanisms operating in neoplastic and immunity-related disorders.

  2. Darier-Roussy Sarcoidosis Mimicking Metastatic Breast Cancer.

    PubMed

    Viswanath, Lokesh; Pallade, Siddanna; Krishnamurthy, B; Naveen, T; Preethi, B L; Pramod, K P R; Reddy, Obula; Padma, G

    2009-01-01

    Subcutaneous sarcoidosis (also known as 'Darier-Roussy sarcoid') is a cutaneous condition characterized by numerous deep-seated nodules on the trunk and extremities. Coexistence of sarcoidosis and breast cancer are reported in the literature, but there will always be a chance of misdiagnosis. It is very crucial to obtain a tissue diagnosis of suspicious metastatic lesions. We report a case of breast cancer presenting with a subcutaneous sarcoid lesion masquerading as a metastatic lesion. A 50-year-old female patient, who had had cancer of the left breast, was on hormone therapy 2 years after treatment with surgery, radiotherapy and chemotherapy. The patient presented with a sudden onset of a forehead subcutaneous swelling mimicking metastasis which on excision biopsy was proved to be sarcoidosis. In India, sarcoidosis is reported rarely. We have to keep in mind that there is a chance of the metastatic lesions being of sarcoidosis origin or another granulomatous disease. To avoid misdiagnosis, it is better to obtain a tissue diagnosis. PMID:20737045

  3. Sarcoidosis Occurring After Solid Cancer: A Nonfortuitous Association: Report of 12 Cases and Review of the Literature.

    PubMed

    Grados, Aurélie; Ebbo, Mikael; Bernit, Emmanuelle; Veit, Véronique; Mazodier, Karin; Jean, Rodolphe; Coso, Diane; Aurran-Schleinitz, Thérèse; Broussais, Florence; Bouabdallah, Reda; Gravis, Gwenaelle; Goncalves, Anthony; Giovaninni, Marc; Sève, Pascal; Chetaille, Bruno; Gavet-Bongo, Florence; Weitten, Thierry; Pavic, Michel; Harlé, Jean-Robert; Schleinitz, Nicolas

    2015-07-01

    The association between cancer and sarcoidosis is controversial. Some epidemiological studies show an increase of the incidence of cancer in patients with sarcoidosis but only few cases of sarcoidosis following cancer treatment have been reported. We conducted a retrospective case study from internal medicine and oncology departments for patients presenting sarcoidosis after solid cancer treatment. We also performed a literature review to search for patients who developed sarcoidosis after solid cancer. We describe the clinical, biological, and radiological characteristics and outcome of these patients. Twelve patients were included in our study. Various cancers were observed with a predominance of breast cancer. Development of sarcoidosis appeared in the 3 years following cancer and was asymptomatic in half of the patients. The disease was frequently identified after a follow-up positron emission tomography computerized tomography evaluation. Various manifestations were observed but all patients presented lymph node involvement. Half of the patients required systemic therapy. With a median follow-up of 73 months, no patient developed cancer relapse. Review of the literature identified 61 other patients for which the characteristics of both solid cancer and sarcoidosis were similar to those observed in our series. This report demonstrates that sarcoidosis must be considered in the differential diagnosis of patients with a history of malignancy who have developed lymphadenopathy or other lesions on positron emission tomography computerized tomography. Histological confirmation of cancer relapse is mandatory in order to avoid unjustified treatments. This association should be consider as a protective factor against cancer relapse.

  4. Rare manifestation of the thrombotic microangiopathy in a patient with sarcoidosis, common variable immunodeficiency and large B-cell non-hodgkin lymphoma: case report.

    PubMed

    Ekart, Robert; Grobelšek, Vesna Kovačič; Dai, Klara; Cernelč, Peter; Hojs, Radovan

    2013-06-01

    58-year old Caucasian woman was diagnosed with sarcoidosis. Low immunoglobulin levels were found and common variable immunodeficiency (CVID) was diagnosed 1year later. Laboratory tests and clinical course at this time revealed thrombotic microangiopathy (TMA). Therapeutic plasma exchange was started and her clinical status and laboratory parameters improved. According to CVID she received human immunoglobulin intravenously. Four months later we noticed swelling of the parotid glands and generalized lymphadenopathy. Histology of cervical lymph node confirmed large B-cell non-Hodgkin lymphoma (B-cell NHL). To the best of our knowledge, TMA complicating the course of sarcoidosis, CVID and B-cell NHL has never been reported. PMID:23619325

  5. Variation in the Lymphotoxin-α/Tumor Necrosis Factor Locus Modifies Risk of Erythema Nodosum in Sarcoidosis

    PubMed Central

    McDougal, Kathryn E.; Fallin, M. Daniele; Moller, David R.; Song, Zhimin; Cutler, David J.; Steiner, Lori L.; Cutting, Garry R.

    2010-01-01

    Sarcoidosis is a multi-system inflammatory disease with organ involvement that varies by race and sex. Family studies indicate that genes play a role in the etiology and extent of organ involvement in sarcoidosis. In this study, we evaluated whether 25 variants distributed in 19 genes with a known role in inflammation were associated with erythema nodosum status in 659 sarcoidosis patients and 658 controls from A Case–Control Etiologic Study of Sarcoidosis (ACCESS). We found no association with affectation status; however, a variant in the promoter of tumor necrosis factor (TNF) at position −308 was found to be associated with erythema nodosum in Caucasian sarcoidosis patients (study-wide P = 0.027). When separated by sex, a variant in intron 1 of lymphotoxin-α (LTA), a gene adjacent to TNF, was associated with erythema nodosum in female Caucasian sarcoidosis patients (study-wide P = 0.027). These DNA variants frequently occur together in Caucasians, and each variant has individually been associated with erythema nodosum in sarcoidosis patients. These results confirm that variation in the LTA/TNF gene cluster modifies a major skin manifestation of sarcoidosis and may explain the higher rate of erythema nodosum in females with sarcoidosis. PMID:19225544

  6. Paracoccidioidomycosis Mimicking Sarcoidosis: A Review of 8 Cases.

    PubMed

    Coelho, Mariana Guimarães; Severo, Cecília Bittencourt; de Mattos Oliveira, Flávio; Hochhegger, Bruno; Severo, Luiz Carlos

    2016-02-01

    Sarcoidosis is a multisystem disorder that is characterized by noncaseous epithelioid cell granulomas, which may affect almost any organ. Thoracic involvement is common and accounts for most of the morbidity and mortality associated with this disease. The diagnosis is based on exhaustive exclusion of differential diagnoses, particularly granulomatous infections. We report data on eight patients with paracoccidioidomycosis mimicking sarcoidosis. Five patients presented with a chronic pulmonary type infection and three had a disseminated form after immunosuppressive treatment. The mycological diagnosis in noncaseating granulomas is emphasized and reviewed.

  7. ACE Inhibitor in the treatment of cutaneous and lymphatic sarcoidosis.

    PubMed

    Kaura, Vinod; Kaura, Samantha H; Kaura, Claire S

    2007-01-01

    Angiotensin-converting enzyme is used as a marker for sarcoid activity. We describe a case of remission of cutaneous and lymphatic sarcoidosis in a patient treated with an ACE inhibitor for congestive heart failure and hypertension; the remission has continued over 4 years of follow-up. Because this is a report of only one case, there is a possibility of sampling error. Whether the patient's remission in this case was a serendipitous spontaneous remission that happened to occur during ACE inhibitor therapy or whether ACE inhibitor therapy can play a role in the treatment of sarcoidosis needs to be determined in a large clinical trial.

  8. A rare case of pulmonary tuberculosis with simultaneous pulmonary and skin sarcoidosis: a case report

    PubMed Central

    2010-01-01

    Background Tuberculosis and sarcoidosis are chronic diseases that rarely occur concomitantly. Sarcoidosis is a multisystem granulomatous disorder characterized pathologically by the presence of non-caseating granulomas in involved tissues. Tuberculosis is infectious disease caused by Mycobacterium tuberculosis characterized by granulomas with caseous necrosis. Case presentation We present a case of 43-year-old female refugee from Kosovo with microbiological confirmation of pulmonary tuberculosis and pulmonary and skin sarcoidosis at the same time. Three weeks after corticosteroid therapy for pulmonary sarcoidosis was introduced, positive finding of mycobacterium culture of bronchial aspirate was observed. Based on these results, corticosteroid therapy was excluded and antituberculous therapy was introduced for six months. In the meantime, new nodes on face and nose appeared and skin sarcoidosis was diagnosed. The patient was given corticosteroids and colchicine according to the skin and pulmonary sarcoidosis therapy recommendation. Conclusion The authors of this study suggest that in cases when there is a dilemma in diagnosis between tuberculosis and sarcoidosis we should advance with corticosteroid therapy until we have microbiological confirmation of mycobacterium culture. This case is remarkable because this is a third described case of sarcoidosis and tuberculosis together (the first reported in Asia, the second in South Africa), and to authors knowledge, this is a first case report in Europe. PMID:20205764

  9. Redistribution on the thallium scan in myocardial sarcoidosis: concise communication

    SciTech Connect

    Makler, P.T.; Lavine, S.J.; Denenberg, B.S.; Bove, A.A.; Idell, S.

    1981-05-01

    Resting and redistribution thallium studies were performed in four young patients with sarcoidosis to evaluate the possibility of myocardial involvement. In each case the resting scan showed marked defects that resolved on the redistribution studies. In a different patient population, these results would have implied significant coronary artery disease.

  10. Colonic sarcoidosis: unusual onset of a systemic disease.

    PubMed

    Erra, Paola; Crusco, Sonia; Nugnes, Loredana; Pollio, Anna Maria; Di Pilla, Gianni; Biondi, Giuseppe; Vigliardi, Giovanni

    2015-03-21

    Sarcoidosis is a multisystem chronic inflammatory condition of unknown etiology that has the potential to involve every tissue in the body. Sarcoidosis in the gastrointestinal system, and particularly the colon, is very rare. Here, we report the case of a 57-year-old man with no previous diagnosis of sarcoidosis who presented with new onset of abdominal pain and constipation. A colonoscopy revealed that the abdominal pain was caused by an obstructing lesion in the cecum-ascending colon and lacked a clear histologic diagnosis. Radiologic investigation revealed concentric wall thickening of the cecum-ascending colon with multiple satellite lymphadenopathies, highly suggestive of a malignancy. The patient underwent a laparotomy and a right hemicolectomy was performed. A diagnosis of colonic sarcoidosis was made after the resected specimen was examined. Additionally, a chest computed tomography scan revealed lung involvement with atypical radiologic features in the absence of respiratory symptoms. Only histologic examination of the surgical specimen can yield a diagnosis of gastrointestinal sarcoidosis due to the non-specificity of endoscopic and radiologic findings.

  11. Sarcoidosis of the upper respiratory tract and its association with lupus pernio.

    PubMed Central

    Neville, E; Mills, R G; Jash, D K; Mackinnon, D M; Carstairs, L S; James, D G

    1976-01-01

    In a series of 34 patients with sarcoidosis affecting the upper respiratory tract and nose, 26 had lupus pernio (LP) and 17 had sarcoidosis of the upper respiratory tract (SURT). In nine patients these features coexisted. A patient presenting with SURT carried a 50% risk of developing LP although one feature could be present without the other. Both were disorders of women of the child-bearing years of life. SURT, like LP, was an indicator of chronic fibrotic sarcoidosis, developing insidiously and progressing indolently over the years. It was complicated by ulceration, septal perforation, and LP. Three patients had nasal septal perforations, in two instances following submucous resection. This operation is contraindicated in patients with active sarcoidosis, particularly when granulomas are found on nasal biopsy. The Kveim-Siltzbach skin test was positive in all patients with SURT, making it invaluable in the differential diagnosis of granuloma of the nasal cavity. PMID:1013937

  12. Esophageal Sarcoidosis: A Review of Cases and an Update

    PubMed Central

    Hajar, Rabab; Virdi, Ravi; Singh, Jaspreet; Mustacchia, Paul

    2013-01-01

    Sarcoidosis is a chronic disorder that can virtually affect any organ system in the body. Histologically, it is characterized by the presence of T lymphocytes, mononuclear phagocytes, and noncaseating granulomas. Most commonly affected are the intrathoracic structures, with 90% of the reported cases involving the lungs. Esophageal involvement in sarcoidosis is extremely rare. Dysphagia is the most common presentation in these patients and can be attributed to various mechanisms such as direct esophageal wall infiltration, extrinsic compression, cranial neuropathy, and brainstem involvement. A thorough online literature review revealed only 23 reported cases of esophageal involvement in sarcoidosis. This paper reviews these reported cases in detail along with newer diagnostic and treatment options, including direction of future therapy. PMID:23533794

  13. Sarcoidosis detected due to tattoo swellings in an Indian female.

    PubMed

    Ghorpade, Ashok

    2015-01-01

    A female patient presented with asymptomatic swellings in old blue-black tattoo marks of her forearms. On careful skin examination, few raised erythematous, circular plaques were also found on the non-tattooed skin on her back, about which she was unaware. The diagnosis of cutaneous and systemic sarcoidosis was confirmed after classical histopathological findings from skin lesions from her non tattooed back and from papulonodular tattooed lesions, ruling out other causes of granulomas and supported by a negative Mantoux test, raised serum angiotensin converting enzyme level, high resolution computed tomography thorax, etc. She responded well to oral steroid therapy. The importance of a careful skin examination, easy access provided by skin lesions in suspected cases of cutaneous/systemic sarcoidosis, the value of sudden tattoo changes in the diagnosis here and the possible contribution of tattoos toward the causation of cutaneous and/or systemic sarcoidosis are highlighted. PMID:26275266

  14. Advances in imaging for diagnosis and management of cardiac sarcoidosis.

    PubMed

    Aggarwal, Niti R; Snipelisky, David; Young, Phillip M; Gersh, Bernard J; Cooper, Leslie T; Chareonthaitawee, Panithaya

    2015-09-01

    Sarcoidosis is a multisystem granulomatous disorder with a high prevalence of cardiac involvement. Cardiac sarcoidosis (CS) may be life threatening due to end-stage cardiomyopathy and sudden cardiac death. The frequent absence of specific symptoms and lack of a diagnostic 'gold standard' pose challenges in the diagnosis of CS. Endomyocardial biopsy, although specific, has an unacceptably low sensitivity. Non-invasive cardiac imaging has a huge role in the assessment of patients with known or suspected CS. This comprehensive review compares the diagnostic accuracy, along with advantages and disadvantages, of established and emerging imaging modalities for CS.

  15. The Management of Sarcoidosis: A Primary Care Approach.

    PubMed

    Shinn, Justin; Paauw, Douglas S

    2015-09-01

    Sarcoidosis is an idiopathic inflammatory disorder characterized by noncaseating granulomas, which can affect any organ system. The lungs are most commonly affected but extrapulmonary sites may cause the initial and/or sole symptoms. In this review, the disease manifestations and treatment are described, with particular emphasis on the management of each affected organ system. Diagnosis and management can be difficult and greatly affect quality of life, but despite these challenges, it is possible to successfully manage patients with sarcoidosis in the primary care setting.

  16. Sarcoidal granulomas in a cosmetic tattoo in association with pulmonary sarcoidosis.

    PubMed

    Baumgartner, Magdalena; Feldmann, Robert; Breier, Friedrich; Steiner, Andreas

    2010-11-01

    Sarcoidosis is a granulomatous disease of unknown etiology, which may affect multiple organ systems. The skin is involved in up to one-third of cases. The development of granulomatous lesions within tattoos is a well-recognized phenomenon in patients with sarcoidosis. The characteristic histology shows non-caseating granulomas; however similar findings may be seen in foreign body granulomas. We report on a patient, who developed sarcoidal granulomas within the black pigment of his tattoos. This subsequently led to the diagnosis of pulmonary sarcoidosis.

  17. A Case of Sarcoidosis Associated With Anti–Tumor Necrosis Factor Treatment

    PubMed Central

    Hanazay, Cigdem; Kokturk, Nurdan; Turktas, Haluk

    2015-01-01

    Sarcoidosis is a systemic chronic granulomatous disease of unknown etiology. It predominantly involves the lungs but can affect many organs or tissues in the body, such as the lymphatic system, skin, eyes, and liver. Typical histopathological lesions are noncaseating granulomas in the affected organ or tissue. Indications, type of treatment, and duration of sarcoidosis treatment is currently debated. Despite studies showing that anti–tumor necrosis factor-α (TNF-α) treatment can successfully be used in refractory sarcoidosis, there are some case reports regarding the development of sarcoidosis with these agents. There have been reports of 47 anti-TNF-associated cases of sarcoidosis until 2012. The patient is a 54-year-old Caucasian male. During routine examinations of the patient who had been followed for psoriasis vulgaris for 20 years and who had been on several anti-TNF regimens thereafter, new pulmonary pathologies due to sarcoidosis were detected. We present here a case of sarcoidosis that developed after infliximab treatment and showed obvious radiologic regression with discontinuation of treatment. During anti-TNF treatment, it should be kept in mind that autoimmune and granulomatous diseases may develop and particular care should be given to patient follow-ups. PMID:26425632

  18. Dermoscopy could be useful in differentiating sarcoidosis from necrobiotic granulomas even after treatment with systemic steroids

    PubMed Central

    Ramadan, Shahira; Hossam, Dalia; Saleh, Marwah A.

    2016-01-01

    Background: Diagnosing cutaneous sarcoidosis and necrobiotic granulomas is challenging. Objective: Assessing the value of dermoscopy in differentiating cutaneous sarcoidosis from necrobiotic granulomas and evaluating whether their dermoscopic features will be altered after treatment. Methods: Nineteen cutaneous sarcoidosis and 11 necrobiotic granuloma patients (2 necrobiosis lipoidica, 4 granuloma annulare and 5 rheumatoid nodule) were included in this study. The diagnosis was confirmed by skin biopsy. The lesions were examined using non-contact polarized dermoscope (Dermlite 2 HR-Pro; 3Gen, San Juan Capistrano, CA). Results: Ten out of 19 cutaneous sarcoidosis patients and 7/11 necrobiotic cases group were receiving treatments (topical, intralesional or systemic steroids ± chloroquine) but still have cutaneous lesions. Treatment duration in the sarcoidosis group ranged from 2 months to 10 years (median 3 years) and in the necrobiotic cases group ranged from 3 months to 16 years (median 2 years). Pink homogenous background, translucent orange areas, white scar-like depigmentation and fine white scales were significantly associated with the cutaneous sarcoidosis compared to necrobiotic cases group. On the other hand mixed pink, white and yellowish background was significantly associated with the necrobiotic cases group. No significant difference in the dermoscopic findings was detected between treated and non-treated patients. Conclusion: Some dermoscopic findings are shared between the cutaneous sarcoidosis group and the necrobiotic cases group, yet dermoscopy could be a useful aid in differentiating them even after treatment.

  19. Dermoscopy could be useful in differentiating sarcoidosis from necrobiotic granulomas even after treatment with systemic steroids

    PubMed Central

    Ramadan, Shahira; Hossam, Dalia; Saleh, Marwah A.

    2016-01-01

    Background: Diagnosing cutaneous sarcoidosis and necrobiotic granulomas is challenging. Objective: Assessing the value of dermoscopy in differentiating cutaneous sarcoidosis from necrobiotic granulomas and evaluating whether their dermoscopic features will be altered after treatment. Methods: Nineteen cutaneous sarcoidosis and 11 necrobiotic granuloma patients (2 necrobiosis lipoidica, 4 granuloma annulare and 5 rheumatoid nodule) were included in this study. The diagnosis was confirmed by skin biopsy. The lesions were examined using non-contact polarized dermoscope (Dermlite 2 HR-Pro; 3Gen, San Juan Capistrano, CA). Results: Ten out of 19 cutaneous sarcoidosis patients and 7/11 necrobiotic cases group were receiving treatments (topical, intralesional or systemic steroids ± chloroquine) but still have cutaneous lesions. Treatment duration in the sarcoidosis group ranged from 2 months to 10 years (median 3 years) and in the necrobiotic cases group ranged from 3 months to 16 years (median 2 years). Pink homogenous background, translucent orange areas, white scar-like depigmentation and fine white scales were significantly associated with the cutaneous sarcoidosis compared to necrobiotic cases group. On the other hand mixed pink, white and yellowish background was significantly associated with the necrobiotic cases group. No significant difference in the dermoscopic findings was detected between treated and non-treated patients. Conclusion: Some dermoscopic findings are shared between the cutaneous sarcoidosis group and the necrobiotic cases group, yet dermoscopy could be a useful aid in differentiating them even after treatment. PMID:27648379

  20. Sarcoidosis in Caucasians, Blacks and Asians in London.

    PubMed

    Edmondstone, W M; Wilson, A G

    1985-01-01

    The incidence and clinical features of sarcoidosis were studied in a retrospective survey of 156 patients attending two South London hospitals between 1969 and 1982. Sixty-eight patients were Caucasian, 59 were Black West Indian or African immigrants and 29 were immigrants racially derived from Indo-Pakistan (Asians). The annual incidence of sarcoidosis in the local community in 1977-78 was 19.8 per 10(5) for Blacks and 16.8 per 10(5) for Asians compared with 1.5 per 10(5) for Caucasians. Erythema nodosum was a more common presenting feature in Caucasians than in Blacks (P less than 0.001) or Asians (P less than 0.05). Blacks developed sarcoidosis at a later age than Caucasians (P less than 0.05) and were less likely to present as a chance finding on a chest radiograph (P less than 0.05). They had more widespread extrathoracic disease than Caucasians (P less than 0.001) and were more commonly treated with corticosteroids (P less than 0.001). In the Asians there was a male predominance compared with Caucasians (P less than 0.01). Asians also had more extrathoracic sarcoidosis than Caucasians (P less than 0.001) and more of them were treated with corticosteroids (P less than 0.05). A stage 2 chest radiograph at presentation (P less than 0.05) and skin sarcoidosis (P less than 0.05) were less common in Asians than in Blacks, but otherwise there were no significant differences between the two groups. In this study the incidence of sarcoidosis in Asians approached that in Blacks, while the clinical features were intermediate in severity between Blacks and Caucasians. In both Blacks and Asians the disease was more common, more severe and more extensive than in Caucasians.

  1. Systemic sarcoidosis complicated of acute renal failure: about 12 cases.

    PubMed

    Mahfoudhi, Madiha; Mamlouk, Habiba; Turki, Sami; Kheder, Adel

    2015-01-01

    The sarcoidosis is a systemic granulomatosis affecting most frequently the lungs and the mediastinum. An acute renal failure reveals exceptionally this disease. It's a retrospective study implicating 12 cases of sarcoidosis complicated of acute renal failure. The aim of this study is to determine epidemiological, clinical, biological and histological profile in these cases and then to indicate the interest to consider the diagnosis of sarcoidosis in cases of unexplained renal failure. Extra-renal complications, therapeutic modalities and the outcome were determined in all patients. Our series involved 12 women with an average age of 40 years. Biological investigations showed an abnormal normocalcemia in 7 cases, a hypercalcemia in 5 cases, a hypercalciuria in 10 cases and polyclonal hypergammaglobulinemia in 7 cases. An acute renal failure was found in all patients with a median creatinin of 520 umol/L. For all patients, the renal echography was normal however, the kidney biopsy showed tubulo-interstitial nephritis. The extra-renal signs highlighting pulmonary interstitial syndrome in 5 cases, a sicca syndrome in 4 cases, mediastinal lymph nodes in 2 cases, a lymphocytic alveolitis in 3 cases, an anterior granulomatous uveitis in 2 cases and a polyarthritis in 5 cases. Five patients benefited of hemodialysis. The treatment consisted of corticosteroid in all cases. The follow up was marked by complete resolution of clinical and biological signs. The diagnosis of renal sarcoidosis must be done quickly to prevent renal failure.

  2. An epidemiological perspective of the pathology and etiology of sarcoidosis.

    PubMed

    Sawahata, Michiru; Sugiyama, Yukihiko

    2016-01-01

    To update current knowledge on the pathology and etiology of sarcoidosis, here we review previous epidemiological research and discuss age-related differences and historical changes in the clinical characteristics of sarcoidosis we identified over the last four decades in Japan. Extrathoracic lymph node involvement was more common in young patients, while extrathoracic involvement of non-lymphatic organs and hypercalcemia were more common in older patients. Most patients in their 20s presented with bilateral hilar lymphadenopathy, but this was consistently less common among older patients. Over time, the distribution of age at diagnosis has shifted toward the older age group in the United States, Denmark, and Japan. In Japan, the incidence rate has been decreasing among young people, but there has consistently been a second peak among postmenopausal women. Age-related differences in the clinical presentation of sarcoidosis may reflect the pathways of causative antigens and the strengthening of immunoregulatory mechanisms with age. Internal and external environmental factors, such as exposure to diverse microorganisms, ovarian insufficiency, and active vitamin D deficiency, that may contribute to the onset of sarcoidosis must be identified in order to develop strategies for prevention and treatment. PMID:27537712

  3. Systemic sarcoidosis complicated of acute renal failure: about 12 cases

    PubMed Central

    Mahfoudhi, Madiha; Mamlouk, Habiba; Turki, Sami; Kheder, Adel

    2015-01-01

    The sarcoidosis is a systemic granulomatosis affecting most frequently the lungs and the mediastinum. An acute renal failure reveals exceptionally this disease. It's a retrospective study implicating 12 cases of sarcoidosis complicated of acute renal failure. The aim of this study is to determine epidemiological, clinical, biological and histological profile in these cases and then to indicate the interest to consider the diagnosis of sarcoidosis in cases of unexplained renal failure. Extra-renal complications, therapeutic modalities and the outcome were determined in all patients. Our series involved 12 women with an average age of 40 years. Biological investigations showed an abnormal normocalcemia in 7 cases, a hypercalcemia in 5 cases, a hypercalciuria in 10 cases and polyclonal hypergammaglobulinemia in 7 cases. An acute renal failure was found in all patients with a median creatinin of 520 umol/L. For all patients, the renal echography was normaln however, the kidney biopsy showed tubulo-interstitial nephritis. The extra-renal signs highlighting pulmonary interstitial syndrome in 5 cases, a sicca syndrome in 4 cases, mediastinal lymph nodes in 2 cases, a lymphocytic alveolitis in 3 cases, an anterior granulomatous uveitis in 2 cases and a polyarthritis in 5 cases. Five patients benefited of hemodialysis. The treatment consisted of corticosteroid in all cases. The follow up was marked by complete resolution of clinical and biological signs. The diagnosis of renal sarcoidosis must be done quickly to prevent renal failure. PMID:26834928

  4. Sarcoidosis in Native and Transplanted Kidneys: Incidence, Pathologic Findings, and Clinical Course

    PubMed Central

    Bagnasco, Serena M.; Gottipati, Srinivas; Kraus, Edward; Alachkar, Nada; Montgomery, Robert A.; Racusen, Lorraine C.; Arend, Lois J.

    2014-01-01

    Renal involvement by sarcoidosis in native and transplanted kidneys classically presents as non caseating granulomatous interstitial nephritis. However, the incidence of sarcoidosis in native and transplant kidney biopsies, its frequency as a cause of end stage renal disease and its recurrence in renal allograft are not well defined, which prompted this study. The electronic medical records and the pathology findings in native and transplant kidney biopsies reviewed at the Johns Hopkins Hospital from 1/1/2000 to 6/30/2011 were searched. A total of 51 patients with a diagnosis of sarcoidosis and renal abnormalities requiring a native kidney biopsy were identified. Granulomatous interstitial nephritis, consistent with renal sarcoidosis was identified in kidney biopsies from 19 of these subjects (37%). This is equivalent to a frequency of 0.18% of this diagnosis in a total of 10,023 biopsies from native kidney reviewed at our institution. Follow-up information was available in 10 patients with biopsy-proven renal sarcoidosis: 6 responded to treatment with prednisone, one progressed to end stage renal disease. Renal sarcoidosis was the primary cause of end stage renal disease in only 2 out of 2,331 transplants performed. Only one biopsy-proven recurrence of sarcoidosis granulomatous interstitial nephritis was identified. Conclusions Renal involvement by sarcoidosis in the form of granulomatous interstitial nephritis was a rare finding in biopsies from native kidneys reviewed at our center, and was found to be a rare cause of end stage renal disease. However, our observations indicate that recurrence of sarcoid granulomatous inflammation may occur in the transplanted kidney of patients with sarcoidosis as the original kidney disease. PMID:25329890

  5. Unmasking sarcoidosis following surgery for Cushing disease

    PubMed Central

    Diernaes, Jon E.F.; Bygum, Anette; Poulsen, Per L.

    2016-01-01

    ABSTRACT We present a patient with Cushing disease apparently suppressing sarcoidosis, which was unmasked following surgical resection of a pituitary adrenocorticotropin (ACTH)-producing microadenoma. Case report and a short review of the literature published in this area. A 46-year-old Caucasian woman presented with symptoms of hypercortisolism such as progressive weight gain, Cushingoid appearance, proximal myopathy, easy bruising, and amenorrhea. Blood testing including inferior petrosal sinus sampling uncovered an ACTH-producing microadenoma in the right aspect of the anterior pituitary gland for which the patient underwent transphenoidal resection. Maintenance corticosteroid therapy was implemented, and the signs and symptoms of Cushing disease began to resolve. Three months after surgery, multiple erythematous painful nodules developed on the patient's arms. Erythema nodosum (EN) was diagnosed clinically and a suspicion of underlying sarcoidosis was substantiated by lung imaging and elevated plasma interleukin (IL)-2 receptor. One month later, the lesions spontaneously resolved without therapy other than maintenance glucocorticoid replacement. Physicians should be aware that patients undergoing successful treatment of Cushing syndrome may have a flare-up or emergence of a corticosteroid-responsive disease. PMID:27375832

  6. Unmasking sarcoidosis following surgery for Cushing disease.

    PubMed

    Diernaes, Jon E F; Bygum, Anette; Poulsen, Per L

    2016-01-01

    We present a patient with Cushing disease apparently suppressing sarcoidosis, which was unmasked following surgical resection of a pituitary adrenocorticotropin (ACTH)-producing microadenoma. Case report and a short review of the literature published in this area. A 46-year-old Caucasian woman presented with symptoms of hypercortisolism such as progressive weight gain, Cushingoid appearance, proximal myopathy, easy bruising, and amenorrhea. Blood testing including inferior petrosal sinus sampling uncovered an ACTH-producing microadenoma in the right aspect of the anterior pituitary gland for which the patient underwent transphenoidal resection. Maintenance corticosteroid therapy was implemented, and the signs and symptoms of Cushing disease began to resolve. Three months after surgery, multiple erythematous painful nodules developed on the patient's arms. Erythema nodosum (EN) was diagnosed clinically and a suspicion of underlying sarcoidosis was substantiated by lung imaging and elevated plasma interleukin (IL)-2 receptor. One month later, the lesions spontaneously resolved without therapy other than maintenance glucocorticoid replacement. Physicians should be aware that patients undergoing successful treatment of Cushing syndrome may have a flare-up or emergence of a corticosteroid-responsive disease. PMID:27375832

  7. The Klatskin Tumor That Wasn’t: An Unusual Presentation of Sarcoidosis

    PubMed Central

    Potosky, Darryn R.

    2016-01-01

    We present the case of a patient who presented with signs and symptoms associated with a Klatskin tumor. After endoscopic retrograde cholangiopancreatography (ERCP) and biopsy, she was found instead to have granulomatous infiltration of the extrahepatic biliary tree consistent with biliary sarcoidosis. The patient was treated successfully with systemic corticosteroids and azathioprine. She later developed cutaneous, lymphatic, and pulmonary granulomatous disease. Isolated biliary disease is a rare initial presentation of systemic sarcoidosis. PMID:27761479

  8. Interleukin-2 Receptor and Angiotensin-Converting Enzyme as Markers for Ocular Sarcoidosis

    PubMed Central

    Gundlach, Enken; Hoffmann, Michael Marcus; Prasse, Antje; Heinzelmann, Sonja; Ness, Thomas

    2016-01-01

    Purpose To study the impact of soluble IL2 receptor (sIL2R), chest x-ray (CxR), and angiotensin-converting enzyme (ACE) as markers for sarcoidosis in uveitis patients. Design Retrospective study. Methods Serum concentrations of sIL2R and ACE were measured in patients with active uveitis. Those with elevated sIL2R and /or ACE values were examined for suspected systemic sarcoidosis. Main Outcome Measure Our main outcome parameters were the specificity and sensitivity of sIL2R, CxR and ACE in screening for ocular sarcoidosis. Results We measured 261 patients with uveitis for sarcoidosis using sIL2R and ACE between January 2008 and November 2011; sarcoidosis was been diagnosed using other tests (e.g. computer tomography, brochoalveolar lavage, biopsy) in 41 of 53 patients with elevated sIL2R values (>639 U/ml) and in one patient with normal sIL2R (582 U/ml). Their mean sIL2R value was 1310 U/ml, extending from 582 to 8659 U/ml. Only 9 patients, however, presented elevated ACE (>82 U/l). Their mean ACE value was 116.4 U/l, ranging from 84.1 to 175.5 U/l. IL2R specificity was 94% with 98% sensitivity. In contrast, ACE had a specificity of 99.5%, but a sensitivity of only 22%; the chest x-ray had a specificity of 100% with 50% sensitivity in detecting sarcoidosis. We observed the entire spectrum of uveitis: sixteen patients suffered from anterior, 8 from intermediate, 16 from posterior, and 2 from panuveitis. Conclusions An elevated level of soluble IL2R suggests sarcoidosis with uveitis more convincingly than ACE, making sIL2R a more effective marker parameter for sarcoidosis than ACE or chest x-ray in uveitis patients. PMID:26799486

  9. [Radiation studies in the detection of early signs of lung tissue lesion in sarcoidosis].

    PubMed

    Stashuk, G A; Dubrova, S E

    2007-01-01

    The capacities of various radiation techniques in the study of patients with pulmonary sarcoidosis are analyzed. The sequence of their use, which ensures a high informative value, is proposed. By analyzing the X-ray semiotics in 45 patients with pulmonary sarcoidosis, the authors give the diagnostic signs of early lung parenchymal damage in this disease. Particular emphasis is placed on the procedure of lung X-ray computed tomography.

  10. Steroid Refractory Autoimmune Haemolytic Anaemia Secondary to Sarcoidosis Successfully Treated with Rituximab and Mycophenolate Mofetil.

    PubMed

    Green, Sarah; Partridge, Erica; Idedevbo, Edore; Borg, Anton

    2016-01-01

    Autoimmune haemolytic anaemia is not a well-recognised complication of sarcoidosis. We describe the case of a 30-year-old female who presented with acute warm haemolytic anaemia and widespread lymphadenopathy. Sarcoidosis was diagnosed on lymph node biopsy and further investigation. The haemolytic anaemia responded only to a high dose of steroids. Evidence regarding treatment of steroid refractory autoimmune haemolysis secondary to sarcoidosis is lacking. Based on the emergent evidence that both disorders share common immunopathogenic mechanisms involving Th1 and Th17 lymphocytes, our patient was given rituximab and mycophenolate mofetil to successfully suppress the haemolysis and sarcoid activity. PMID:27563474

  11. Steroid Refractory Autoimmune Haemolytic Anaemia Secondary to Sarcoidosis Successfully Treated with Rituximab and Mycophenolate Mofetil

    PubMed Central

    Idedevbo, Edore; Borg, Anton

    2016-01-01

    Autoimmune haemolytic anaemia is not a well-recognised complication of sarcoidosis. We describe the case of a 30-year-old female who presented with acute warm haemolytic anaemia and widespread lymphadenopathy. Sarcoidosis was diagnosed on lymph node biopsy and further investigation. The haemolytic anaemia responded only to a high dose of steroids. Evidence regarding treatment of steroid refractory autoimmune haemolysis secondary to sarcoidosis is lacking. Based on the emergent evidence that both disorders share common immunopathogenic mechanisms involving Th1 and Th17 lymphocytes, our patient was given rituximab and mycophenolate mofetil to successfully suppress the haemolysis and sarcoid activity. PMID:27563474

  12. Sarcoidosis presenting as severe renin-dependent hypertension due to kidney vascular injury

    PubMed Central

    Rafat, Cedric; Bobrie, Guillaume; Chedid, Antoine; Nochy, Dominique; Hernigou, Anne; Plouin, Pierre-François

    2014-01-01

    Renal sarcoidosis embraces a wide variety of clinical patterns. Renal vascular involvement has seldom been reported and usually in the setting of systemic vasculitis. We report the case of a 22-year-old patient in whom inaugural manifestation of renal sarcoidosis consisted of severe hypertension associated with bilateral perfusion defects and tumour-like nodules. In the setting of renal sarcoidosis, our case suggests that renin-dependant hypertension may arise from renal ischaemia as a result of extrinsic compression of kidney blood vessels due to severe granulomatous inflammation. PMID:25852913

  13. "End-stage" pulmonary fibrosis in sarcoidosis.

    PubMed

    Teirstein, Alvin T; Morgenthau, Adam S

    2009-02-01

    Pulmonary fibrosis is an unusual "end stage" in patients with sarcoidosis. Fibrosis occurs in a minority of patients, and presents with a unique physiologic combination of airways dysfunction (obstruction) superimposed on the more common restrictive dysfunction. Imagin techniques are essential to the diagnosis, assessment and treatment of pulmonary fibrosis. Standard chest radiographs and CT scans may reveal streaks, bullae, cephalad retraction of the hilar areas, deviation of the trachea and tented diaphragm. Positive gallium and PET scans indicate residual reversible granulomatous disease and are important guides to therapy decisions. Treatment, usually with corticosteroids, is effective in those patients with positive scans, but fibrosis does not improve with any treatment. With severe functional impariment and patient disability, pulmonary hypertension and right heart failure may supervene for which the patient will require treatment. Oxygen, careful diuresis, sildenafil and bosentan may be salutary. These patients are candidates for lung transplantation. PMID:19170216

  14. Granulomatous cutaneous sarcoidosis: diagnosis, relationship to systemic disease, prognosis and treatment.

    PubMed

    Mañá, Juan; Marcoval, Joaquim; Rubio, Manuel; Labori, Maria; Fanlo, Marta; Pujol, Ramón

    2013-12-17

    Sarcoidosis is an antigen-mediated disease of unknown aetiology, characterized by the presence of non-caseating epithelioid cell granulomas in multiple organs. Cutaneous involvement in sarcoidosis is classified as specific, when biopsy reveals non-caseating granulomas, and non-specific, typically erythema nodosum. Granulomatous skin lesions occur in 9% to 37% of patients. The skin is the second most commonly involved organ after the lung. A skin biopsy is easy to perform and enables an early diagnosis with a minor invasive procedure. Some types of specific lesions have prognostic significance and may help to predict the outcome of the systemic disease. Maculopapules, subcutaneous nodules and scar sarcoidosis are usually transient or tend to follow the course of the systemic disease. Skin plaques and lupus pernio are associated with chronic sarcoidosis. Although most cutaneous lesions of sarcoidosis do not cause significant morbidity and do not require treatment, some have cosmetic importance because they may be disfiguring and can have a strong psychosocial impact. Treatment of these lesions is a challenge since they do not respond well to conventional treatments. This manuscript reviews the clinical characteristics of the more frequent types of specific cutaneous lesions of sarcoidosis, the relationship between cutaneous involvement and systemic disease, the prognostic significance of lesions and the present state of treatment of difficult cases of cutaneous sarcoidosis.

  15. A case of scar sarcoidosis

    PubMed Central

    Hong, Young Chul; Han, Sang Hoon; Lee, Yang Deok; Cho, Yong Seon; Han, Min Soo

    2008-01-01

    Infiltration of sarcoid granuloma in old cutaneous scars is one of the uncommon cutaneous manifestations of sarcoidosis. Here, we report the case of a 47-year-old female who presented with swelling and irritation in 5 old scars. She had acquired these scars 9 years ago in a traffic accident. An incisional scar biopsy revealed noncaseating granulomas consistent with sarcoidosis. High-resolution CT (HRCT) revealed right paratracheal, both hilar, paraaortic, and subcarinal lymphadenopathy without any nodular densities in both lung fields. Successful regression of cutaneous inflammation was achieved using a short course of oral steroids. PMID:19119259

  16. Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis

    PubMed Central

    Nunes, H; Humbert, M; Capron, F; Brauner, M; Sitbon, O; Battesti, J‐P; Simonneau, G; Valeyre, D

    2006-01-01

    Background Pulmonary hypertension (PH) is a rare complication of sarcoidosis, although it is not uncommon in advanced disease. Methods A retrospective series of 22 sarcoidosis patients (16 men) of mean (SD) age 46 (13) years with PH was divided into two groups depending on the absence (stage 0: n = 2, stage II: n = 4, stage III: n = 1) or presence (n = 15) of radiographic pulmonary fibrosis at the time of PH diagnosis. Results In both groups PH was moderate to severe and there was no response to acute vasodilator challenge. In non‐fibrotic cases no other cause of PH was found, suggesting a specific sarcoidosis vasculopathy, although no histological specimens were available. In cases with fibrosis there was no correlation between haemodynamics and lung volumes or arterial oxygen tensions, suggesting other mechanisms for PH in addition to pulmonary destruction and hypoxaemia. These included extrinsic arterial compression by lymphadenopathies in three cases and histologically proven pulmonary veno‐occlusive disease in the five patients who underwent lung transplantation. Ten patients received high doses of oral prednisone for PH (stage 0: n  =  1, stage II: n  =  4 and stage IV: n  =  5); three patients without pulmonary fibrosis experienced a sustained haemodynamic response. Survival of the overall population was poor (59% at 5 years). Mortality was associated with NYHA functional class IV but not with haemodynamic parameters or with lung function. Conclusion Two very different phenotypes of sarcoidosis combined with PH are observed depending on the presence or absence of pulmonary fibrosis. PH is a severe complication of sarcoidosis. PMID:16227329

  17. Optic neuropathy associated with systemic sarcoidosis

    PubMed Central

    Burton, Ben J.; Graham, Elizabeth M.; Plant, Gordon T.

    2016-01-01

    Objective: To identify and follow a series of 52 patients with optic neuropathy related to sarcoidosis. Methods: Prospective observational cohort study. Results: The disorder was more common in women and affected a wide age range. It was proportionately more common in African and Caribbean ethnic groups. Two clinical subtypes were identified: the more common was a subacute optic neuropathy resembling optic neuritis; a more slowly progressive optic neuropathy arose in the remaining 17%. Sixteen (31%) were bilateral. Concurrent intraocular inflammation was seen in 36%. Pain arose in only 27% of cases. An optic perineuritis was seen in 2 cases, and predominate involvement of the chiasm in one. MRI findings showed optic nerve involvement in 75% of cases, with adjacent and more widespread inflammation in 31%. Treatment with corticosteroids was helpful in those with an inflammatory optic neuropathy, but not those with mass lesions. Relapse of visual signs arose in 25% of cases, necessitating an increase or escalation of treatment, but relapse was not a poor prognostic factor. Conclusions: This is a large prospective study of the clinical characteristics and outcome of treatment in optic neuropathy associated with sarcoidosis. Patients who experience an inflammatory optic neuropathy respond to treatment but may relapse. Those with infiltrative or progressive optic neuropathies improve less well even though the inflammatory disorder responds to therapy. PMID:27536707

  18. Molecular analysis of sarcoidosis lymph nodes for microorganisms: a case–control study with clinical correlates

    PubMed Central

    Robinson, Lary A; Smith, Prudence; SenGupta, Dhruba J; Prentice, Jennifer L; Sandin, Ramon L

    2013-01-01

    Introduction Sarcoidosis is an incurable, chronic granulomatous disease primarily involving the lungs and lymph nodes of unknown aetiology, treated with non-specific anti-inflammatory/immunosuppressive drugs. Persistently symptomatic patients worsen with a disabling, potentially fatal clinical course. To determine a possible infectious cause, we correlated in a case-control study the clinical information with the presence of bacterial DNA in sarcoidosis mediastinal lymph nodes compared with control lymph nodes resected during cancer surgery. Methods We retrospectively studied formalin-fixed, paraffin-embedded, mediastinal lymph nodes from 30 patients with sarcoidosis and 30 control patients with lung cancer. Nucleic acids were extracted from nodes, evaluated by ribosomal RNA PCR for bacterial 16S ribosomal DNA and the results were sequenced and compared with a bacterial sequence library. Clinical information was correlated. Results 11/30 (36.7%) of lymph nodes from patients with sarcoidosis had detectable bacterial DNA, significantly more than control patient lymph nodes (2/30, 6.7%), p=0.00516. At presentation, 19/30 (63.3%) patients with sarcoidosis were symptomatic including all patients with detectable bacterial DNA. Radiographically, there were 18 stage I and 12 stage II patients. All stage II patients were symptomatic and 75% had PCR-detectable bacteria. After a mean follow-up of 52.8±32.8 months, all patients with PCR-detectable bacteria in this series were persistently symptomatic requiring treatment. Discussion 36.6% of patients with sarcoidosis had detectable bacterial DNA on presentation, all of these patients were quite symptomatic and most were radiographically advanced stage II. These findings suggest that bacterial DNA-positive, symptomatic patients have more aggressive sarcoidosis that persists long term and might benefit from antimicrobial treatment directed against this presumed chronic granulomatous infection. PMID:24366580

  19. What Are the Signs and Symptoms of Sarcoidosis?

    MedlinePlus

    ... Twitter. What Are the Signs and Symptoms of Sarcoidosis? Many people who have sarcoidosis have no signs ... symptom is more common in women than men. Sarcoidosis Signs and Symptoms The illustration shows the major ...

  20. Activated alveolar macrophage and lymphocyte alveolitis in extrathoracic sarcoidosis without radiological mediastinopulmonary involvement

    SciTech Connect

    Wallaert, B.; Ramon, P.; Fournier, E.C.; Prin, L.; Tonnel, A.B.; Voisin, C.

    1986-01-01

    Cellular characteristics of BAL were investigated in 18 patients with proved extrathoracic sarcoidosis (that is, sarcoidosis that affected the skin, eyes, parotid glands, stomach, nose, kidneys, or meninges) without clinical or radiological mediastinopulmonary involvement. Computed tomography of the thorax was performed on five patients: four patients were normal, and one had enlarged lymph nodes (these enlargements were not detectable on the patient's chest roentgenogram). The results of pulmonary function tests were normal in all patients. The total BAL cell count did not differ significantly between controls and patients. Abnormal percentages of alveolar lymphocytes (from 18 to 87%) were noted in 15 out of 18 patients. SACE levels were normal in 15 patients. No pulmonary gallium uptake was detected. The chemiluminescence of AM's, whether spontaneous or PMA induced, was increased in five out of seven patients. The percentages of T3+ lymphocytes in sarcoidosis patients did not significantly differ from those in controls. The T4+:T8+ ratio was normal in four patients and slightly increased in one. Follow-up of patients showed that alveolar lymphocytosis is as lasting as extrathoracic involvement. Our data demonstrate increased percentages of lymphocytes and activated AM's in the BAL of patients with extrathoracic sarcoidosis. This may be due to the initial involvement of the respiratory tract in extrathoracic sarcoidosis or to the diffusion of activated macrophages and lymphocytes from an extrathoracic site into the lung.

  1. SPECT imaging with Tl-201 and Ga-67 in myocardial sarcoidosis

    SciTech Connect

    Kurata, C.; Sakata, K.; Taguchi, T.; Fukumoto, Y.; Miyata, H.; Aoshima, S.; Yamazaki, N. )

    1990-06-01

    Two patients with myocardial sarcoidosis are presented, both of whom underwent SPECT imaging with Tl-201 and Ga-67. The first had Ga-67 myocardial uptake with a Tl-201 defect, which disappeared with corticosteroid therapy. The second had multiple Tl-201 defects without Ga-67 uptake, which persisted despite corticosteroid therapy. Therefore, the combination of Tl-201 and Ga-67 imaging may be useful for recognizing myocardial sarcoidosis and for predicting the response to corticosteroid therapy.

  2. Tumoral calcinosis associated with sarcoidosis and positive bone and gallium imaging

    SciTech Connect

    Wolpe, F.M.; Khedkar, N.Y.; Gordon, D.; Werner, P.; Shirazi, P.; Al-Sabban, M.H.

    1987-07-01

    A 63-year-old female with biopsy proven tumoral calcinosis presented with progressive and recurrent swelling and tenderness of the right hip, thigh, elbow, and wrist. Both gallium and bone imaging demonstrated intense, congruent uptake in these areas. This is the third case of tumoral calcinosis with sarcoidosis documented in the literature. However, these are the first published bone and gallium scans in a patient with a history of sarcoidosis and tumoral calcinosis.

  3. Isolated oculocutaneous sarcoidosis in a teenage male: a rare case report.

    PubMed

    Aggarwal, H K; Jain, D; Mittal, A; Dabas, G

    2016-01-01

    Sarcoidosis is an inflammatory disease with multisystem involvement characterized by the presence of noncaseating granulomas. It can affect virtually every organ of the body, with lung involvement being most common occurring in >90% of patients. Other organs affected are skin, eye and liver. Skin involvement is common, affecting 25-35%. Here we present a rare case of a 15 year-old male with isolated oculocutaneous sarcoidosis without systemic involvement. PMID:27608799

  4. Isolated oculocutaneous sarcoidosis in a teenage male: a rare case report.

    PubMed

    Aggarwal, H K; Jain, D; Mittal, A; Dabas, G

    2016-01-01

    Sarcoidosis is an inflammatory disease with multisystem involvement characterized by the presence of noncaseating granulomas. It can affect virtually every organ of the body, with lung involvement being most common occurring in >90% of patients. Other organs affected are skin, eye and liver. Skin involvement is common, affecting 25-35%. Here we present a rare case of a 15 year-old male with isolated oculocutaneous sarcoidosis without systemic involvement.

  5. Cardiac sarcoidosis: diagnosis and management.

    PubMed

    Dubrey, S W; Sharma, R; Underwood, R; Mittal, T

    2015-07-01

    Cardiac sarcoidosis is one of the most serious and unpredictable aspects of this disease state. Heart involvement frequently presents with arrhythmias or conduction disease, although myocardial infiltration resulting in congestive heart failure may also occur. The prognosis in cardiac sarcoidosis is highly variable, which relates to the heterogeneous nature of heart involvement and marked differences between racial groups. Electrocardiography and echocardiography often provide the first clue to the diagnosis, but advanced imaging studies using positron emission tomography and MRI, in combination with nuclear isotope perfusion scanning are now essential to the diagnosis and management of this condition. The identification of clinically occult cardiac sarcoidosis and the management of isolated and/or asymptomatic heart involvement remain both challenging and contentious. Corticosteroids remain the first treatment choice with the later substitution of immunosuppressive and steroid-sparing therapies. Heart transplantation is an unusual outcome, but when performed, the results are comparable or better than heart transplantation for other disease states. We review the epidemiology, developments in diagnostic techniques and the management of cardiac sarcoidosis.

  6. Assessment of gallium-67 scanning in pulmonary and extrapulmonary sarcoidosis

    SciTech Connect

    Israel, H.L.; Gushue, G.F.; Park, C.H.

    1986-01-01

    Gallium-67 scans have been widely employed in patients with sarcoidosis as a means of indicating alveolitis and the need for corticosteroid therapy. Observation of 32 patients followed 3 or more years after gallium scans showed no correlation between findings and later course: of 10 patients with pulmonary uptake, 7 recovered with minor residuals; of 18 patients with mediastinal of extrathoracic uptake, 10 had persistent or progressive disease; of 4 patients with negative initial scans, 2 had later progression. The value of gallium-67 scans as an aid to diagnosis was studied in 40 patients with extrapulmonary sarcoidosis. In 12 patients, abnormal lacrimal, nodal, or pulmonary uptake aided in selection of biopsy sites. Gallium-67 scans and serum ACE levels were compared in 97 patients as indices of clinical activity. Abnormal gallium-67 uptake was observed in 96.3% of the tests in active disease, and ACE level elevation occurred in 56.3%. In 24 patients with inactive or recovered disease, abnormal gallium-67 uptake occurred in 62.5% and ACE level elevation in 37.5%. Gallium-67 scans have a limited but valuable role in the diagnosis and management of sarcoidosis.

  7. Sarcoidosis and multiple myeloma: Concurrent presentation of an unusual association.

    PubMed

    Nair, Vidya; Prajapat, Deepak; Talwar, Deepak

    2016-01-01

    Literature on concurrent association of sarcoidosis with lymphoproliferative malignancies other than lymphoma e.g. multiple myeloma is meager. The rarity of the situation prompted us to report this patient who was a 51-year-old woman with a 2-years history of breathlessness, cough with expectoration, chest pain and backache. Initial evaluation revealed mild anemia, increased alkaline phosphatase with chest skiagram showing both lower zone non homogenous opacities with calcified hilar lymph nodes. CECT chest showed mediastinal with bilateral hilar lymphadenopathy, parenchymal fibrosis, traction bronchiectasis, ground glass opacities, septal and peribronchovascular thickening affecting mid and lower lung zones bilaterally. MRI Dorsolumbar spine was suggestive of marrow infiltrative disorder. EBUS FNA of intrathoracic nodes, EBB and TBLB confirmed sarcoidosis. PET CT revealed hyper metabolic activity in lung, multiple lymph nodes and lytic bone lesions. Serum protein electrophoresis and immunofixation revealed a monoclonal paraprotein, immunoglobulin IgG kappa type. Bone marrow biopsy revealed an increase in plasma cells (15%), but no granulomas. Diagnosis of Indolent or multiple myeloma with sarcoidosis was established. 12 cases of sarcoidosis and multiple myeloma have been reported in literature, and mostly preceding the onset of multiple myeloma by many years, in our case both were diagnosed concurrently. PMID:26933313

  8. Sarcoidosis and multiple myeloma: Concurrent presentation of an unusual association

    PubMed Central

    Nair, Vidya; Prajapat, Deepak; Talwar, Deepak

    2016-01-01

    Literature on concurrent association of sarcoidosis with lymphoproliferative malignancies other than lymphoma e.g. multiple myeloma is meager. The rarity of the situation prompted us to report this patient who was a 51-year-old woman with a 2-years history of breathlessness, cough with expectoration, chest pain and backache. Initial evaluation revealed mild anemia, increased alkaline phosphatase with chest skiagram showing both lower zone non homogenous opacities with calcified hilar lymph nodes. CECT chest showed mediastinal with bilateral hilar lymphadenopathy, parenchymal fibrosis, traction bronchiectasis, ground glass opacities, septal and peribronchovascular thickening affecting mid and lower lung zones bilaterally. MRI Dorsolumbar spine was suggestive of marrow infiltrative disorder. EBUS FNA of intrathoracic nodes, EBB and TBLB confirmed sarcoidosis. PET CT revealed hyper metabolic activity in lung, multiple lymph nodes and lytic bone lesions. Serum protein electrophoresis and immunofixation revealed a monoclonal paraprotein, immunoglobulin IgG kappa type. Bone marrow biopsy revealed an increase in plasma cells (15%), but no granulomas. Diagnosis of Indolent or multiple myeloma with sarcoidosis was established. 12 cases of sarcoidosis and multiple myeloma have been reported in literature, and mostly preceding the onset of multiple myeloma by many years, in our case both were diagnosed concurrently. PMID:26933313

  9. Primary Cardiac Sarcoidosis with Syncope and Refractory Atrial Arrhythmia: A Case Report and Review of the Literature

    PubMed Central

    Thangam, Manoj; Nathan, Sriram; Kar, Biswajit; Petrovic, Marija; Patel, Manish; Loyalka, Pranav; Buja, L. Maximilian

    2016-01-01

    We discuss the case of a 38-year-old black man who presented at our hospital with his first episode of syncope, recently developed atrial arrhythmias refractory to pharmacologic therapy, and a left atrial thrombus. He was diagnosed with primary cardiac sarcoidosis characterized by predominant involvement of the epicardium that caused atrial fibrillation and atrial flutter. Histologic analysis of his epicardial lesions yielded a diagnosis of sarcoidosis. This patient's atrial arrhythmia was successfully treated with a hybrid operation that involved resection of his atrial appendage, an Epicor maze procedure, and radiofrequency ablation during a catheter-based electrophysiologic study. The cardiac sarcoidosis was successfully managed with corticosteroid therapy. Our case report shows that sarcoidosis can initially manifest itself as syncope with new-onset atrial arrhythmia. Sarcoidosis is important in the differential diagnosis because of its progressive nature and its potential for treatment with pharmacologic, surgical, and catheter-based interventions. PMID:27303240

  10. Cigarette smoking and male sex are independent and age concomitant risk factors for the development of ocular sarcoidosis in a new orleans sarcoidosis population

    PubMed Central

    Janot, Adam C.; Huscher, Dörte; Walker, McCall; Grewal, Harmanjot K.; Yu, Mary; Lammi, Matthew R.; Saketkoo, Lesley Ann

    2016-01-01

    Introduction Sarcoidosis is a multi-organ system granulomatous disease of unknown origin with an incidence of 1–40/100,000. Though pulmonary manifestations are predominant, ocular sarcoidosis (OS) affects 25–50% of patients with sarcoidosis and can lead to blindness. Methods A retrospective, single-center chart review of sarcoidosis cases investigated variables associated with the development of OS. Inclusion criteria were biopsy-proven sarcoidosis, disease duration greater than 1 year, documented smoking status on chart review and documentation of sarcoid-related eye disease. Multivariate analysis identified independent risk factors for OS. Results Of 269 charts reviewed, 109 patients met inclusion criteria. The OS group had a significantly higher proportion of smokers (71.4%) than without OS (42.0%, p=0.027) with no difference (p=0.61) in median number of pack years. Male sex was significantly higher in the OS group (57.1% versus 26.1%, p=0.009). Median duration of sarcoidosis was higher in the OS group (10 versus 4 years, p=0.031). Multivariate regression identified tobacco exposure (OR=5.25, p=0.007, 95% CI 1.58–17.41), male sex (OR=7.48, p=0.002, 95% CI 2.15–26.01), and age (OR=1.114, p=0.002, 95% CI 1.04–1.19) as concomitant risk factors for the development of OS. Conclusion To date, there are few dedicated investigations of risk factors for OS, especially smoking. This investigation identified male sex, age, and tobacco exposure as independent risk factors for OS. Though disease duration did not withstand regression analysis in this moderately sized group, age at chart review suggests screening for OS should not remit but rather intensify in aging patients with sarcoidosis. PMID:26278693

  11. Isolated sarcoidosis of accessory spleen in the greater omentum: A case report

    PubMed Central

    TU, CHAOYONG; LIN, QIAOMEI; ZHU, JINGDE; SHAO, CHUXIAO; ZHANG, KUN; JIANG, CHUAN; DING, ZHIYONG; ZHOU, XINGMU; TU, JIEFEI; ZHU, WANLIN; CHEN, WEI

    2016-01-01

    Sarcoidosis is a multisystemic disease of unknown origin characterized by the formation of non-caseating granulomas. Thoracic involvement is the most common presentation; however, sarcoidosis can involve almost any other organ. To the best of our knowledge there have been only 10 cases of splenic sarcoidosis reported in the English literature, with no reports of sarcoidosis of an accessory spleen. The present study reports a case of isolated sarcoidosis of an accessory spleen in the greater omentum, which was identified postoperatively in a 44-year-old female. Chest X-ray results were normal. Gastric endoscopy demonstrated an ulcer in the antrum, which was confirmed to be a signet-ring cell carcinoma via biopsy. Computed tomography of the abdomen revealed mild thickening of the posterior antrum, and a mass in the inferior pole of the left kidney. Intraoperatively, no masses were detected in the liver and spleen. Moreover, no enlarged lymph nodes were detected in the abdominal cavity, pelvic cavity, mesenteric and para-aorta. Following a radical distal gastrectomy and left radical nephrectomy, postoperative pathology demonstrated signet-ring cell carcinoma in the antrum, left renal clear cell cancer and a red lesion measuring 0.5×0.5 cm in the greater omentum, which was similar to the spleen in the splenic cavity and was regarded as an accessory spleen. Following exclusion of fungi and acid-fast bacilli as causative agents, sarcoidosis of the accessory spleen in the greater omentum was confirmed. The patient recovered uneventfully and was discharged on day 8 postoperation. The patient remained alive after two-year follow-up without sarcoidosis and malignant tumor recurrence. The present case demonstrated that, intraoperatively, comprehensive exploration should be conducted to exclude the accessory spleen, which may also suffer from sarcoidosis. PMID:27284324

  12. [Hypercalcemia in sarcoidosis--case report, prevalence, pathophysiology and therapeutic options].

    PubMed

    Ackermann, D

    2007-05-01

    Hypercalcemia is a highly prevalent complication of sarcoidosis. A medical history of a patient with sarcoidosis is shown as case report. Depending on the population studied about 2-63% of sarcoidosis patients show hypercalcemia. The major difference in the prevalence of hypercalcemia may be in part due to the undulating course of subacute sarcoidosis, so hypercalcemia may be missed when serum calcium is not frequently measured. Hypercalciuria appears to be twice as prevalent then hypercalcemia and should be looked for in every sarcoidosis patient. Hypercalcemia in sarcoidosis is due to the uncontrolled synthesis of 1,25-dihydroxyvitamin D3 by macrophages. 1,25-dihydroxyvitamin D3 leads to an increased absorption of calcium in the intestine and to an increased resorption of calcium in the bone. Immunoregulatory properties have been ascribed to 1,25-dihydroxyvitamin D3. It is an important inhibitor of interleukin-2 and of interferon-gamma-synthesis, two cytokines that are important in granuloma formation in sarcoidosis. It is thought that 1,25-dihydroxyvitamin D3 counterregulates uncontrolled granuloma formation. Treatment of hypercalcemia depends on the serum level of hypercalcemia and its persistence. Generally sarcoidotic patients should be advised to avoid sun exposition to reduce vitamin D3 synthesis in the skin, to omit fish oils that are rich of vitamin D and to produce more than two liters urine a day by adapting fluid intake. Although severe hypercalcemia seems to be rare, glucocorticosteroid treatment should be started if corrected total calcium level rises beyond 3 mmol/l. If hypercalcemia is symptomatic, treatment should be started even at lower levels. Glucocorticosteroids act by inhibition of the overly 1alpha-hydroxylase activity of macrophages. Alternatively, treatment with chloroquine or ketoconazole can be established. If isolated hypercalciuria without hypercalcemia is present with evidence for recurrent nephrolithiasis, patients can be treated

  13. Bronchial arterial embolisation for massive haemoptysis in cavitary sarcoidosis

    PubMed Central

    Loh, Geoffrey Andrew; Lettieri, Christopher J; Shah, Anita A

    2013-01-01

    A 48-year-old non-smoking man with a 6-year history of pulmonary cavitary sarcoidosis presented with acute onset of haemoptysis of approximately 600 ml. Prior episodes of haemoptysis had resolved only after serial upper lobe wedge resections bilaterally and steroids. A chest CT identified bilateral upper lobe cavitary lesions with extravasation of contrast from a large right upper lobe cavity. The patient underwent urgent bronchial angiography and subsequent bronchial artery embolisation of a left bronchial artery and three right bronchial arteries. He was started on methotrexate and steroids for refractory sarcoidosis. Two years after embolisation, the patient remained haemoptysis-free with his sarcoid well controlled on methotrexate monotherapy. PMID:23355590

  14. On the nature of sarcoidosis.

    PubMed

    Reich, Jerome M

    2012-03-01

    More than 140 years since its recognition as a clinical entity, sarcoidosis remains enigmatic. Its classification as a disease vs. a syndrome is uncertain. Its etiology remains undefined. The "immune paradox" (delayed type hypersensitivity anergy in a setting of exuberant systemic granulomatous response) resists explanation. Its relationship to the Kveim test is poorly understood. Its prognostic determinants and treatment indications are among the unsolved or disputed problems. Immunological investigations generated the thesis that the characterizing systemic granuloma arise as a fallback reaction to inefficient cellular immune processing, due most often to impaired myeloid dendritic cell function of unknown cause. The concept that sarcoidosis represents a (genetically conditioned) default to a more primitive immunological response provides a unifying explanation for its development in persons with a variety of antigenic exposures and in individuals with cellular immune deficiencies. It furnishes a coherent explanation for the apparent paradox that individuals exhibiting the most intense cellular response experience the most favorable outcomes and for the adverse effect of corticosteroid-suppression in recent onset sarcoidosis.

  15. Flexible transbronchial needle aspiration in the diagnosis of sarcoidosis.

    PubMed

    Morales, C F; Patefield, A J; Strollo, P J; Schenk, D A

    1994-09-01

    The histopathologic diagnosis of sarcoidosis requires the presence of noncaseating granulomas. Transbronchoscopic lung biopsy (TBLB) has been considered the procedure of choice when less invasive tissue samples are unavailable. A total of 51 consecutive patients suspected of having sarcoidosis underwent combined TBLB and flexible transbronchial needle aspirate (TBNA). In 18 of the 30 patients (60 percent) with stage I disease, the diagnosis was confirmed by TBLB and 16 (53 percent) were confirmed by TBNA. The combined use of both procedures increased the diagnostic yield to 83 percent. The remaining 21 patients with stage II disease had their diagnosis confirmed in 16 (76 percent) cases by TBLB and 10 (48 percent) by TBNA with a combined diagnostic yield of 86 percent. Seven (23 percent) patients with stage I disease and 2 (10 percent) with stage II disease had their conditions diagnosed by TBNA. We conclude that combining TBNA with TBLB increases the diagnostic yield in pulmonary sarcoidosis; TBNA should complement TBLB in the diagnosis of this disease. PMID:8082345

  16. Systemic Sarcoidosis Unmasked by Cushing's Disease Surgical Treatment.

    PubMed

    Bongetta, Daniele; Zoia, Cesare; Lombardi, Francesco; Lovati, Elisabetta; Lucotti, Pietro; Gaetani, Paolo

    2016-01-01

    Diseases responsive to glucocorticoids, like sarcoidosis, are rarely masked by Cushing's syndrome. An ACTH secreting pituitary adenoma is a possible cause of Cushing's syndrome and its resection can make a subclinical sarcoidosis clear. Only few cases of sarcoidosis following the treatment of hypercortisolism are reported in literature. We report a case of sarcoidosis after the resection of an ACTH secreting pituitary adenoma. PMID:27525010

  17. Systemic Sarcoidosis Unmasked by Cushing's Disease Surgical Treatment

    PubMed Central

    Lombardi, Francesco; Lovati, Elisabetta; Gaetani, Paolo

    2016-01-01

    Diseases responsive to glucocorticoids, like sarcoidosis, are rarely masked by Cushing's syndrome. An ACTH secreting pituitary adenoma is a possible cause of Cushing's syndrome and its resection can make a subclinical sarcoidosis clear. Only few cases of sarcoidosis following the treatment of hypercortisolism are reported in literature. We report a case of sarcoidosis after the resection of an ACTH secreting pituitary adenoma. PMID:27525010

  18. Parotid gland biopsy and /sup 67/Ga imaging correlation in systemic sarcoidosis

    SciTech Connect

    Brantley, S.D.; Orzel, J.A.; Weiland, F.L.; Bower, J.H.

    1987-03-01

    We correlated the results of parotid gland biopsy, chest roentgenograms, and gallium citrate scintigraphy in 24 patients evaluated for possible systemic sarcoidosis. Of 19 patients ultimately proven to have sarcoidosis, 11 (57.9 percent) had positive parotid gland biopsy. The yield of parotid gland biopsy in patients with abnormal gallium parotid activity was only marginally higher (64.7 percent). Abnormal parotid gland uptake of gallium citrate was seen in 17 of these 19 patients (89.5 percent) and was always associated with abnormal lung or perihilar activity. The parotid gland biopsy is a useful technique for obtaining the tissue diagnosis of sarcoidosis; however, gallium scintigraphy should not be performed to select patients as this will only marginally increase the biopsy yield.

  19. Shortfalls in imputing sarcoidosis to occupational exposures.

    PubMed

    Reich, Jerome M

    2013-04-01

    The cause of sarcoidosis remains elusive. Reports of elevated sarcoidosis incidence in New York City firefighters and World Trade Center disaster responders have been advanced to support a causal relationship. This inference is open to question due to methodological differences in assessing and computing sarcoidosis incidence in populations versus putative occupational exposures. The magnitude of the odds ratio (OR; ca. 1.5) of causal candidates in the ACCESS case-control study of occupational and environmental exposures is sufficiently small that it might easily be attributable to confounders. Additionally, multiplicity of comparisons, difficulty in assembling a valid control population and the potential for recall bias critically limit causal inferences. A possible explanation for etiological elusiveness and multiplicity of elevated OR is that individuals with sarcoidosis, lacking components of efficient cellular immunity, respond with systemic granulomas to a variety of ubiquitous, frequently unidentifiable environmental antigens. Epidemiological methods for the identification of sarcoidosis causal candidates are potentially misleading and are unlikely to prove useful.

  20. Radionuclide imaging in myocardial sarcoidosis. Demonstration of myocardial uptake of /sup 99m/Tc pyrophosphate and gallium

    SciTech Connect

    Forman, M.B.; Sandler, M.P.; Sacks, G.A.; Kronenberg, M.W.; Powers, T.A.

    1983-03-01

    A patient had severe congestive cardiomyopathy secondary to myocardial sarcoidosis. The clinical diagnosis was confirmed by radionuclide ventriculography, /sup 201/Tl, /sup 67/Ga, and /sup 99m/Tc pyrophosphate (TcPYP) scintigraphy. Myocardial TcPYP uptake has not been reported previously in sarcoidosis. In this patient, TcPYP was as useful as gallium scanning and thallium imaging in documenting the myocardial process.

  1. Ga-67 uptake in the lung in sarcoidosis

    SciTech Connect

    Johnson, D.G.; Johnson, S.M.; Harris, C.C.; Piantadosi, C.A.; Blinder, R.A.; Coleman, R.E.

    1984-02-01

    Images were obtained with Ga-67 and bronchopulmonary lavage performed in 21 patients with sarcoidosis (31 studies). The Ga-67 index, a semiquantitative criterion, was compared to a quantitative computer index based on lung:liver activity ratios; accuracy in predicting active alveolitis (defined by lavage lymphocyte counts) was assessed and differences between 24- and 48-hour studies examined. Computer activity ratios correlated well with the Ga-67 index, which had a sensitivity of 64%, specificity of 71%, 82%, and 77%, respectively, for the computer scores. Scores at 24 and 48 hours were similar. These results suggest that (a) Ga-67 scanning is useful in staging activity in pulmonary sarcoidosis, (b) quantitative computer scores are accurate in predicting disease activity, and (c) scanning can be performed 24 or 48 hours after injection.

  2. Expression Profile of Six RNA-Binding Proteins in Pulmonary Sarcoidosis

    PubMed Central

    Novosadova, Eva; Hagemann-Jensen, Michael; Kullberg, Susanna; Kolek, Vitezslav; Grunewald, Johan; Petrek, Martin

    2016-01-01

    Background Sarcoidosis is characterised by up-regulation of cytokines and chemokine ligands/receptors and proteolytic enzymes. This pro-inflammatory profile is regulated post-transcriptionally by RNA-binding proteins (RBPs). We investigated in vivo expression of six RBPs (AUF1, HuR, NCL, TIA, TIAR, PCBP2) and two inhibitors of proteolytic enzymes (RECK, PTEN) in pulmonary sarcoidosis and compared it to the expression in four control groups of healthy individuals and patients with other respiratory diseases: chronic obstructive pulmonary disease (COPD), asthma and idiopathic interstitial pneumonias (IIPs). Methods RT-PCR was used to quantify the mRNAs in bronchoalveolar (BA) cells obtained from 50 sarcoidosis patients, 23 healthy controls, 30 COPD, 19 asthmatic and 19 IIPs patients. Flow cytometry was used to assess intracellular protein expression of AUF1 and HuR in peripheral blood T lymphocytes (PBTLs) obtained from 9 sarcoidosis patients and 6 healthy controls. Results Taking the stringent conditions for multiple comparisons into consideration, we consistently observed in the primary analysis including all patients regardless of smoking status as well as in the subsequent sub-analysis limited for never smokers that the BA mRNA expression of AUF1 (p<0.001), TIA (p<0.001), NCL (p<0.01) and RECK (p<0.05) was decreased in sarcoidosis compared to healthy controls. TIA mRNA was also decreased in sarcoidosis compared to both obstructive pulmonary diseases (COPD and asthma; p<0.001) but not compared to IIPs. There were several positive correlations between RECK mRNA and RBP mRNAs in BA cells. Also sarcoidosis CD3+, CD4+ and CD8+ PBTLs displayed lower mean fluorescence intensity of AUF1 (p≤0.02) and HuR (p≤0.03) proteins than control healthy PBTLs. Conclusion mRNA expressions of three RBPs (AUF1, TIA and NCL) and their potential target mRNA encoding RECK in BA cells and additionally protein expression of AUF1 and HuR in PBTLs were down-regulated in our sarcoidosis

  3. Mycobacterium tuberculosis as a sarcoid factor? A case report of family sarcoidosis

    PubMed Central

    Piotrowski, Wojciech J.; Górski, Paweł; Duda-Szymańska, Joanna; Kwiatkowska, Sylwia

    2014-01-01

    Patient: Male, 26 Final Diagnosis: Sarcoidosis Symptoms: Disseminated lung parenchymal changes Medication: — Clinical Procedure: — Specialty: Pulmonology Objective: Rare disease Background: Sarcoidosis is a granulomatous inflammatory disease that is induced by unknown antigen(s) in a genetically susceptible host. Although the direct link between Mycobacterium tuberculosis (MTB) infection and sarcoidosis can be excluded on the basis of a current knowledge, the non-infectious mechanisms may explain the causative role of mycobacterial antigens. The co-incidence of tuberculosis (TB) and sarcoidosis, and higher incidence of mycobacterial DNA in biological samples of sarcoid patients, have been reported by many authors. Case Report: We present a case in which MTB infection in 1 family member triggered a sarcoid reaction in the infected subject and 2 other non-infected family members. We discuss different aspects of diagnosis and differentiation, as well as up-to-date hypotheses on the possible mechanisms leading to sarcoid inflammation in patients exposed to MTB. Conclusions: This case series documents the possibility of familial spreading of sarcoidosis, and points to MTB as a potential etiological factor. PMID:24847413

  4. Granulomatous thyroiditis: an unexpected finding leading to the diagnosis of sarcoidosis.

    PubMed

    Gentilucci, Umberto Vespasiani; Picardi, Antonio; Manfiini, Silvia; D'Avola, Delia; Costantino, Sebastiano; Pozzilli, Paolo

    2004-04-01

    A 59-year old male presented with dyspnoea, dull pain at the base of the neck, fever, and a history of severe recurrent coronary disease. In the past he had undergone two angioplasties, multiple stenting, and a triple aorto-coronary bypass. The patient also experienced a painful enlargement of the thyroid gland with bilateral multiple lymphoadenopathies and thyrotoxicosis. Thyroid histology revealed interstitial non-caseating granulomas that are typical of thyroid sarcoidosis. The finding ofgranulomas in the thyroid allowed us to interpret the clinical manifestations of our patient leading to the diagnosis of sarcoidosis. The patient was ultimately treated for sarcoidosis and after one year of specific therapy has completely recovered without recurrence of cardiac or respiratory symptoms up to date.

  5. Immunological Evidence for the Role of Mycobacteria in Sarcoidosis: A Meta-Analysis

    PubMed Central

    Fang, Chuling; Huang, Hui; Xu, Zuojun

    2016-01-01

    Background Sarcoidosis is a granulomatous disease, the etiology of which is currently unknown. The role of mycobacteria in the etiology of sarcoidosis has been extensively investigated. In this meta-analysis, we assessed the immunological evidence of the possible role of mycobacteria in the pathogenesis and development of sarcoidosis. Methods We performed a systematic search of relevant articles from PubMed, Embase and Cochrane Library databases published between January 1990 and October 2015. Data extracted from the articles were analyzed with Review Manager 5.3 (Cochrane Collaboration, Oxford, UK). Results In this meta-analysis, 13 case-control studies (733 participants) were considered eligible according to our criteria. Methodological quality was assessed using the Newcastle-Ottawa Scale (NOS). The positivity incidence of the immune response (either the cell-mediated response or humoral response) in sarcoidosis patients was significantly higher than that in controls, as determined using fixed-effects model. The odds ratio (OR) of the positivity incidence of T-cell response in the patients with sarcoidosis versus the controls with PPD- or unknown PPD status was 5.54 (95% CI 3.56–8.61); the ORs were 16.70 (95% CI 8.19–34.08) and 1.48 (95% CI 0.74–2.96) for the two subgroups with PPD- controls and unknown PPD status respectively. However, the OR of the positivity incidence in patients with sarcoidosis versus PPD+ controls (latent tuberculosis infection; LTBI) was 0.26 (95% 0.10–0.66). Regarding the humoral response, pooled analysis of the positivity incidence revealed an OR (95%CI) of 20.43 (5.53–75.53) for the patients with sarcoidosis versus controls; the ORs were 11.93 (95% CI 2.15–66.27) and 41.97 (95% CI 5.24–336.15) in two subgroups of controls with PPD- and unknown PPD statuses respectively. Data on heterogeneity and evidence of publication bias were examined. Conclusions This meta-analysis confirmed the existence of an association between

  6. A critical analysis of sarcoidosis incidence assessment.

    PubMed

    Reich, Jerome M

    2013-01-01

    Valid sarcoidosis incidence assessment is contingent on access to medical care, thoroughness of reportage, assiduity of radiographic interpretation, employment and health care screening policies, misclassification, and population ethnicity. To diminish ambiguity and foster inter-population comparison, the term "sarcoidosis incidence" must be modified to convey the methodology employed in compiling the numerator. In age-delimited cohorts, valid comparison to population incidence requires age adjustment due to the age-dependency of incidence. The "true incidence" of sarcoidosis is a notional concept: more than 90% of cases are subclinical and radiographically inevident. Occupational causal inference based on incidence differential vs. populations has been undermined by methodological differences in ascertainment and computation.

  7. Sarcoidosis in a dental surgeon: a case report

    PubMed Central

    2010-01-01

    Introduction Although the causes of sarcoidosis are still unknown, past and current studies have provided evidence that this disease may be associated with occupational exposure to specific environmental agents. We describe a case of sarcoidosis in a dental surgeon with long exposure to inorganic dusts. To the best of our knowledge, this is the first report of this kind in the literature. Case presentation At the beginning of 2000, a 52-year-old Caucasian man, who worked as a dental surgeon, presented with shortness of breath during exercise, cough and retrosternal pain. After diagnosis of sarcoidosis, a scanning electronic microscopy with X-ray microanalysis of biopsy specimens was used in order to determine whether the disease could be traced to an occupational environmental agent. Results showed the presence of inorganic dust particles within sarcoidotic granulomas, and demonstrated that the material detected was identical to that found in a powder used by our patient for several years. Conclusions Although these results cannot be considered as definitive proof, they do however provide strong evidence that this disease may be associated with material used by dental surgeons. PMID:20698948

  8. Isolated muscular sarcoidosis causing fever of unknown origin: The value of gallium-67 imaging

    SciTech Connect

    Patel, N.; Krasnow, A.; Sebastian, J.L.; Collier, B.D.; Hellman, R.S.; Isitman, A.T. )

    1991-02-01

    An unusual case of a patient with a long-standing fever of unknown origin (FUO) is presented whose gallium-67 ({sup 67}Ga) images revealed increased activity only in the calf muscles bilaterally. Other imaging modalities also failed to show chest or other abnormal findings. Subsequent biopsy of the right gastrocnemius muscle revealed noncaseating granulomas consistent with the diagnosis of sarcoidosis. When using {sup 67}Ga to evaluate a patient with a FUO, imaging of the extremities should always be included. Also, when abnormal Ga-67 uptake is present in the extremities, sarcoidosis should be included in the differential diagnosis.

  9. Thallium-201 scintigraphy in the diagnosis and management of myocardial sarcoidosis

    SciTech Connect

    Fields, C.L.; Ossorio, M.A.; Roy, T.M.; Denny, D.M.; Varga, D.W. )

    1990-03-01

    We have described three patients with clinical evidence of myocardial sarcoidosis to illustrate the utility of thallium-201 scintigraphy in demonstrating the myocardial lesions. Both the symptomatic and asymptomatic individuals studied showed the characteristic reverse redistribution phenomenon. No abnormalities were seen during the exercise phase of the thallium study, but myocardial defects were detected in each patient when repeat studies were obtained at rest six hours later. Steroid therapy resolved the defects in each case. We propose thallium-201 scintigraphy of the heart as a safe and useful tool for documenting myocardial involvement in sarcoidosis and following the effects of therapy.

  10. Sarcoidosis occuring during anti-TNF-alpha treatment for inflammatory rheumatic diseases: report of two cases.

    PubMed

    Toussirot, E; Pertuiset, E; Kantelip, B; Wendling, D

    2008-01-01

    Anti-TNF-alpha agents have been tried in cases of refractory sarcoidosis, giving favourable results. Thus, the occurrence of a granulomatous disease in a patient receiving such drug seems paradoxical. We describe 2 patients with inflammatory rheumatic disease, the first with ankylosing spondylitis, the second with rheumatoid arthritis, under anti-TNF-alpha treatment (infliximab and etanercept respectively) who developed non-caseating granulomas of the lungs and lymph nodes consistent with the diagnosis of sarcoidosis. Limited and various similar cases have been reported. It is generally considered that these granulomatous diseases are related to the anti-TNF-alpha agent.

  11. A case of sarcoidosis diagnosed by positron emission tomography/computed tomography

    PubMed Central

    Aksoy, Sabire Yilmaz; Özdemir, Elif; Sentürk, Aysegül; Türkölmez, Seyda

    2016-01-01

    Sarcoidosis is a multisystem granulomatous disorder of unknown cause which may affect any organ or system but primarily involve the lungs and the lymphatic system. Extrapulmonary sarcoidosis represents approximately 30-50% of patients. We report the case of a 51-year-old female who presented with increasing complaints of a cough, weakness, weight loss, and chest pain and who was found to have a suspicious lesion on thorax computed tomography(CT). Fluorodeoxyglucose (FDG) positron emission tomography/CT performed for diagnostic purposes demonstrated increased FDG accumulation at the bilateral enlarged parotid and lacrimal gland and in the reticulonodular infiltration area located in the left lung as well as multiple lymphadenopathies with increased FDG accumulation. There were also hepatosplenomegaly and splenic uptake. Skin biopsy showed noncaseating granulomas, and the patient was diagnosed as stage 2 sarcoidosis. PMID:27385890

  12. A case of sarcoidosis diagnosed by positron emission tomography/computed tomography.

    PubMed

    Aksoy, Sabire Yilmaz; Özdemir, Elif; Sentürk, Aysegül; Türkölmez, Seyda

    2016-01-01

    Sarcoidosis is a multisystem granulomatous disorder of unknown cause which may affect any organ or system but primarily involve the lungs and the lymphatic system. Extrapulmonary sarcoidosis represents approximately 30-50% of patients. We report the case of a 51-year-old female who presented with increasing complaints of a cough, weakness, weight loss, and chest pain and who was found to have a suspicious lesion on thorax computed tomography(CT). Fluorodeoxyglucose (FDG) positron emission tomography/CT performed for diagnostic purposes demonstrated increased FDG accumulation at the bilateral enlarged parotid and lacrimal gland and in the reticulonodular infiltration area located in the left lung as well as multiple lymphadenopathies with increased FDG accumulation. There were also hepatosplenomegaly and splenic uptake. Skin biopsy showed noncaseating granulomas, and the patient was diagnosed as stage 2 sarcoidosis. PMID:27385890

  13. Intramedullary Sarcoidosis Presenting with Delayed Spinal Cord Swelling after Cervical Laminoplasty for Compressive Cervical Myelopathy

    PubMed Central

    Kwon, Du Ho; Kim, Eun-Sang; Eoh, Whan

    2014-01-01

    Sarcoidosis is a systemic disease of unknown etiology that may affect any organ in the body. The nervous system is involved in 5-16% of cases of sarcoidosis. Here, we report a case of intramedullary sarcoidosis presenting with delayed spinal cord swelling after laminoplasty for the treatment of compressive cervical myelopathy. A 56-year-old woman was admitted to our hospital complaining of upper extremity pain and gait disturbance. The patient had undergone laminoplasty for compressive cervical myelopathy 3 months previously. Follow-up magnetic resonance imaging revealed a large solitary intramedullary lesion with associated extensive cord swelling, signal changes, and heterogeneous enhancement of spinal cord from C2 to C7. Spinal cord biopsy revealed non-necrotizing granulomas with signs of chronic inflammation. The final diagnosis of sarcoidosis was based upon laboratory data, imaging findings, histological findings, and the exclusion of other diagnoses. Awareness of such presentations and a high degree of suspicion of sarcoidosis may help arrive at the correct diagnosis. PMID:25535524

  14. Bronchoalveolar lavage, serum angiotensin-converting enzyme, and /sup 67/Ga scanning in extrathoracic sarcoidosis

    SciTech Connect

    Wallaert, B.; Ramon, P.; Fournier, E.; Tonnel, A.B.; Voisin, C.

    1982-11-01

    Results of bronchoalveolar lavage (BAL), 67Ga scanning, and serum angiotensin-converting enzyme (SACE) assay are compared in the assessment of pulmonary involvement in ten cases of extrathoracic sarcoidosis. Standard clinical, radiologic, and pulmonary function tests detected no pulmonary changes in these patients, but BAL demonstrated an increased alveolar lymphocytosis in eight of ten cases. SACE levels were increased in two cases, and the thoracic gallium uptake was normal in all cases. BAL appears to be the best technique for diagnosing latent pulmonary involvement in extrathoracic sarcoidosis.

  15. Sarcoidosis imitating breast cancer metastasis: a case report and literature review

    PubMed Central

    Kochoyan, Teimuraz; Akhmedov, Mobil; Shabanov, Alexander; Terekhov, Ilya

    2016-01-01

    Sarcoidosis is a benign systematic granulomatous disorder of unknown etiology and is associated with various malignancies. However, granulomatous and metastatic lymph node lesions are difficult to distinguish even when using precise and modern diagnostic methods, such as positron emission tomography. Thus, histological verification is the only method that can be used to accurately describe the nature of this disease. In this article, we report a case of non-luminal HER-2/neu-positive breast cancer in a patient without history of sarcoidosis and suspected to have metastatic disease. PMID:27807507

  16. [The x-ray morphological characteristics of the early manifestations of sarcoidosis].

    PubMed

    Khomenko, A G; Dmitrieva, L I; Khikkel', G G; Gedymin, L E

    1990-01-01

    The paper is devoted to an analysis of combined x-ray findings supplemented by CT findings in patients with sarcoidosis detected for the first time with early signs of this disease. A conclusion has been made that apart from lymph node enlargement, sarcoidosis is characterized by lung parenchymal changes which manifest themselves as symptoms of broncho-alveolitis. Characteristic x-ray symptom complexes are described. Roentgeno-semiotics of these changes were compared with the results of morphological investigation of transbronchial puncture specimens. Roentgenologically detectable changes showed good correlation with morphology findings. The authors have also emphasized the importance of morphological verification of this disease at early stages.

  17. Sonographic Findings of Subcutaneous Sarcoidosis in 3 Cases.

    PubMed

    Tada, Saori; Watanabe, Tsuneo; Suzuki, Chigusa; Nohisa, Yuzuru; Shinoda, Koichi; Furuta, Nobuyuki; Ito, Hiroyasu; Okumura, Yoko; Shu, En; Seishima, Mariko

    2015-08-01

    Subcutaneous sarcoidosis occurs infrequently among cases of cutaneous sarcoidosis. To our knowledge, few studies have described the sonographic characteristics of subcutaneous sarcoidosis. Here we report the sonographic characteristics of 3 cases of this condition. Our results revealed 4 features: (1) an irregular hypoechoic appearance, (2) heterogeneous echogenicity, (3) perilesional hyperechoic changes, and (4) abnormal Doppler signals. Sonography is a rapid, simple, and noninvasive procedure that is useful for initial evaluation of granulomatous lesions such as subcutaneous sarcoidosis.

  18. Concurrence of sarcoidosis and aortitis: case report and review of the literature

    PubMed Central

    Weiler, V; Redtenbacher, S; Bancher, C; Fischer, M; Smolen, J

    2000-01-01

    Takayasu arteritis (TA) is a rare manifestation of systemic large vessel vasculitis which affects predominantly the aorta and its main branches, but often remains unrecognised owing to delayed diagnosis and non-characteristic clinical features. Sarcoidosis, too, is a systemic inflammatory disease which can affect virtually any organ system. Reports about the coincidence of both diseases have appeared.
  The case presented here is characterised by a significant time lag between detection of TA and appearance of clinical signs of sarcoidosis. The woman, now 39 years old, had erythema nodosum, circumscript alopecia, and recurrent uveitis, which dated back to 1980 and was attributed to sarcoidosis. At least 12 years later aortic valve insufficiency with progressive cardiac failure developed. Histology performed at the time of aortic valve prosthesis in 1997 disclosed a diagnosis of TA, which was confined to the aortic root. Incidentally, sarcoidosis was diagnosed in adjacent lymph nodes. A thorough check up failed to detect further manifestations of TA; thus, possibly, the patients had aortitis similar to, but not identical with, TA.
Several related cases previously reported are discussed, suggesting that both diseases may be inherently related as they are characterised by certain non-specific, immunoinflammatory abnormalities. This case report suggests that the prevalence of TA, or related forms of arteritis, may be higher than expected and should be considered, especially in younger patients with non-characteristic cardiovascular symptoms and suspected systemic inflammatory disease. Moreover, the association with sarcoidosis in this and other previously described cases suggests that the two diseases may be related and that TA or TA-like vasculitis may even be a complication of sarcoidosis.

 PMID:11053059

  19. Yield of EBUS-TBNA for the diagnosis of sarcoidosis: impact of operator and cytopathologist experience

    PubMed Central

    Navasakulpong, Asma; Auger, Manon; Gonzalez, Anne V

    2016-01-01

    Background Studies have reported a high diagnostic yield of endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) for the diagnosis of sarcoidosis. We sought to review the yield of EBUS-TBNA for the diagnosis of sarcoidosis at our institution over time, and examine factors that may influence this yield. Methods Patients who underwent EBUS-TBNA for suspected sarcoidosis between December 2008 and November 2011 were identified. EBUS was performed without rapid on-site evaluation (ROSE) of samples. The final diagnosis was based on the results of all invasive diagnostic procedures and/or clinical follow-up. Logistic regression analysis was used to examine the effect of various factors on yield. Results 43 patients underwent 45 EBUS-TBNA procedures for suspected sarcoidosis. A total of 115 lymph nodes were sampled. The 21 G needle was used in 51% of procedures. The mean number of lymph node stations sampled was 2.6 (SD 0.7) and the mean number of needle passes per procedure was 7.8 (SD 2.0). Non-necrotising granulomatous inflammation was detected in EBUS-TBNA samples from 34/45 (76%) procedures. The overall diagnostic yield increased to 36/45 (80%) following a cytopathology review for this study. Needle gauge, number of lymph node stations sampled and number of needle passes were not associated with diagnostic yield. The yield of EBUS-TBNA increased significantly after the first 15 procedures performed for suspected sarcoidosis; the 2 additional cases diagnosed after the cytopathology review were part of this early experience. Conclusions EBUS-TBNA is a valuable technique for the diagnosis of sarcoidosis when performed without ROSE. The yield of the procedure improved significantly over time, based on operator and cytopathologist experience. PMID:27547408

  20. Increased expression of CCL4/MIP-1β in CD8+ cells and CD4+ cells in sarcoidosis.

    PubMed

    Barczyk, A; Pierzchała, E; Caramori, G; Sozańska, E

    2014-01-01

    Sarcoidosis is a granulomatous disease with an increased accumulation of T cells in lungs as a result of on-site proliferation and chemotaxis induced by chemokines. It has already been demonstrated that CCL3-5 levels were increased in BAL fluid of sarcoidosis patients. To analyze the expression of CCL3-5 chemokines by T-cell subtypes (CD4+, CD8+, Th1, Th2, Tc1 or Tc2) in the lungs of sarcoidosis patients, fifteen untreated sarcoidosis patients and eighteen control subjects were enrolled in this study. CD4+ and CD8+ cells were isolated from BAL fluid by positive magnetic selection. The expression of CCL3-5 and other cytokines in CD4+ and CD8+ cells were measured by flow cytometry. The percentage of CD4+ or CD8+ cells expressing CCL4 were significantly higher in sarcoidosis patients (22.3% and 58.1%) compared to those seen in healthy subjects (11.1% and 16.5%, P = 0.04 and P = 0.02, respectively). In addition, the expression of CCL3, CCL4 and CCL5 was significantly elevated in CD8+ cells (8.9%, 58.1% and 2.1%) compared to CD4+ cells (2.1%, 22.3% and 0.7%; P = 0.04, P = 0.009 and P = 0.04, respectively), whereas CCL4 was expressed by significantly more Tc1 than Th1 cells in sarcoidosis patients (P = 0.006). Our study shows the possible role of CD8+ cells and CD4+ cells in recruiting T cells to the site of inflammation in sarcoidosis through the release of CCL4, either alone or together with Th1/Tc1-associated cytokines.

  1. Vitamin-D status and mineral metabolism in two ethnic populations with sarcoidosis.

    PubMed

    Capolongo, Giovanna; Xu, Li Hao Richie; Accardo, Mariasofia; Sanduzzi, Alessandro; Stanziola, Anna Agnese; Colao, Annamaria; Agostini, Carlo; Zacchia, Miriam; Capasso, Giovambattista; Adams-Huet, Beverley; Moe, Orson W; Maalouf, Naim M; Sakhaee, Khashayar; Hsia, Connie C W

    2016-06-01

    Vitamin-D insufficiency and sarcoidosis are more common and severe in African Americans (AA) than Caucasians. In sarcoidosis, substrate-dependent extrarenal 1,25-dihydroxyvitamin-D (1,25-(OH)2D) production is thought to contribute to hypercalciuria and hypercalcemia, and vitamin-D repletion is often avoided. However, the anti-inflammatory properties of vitamin-D may also be beneficial. We prospectively examined serum vitamin-D levels, calcium balance, and the effects of vitamin-D repletion in 86 AA and Caucasian patients with biopsy-proven active sarcoidosis from the USA (US) and Italy (IT) in university-affiliated outpatient clinics. Clinical features, pulmonary function, and calciotropic hormones were measured. 16 patients with vitamin-D deficiency and normal serum ionized calcium (Ca(2+)) were treated with oral ergocalciferol (50,000 IU/week) for 12 weeks. Baseline mineral parameters were similar in US (93% AA) and IT (95% Caucasian) patients irrespective of glucocorticoid treatment. Pulmonary dysfunction was less pronounced in IT patients. Nephrolithiasis (in 11% US, 17% IT patients) was associated with higher urinary calcium excretion. Vitamin-D deficiency was not more prevalent in patients compared to the respective general populations. As serum 25-hydroxyvitamin-D (25-OHD) rose postrepletion, serum 1,25-(OH)2D, γ-globulins, and the previously elevated angiotensin converting enzyme (ACE) levels declined. Asymptomatic reversible increases in Ca(2+) or urinary calcium/creatinine (Ca/Cr) developed in three patients during repletion. In conclusion, Caucasian and AA patients show similar calcium and vitamin D profiles. The higher prevalence of hypercalciuria and nephrolithiasis in sarcoidosis is unrelated to endogenous vitamin-D levels. Vitamin-D repletion in sarcoidosis is generally safe, although calcium balance should be monitored. A hypothesis that 25-OHD repletion suppresses granulomatous immune activity is provided. PMID:27053725

  2. Prevalence of cardiac sarcoidosis in white population: a case–control study

    PubMed Central

    Martusewicz-Boros, Magdalena M.; Boros, Piotr W.; Wiatr, Elżbieta; Zych, Jacek; Piotrowska-Kownacka, Dorota; Roszkowski-Śliż, Kazimierz

    2016-01-01

    Abstract Cardiac sarcoidosis (CS) is a life-threatening and underdiagnosed manifestation of the disease, which requires a complicated and expensive diagnostic pathway. There is a need for simple tool for practitioners to determine the risk of CS without access to specialized equipment. The aim of study was to determine the prevalence of CS in a group of patients diagnosed with or followed up because of sarcoidosis. A secondary objective was the search for factors associated with heart involvement. We performed a prospective case–control study (screening analysis) in consecutive sarcoidosis patients collected from October 2012 to September 2015. Cardiac magnetic resonance (CMR) imaging was performed to confirm or exclude cardiac involvement in all patients. The study was conducted in a hospital-based referral center for patients with sarcoidosis and other interstitial lung diseases. Analysis was performed in a group of 201 patients (all white) with biopsy-proven sarcoidosis, mean age 41.4 ± 10.2, 121 of them (60.2%) males. Four patients with previously recognized cardiac diseases, which make CMR imaging for CS inconclusive, were not included. Cardiac involvement was detected by CMR in 49 patients (24.4%). Factors associated with an increased risk of CS (univariate analyses) included male sex (odds ratio [OR]: 2.5; 1.21–5.16, P = 0.01), cardiac-related symptoms (OR: 3.53; 1.81–6.89, P = 0.0002), extrathoracic sarcoidosis (OR: 3.48; 1.77–6.84, P = 0.0003), elevated serum NT-proBNP (OR: 3.82; 1.55–9.42, P = 0.004), any electrocardiography abnormality (OR: 5.38; 2.48–11.67, P = 0.0001), and contemporary radiological progression sarcoidosis in the lungs (OR: 2.98; 1.52–5.84, P = 0.001). Abnormalities in echocardiography and Holter ECG were also risk factors, but not significant in multivariate analyses. A CS Risk Index was developed using a multivariate model to predict CS, achieving an accuracy of 82%, sensitivity of 50

  3. Multiplex immune serum biomarker profiling in sarcoidosis and systemic sclerosis.

    PubMed

    Beirne, P; Pantelidis, P; Charles, P; Wells, A U; Abraham, D J; Denton, C P; Welsh, K I; Shah, P L; du Bois, R M; Kelleher, P

    2009-12-01

    Multiplex protein technology has the potential to identify biomarkers for the differentiation, classification and improved understanding of the pathogenesis of interstitial lung disease. The aim of this study was to determine whether a 30-inflammatory biomarker panel could discriminate between healthy controls, sarcoidosis and systemic sclerosis (SSc) patients independently of other clinical indicators. We also evaluated whether a panel of biomarkers could differentiate between the presence or absence of lung fibrosis in SSc patients. We measured 30 circulating biomarkers in 20 SSc patients, 21 sarcoidosis patients and 20 healthy controls using Luminex bead technology and used Fisher's discriminant function analysis to establish the groups of classification mediators. There were significant differences in median concentration measurements between study groups for 20 of the mediators but with considerable range overlap between the groups, limiting group differentiation by single analyte measurements. However, a 17-analyte biomarker model correctly classified 90% of study individuals to their respective group and another 14-biomarker panel correctly identified the presence of lung fibrosis in SSc patients. These findings, if they are corroborated by independent studies in other centres, have potential for clinical application and may generate novel insights into the modulation of immune profiles during disease evolution. PMID:19541722

  4. Verrucous cutaneous sarcoidosis: case report and review of this unusual variant of cutaneous sarcoidosis.

    PubMed

    Stockman, David L; Rosenberg, Jason; Bengana, Chafik; Suster, Saul; Plaza, Jose A

    2013-04-01

    Sarcoidosis is a multisystem disorder of unknown origin, characterized by the accumulation of lymphocytes and mononuclear histiocytes inducing the formation of noncaseating "naked" epithelioid granulomas. The lungs, lymphatic system, and skin are most often affected, but sarcoidosis may affect any organ. Cutaneous involvement of sarcoidosis is often the sentinel sign of the disease, with the skin sometimes being exclusively affected. We present a case of a 54-year-old African American woman with long-standing history of pulmonary sarcoidosis that presented with multiple verrucous cutaneous lesions on the upper and lower extremities mimicking carcinoma. The initial cutaneous biopsy was superficial in nature, and the pathologist raised the consideration of a possible keratoacanthoma. A deeper skin shave biopsy was performed, and the histopathology showed verrucous pseudoepitheliomatous epidermal hyperplasia with scattered noncaseating granulomas in the superficial dermis. Stains (acid-fast bacillus, Periodic acid-Schiff, and Gomori-Grocott methenamine silver stains) were negative for microorganisms. Given the clinical setting and histomorphology of the cutaneous lesions, the diagnosis of verrucous sarcoidosis was rendered. Verrucous sarcoidosis is a rare cutaneous manifestation of sarcoidosis that could be easily misdiagnosed if it is not appropriately biopsied. This hinders the precise evaluation of the histological specimen, overall clinical picture, and administration of appropriate therapy. PMID:23344007

  5. The Etiologic Role of Infectious Antigens in Sarcoidosis Pathogenesis.

    PubMed

    Celada, Lindsay J; Hawkins, Charlene; Drake, Wonder P

    2015-12-01

    Sarcoidosis is a granulomatous disease of unknown etiology, most commonly involving the lung, skin, lymph node, and eyes. Molecular and immunologic studies continue to strengthen the association of sarcoidosis with infectious antigens. Independent studies report the presence of microbial nucleic acids and proteins within sarcoidosis specimens. Complementary immunologic studies also support the role of infectious agents in sarcoidosis pathogenesis. Case reports and clinical trials have emerged regarding the efficacy of antimicrobials. They support increasing efforts to identify novel therapeutics, such as antimicrobials, that will have an impact on the observed increase in sarcoidosis morbidity and mortality.

  6. Gallium-67 scanning to stage the alveolitis of sarcoidosis: correlation with clinical studies, pulmonary function studies, and bronchoalveolar lavage

    SciTech Connect

    Line, B.R.; Hunninghake, G.W.; Keogh, B.A.; Jones, A.E.; Johnston, G.S.; Crystal, R.G.

    1981-04-01

    Current concepts of the pathogenesis of sarcoidosis suggest that the alveolitis of this disorder is related to increased numbers of mononuclear phagocytes and activated T-lymphocytes within the lung. To determine if 67Ga scanning, a procedure commonly used in the evaluation of inflammation, would be useful in staging the alveolitis of sarcoidosis, we studied 41 patients with this disorder and correlated estimates of pulmonary 67Ga accumulation with clinical, roentgenographic, physiologic, and bronchoalveolar lavage studies in these patients. Although 65% of patients with sarcoidosis showed increased amounts of 67Ga accumulation in the lung compared with control subjects, only weak correlations (r less than +/- 0.42, all comparisons) were found between the degree of gallium uptake and the clinical, roentgenographic, or physiologic data. In contrast, there was a strong correlation of 67Ga uptake and the number of lymphocytes and T-lymphocytes recovered from the lungs of these patients by bronchoalveolar lavage (p less than 0.0001, r greater than or equal to 0.67, both comparisons). This data suggested that gallium uptake reflects the intensity of the T-lymphocytes mediated component of the alveolitis in sarcoidosis. Because 67Ga scans are noninvasive, simple to perform, and widely available, they should prove useful to stage the activity of sarcoidosis and to make decisions regarding therapy directed against the alveolitis of the disease.

  7. Gallium-67 scanning to stage the alveolitis of sarcoidosis: correlation with clinical studies, pulmonary function studies, and bronchoalveolar lavage

    SciTech Connect

    Line, B.R.; Hunninghake, G.W.; Keogh, B.A.; Jones, A.E.; Johnston, G.S.; Crystal, R.G.

    1981-04-01

    Current concepts of the pathogenesis of sarcoidosis suggest that the alveolitis of this disorder is related to increased numbers of mononuclear phagocytes and activated T-lymphocytes within the lung. To determine if 67Ga scanning, a procedure commonly used in the evaluation of inflammation, would be useful in staging the alveolitis of sarcoidosis, researchers studied 41 patients with this disorder and correlated estimates of pulmonary /sup 67/Ga accumulation with clinical, roentgenographic, physiologic, and bronchoalveolar lavage studies in these patients. Although 65% of patients with sarcoidosis showed increased amounts of /sup 67/Ga accumulation in the lung compared with control subjects, only weak correlations (r less than +/- 0.42, all comparisons) were found between the degree of gallium uptake and the clinical, roentgenographic, or physiologic data. In contrast, there was a strong correlation of /sup 67/Ga uptake and the number of lymphocytes and T-lymphocytes recovered from the lungs of these patients by bronchoalveolar lavage (p less than 0.0001, r greater than or equal to 0.67, both comparisons). This data suggested that gallium uptake reflects the intensity of the T-lymphocytes mediated component of the alveolitis in sarcoidosis. Because /sup 67/Ga scans are noninvasive, simple to perform, and widely available, they should prove useful to stage the activity of sarcoidosis and to make decisions regarding therapy directed against the alveolitis of the disease.

  8. Cardiac sarcoidosis mimicking right ventricular dysplasia.

    PubMed

    Shiraishi, Jun; Tatsumi, Tetsuya; Shimoo, Kazutoshi; Katsume, Asako; Mani, Hiroki; Kobara, Miyuki; Shirayama, Takeshi; Azuma, Akihiro; Nakagawa, Masao

    2003-02-01

    A 59-year-old woman with skin sarcoidosis was admitted to hospital for assessment of complete atrioventricular block. Cross-sectional echocardiography showed that the apical free wall of the right ventricle was thin and dyskinetic with dilation of the right ventricle. Thallium-201 myocardial imaging revealed a normal distribution. Both gallium-67 and technetium-99m pyrophosphate scintigraphy revealed no abnormal uptake in the myocardium. Right ventriculography showed chamber dilation and dyskinesis of the apical free wall, whereas left ventriculography showed normokinesis, mimicking right ventricular dysplasia. Cardiac sarcoidosis was diagnosed on examination of an endomyocardial biopsy specimen from the right ventricle. A permanent pacemaker was implanted to manage the complete atrioventricular block. After steroid treatment, electrocardiography showed first-degree atrioventricular block and echocardiography revealed an improvement in the right ventricular chamber dilation. Reports of cardiac sarcoidosis mimicking right ventricular dysplasia are extremely rare and as this case shows, right ventricular involvement may be one of its manifestations.

  9. Sarcoidosis in tuberculosis-endemic regions: India

    PubMed Central

    2013-01-01

    Sarcoidosis is a multisystem inflammatory disease of unknown etiology affecting multiple organs. Earlier reports suggested that sarcoidosis was a disease of the developed world. However, recent reports suggest that the disease is found in the developing countries as well. Clinical, radiological, and histopathological similarities with tuberculosis pose a great challenge in countries endemic for tuberculosis. Mantoux test, high resolution computed tomography, and transbronchial lymph node and lung biopsies are diagnostic modalities, which play an important role in the diagnosis of sarcoid. In this review, we look at the epidemiology of sarcoid in tuberculosis-endemic regions, the sarcoidosis-tuberculosis link, clinical profile, diagnostic modalities, dilemma in the diagnosis, and the treatment of this disease. PMID:23803558

  10. Immunophenotyping of macrophages in human pulmonary tuberculosis and sarcoidosis

    PubMed Central

    Stanton, Lee-Anne; Fenhalls, Gael; Lucas, Andrew; Gough, Peter; Greaves, David R; Mahoney, James A; Helden, Paul Van; Gordon, Siamon

    2003-01-01

    Classic studies of tuberculosis (TB) revealed morphologic evidence of considerable heterogeneity of macrophages (MØs), but the functional significance of this heterogeneity remains unknown. We have used newly available specific antibodies for selected membrane and secretory molecules to examine the phenotype of MØs in situ in a range of South African patients with TB, compared with sarcoidosis. Patients were human immunodeficiency virus-negative adults and children, and the examined biopsy specimens included lung and lymph nodes. Mature pulmonary MØs (alveolar, interstitial, epithelioid and multinucleated giant cells) selectively expressed scavenger receptor type A and a novel carboxypeptidase-like antigen called carboxypeptidase-related vitellogenin-like MØ molecule (CPVL). CPVL did not display enhanced expression in sarcoidosis, vs. TB patients, as observed with angiotensin-converting enzyme (ACE), a related molecule. Immunocytochemical studies with surfactant proteins (SP)-A and -D showed that type II alveolar cells expressed these collectins, as did MØs, possibly after binding of secreted proteins. Studies with an antibody specific for the C-terminus of fractalkine, a tethered CX3C chemokine, confirmed synthesis of this molecule by bronchiolar epithelial cells and occasional endothelial cells. These studies provide new marker antigens and extend previous studies on MØ differentiation, activation and local interactions in chronic human granulomatous inflammation in the lung. PMID:14748748

  11. [Types of skin lesions in pulmonary sarcoidosis and their prognostic value].

    PubMed

    Garmash, Iu Iu; Soldatskaia, L V; Enikeeva, E G; Agadzhanova, I S; Slutskaia, O M

    2007-01-01

    When sarcoidosis is suspected, a patient should be meticulously examined for dermal changes, the favorite sites of which are the skin of the face, ears, upper and lower extremities, and trunk. Elements may vary in number from solitary to multiple. When dermal changes are suspected of sarcoidosis, a dermatologist should examine the patient and, if there are some doubts about the nature of dermal elements, biopsy and histological study should be made since the morphological verification of the diagnosis primarily requires the use of extrapulmonary sites of the process, namely superficial peripheral lymph nodes, as well as the skin. The findings suggest that skin sarcoidosis characterized by the long-term chronic or progressive course is encountered at different (X-ray) stages of sarcoidosis of respiratory organs and is of poor prognostic value. The severity of dermal manifestations is no less and, in individual cases, more significant than pulmonary symptoms. Great or ugly skin lesions require prednisolone treatment. In some cases, long-term therapy cannot yield a result. Delagil treatment of dermal manifestations remains to be effective.

  12. In-Hospital Disease Burden of Sarcoidosis in Switzerland from 2002 to 2012

    PubMed Central

    Pohle, Susanne; Baty, Florent; Brutsche, Martin

    2016-01-01

    Sarcoidosis is a multisystem disease with an unpredictable and sometimes fatal course while the underlying pathomechanism is still unclear. Reasons of the increasing hospitalization rate and mortality in the United States remain in dispute but incriminated are a number of distinct comorbidities and risk factors as well as the application of more aggressive therapeutic agents. Studies reflecting the recent development in central Europe are lacking. Our aim was to investigate the recent mortality and hospitalization rates as well as the underlying comorbidities of hospitalized sarcoidosis patients in Switzerland. In this longitudinal, nested case-control study, a nation-wide database provided by the Swiss Federal Office for Statistics enclosing every hospital entry covering the years 2002–2012 (n = 15,627,573) was analyzed. There were 8,385 cases with a diagnosis of sarcoidosis representing 0.054% (8,385 / 15,627,573) of all hospitalizations in Switzerland. These cases were compared with age- and sex-matched controls without the diagnosis of sarcoidosis. Hospitalization and mortality rates in Switzerland remained stable over the observed time period. Comorbidity analysis revealed that sarcoidosis patients had significantly higher medication-related comorbidities compared to matched controls, probably due to systemic corticosteroids and immunosuppressive therapy. Sarcoidosis patients were also more frequently re-hospitalized (median annual hospitalization rate 0.28 [IQR 0.15-0.65] vs. 0.19 [IQR 0.13-0.36] per year; p < 0.001), had a longer hospital stay (6 [IQR 2-13] vs. 4 [IQR 1-8] days; p < 0.001), had more comorbidities (4 [IQR 2-7] vs. 2 [IQR 1-5]; p < 0.001), and had a significantly higher in-hospital mortality (2.6% [95% CI 2.3%-2.9%] vs. 1.8% [95% CI 1.5%-2.1%] (p < 0.001). A worse outcome was observed among sarcoidosis patients having co-occurrence of associated respiratory diseases. Moreover, age was an important risk factor for re-hospitalization. PMID

  13. In-Hospital Disease Burden of Sarcoidosis in Switzerland from 2002 to 2012.

    PubMed

    Pohle, Susanne; Baty, Florent; Brutsche, Martin

    2016-01-01

    Sarcoidosis is a multisystem disease with an unpredictable and sometimes fatal course while the underlying pathomechanism is still unclear. Reasons of the increasing hospitalization rate and mortality in the United States remain in dispute but incriminated are a number of distinct comorbidities and risk factors as well as the application of more aggressive therapeutic agents. Studies reflecting the recent development in central Europe are lacking. Our aim was to investigate the recent mortality and hospitalization rates as well as the underlying comorbidities of hospitalized sarcoidosis patients in Switzerland. In this longitudinal, nested case-control study, a nation-wide database provided by the Swiss Federal Office for Statistics enclosing every hospital entry covering the years 2002-2012 (n = 15,627,573) was analyzed. There were 8,385 cases with a diagnosis of sarcoidosis representing 0.054% (8,385 / 15,627,573) of all hospitalizations in Switzerland. These cases were compared with age- and sex-matched controls without the diagnosis of sarcoidosis. Hospitalization and mortality rates in Switzerland remained stable over the observed time period. Comorbidity analysis revealed that sarcoidosis patients had significantly higher medication-related comorbidities compared to matched controls, probably due to systemic corticosteroids and immunosuppressive therapy. Sarcoidosis patients were also more frequently re-hospitalized (median annual hospitalization rate 0.28 [IQR 0.15-0.65] vs. 0.19 [IQR 0.13-0.36] per year; p < 0.001), had a longer hospital stay (6 [IQR 2-13] vs. 4 [IQR 1-8] days; p < 0.001), had more comorbidities (4 [IQR 2-7] vs. 2 [IQR 1-5]; p < 0.001), and had a significantly higher in-hospital mortality (2.6% [95% CI 2.3%-2.9%] vs. 1.8% [95% CI 1.5%-2.1%] (p < 0.001). A worse outcome was observed among sarcoidosis patients having co-occurrence of associated respiratory diseases. Moreover, age was an important risk factor for re-hospitalization.

  14. Bronchoalveolar lavage cell counts as a predictor of short term outcome in pulmonary sarcoidosis.

    PubMed

    Foley, N M; Coral, A P; Tung, K; Hudspith, B N; James, D G; Johnson, N M

    1989-09-01

    Sixty seven patients with biopsy proven pulmonary sarcoidosis were prospectively studied to determine whether single point bronchoalveolar lavage cell counts were a useful indicator of functional outcome and whether repeated lavage helped in management. The mean follow up period was 25 (range 13-37) months. No patient was having corticosteroid treatment at the time of initial bronchoalveolar lavage. "High intensity alveolitis" (lymphocyte count greater than or equal to 28%) was present at the initial lavage in 42 patients. These patients showed a significant improvement in their pulmonary function and chest radiographs over the follow up period whereas patients with "low intensity alveolitis" did not. Of the 42 patients with high intensity alveolitis, 31 had chronic sarcoidosis (duration over two years, mean 80 months). These patients showed a significant improvement in FVC but not in TLCO. Corticosteroids resulted in greater functional and radiological improvement in the patients with high intensity alveolitis than in those with low intensity alveolitis. Repeat bronchoalveolar lavage in 34 patients, mean 8.4 months after the original lavage, showed a weak inverse relation between a reduced lymphocyte count and change in forced vital capacity and isotope uptake on a gallium scan. These correlations were too weak to make repeated cell counts useful in management. Our results suggest that high intensity alveolitis may be a favourable prognostic factor for lung function in pulmonary sarcoidosis, even in patients with chronic disease, but that repeat lavage adds little to the management of the individual patient. PMID:2588210

  15. Vertebral sarcoidosis: demonstration of bone involvement by computerized axial tomography

    SciTech Connect

    Dinerstein, S.L.; Kovarsky, J.

    1984-08-01

    A report is given of a rare case of vertebral sarcoidosis with negative conventional spinal x-ray films, yet with typical cystic lesions of the spine found incidentally during abdominal computerized axial tomography (CAT). The patient was a 28-year-old black man, who was admitted for evaluation of a 1 1/2-year history of diffuse myalgias, intermittent fever to 102 F orally, bilateral hilar adenopathy, and leukopenia. A technetium polyphosphate bone scan revealed diffuse areas of increased uptake over the sternum, entire vertebral column, and pelvis. Conventional x-ray films of the cervical, thoracic, and lumbar spine, and an AP view of the pelvis were all normal. Chest x-ray film revealed only bilateral hilar adenopathy. During the course of an extensive negative evaluation for infection, an abdominal CAT scan was done, showing multiple, small, sclerotic-rimmed cysts at multiple levels of the lower thoracic and lumbar spine. Bone marrow biopsy revealed only changes consistent with anemia of chronic disease. Mediastinal lymph node biopsy revealed noncaseating granulomas. A tentative diagnosis of sarcoidosis was made, and treatment with prednisone, isoniazid and rifampin was begun. Within two weeks of initiation of prednisone therapy, the patient was symptom-free. A repeat technetium polyphosphate bone scan revealed only a small residual area of mildly increased uptake over the upper thoracic vertebrae.

  16. Cardiac Sarcoidosis or Giant Cell Myocarditis? On Treatment Improvement of Fulminant Myocarditis as Demonstrated by Cardiovascular Magnetic Resonance Imaging

    PubMed Central

    Bogabathina, Hari; Olson, Peter; Rathi, Vikas K.; Biederman, Robert W. W.

    2012-01-01

    Giant cell myocarditis, but not cardiac sarcoidosis, is known to cause fulminant myocarditis resulting in severe heart failure. However, giant cell myocarditis and cardiac sarcoidosis are pathologically similar, and attempts at pathological differentiation between the two remain difficult. We are presenting a case of fulminant myocarditis that has pathological features suggestive of cardiac sarcoidosis, but clinically mimicking giant cell myocarditis. This patient was treated with cyclosporine and prednisone and recovered well. This case we believe challenges our current understanding of these intertwined conditions. By obtaining a sense of severity of cardiac involvement via delayed hyperenhancement of cardiac magnetic resonance imaging, we were more inclined to treat this patient as giant cell myocarditis with cyclosporine. This resulted in excellent improvement of patient's cardiac function as shown by delayed hyperenhancement images, early perfusion images, and SSFP videos. PMID:24826266

  17. Isolated cardiac sarcoidosis: clinical characteristics, diagnosis and treatment.

    PubMed

    Isobe, Mitsuaki; Tezuka, Daisuke

    2015-03-01

    Sarcoidosis is a systemic disease characterized by the development of noncaseating epithelioid granulomas in multiple organs. Despite extensive investigations over a long period of time, the etiology of this disease remains unknown. Cardiac involvement of this disease is the most ominous complication leading to fatal outcome. Recently, attention has been focused on isolated cardiac sarcoidosis, which exists without clinically apparent sarcoidosis elsewhere. One of the critical issues of isolated cardiac sarcoidosis is difficulty in diagnosis, since existence of the cardiac lesion should be detected from cardiac manifestations alone. Because specificity of biomarkers or sensitivity of histological examination of biopsied sample is very low, diagnosis of isolated cardiac sarcoidosis mainly depends on imaging modalities. In this review article we summarized current knowledge on the pathogenesis of sarcoidosis, clinical features of cardiac sarcoidosis especially that isolated to the heart by showing some typical cases. Utilities and problems of diagnostic imaging tests for this condition including echocardiography, cardiac magnetic resonance imaging, and fluorodeoxyglucose-positron emission tomography are discussed. Advances in pharmacological and nonpharmacological treatment for cardiac sarcoidosis have improved the prognosis of cardiac sarcoidosis to a great deal; however, there are many issues that remain to be solved in the management of isolated cardiac sarcoidosis.

  18. Rare manifestations of sarcoidosis in modern era of new diagnostic tools

    PubMed Central

    Sharma, Surendra K; Soneja, Manish; Sharma, Abhishek; Sharma, Mehar C.; Hari, Smriti

    2012-01-01

    Background & objectives: Growing body of literature on sarcoidosis in India has led to an increased awareness of the disease. With the advent of better imaging tools hitherto under-recognized manifestations of sarcoidosis are likely to be better recognized. We sought to study the rare clinical and radiological manifestations (<5%) in patients with sarcoidosis. Methods: Retrospective review of records of 164 patients with histopathologically proven sarcoidosis seen over six years in a tertiary care centre in north India, was done. Results: Fifty four rare manifestations were observed in 164 patients. Acute presentation in the form of Lofgren syndrome was seen in eight (4.9%) and Heerfordt's syndrome in two (1.2%) patients. Musculoskeletal manifestations included chronic sarcoid arthritis in three (1.8%), deforming arthritis and bone erosion in one (0.6%) each. Rare initial presentation with dilated cardiomyopathy in one (0.6%), complete heart block in two (1.2%), bilateral sequential facial nerve palsy in two (1.2%), and pyrexia of unknown origin was seen in one (0.6%) patient. Other rare manifestations included chronic respiratory failure in one (0.6%), dysphagia in one (0.6%), sicca syndrome in five (3%), massive splenomegaly in one (0.6%), portal hypertension in two (1.2%), hypersplenism, gastric sarcoidosis, ninth and tenth cranial nerve palsies, moderate pericardial effusion and nephrocalcinosis in one (0.6%) each, and pulmonary artery hypertension in two (1.2%) patients. Rare radiological manifestations included moderate pleural effusion in two (1.2%), pleural thickening in five (3%), calcification of intrathoracic lymph nodes in four (2.4%), alveolar (nodular) sarcoidosis in three (1.8%), and myocardial uptake of 18F-fluorodeoxyglucose (F-18 FDG) in two (1.2%) patients. Fourteen patients had airways obstruction and behaved typically like seasonal bronchial asthma with excellent response to corticosteroids. Interpretation & conclusions: Increased awareness of

  19. Interleukin-12 family cytokines and sarcoidosis.

    PubMed

    Ringkowski, Sabine; Thomas, Paul S; Herbert, Cristan

    2014-01-01

    Sarcoidosis is a systemic granulomatous disease predominantly affecting the lungs. It is believed to be caused by exposure to pathogenic antigens in genetically susceptible individuals but the causative antigen has not been identified. The formation of non-caseating granulomas at sites of ongoing inflammation is the key feature of the disease. Other aspects of the pathogenesis are peripheral T-cell anergy and disease progression to fibrosis. Many T-cell-associated cytokines have been implicated in the immunopathogenesis of sarcoidosis, but it is becoming apparent that IL-12 cytokine family members including IL-12, IL-23, IL-27, and IL-35 are also involved. Although the members of this unique cytokine family are heterodimers of similar subunits, their biological functions are very diverse. Whilst IL-23 and IL-12 are pro-inflammatory regulators of Th1 and Th17 responses, IL-27 is bidirectional for inflammation and the most recent family member IL-35 is inhibitory. This review will discuss the current understanding of etiology and immunopathogenesis of sarcoidosis with a specific focus on the bidirectional impact of IL-12 family cytokines on the pathogenesis of sarcoidosis.

  20. Granulomatosis-associated common variable immunodeficiency disorder: a case-control study versus sarcoidosis.

    PubMed

    Bouvry, Diane; Mouthon, Luc; Brillet, Pierre-Yves; Kambouchner, Marianne; Ducroix, Jean-Pierre; Cottin, Vincent; Haroche, Julien; Viallard, Jean-Francois; Lazor, Romain; Lebargy, François; Tazi, Abdellatif; Wallaert, Benoît; Smail, Amar; Pellegrin, Jean-Luc; Nunes, Hilario; Amoura, Zahir; Cordier, Jean-François; Valeyre, Dominique; Naccache, Jean-Marc

    2013-01-01

    The aim of the present study was to investigate to what extent interstitial lung disease (ILD) in common variable immunodeficiency disorder (CVID)-associated granulomatous disease (GD) is similar to pulmonary sarcoidosis 20 patients with CVID/GD were included in a retrospective study conducted by the Groupe Sarcoïdose Francophone. Medical records were centralised. Patients were compared with 60 controls with sarcoidosis. Clinical examination showed more frequent crackles in patients than controls (45% versus 1.7%, respectively; p<0.001). On thoracic computed tomography scans, nodules (often multiple and with smooth margins), air bronchograms and halo signs were more frequent in patients than controls (80% versus 42%, respectively; p=0.004) as well as bronchiectasis (65% versus 23%, respectively; p<0.001). The micronodule distribution was perilymphatic in 100% of controls and in 42% of patients (p<0.001). Bronchoalveolar lavage analysis showed lower T-cell CD4/CD8 ratios in patients than in controls (mean ± sd 1.6 ± 1.1 versus 5.3 ± 4, respectively; p<0.01). On pathological analysis, nodules and consolidations corresponded to granulomatous lesions with or without lymphocytic disorders in most cases. Mortality was higher in patients than controls (30% versus 0%, respectively) and resulted from common variable immunodeficiency complications. ILD in CVID/GD presents a specific clinical picture and evolution that are markedly different from those of sarcoidosis. PMID:22903958

  1. Tenth international conference on sarcoidosis and other granulomatous disorder

    SciTech Connect

    Johns, C.J.

    1986-01-01

    This book contains papers divided among three sections: Basic Mechanisms of Sarcoidosis; Other Granulomatous Disorders; and Clinical Studies of Sarcoidosis. Some of the paper titles are: Radionuclides in Detecting Active Granuloma Formation: Gallium-67 Scintigraphy and Histopathology with Autoradiographic; a European Survey on the Usefulness of /sup 67/Ga Lung Scans in Assessing Sarcoidosis: Experience in 14 Research Centers in Seven Different Countries; and Reassessing the Standard Chest Radiograph for Intraparenchymal Activity.

  2. Sarcoidosis presenting as isolated gingival enlargement: a rare case entity.

    PubMed

    Tripathi, Pragya; Aggarwal, Jaihans; Chopra, Deepak; Bagga, Sukhchain; Sethi, Kanika

    2014-11-01

    Sarcoidosis is a non-caseating granulomatous disease . It is a multiorgan inflammatory disorder of unknown etiology. Conditions affecting skin or other organs frequently involve oral cavity and rarely manifest as gingival disease. Here we are reporting a rare case in which gingival hyperplasia was the initial symptom which finally led to the diagnosis of sarcoidosis. Oral mucous membrane needs to be examined carefully as it may constitute in presenting first sign of systemic sarcoidosis.

  3. Non-caseating granulomas in patients with hematologic malignancies. A report of three cases.

    PubMed

    Tangen, J M; Naess, A; Aasen, T; Morild, I

    1988-01-01

    Sarcoidosis or sarcoid-like lesions occurred in three patients with hematologic malignancies. In one patient, an overlap syndrome sarcoidosis/polycythemia vera was the most probable diagnosis. In the other two cases, the granulomas were probably secondary to myelofibrosis and Waldenström's disease, respectively. Recent epidemiological studies have failed to show a causal relationship between sarcoidosis and malignancy. The co-existence of sarcoidosis and malignancy is probably fortuitous, but non-caseating granulomas may occur secondary to malignant diseases.

  4. The Association between ESR and CRP and Systemic Hypertension in Sarcoidosis

    PubMed Central

    Mirsaeidi, Mehdi; Omar, Hesham R.; Ebrahimi, Golnaz; Campos, Micheal

    2016-01-01

    Introduction. The association between the level of systemic inflammation and systemic hypertension (sHTN) among subjects with sarcoidosis has not been previously explored. Methods. A retrospective study was conducted to investigate the relation between the level of systemic inflammation in sarcoidosis, measured by various serum inflammatory markers, and sHTN. Results. Among a total of 108 cases with sarcoidosis (mean age: 53.4 years, 76.9% females), 55 (50.9%) had sHTN and 53 (49.1%) were normotensive. ESR was highly associated with sHTN. The patients with sHTN had higher mean ESR levels compared with normotensives (48.8 ± 35 versus 23.2 ± 27 mm/hr, resp.; P = 0.001). ROC curve analysis for ESR revealed an AUC value of 0.795 (95% CI: 0.692–0.897; P = 0.0001). With regard to CRP, there was a trend towards higher mean values in sHTN group (3.4 versus 1.7 mg/L; P = 0.067) and significantly higher prevalence of sHTN in the highest CRP quartile compared to the lowest one (69.6% versus 30%; OR 4.95; P = 0.017). ROC curve analysis for CRP revealed an AUC value of 0.644 (95% CI: 0.518–0.769; P = 0.03). On multivariate analysis, ESR and the CRP remained independent predictors for sHTN among subjects with sarcoidosis. Conclusion. Systemic inflammation is associated with the presence of sHTN in sarcoidosis. PMID:27433355

  5. A rare manifestation of sarcoidosis with sensomotoric neuropathy of the ulnar nerve as the only symptom

    PubMed Central

    Mattiassich, Georg; Schubert, Heinrich; Hutarew, Georg; Wechselberger, Gottfried

    2012-01-01

    A 79-year-old woman was admitted complaining of progressive weakness and numbness of the right hand. The patient was otherwise healthy. The patient's history was unremarkable. Clinical and electrophysiological examination revealed a compression of the ulnar nerve in the ulnar sulcus and in Guyon's canal. Ultrasound evaluation showed a suspicious tumour proximal to the elbow close to the ulnar nerve. The ulnar sulcus was then released and an epineural and perineural lesion 3–4 cm proximal to the sulcus was excised under microscope. The histopathology confirmed the lesion as non-caseating sarcoid granulomas. The patient showed no other signs of systemic sarcoidosis, as neuropathy was the only symptom and the condition improved postoperatively. Sensory deficits and paraesthesia resolved fully. The extension of the minor finger remained slightly inferior compared with the not affected side. Sarcoid neuropathy is a rare neurological complication of sarcoidosis and has to be included in differential diagnosis of nerve conduction impairments. PMID:23192580

  6. A rare manifestation of sarcoidosis with sensomotoric neuropathy of the ulnar nerve as the only symptom.

    PubMed

    Mattiassich, Georg; Schubert, Heinrich; Hutarew, Georg; Wechselberger, Gottfried

    2012-01-01

    A 79-year-old woman was admitted complaining of progressive weakness and numbness of the right hand. The patient was otherwise healthy. The patient's history was unremarkable. Clinical and electrophysiological examination revealed a compression of the ulnar nerve in the ulnar sulcus and in Guyon's canal. Ultrasound evaluation showed a suspicious tumour proximal to the elbow close to the ulnar nerve. The ulnar sulcus was then released and an epineural and perineural lesion 3-4 cm proximal to the sulcus was excised under microscope. The histopathology confirmed the lesion as non-caseating sarcoid granulomas. The patient showed no other signs of systemic sarcoidosis, as neuropathy was the only symptom and the condition improved postoperatively. Sensory deficits and paraesthesia resolved fully. The extension of the minor finger remained slightly inferior compared with the not affected side. Sarcoid neuropathy is a rare neurological complication of sarcoidosis and has to be included in differential diagnosis of nerve conduction impairments. PMID:23192580

  7. [The cytoarchitectonics of the epidermis in sarcoidosis of the skin].

    PubMed

    Samtsov, A V; Shiliaeva, L V; Burkova, N P

    1990-01-01

    Histologic and electron microscopic study of the epidermis in, respectively, 30 and 4 patients with skin sarcoidosis has revealed that atrophy of the epidermis is the most incident condition, whose severity depends on microcirculation disorders that are explained by the location of the epithelioid-cell granulomas relative to skin microcirculatory bed. Moderate atrophy involves the following ultrastructural changes in the keratinocytes: increased count of ribosomes and mitochondria, tonofibril condensation, perinuclear vacuolization, separation of individual cellular contact sites. If atrophy is well manifest, basal keratinocytes grow in size and the nuclei in them are positioned apically. These data suggest that drugs improving the microcirculation be prescribed to such patients in order to obliterate possible cosmetic defects.

  8. Gallium 67 citrate scanning and serum angiotensin converting enzyme levels in sarcoidosis

    SciTech Connect

    Gupta, R.G.; Bekerman, C.; Sicilian, L.; Oparil, S.; Pinsky, S.M.; Szidon, J.P.

    1982-09-01

    Gallium 67 citrate scans and serum angiotension converting enzyme (ACE) levels were obtained in 54 patients with sarcoidosis and analyzed in relation to clinical manifestation. /sup 67/Ga scans were abnormal in 97% of patients with clinically active disease (n = 30) and in 71% of patients with inactive disease (n = 24). Serum ACE levels were abnormally high (2 standard deviations above the control mean) in 73% of patients with clinically active disease and in 54% of patients with inactive disease. Serum ACE levels correlated significantly with /sup 67/Ga uptake score (r = .436; p < .005). The frequency of abnormal /sup 67/Ga scans and elevated serum ACE levels suggests that inflammatory activity with little or no clinical expression is common in sarcoidosis. Abnormal /sup 67/Ga scans were highly sensitive (97%) but had poor specificity (29%) to clinical disease activity. The accuracy of negative prediction of clinical activity by normal scans (87%) was better than the accuracy of positive prediction of clinical activity by abnormal scans (63%). /sup 67/Ga scans can be used to support the clinical identification of inactive sarcoidosis.

  9. Gallium-67 uptake in meningeal sarcoidosis

    SciTech Connect

    Ayres, J.G.; Hicks, B.H.; Maisey, M.N.

    1986-07-01

    A case of sarcoidosis limited to the central nervous system is described in which the diagnosis was suggested by high Ga-67 uptake in the cranial and spinal meninges. The diagnosis was confirmed by meningeal biopsy. Treatment with oral corticosteroids resulted in clinical improvement and marked reduction in Ga-67 uptake in the meninges. This is the first reported case of the central nervous system sarcoid diagnosed by Ga-67 imaging.

  10. Gene-environment interactions in sarcoidosis

    PubMed Central

    Culver, Daniel A.; Newman, Lee S.; Kavuru, Mani S.

    2007-01-01

    Susceptibility to most human diseases is polygenic, with complex interactions between functional polymorphisms of single genes governing disease incidence, phenotype, or both. In this context, the contribution of any discrete gene is generally modest for a single individual, but may confer substantial attributable risk on a population level. Environmental exposure can modify the effects of a polymorphism, either by providing a necessary substrate for development of human disease or because the effects of a given exposure modulate the effects of the gene. In several diseases, genetic polymorphisms have been shown to be context-dependent, i.e. the effects of a genetic variant are realized only in the setting of a relevant exposure. Since sarcoidosis susceptibility is dependent on both genetic and environmental modifiers, the study of gene-environment interactions may yield important pathogenetic information and will likely be crucial for uncovering the range of genetic susceptibility loci. However, the complexity of these relationships implies that investigations of gene-environment interactions will require the study of large cohorts with carefully-defined exposures and similar clinical phenotypes. A general principle is that the study of gene-environment interactions requires a sample size at least several-fold greater than for either factor alone. To date, the presence of environmental modifiers has been demonstrated for one sarcoidosis susceptibility locus, HLA-DQB1, in African-American families. This article reviews general considerations obtaining for the study of gene-environment interactions in sarcoidosis. It also describes the limited current understanding of the role of environmental influences on sarcoidosis susceptibility genes. PMID:17560304

  11. [A comparative evaluation of the efficacy of different therapeutic methods in sarcoidosis of the skin].

    PubMed

    Samtsov, A V

    1990-01-01

    Twenty-eight patients with skin sarcoidosis were treated with thymalin, thymogen, levamisole, dimocifon, with due consideration for their immune status. Immunostimulating therapy has failed. Plaquenil, phthivazide and duplex therapy did not result in noticeable improvement either. An individual pattern of mononuclear phagocyte response to various immunoregulators was revealed. The highest sensitivity to human interferon, hydrocortisone, and prospidine was determined in vitro. The highest effect was achieved by a combination of corticosteroids with prospidine, though in 20 percent of cases the disease recurred within 2 years. Since these drugs are not harmless for the body, the major indications for their prescription to patients with skin sarcoidosis (provided there are no direct indications because of other conditions) are the process activity and dissemination, cosmetic defects because of the disease. In case of a local involvement and uneventful course of the process a follow-up is advisable.

  12. Pediatric Gastrointestinal Sarcoidosis: Successful Treatment with Infliximab

    PubMed Central

    Alawdah, Laila; Nahari, Ahmad; Alshahrani, Dayel; Fagih, Musa; Ghazi, Shahid; Al-Hussaini, Abdulrahman

    2016-01-01

    Gastrointestinal sarcoidosis is a rare disease with very limited data in children. Here we report the first pediatric case of successful treatment with infliximab. The first case was an 8-year-old Saudi girl who presented with fever, weight loss, and abdominal pain that was followed in a few months with hematemesis and development of hepatosplenomegaly. The second case was a 9-year-old Sudanese boy who manifested with vomiting, epigastric pain, and weight loss. On upper endoscopy, both cases demonstrated severe erosive nodular gastric mucosa. Gastric and esophageal biopsies had shown noncaseating granulomatous inflammation. The first case had histopathological evidence of granulomatous hepatitis, and both cases demonstrated lung nodularity on computed tomography chest. The boy had elevated angiotensin-converting enzyme level. Given the multisystem involvement with significant chest findings, tissue findings of granulomatous disease, and negative workup for other causes of granulomatous inflammation, both cases were diagnosed with active disseminated sarcoidosis, and treated with corticosteroids. The girl continued to be symptom-free for 4 years after tapering steroid therapy. The boy had relapses off steroids and the disease was brought into remission for 5 years off steroid therapy by infliximab. Pediatric GI sarcoidosis is a rare disease that exhibits heterogeneity in natural course. The chronic relapsing progressive form of the disease might benefit from infliximab therapy. PMID:27748327

  13. Sarcoidosis: correlation of pulmonary parenchymal pattern at CT with results of pulmonary function tests

    SciTech Connect

    Bergin, C.J.; Bell, D.Y.; Coblentz, C.L.; Chiles, C.; Gamsu, G.; MacIntyre, N.R.; Coleman, R.E.; Putman, C.E.

    1989-06-01

    The appearances of the lungs on radiographs and computed tomographic (CT) scans were correlated with degree of uptake on gallium scans and results of pulmonary function tests (PFTs) in 27 patients with sarcoidosis. CT scans were evaluated both qualitatively and quantitatively. Patients were divided into five categories on the basis of the pattern of abnormality at CT: 1 = normal (n = 4); 2 = segmental air-space disease (n = 4); 3 = spherical (alveolar) masslike opacities (n = 4); 4 = multiple, discrete, small nodules (n = 6); and 5 = distortion of parenchymal structures (fibrotic end-stage sarcoidosis) (n = 9). The percentage of the volume judged to be abnormal (CT grade) was correlated with PFT results for each CT and radiographic category. CT grades were also correlated with gallium scanning results and percentage of lymphocytes recovered from bronchoalveolar lavage (BAL). Patients in CT categories 1 and 2 had normal lung function, those in category 3 had mild functional impairment, and those in categories 4 and 5 showed moderate to severe dysfunction. The overall CT grade correlated well with PFT results expressed as a percentage of the predicted value. In five patients, CT scans showed extensive parenchymal disease not seen on radiographs. CT grades did not correlate with the results of gallium scanning or BAL lymphocytes. The authors conclude that patterns of parenchymal sarcoidosis seen at CT correlate with the PFT results and can be used to indicate respiratory impairment.

  14. Evaluation and management of heart rhythm disturbances due to cardiac sarcoidosis.

    PubMed

    Selan, Jeffrey C; Michaelson, Melody; Fanburg, Barry L; Estes, N A Mark

    2014-12-01

    Cardiac sarcoidosis (CS) affects less than 5% of patients with pulmonary or systemic sarcoidosis, but when present is often associated with a spectrum of clinically significant conduction abnormalities and arrhythmias. The cardinal manifestations of CS include conduction disturbances, arrhythmias, or congestive heart failure. Less commonly, there is concealed subclinical disease. The electrophysiologic evaluation for CS includes a history and physical exam, ECG, and echocardiogram for all sarcoidosis patients, along with MRI, PET/nuclear scans, and EPS for certain subsets of patients. Despite variable data to support their efficacy, glucocorticoids should still be considered in the treatment plan of CS. Antiarrhythmics in isolation are often ineffective in controlling ventricular arrhythmias. Cardiac pacemakers have provided important therapy for patients with conduction defects and implantable cardioverter defibrillator (ICD) therapy provides the strongest insurance to prevent fatal arrhythmias from CS. A recent consensus statement provides guidance for clinicians on the diagnosis and management of arrhythmias associated with CS including indications for ICDs. The use of pacemakers, ICD implantation and early implementation of corticosteroid therapy have led to an improvement in the overall prognosis and clinical outcomes of CS.

  15. Expression of CD14 correlates with lung function impairment in pulmonary sarcoidosis.

    PubMed

    Pforte, A; Schiessler, A; Gais, P; Beer, B; Ehlers, M; Schütt, C; Ziegler-Heitbrock, H W

    1994-02-01

    CD14 expression on alveolar macrophages (AM) was studied in patients with sarcoidosis using immunocytochemistry and cytometric analysis. Compared with healthy control donors, patients had elevated percentages of CD14-positive AM (22 percent vs 34 percent), and the antigen density was threefold higher (92 vs 297 channels). Furthermore, soluble serum CD14 (ssCD14) was significantly elevated in patients with sarcoidosis with an average of 5.3 +/- 1.6 mg/L vs 3.2 +/- 0.7 mg/L in healthy control subjects. Follow-up of one patient, whose lung function test results improved during therapy with corticosteroids, revealed a concomitant decrease of CD14 staining on AM and of ssCD14. Statistical analysis revealed a negative correlation between CD14 expression on AM and PO2 at rest (p = 0.0005), and after labor (p = 0.02). Levels of ssCD14 gave a positive correlation to reduction of Dco (p = 0.006) and VC (p = 0.05). These data suggest that CD14 expression is related to severity of disease and that it may be useful for monitoring in sarcoidosis. PMID:7508361

  16. Biochemical and immunohistochemical comparison of collagen in granuloma annulare and skin sarcoidosis.

    PubMed

    Oikarinen, A; Kinnunen, T; Kallioinen, M

    1989-01-01

    Collagen was studied by biochemical and immunohistochemical means in 5 patients with granuloma annulare (GA) and 3 with cutaneous sarcoidosis (SA). The solubility of collagen from the lesional skin in acetic acid was higher than that of collagen from unaffected skin from both patients and control subjects. Collagen concentration in the skin lesions, measured in terms of hydroxyproline content, was reduced in 3 patients with granuloma annulare and one with sarcoidosis, but the ratio of type III/I collagen was unchanged vis-à-vis non-affected skin. The collagen concentration in non-affected skin of both GA and SA-patients was also lower than in controls. The most typical immunohistochemical finding was the association of type III procollagen and fibronectin with granulomas in the lesional skin of both GA and SA cases. The activity of prolyl hydroxylase, a key enzyme in collagen biosynthesis, was markedly increased in the lesional skin, indicating that collagen synthesis in vivo was also increased. Surprisingly, collagen synthesis was not increased in cell culture studies. This could be due to cell selection as observed previously in scleroderma. Another possibility could be that various mediators released in vivo from inflammatory cells activate fibroblasts. However, when cells are subcultivated, this effect is not maintained. In conclusion, marked changes in collagen could be observed in granuloma annulare and skin sarcoidosis, reflecting increased turnover of collagen in vivo.

  17. Sarcoidosis: a diagnostic challenge in atypical radiologic findings of unilateral lymphadenopathy

    PubMed Central

    Meillier, Andrew; Commodore, Marius

    2015-01-01

    Sarcoidosis is a chronic systemic disease with a wide array of clinical findings. Given that the clinical symptoms are not pathognomonic, chest radiographs have become essential to the initial diagnosis and choice of treatment modality. Diagnosis hinges on ruling out alternative diagnoses; sometimes, advanced radiologic techniques and histopathology are required. On this occasion, we present a case of a patient with generalized symptoms, no significant chest radiograph findings and lymphadenopathy where advanced imaging and pathology assisted in the diagnosis. PMID:26719811

  18. Increased serum thymidine kinase activity in acute sarcoidosis.

    PubMed

    Tajima, Syunji; Sando, Yoshichika; Maeno, Toshitaka; Sagawa, Naoki; Nara, Mami; Maeno, Yuri; Nakagawa, Junichi; Ito, Toshio; Hoshino, Yoichi; Suga, Tatsuo; Arai, Masashi; Kurabayashi, Masahiko

    2002-02-01

    This is the first case report of acute sarcoidosis with increased serum thymidine kinase (TK) activity. A 43-year-old male presented fever, swelling of parotid glands, lymphadenopathy, and peripheral neuropathy. Sarcoidosis was pathologically diagnosed by lung and parotid gland biopsy. His serum TK, which was increased to 11.2 U/l at diagnosis (normal <5 U/l), normalized after glucocorticoid therapy. Serum TK has been considered as a good marker of the proliferative activity of various types of neoplasms. Its rise in sarcoidosis has, however, not been described. Because acute sarcoidosis sometimes resembles malignant lymphoma, the possible rise of serum TK in sarcoidosis may be worthy of note. PMID:11868600

  19. The alveolitis of pulmonary sarcoidosis. Evaluation of natural history and alveolitis-dependent changes in lung function

    SciTech Connect

    Keogh, B.A.; Hunninghake, G.W.; Line, B.R.; Crystal, R.G.

    1983-08-01

    Current concepts of the pathogenesis of pulmonary sarcoidosis suggest that a mononuclear cell alveolitis, comprised of activated T-lymphocytes and activated alveolar macrophages, precedes and modulates the formation of granuloma and fibrosis. To evaluate the natural history of this alveolitis and determine the relationship it has to subsequent changes in lung function, 19 untreated patients with pulmonary sarcoidosis without extrapulmonary manifestations were studied with bronchoalveolar lavage, /sup 67/Ga scanning, and pulmonary function tests to evaluate lung T-cells, lung alveolar macrophages, and lung function, respectively. In patients with sarcoidosis, low intensity alveolitis (lung T-cells less than or equal to 28% of all lung effector cells and/or /sup 67/Ga scan negative) was much more common (80% of all observations) than high intensity alveolitis (lung T-cells greater than 28% and /sup 67/Ga scan positive, 20% of all observations). Conventional clinical, roentgenographic, or physiologic studies could not predict the alveolitis status. Interestingly, of the 51 alveolitis evaluations in the 19 patients, there were 24 occurrences (47%) where the alveolitis was ''split,'' i.e., /sup 67/Ga scans positive and T-cells low (39%) or /sup 67/Ga negative and T-cells high (8%). Most untreated patients with sarcoidosis without extrapulmonary symptoms may have some inflammatory processes ongoing in their alveolar structures. Overall, whenever a high intensity alveolitis episode occurred, it was followed by deterioration over the next 6 months in at least one lung function parameter. A low intensity alveolitis episode was followed by functional deterioration only 8% of the time. The alveolitis parameters (lavage and /sup 67/Ga scanning) clearly predicted prognosis. These observations should prove useful in understanding the natural history of pulmonary sarcoidosis, in staging patients with this disease, and in making rational therapy decisions.

  20. Assessment of activity in Sarcoidosis. Sensitivity and specificity of 67Gallium scintigraphy, serum ACE levels, chest roentgenography, and blood lymphocyte subpopulations

    SciTech Connect

    Klech, H.; Kohn, H.; Kummer, F.; Mostbeck, A.

    1982-12-01

    The value of different factors are examined to assess activity in 60 patients with biopsy-proven sarcoidosis. In patients with active sarcoidosis (n . 35), /sup 67/Ga scans proved to be the most sensitive method (94 percent sensitivity), followed by serum angiotensin I converting enzyme (S-ACE) levels, chest x-ray films, and lymphocyte assays. In patients with peripheral pulmonary lesions, chest x-ray films failed in 32 percent of cases to document activity (68 percent sensitivity) whereas /sup 67/Ga scans and S-ACE levels remained to give reliable results. Despite poor specificity, negative /sup 67/Ga scans together with normal ACE levels have a high predictive value for exclusion of active sarcoidosis. In patients with peripheral pulmonary lesions, chest roentgenography is of doubtful value for staging lung involvement and assessment of activity including monitoring and control of therapy.

  1. Development of an enzyme-linked immunosorbent assay for Clara cell 10-kDa protein: in pursuit of clinical significance of sera in patients with asthma and sarcoidosis.

    PubMed

    Shijubo, N; Itoh, Y; Yamaguchi, T; Abe, S

    2000-01-01

    We have produced nine monoclonal antibodies to human CC10/protein-1 and analyzed their characterization. TY-5, TY-7, and TY-8 recognized restricted possible hydrophobic epitopes and their binding to CC10 prevented the other clones from CC10 binding, suggesting that these antibodies induce strong conformational change. TY-1, TY-2, TY-3, TY-6, and 6D4 recognize amino acid residues 61-68 and the presence of disulfide bonds might be essential for epitope expression of these five clones. The best combination was TY-1 and TY-2 in developing an enzyme-linked immunosorbent assay (ELISA), whereas TY-5 was most suitable for immunohistochemistry and immunoblotting. We found significantly lower serum CC10 levels in asthmatic subjects and higher serum CC10 levels in sarcoidosis subjects than in controls. Data of CC10 levels in BAL fluids of sarcoidosis subjects were similar to those in the circulation. CC10-positive epithelial cells were significantly lower in small airways of asthmatic subjects than in controls, and CC10-positive epithelial cells were inversely correlated with T cell and mast cell accumulation in the airways of asthmatic subjects. CC10 may be a downregulator in both Th1- and Th2-mediated chronic inflammatory diseases. The use of these MoAbs and recombinant CC10 is a powerful tool to investigate the clinical roles of CC10/P1 and the structure and function of CC10/P1. PMID:11193763

  2. Pitfalls of contrast-enhanced ultrasound (CEUS) in the diagnosis of splenic sarcoidosis.

    PubMed

    Tana, C; Iannetti, G; D'Alessandro, P; Tana, M; Mezzetti, A; Schiavone, C

    2013-01-01

    By observing the real-time behavior of focal liver lesions at three vascular phases (arterial, portal-venous, and late), contrast-enhanced ultrasound (CEUS) has been successfully applied to differentiate benign from malignant hepatic nodules. In recent years, numerous studies highlighted the usefulness of CEUS also for other applications such as abdominal trauma, renal, pancreatic, thyroid, and inflammatory bowel diseases, supporting its role even in differentiating benign from malignant splenic nodules. Therefore, the European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB) recently updated the guidelines for the use of ultrasound contrast agents in clinical practice, pointing out the indication to characterize splenic parenchymal inhomogeneity or suspected lesions found on conventional ultrasound (BUS). We describe the case of a patient with a history of colon cancer and finding, at BUS and CEUS, of hypoechoic lesions with a highly suggestive pattern for metastases, subsequently histologically proved to be splenic localizations of a benign and multisystemic granulomatous disease such as sarcoidosis. We therefore reviewed the current literature focusing on the role of CEUS in differentiating benign from malignant splenic lesions, emphasizing on the lack of data and numerical shortage of sarcoidosis derived-lesions in the available studies. We conclude that sarcoidosis remains a diagnosis of exclusion and new studies are needed before defining precise indications of CEUS in these patients.

  3. Does serum angiotensin converting enzyme reflect intensity of alveolitis in sarcoidosis?

    PubMed Central

    Cohen, R D; Bunting, P S; Meindok, H O; Chamberlain, D W; Rebuck, A S

    1985-01-01

    Serum angiotensin converting enzyme activity is increased in many patients with pulmonary sarcoidosis and has been proposed as a measure of disease activity. Assay of serum angiotensin converting enzyme, bronchoalveolar lavage, and gallium scans were performed in 27 patients with biopsy proved pulmonary sarcoidosis. There was a positive correlation between serum angiotensin converting enzyme activity and an index of pulmonary gallium uptake assessed by the National Institutes of Health method (r = 0.7, p less than 0.001). There was no significant relationship (r = 0.19) between serum angiotensin converting enzyme activity and bronchoalveolar lavage lymphocytes expressed as a proportion of cells recovered. Increase in the enzyme activity had a sensitivity of 50% as a means of detecting high intensity alveolitis but specificity was only 45%. There was no significant difference in mean angiotensin converting enzyme activity between the following groups: those with positive and those with negative gallium scans; those with bronchoalveolar lavage lymphocyte counts less than or equal to 28% and those with counts greater than 28%. Although there was a significant correlation between the enzyme activity and one component of the alveolitis of sarcoidosis, the data suggest that serum angiotensin converting enzyme activity alone is neither sensitive nor specific enough for high intensity alveolitis. PMID:2994247

  4. Prostate-Specific Membrane Antigen PET/CT: False-Positive Results due to Sarcoidosis?

    PubMed Central

    Hermann, Robert M.; Djannatian, Manoutschehr; Czech, Norbert; Nitsche, Mirko

    2016-01-01

    We report on a 72-year-old male patient who developed sarcoidosis of the mediastinal lymph nodes, the liver, and the prostate 11 years ago. Seven years later, he underwent transurethral resection of the prostate by laser due to hematuria. Pathology of the resected chips showed a ‘granulomatous prostatitis with epitheloid cells’. Malignancy was histologically excluded at that time. Four years later, he was diagnosed with an undifferentiated prostate carcinoma, with a Gleason score of 5 + 4 = 9. After initiation of antihormonal therapy, he underwent radical prostatectomy and pelvic lymphadenectomy, which revealed a pT3b pN1 carcinoma with infiltrated resection margins. Three months later, the prostate-specific antigen level was 1.4 ng/ml, and a local recurrence was suspected by ultrasound; consequently, a 68Ga-prostate-specific membrane antigen (PSMA) PET/CT was performed. This examination seemed to confirm the local recurrence, a right pelvic lymph node metastasis, and a hepatic metastasis. However, ultrasound with contrast medium could not confirm the metastatic spread to the liver. In palliative intention, radiotherapy of the pelvis was done. After 50 Gy, the supposed recurrence had markedly shrunk, and an additional boost dose with 16.2 Gy was applied. Two years later, the patient is still free of disease. Due to this clinical development, we doubt the diagnosis of a fulminant progression of the prostate cancer as suspected by PSMA-PET/CT. Instead, we suspect a recurrence of the previously proven sarcoidosis leading to false-positive results. Our focus in this report is on the interaction between PSMA-PET/CT and sarcoidosis. Another report on a case of sarcoidosis of the spleen seems to confirm this possibility [Kobe et al: Clin Nucl Med 2015;40: 897–898]. PMID:27721768

  5. Sarcoidosis: a Critical Review of History and Milestones.

    PubMed

    Spagnolo, Paolo

    2015-08-01

    Sarcoidosis is a chronic systemic disease of unknown origin and uncertain prognosis that most commonly affects young adults, and frequently presents with bilateral hilar lymphadenopathy, pulmonary infiltrates and ocular and skin lesions. The diagnosis is established when characteristic clinical-radiological features are supported by compatible histopathology of epithelioid cell granulomas, following exclusion of known causes of granulomatous inflammation. Indeed, sarcoidosis belongs to a large family of disorders that share granuloma formation as common denominator. Since its first description by Jonathan Hutchinson in 1869, sarcoidosis has generated enormous interest and considerable controversy. In Hutchinson's day, it was considered a dermatological condition, which gradually evolved into a multisystem disorder associated in the majority of cases with respiratory abnormalities. With time, it has also become clear that sarcoidosis occurs throughout the world, affecting individuals of both genders and all races, although its prevalence varies widely across ethnic and racial groups. In recent years, advances in different disciplines, particularly biochemistry, genetics, immunology and molecular biology, have improved dramatically our understanding of the disease. Yet, the critical questions regarding who gets sarcoidosis and whether it has an infectious origin remain unanswered. Sarcoidosis has a distinguished medical history that covers the last 150 years. Right from the time of seminal contributions by Hutchinson, Besnier and Boeck medical discussion on sarcoidosis has always been animated and to a certain extent emotional. Such discussions will inevitable continue until the true cause of the disease has been found, hopefully in the near future.

  6. Budd-Chiari Syndrome: An Unusual Presentation of Multisystemic Sarcoidosis

    PubMed Central

    Bacha, Dhouha; Romdhane, Hayfa; Cheikh, Myriam; Nejma, Houda Ben; BelHadj, Najet

    2015-01-01

    Sarcoidosis is a multisystem granulomatous disease of unknown origin. All organs may be affected. Liver involvement is common but it is rarely symptomatic. Only a few cases of Budd-Chiari syndrome (BCS) secondary to a hepatic sarcoidosis have been described so far. We describe a case of multisystemic sarcoidosis presenting with BCS. A 42-year old female was referred to our department for chronic and anicteric cholestasis. Laboratory and imaging investigations disclosed features of chronic BCS associated with multisystemic sarcoidosis. The positive diagnosis was based on microscopic features, which showed hepatic, gastric and cutaneous non-caseating granulomas. Screening for an underlying thrombophilic disorder was negative. The diagnosis of BCS complicating hepatic sarcoidosis was the most likely. She was put on corticosteroids and anticoagulation therapy. To our knowledge, few cases of sarcoidosis-related BCS have been reported in the literature. In addition to being an uncommon presentation of sarcoidosis, this case illustrates the importance of recognizing an unusual cause of BCS and its therapeutic difficulties. PMID:26900444

  7. Poor accumulation of technetium-99m glucoheptonate in sarcoidosis and other diffuse infiltrative lung diseases as compared with gallium-67 citrate

    SciTech Connect

    Vorne, M.; Sahlstroem, K.A.; Alanko, K.

    1988-02-01

    Forty-two patients with diffuse infiltrative lung diseases were imaged with Ga-67 citrate and Tc-99m glucoheptonate (GH). Twenty patients had sarcoidosis, six had fibrosis, six had tuberculosis, nine had lung infiltration, and one had pleural empyema. The main difference between Ga-67 and Tc-99m GH was the much greater uptake of Ga-67 in sarcoidosis than that of Tc-99m GH. Fifteen patients with sarcoidosis had positive Ga-67 scans but only six had positive Tc-99m GH scans. The results in other diffuse infiltrative lung diseases were almost equal with Ga-67 and Tc-99m GH. Although Tc-99m GH is less expensive and simpler to use, it is not an adequate substitute for Ga-67 in diffuse infiltrative lung diseases.

  8. Infliximab therapy balances regulatory T cells, tumour necrosis factor receptor 2 (TNFR2) expression and soluble TNFR2 in sarcoidosis.

    PubMed

    Verwoerd, A; Hijdra, D; Vorselaars, A D M; Crommelin, H A; van Moorsel, C H M; Grutters, J C; Claessen, A M E

    2016-08-01

    Sarcoidosis is a systemic granulomatous disease of unknown aetiology that most commonly affects the lungs. Although elevated levels of regulatory T cells (Tregs ) have been reported, the extent to which they play a role in sarcoidosis pathogenesis remains unclear. Tumour necrosis factor (TNF) is thought to be one of the driving forces behind granuloma formation, illustrated by the efficacy of infliximab in severe sarcoidosis. Tregs express TNF receptor 2 (TNFR2) highly. Here, we examined the influence of infliximab therapy on Tregs and (soluble) TNFR2 levels in sarcoidosis, and correlated these with response to therapy. We observed that relative frequencies of Tregs were significantly higher in patients (n = 54) compared to healthy controls (n = 26; median 6·73 versus 4·36%; P < 0·001) and decreased following therapy (4·95; P < 0·001). Baseline TNFR2 expression on Tregs was increased significantly in patients versus controls (99·4 versus 96·2%; P = 0·031), and also in responders to therapy versus non-responders (99·6 versus 97·3%; P = 0·012). Furthermore, baseline soluble TNFR2 (sTNFR2) was higher in responders than in non-responders (mean 174 versus 107 pg/ml; P = 0·015). After treatment, responders showed a significant reduction in sTNFR2 levels in peripheral blood (-44·7 pg/ml; P < 0·001), in contrast to non-responders (+3·59 pg/ml). Our results demonstrated that Treg frequencies and TNFR2 expression on Tregs are increased in sarcoidosis, followed by a decline during infliximab therapy, suggesting a pathophysiological role of this T cell subset. Interestingly, sTNFR2 levels at baseline differed significantly between responders and non-responders, making it a potential marker in predicting which patients might benefit from infliximab.

  9. [A case of hepatic sarcoidosis presenting with cirrhotic symptoms].

    PubMed

    Kaji, Kiichiro; Ogino, Hidero; Hirai, Satoshi; Shimatani, Akiyoshi; Horita, Yosuke; Matsuda, Kouichiro; Hiramatsu, Katsushi; Matsuda, Mitsuru; Shimizu, Koichi; Nakanishi, Yuko; Noda, Yatsugi

    2014-03-01

    A man in 40s with skin sarcoidosis presented with signs and symptoms of liver injury and thrombocytopenia. Enhanced computed tomography and magnetic resonance imaging revealed cholecystolithiasis, hepatic deformation, and giant splenomegaly. Gastrointestinal endoscopy showed esophageal varices. Cholecystectomy, splenectomy, and wedge biopsy of the liver were performed. Histopathology of the liver revealed many granulomas and severe periportal fibrosis without lobular reconstruction. These findings were compatible with hepatic sarcoidosis, but not liver cirrhosis. Here we report a rare case of hepatic sarcoidosis presenting with cirrhotic symptoms.

  10. CT of Hepatic Sarcoidosis: Small Nodular Lesions Simulating Metastatic Disease

    PubMed Central

    Ufuk, Furkan; Herek, Duygu

    2015-01-01

    Summary Background Sarcoidosis is a multisystemic inflammatory disease of unknown origin. The lymphoid system and the lungs are the most commonly involved organs. The frequency of signs or symptoms of hepatic involvement is very low. Case Report We present a case of symptomatic granulomatous liver disease secondary to sarcoidosis, mimicking a metastatic disease on ultrasonography and CT. Conclusions Hepatic involvement in sarcoidosis might be a perplexing diagnostic problem. The decisive CT finding with respect to the differential diagnosis was the absence of a mass effect and intact vascular architecture around the lesions. PMID:25908950

  11. Bronchoalveolar mast cells in sarcoidosis: increased numbers and accentuation of mediator release.

    PubMed Central

    Flint, K C; Leung, K B; Hudspith, B N; Brostoff, J; Pearce, F L; Geraint-James, D; Johnson, N M

    1986-01-01

    Bronchoalveolar lavage was carried out in 36 subjects with sarcoidosis and 20 control subjects undergoing bronchoscopy for routine diagnostic purposes. The proportion of mast cells in the lavage fluid of subjects with sarcoidosis (mean (SE) 0.84% 0.09%; p less than 0.01) when compared with that of controls (mean 0.32% (0.05%); p less than 0.01). This increase was greatest in subjects with positive gallium scans but was not correlated with the percentage recovery of lymphocytes or radiographic stage. Anti-IgE induced histamine release from the bronchoalveolar cells of 15 subjects with sarcoidosis was significantly increased at all effective doses of anti-IgE. This accentuation of histamine release was significantly greater in patients with positive gallium scans and correlated directly with the percentage recovery of lymphocytes (r = 0.7, p less than 0.005). The dose-response curve of anti-IgE induced histamine release from bronchoalveolar cells of subjects with more than 20% of lymphocytes in the lavage cell population was significantly greater than the dose-response curves of subjects with fewer than 20% of lymphocytes and of controls. PMID:2422776

  12. Brainstem Infarction and Panuveitis due to Sarcoidosis Successfully Treated with Steroid Pulse Therapy

    PubMed Central

    Yoshida-Hata, Natsuyo; Yashiro, Shigeko; Arai, Noritoshi; Takeuchi, Sousuke

    2012-01-01

    A 36-year-old man visited our hospital because of blurred vision and redness of the conjunctiva. Slit-lamp examination showed panuveitis. Two days later, he suddenly experienced dizziness, speech disturbance, paralysis of his right extremities, and gait disturbances. Neurological examinations suggested that his symptoms were caused by a left lateral medullary lesion. He also had erythema mainly on his trunk. Magnetic resonance imaging (MRI) of his brain demonstrated a small infarct on the left side of the medulla oblongata. Clinical presentation and MRI findings were consistent with the diagnosis of a Wallenberg's syndrome. He also had bilateral hilar lymphadenopathy. A skin biopsy showed granulomatous nodular dermatitis compatible with sarcoidosis. He was treated with steroid pulse therapy and his neurological and ocular symptoms immediately improved. Only seven similar cases of intracranical sarcoidosis have been reported, but none had been treated with steroid pulse therapy. We recommend that steroid pulse therapy be considered to treat patients with sarcoidosis with signs of lesions in the central nervous system. PMID:22431930

  13. Polymorphisms in genes coding for mediators in the interleukin cascade and their effect on susceptibility to sarcoidosis in the Slovenian population.

    PubMed

    Maver, Ales; Medica, Igor; Salobir, Barbara; Sabovic, Miso; Tercelj, Marjeta; Peterlin, Borut

    2007-09-01

    Sarcoidosis is a chronic inflammatory disease characterised by granulomatous inflammation in various organs. As genetic factors have been implicated in its aetiology, in our study we investigated whether the promotor polymorphisms in three genes coding for inflammatory mediators interleukin-6 (IL-6), interleukin-12 (IL-12), and interleukin-18 (IL-18) could be associated with susceptibility to sarcoidosis. The study sample consisted of 104 patients with sarcoidosis and 100 healthy control subjects. Following DNA isolation from peripheral blood the IL-6/-174G>C single nucleotide polymorphism (SNP), the IL-12B/4 bp insertion polymorphism, and the IL-18/-137G>C SNP were characterised by polymerase chain reaction. Our results showed an increased frequency of IL-6/-174C allele (p<0.01, OR=2.05, 95% CI=1.32-3.16), and also an increased genotype frequency of IL-6/-174 CC and CG carriers (p<0.01, OR=2.78, 95% CI=1.49-5.19) among sarcoidosis patients in comparison with healthy controls. Allele and genotype frequencies did not differ significantly between cases and controls for either the IL-12B/4 bp insertion polymorphism or for the IL-18/-137G>C polymorphism. In conclusion, we demonstrated that the -174G>C promotor polymorphism in the IL-6 gene may be a risk factor for sarcoidosis.

  14. FoxP3+ Regulatory T Cells Suppress Early Stages of Granuloma Formation but Have Little Impact on Sarcoidosis Lesions

    PubMed Central

    Taflin, Cécile; Miyara, Makoto; Nochy, Dominique; Valeyre, Dominique; Naccache, Jean-Marc; Altare, Frédéric; Salek-Peyron, Pascale; Badoual, Cécile; Bruneval, Patrick; Haroche, Julien; Mathian, Alexis; Amoura, Zahir; Hill, Gary; Gorochov, Guy

    2009-01-01

    Sarcoidosis is characterized by a disproportionate Th1 granulomatous immune response in involved organs. It is also associated with both peripheral and intratissular regulatory T cell (Treg) expansion. These cells exhibit powerful antiproliferative activity, yet do not completely inhibit the production of either tumor necrosis factor-α or interferon-γ. The origin of the observed Treg amplification and, more importantly, its impact on the evolution of sarcoidosis remain unresolved issues. Here, we show that CD4+CD45RA−FoxP3bright Tregs proliferate and accumulate within granulomas. However, circulating and tissue Treg numbers are neither correlated with the dissemination of the disease nor correlated locally with the extent of granulomatous inflammation. Rather, we found a positive correlation between the presence of Tregs in renal granulomas and the degree of interstitial fibrosis (r = 0.46, P = 0.03, n = 20). Furthermore, Treg depletion accelerates in vitro granuloma growth in mononuclear cell cultures of healthy controls, but not in those from patients with active sarcoidosis. The results of this study show that although healthy Tregs suppress the initial steps of granuloma formation, they have no positive influence on sarcoidosis lesions. Our findings argue for a more preventive than curative effect of Tregs on inflammatory processes. PMID:19147826

  15. [The use of methotrexate for the treatment of sarcoidosis (data from a controlled prospective study)].

    PubMed

    Vizel, A A; Vzel, I Yu

    2015-01-01

    We estimated the effectiveness and safety of methotrexate monotherapy of sarcoidosis in 63 patients refractory to the previous treatment. Methotrexate was given weekly at a dose of 5-20 mg. 54% of the patients reported improvement of their condition. Parameters of spirogram and the ray picture improved in 63.2 and 54% of the cases respectively. Methotrexate proved safe and had to be discontinued only in 12.6% of the patients. It is concluded that methotrexate can be used at least in half of the cases as a safe alternative to systemic steroid therapy.

  16. Diagnosis and treatment of cardiac sarcoidosis.

    PubMed

    Kusano, Kengo F; Satomi, Kazuhiro

    2016-02-01

    Sarcoidosis is a systemic granulomatous disease of unknown aetiology. The frequency of cardiac involvement (cardiac sarcoidosis (CS)) varies in the different geographical regions, but it has been reported that it is an absolutely important prognostic factor in this disease. Complete atrioventricular block is the most common, and ventricular tachycardia/ventricular fibrillation the second most common arrhythmia in this disease, both of which are associated with cardiac sudden death. Diagnosing CS is sometimes difficult because of the non-specific ECG and echocardiographic findings, and CS is sometimes misdiagnosed as dilated cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy or an idiopathic ventricular aneurysm, and therefore, endomyocardial biopsy is important, but has a low sensitivity. Another problem is the recognition of isolated types of CS. Recently, MRI and (18)F-fluorodeoxyglucose positron emission tomography have been demonstrated to be useful tools for the non-invasive diagnosis of CS as well as therapeutic evaluation tools, but are still unsatisfactory. Treatment of CS is usually done by corticosteroid therapy to control inflammation, prevent fibrosis and protect from any deterioration of the cardiac function, but the long-term outcome is still in debate. Despite the advancement of non-pharmacological approaches for CS (pacing, defibrillators and catheter ablation) to improve the prognosis, there are still many issues remaining to resolve diagnosing and managing CS. Here, we attempt a review of the clinical evidence, with special focus on the current understanding of this disease and showing the current strategies and remaining problems of diagnosing and managing CS. PMID:26643814

  17. An Isolated Mass in the Palm, Starting Manifestation of Sarcoidosis

    PubMed Central

    Ghorbanhoseini, Mohammad; Yazdi, Hamidreza; Naseh, Hossein; Hafezi, Poopak

    2016-01-01

    Sarcoidosis is a systemic disease that is characterized with noncaseating granulomatous nodules which present in multiple organs specially lungs (90%). Incidence of masses due to Sarcoidosis in upper extremity is low and most cases present in association with involvement of pulmonary hilary lymph nodes. In this article we present a rare case of Sarcoidosis which presented as a single soft tissue mass in hand without osseous or pulmonary hillary lymph node involvement. Incidence of involvement of musculoskeletal system is 1-5 %, mostly it occurs in small bones in hands and feet. In most cases involvement of soft tissue in extremities is accompanied with bone lesions. Those cases of soft tissue involvement are generally coincide with pulmonary lymph nodules. To the authors` knowledge, this is the first case of Sarcoidosis that presents without spreading in bones or pulmonary hilar lymph nodes. PMID:26894226

  18. Sarcoidosis and Autoimmunity: From Genetic Background to Environmental Factors.

    PubMed

    Bindoli, Sara; Dagan, Amir; Torres-Ruiz, José J; Perricone, Carlo; Bizjak, Mojca; Doria, Andrea; Shoenfeld, Yehuda

    2016-01-01

    Sarcoidosis is a chronic multisystem disease with variable course resulting from the interaction between environmental factors and the immune system of individuals genetically predisposed. The evidence linking sarcoidosis with environmental triggers such as metals is increasing. We describe the case of a 44 year old female with a history. of smoking since age 30 and previous mercury dental filling who presented at physical examination with numerous subcutaneous nodules. Laboratory data showed accelerated erythrocyte sedimentation rate and high titer of anti-U1 ribonucleoprotein antibodies (U1 RNP). Skin biopsy and chest X-ray suggested the diagnosis of sarcoidosis. In this report we illustrate the different causes involved in the onset of sarcoidosis. PMID:27228643

  19. Hepatic sarcoidosis complicating treatment-naive viral hepatitis

    PubMed Central

    Aravinthan, Aloysious; Gelson, William; Limbu, Anita; Brais, Rebecca; Richardson, Paul

    2012-01-01

    Hepatic sarcoidosis is usually asymptomatic but rarely leads to adverse liver-related outcome. Co-existence of viral hepatitis and hepatic sarcoidosis is a rare, but recognised phenomenon. Obtaining a balance between immune suppression and anti-viral therapy may be problematic. Immunosuppression in the presence of viral hepatitis can lead to rapid deterioration of liver disease. Similarly, anti-viral therapy may exacerbate granulomatous hepatitis. Here we present two cases of viral hepatitis co-existing with sarcoidosis that illustrate successful management strategies. In one, hepatitis B replication was suppressed with oral anti-viral therapy before commencing prednisolone. In the second, remission of hepatic sarcoidosis was achieved with prednisolone, before treating hepatitis C and obtaining a sustained virological response with pegylated interferon and ribavirin therapy. PMID:23355920

  20. An Isolated Mass in the Palm, Starting Manifestation of Sarcoidosis.

    PubMed

    Ghorbanhoseini, Mohammad; Yazdi, Hamidreza; Naseh, Hossein; Hafezi, Poopak

    2016-01-01

    Sarcoidosis is a systemic disease that is characterized with noncaseating granulomatous nodules which present in multiple organs specially lungs (90%). Incidence of masses due to Sarcoidosis in upper extremity is low and most cases present in association with involvement of pulmonary hilary lymph nodes. In this article we present a rare case of Sarcoidosis which presented as a single soft tissue mass in hand without osseous or pulmonary hillary lymph node involvement. Incidence of involvement of musculoskeletal system is 1-5 %, mostly it occurs in small bones in hands and feet. In most cases involvement of soft tissue in extremities is accompanied with bone lesions. Those cases of soft tissue involvement are generally coincide with pulmonary lymph nodules. To the authors` knowledge, this is the first case of Sarcoidosis that presents without spreading in bones or pulmonary hilar lymph nodes.

  1. Clinical and pathological features of six cases of sarcoidosis presenting with renal failure

    PubMed Central

    Bear, R.A.; Handelsman, S.; Lang, A.; Cattran, D.; Wilson, D.; Johnson, M.; Lee, K.Y.; Cole, E.H.

    1979-01-01

    Six patients with biopsy-proven renal sarcoidosis presented with renal failure of unknown origin; in none was the diagnosis of sarcoidosis initially considered. The serum creatinine concentration at the time of presentation ranged from 265 to 1380 μmol/l (3.0 to 15.6 mg/dl), with a mean of 787 μmol/l (8.9 mg/dl). Although only two patients were hypercalcemic at the time of presentation, the 24-hour urinary excretion of calcium was increased in three of the four patients in whom it was measured, and renal calculi were present in one case. Renal biopsy revealed interstitial nephritis and tubular atrophy in all cases, as well as nephrocalcinosis in three cases and noncaseating granulomas negative for acid-fast bacilli in four cases. In each patient steroid therapy led to a rapid improvement in renal function (mean post-treatment serum creatinine level 274 μmol/l [3.1 mg/dl]). The follow-up period ranged from 8 months to 8 years (mean 3.0 years). In three patients renal function remained stable with low-dose steroid therapy. In two cases recurrent hypercalcemia and deteriorating renal function accompanied steroid withdrawal but resolved with its reinstitution. In one additional case reversible deterioration in renal function accompanied tapering of the steroid dose; however, there was no hypercalcemia. This report emphasizes the importance of considering sarcoidosis in the differential diagnosis of acute renal failure of unknown origin. Long-term follow-up of such patients is essential, as relapse is common. ImagesFIG. 1FIG. 2FIG. 3 PMID:519562

  2. F-18-fluorodeoxyglucose positron emission tomography-guided sampling of mediastinal lymph nodes in the diagnosis of cardiac sarcoidosis.

    PubMed

    Simonen, Piia; Lehtonen, Jukka; Kandolin, Riina; Schildt, Jukka; Marjasuo, Suvi; Miettinen, Heikki; Airaksinen, Juhani; Vihinen, Tapani; Tuohinen, Suvi; Haataja, Petri; Kupari, Markku

    2015-11-15

    Histologic proof of granulomatous inflammation is prerequisite for the diagnosis of cardiac sarcoidosis (CS). Because of the limited sensitivity of endomyocardial biopsy (EMB), confirmation of sarcoidosis often has to be acquired from extracardiac biopsies. We set out to review our experience of F-18-fluorodeoxyglucose positron emission tomography (F-18-FDG PET) in guiding extracardiac tissue biopsies in suspected CS. We included in this work 68 consecutive patients with proved CS who had undergone cardiac F-18-FDG PET with (n = 57) or without whole-body imaging as part of initial diagnostic evaluation. Their hospital charts, imaging studies, and diagnostic biopsies were reviewed in retrospect. Whole-body PET images showed extracardiac foci of abnormally high F-18-FDG uptake in 39 of 57 patients, of whom 38 had involvement of mediastinal lymph nodes (MLN). Parallel F-18-FDG uptake was found in other lymph nodes (n = 10), lungs (n = 9), liver (n = 3), spleen (n = 2), and thyroid gland (n = 1). Adding the mediastinal findings at cardiac PET without whole-body imaging, abnormal F-18-FDG uptake in MLN was found in totally 43 of the 68 patients with CS (63%). Histology of systemic sarcoidosis was known at presentation of cardiac symptoms in 8 patients. Of the 60 patients with missing histology, 24 patients underwent mediastinoscopy for sampling of PET-positive MLN, most often (n = 20) after nondiagnostic EMB; microscopy revealed diagnostic noncaseating granulomatous inflammation in 24 of the 24 cases (sensitivity 100%). In the remaining 36 patients, sarcoidosis histology was confirmed by EMB (n = 30), by biopsy of lungs (n = 2) or peripheral lymph nodes (n = 2), or at autopsy (n = 1) or post-transplantation (n = 1). In conclusion, MLN accumulate F-18-FDG at PET in most patients with CS and provide a highly productive source for diagnostic biopsies either primarily or subsequent to nondiagnostic EMB. PMID:26411357

  3. Evidence for a genetic component in familial sarcoidosis

    SciTech Connect

    Rybicki, B.A.; Harrington, D.; Major, M.

    1994-09-01

    Sarcoidosis is a disease with reported familial clustering, but a putative hereditary component has not been established. We analyzed 33 sarcoid index cases with a positive family history and their 596 1st and 2nd degree relatives to determine if the distribution of disease was consistent with a genetic etiology. Prevalence of sarcoidosis was twice as high in 1st degree relatives of index cases compared to 2nd degree relatives (8.5% vs. 3.9%; p=0.016). Recurrence risk ratios (defined as the risk of disease in relatives divided by the population prevalence) were calculated in 2nd degree relatives after making an ascertainment correction. A single gene model fit the observed results better than a polygenic model for a range of sarcoidosis prevalance. A greater shared environment among siblings compared to parents and offspring did not appear to increase risk of sarcoidosis based on a higher prevalance of disease in the latter (7.1% vs. 10.7%; p=0.313). However, the age of diagnosis in affected parent-offspring pairs was more consistent with a combined genetic and environmental etiology than either component alone. In summary, these results suggest a genetic component, possibly a single gene, increases disease risk in family members of sarcoidosis cases. A segregation analysis is planned to more thoroughly evaluate the evidence for both genetic and non-genetic transmission of sarcoidosis in these families.

  4. Cardiac Sarcoidosis: Clinical Manifestations, Imaging Characteristics, and Therapeutic Approach

    PubMed Central

    Houston, Brian A; Mukherjee, Monica

    2014-01-01

    Sarcoidosis is a multi-system disease pathologically characterized by the accumulation of T-lymphocytes and mononuclear phagocytes into the sine qua non pathologic structure of the noncaseating granuloma. Cardiac involvement remains a key source of morbidity and mortality in sarcoidosis. Definitive diagnosis of cardiac sarcoidosis, particularly early enough in the disease course to provide maximal therapeutic impact, has proven a particularly difficult challenge. However, major advancements in imaging techniques have been made in the last decade. Advancements in imaging modalities including echocardiography, nuclear spectroscopy, positron emission tomography, and magnetic resonance imaging all have improved our ability to diagnose cardiac sarcoidosis, and in many cases to provide a more accurate prognosis and thus targeted therapy. Likewise, therapy for cardiac sarcoidosis is beginning to advance past a “steroids-only” approach, as novel immunosuppressant agents provide effective steroid-sparing options. The following focused review will provide a brief discussion of the epidemiology and clinical presentation of cardiac sarcoidosis followed by a discussion of up-to-date imaging modalities employed in its assessment and therapeutic approaches. PMID:25452702

  5. Resolution of Cutaneous Sarcoidosis Following Topical Application of Ganoderma lucidum (Reishi Mushroom).

    PubMed

    Saylam Kurtipek, Gulcan; Ataseven, Arzu; Kurtipek, Ercan; Kucukosmanoglu, İlknur; Toksoz, Mustafa Rasid

    2016-03-01

    Ganoderma lucidum (reishi mushroom) has been used in traditional Chinese and Japanese medicine as a herbal remedy for over 2000 years. Studies have shown that G. lucidum has anti-allergic, anti-oxidant, anti-tumor, anti-viral, and anti-inflammatory properties. A review of the literature revealed that there were no studies examining the use of G. lucidum for the treatment of skin diseases. Here, we report the case of a 44-year-old male patient who used soap enriched with G. lucidum and goat's milk for 3 days in treating annular cutaneous sarcoidosis. The patient showed almost complete regression of the lesions.

  6. Cardiopulmonary exercise testing variables as predictors of long-term outcome in thoracic sarcoidosis

    PubMed Central

    Lopes, A.J.; Menezes, S.L.S.; Dias, C.M.; Oliveira, J.F.; Mainenti, M.R.M.; Guimarães, F.S.

    2012-01-01

    Cardiopulmonary exercise testing (CPET) plays an important role in the assessment of functional capacity in patients with interstitial lung disease. The aim of this study was to identify CPET measures that might be helpful in predicting the vital capacity and diffusion capacity outcomes of patients with thoracic sarcoidosis. A longitudinal study was conducted on 42 nonsmoking patients with thoracic sarcoidosis (median age = 46.5 years, 22 females). At the first evaluation, spirometry, the measurement of single-breath carbon monoxide diffusing capacity (DLCOsb) and CPET were performed. Five years later, the patients underwent a second evaluation consisting of spirometry and DLCOsb measurement. After 5 years, forced vital capacity (FVC)% and DLCOsb% had decreased significantly [95.5 (82-105) vs 87.5 (58-103) and 93.5 (79-103) vs 84.5 (44-102), respectively; P < 0.0001 for both]. In CPET, the peak oxygen uptake, maximum respiratory rate, breathing reserve, alveolar-arterial oxygen pressure gradient at peak exercise (P(A-a)O2), and Δ SpO2 values showed a strong correlation with the relative differences for FVC% and DLCOsb% (P < 0.0001 for all). P(A-a)O2 ≥22 mmHg and breathing reserve ≤40% were identified as significant independent variables for the decline in pulmonary function. Patients with thoracic sarcoidosis showed a significant reduction in FVC% and DLCOsb% after 5 years of follow-up. These data show that the outcome measures of CPET are predictors of the decline of pulmonary function. PMID:22331135

  7. Rationale and Design of the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis (GRADS) Study. Sarcoidosis Protocol

    PubMed Central

    Koth, Laura L.; Maier, Lisa A.; Morris, Alison; Drake, Wonder; Rossman, Milton; Leader, Joseph K.; Collman, Ronald G.; Hamzeh, Nabeel; Sweiss, Nadera J.; Zhang, Yingze; O’Neal, Scott; Senior, Robert M.; Becich, Michael; Hochheiser, Harry S.; Kaminski, Naftali; Wisniewski, Stephen R.; Gibson, Kevin F.

    2015-01-01

    Sarcoidosis is a systemic disease characterized by noncaseating granulomatous inflammation with tremendous clinical heterogeneity and uncertain pathobiology and lacking in clinically useful biomarkers. The Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis (GRADS) study is an observational cohort study designed to explore the role of the lung microbiome and genome in these two diseases. This article describes the design and rationale for the GRADS study sarcoidosis protocol. The study addresses the hypothesis that distinct patterns in the lung microbiome are characteristic of sarcoidosis phenotypes and are reflected in changes in systemic inflammatory responses as measured by peripheral blood changes in gene transcription. The goal is to enroll 400 participants, with a minimum of 35 in each of 9 clinical phenotype subgroups prioritized by their clinical relevance to understanding of the pathobiology and clinical heterogeneity of sarcoidosis. Participants with a confirmed diagnosis of sarcoidosis undergo a baseline visit with self-administered questionnaires, chest computed tomography, pulmonary function tests, and blood and urine testing. A research or clinical bronchoscopy with a research bronchoalveolar lavage will be performed to obtain samples for genomic and microbiome analyses. Comparisons will be made by blood genomic analysis and with clinical phenotypic variables. A 6-month follow-up visit is planned to assess each participant’s clinical course. By the use of an integrative approach to the analysis of the microbiome and genome in selected clinical phenotypes, the GRADS study is powerfully positioned to inform and direct studies on the pathobiology of sarcoidosis, identify diagnostic or prognostic biomarkers, and provide novel molecular phenotypes that could lead to improved personalized approaches to therapy for sarcoidosis. PMID:26193069

  8. Rationale and Design of the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis (GRADS) Study. Sarcoidosis Protocol.

    PubMed

    Moller, David R; Koth, Laura L; Maier, Lisa A; Morris, Alison; Drake, Wonder; Rossman, Milton; Leader, Joseph K; Collman, Ronald G; Hamzeh, Nabeel; Sweiss, Nadera J; Zhang, Yingze; O'Neal, Scott; Senior, Robert M; Becich, Michael; Hochheiser, Harry S; Kaminski, Naftali; Wisniewski, Stephen R; Gibson, Kevin F

    2015-10-01

    Sarcoidosis is a systemic disease characterized by noncaseating granulomatous inflammation with tremendous clinical heterogeneity and uncertain pathobiology and lacking in clinically useful biomarkers. The Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis (GRADS) study is an observational cohort study designed to explore the role of the lung microbiome and genome in these two diseases. This article describes the design and rationale for the GRADS study sarcoidosis protocol. The study addresses the hypothesis that distinct patterns in the lung microbiome are characteristic of sarcoidosis phenotypes and are reflected in changes in systemic inflammatory responses as measured by peripheral blood changes in gene transcription. The goal is to enroll 400 participants, with a minimum of 35 in each of 9 clinical phenotype subgroups prioritized by their clinical relevance to understanding of the pathobiology and clinical heterogeneity of sarcoidosis. Participants with a confirmed diagnosis of sarcoidosis undergo a baseline visit with self-administered questionnaires, chest computed tomography, pulmonary function tests, and blood and urine testing. A research or clinical bronchoscopy with a research bronchoalveolar lavage will be performed to obtain samples for genomic and microbiome analyses. Comparisons will be made by blood genomic analysis and with clinical phenotypic variables. A 6-month follow-up visit is planned to assess each participant's clinical course. By the use of an integrative approach to the analysis of the microbiome and genome in selected clinical phenotypes, the GRADS study is powerfully positioned to inform and direct studies on the pathobiology of sarcoidosis, identify diagnostic or prognostic biomarkers, and provide novel molecular phenotypes that could lead to improved personalized approaches to therapy for sarcoidosis. PMID:26193069

  9. Rationale and Design of the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis (GRADS) Study. Sarcoidosis Protocol.

    PubMed

    Moller, David R; Koth, Laura L; Maier, Lisa A; Morris, Alison; Drake, Wonder; Rossman, Milton; Leader, Joseph K; Collman, Ronald G; Hamzeh, Nabeel; Sweiss, Nadera J; Zhang, Yingze; O'Neal, Scott; Senior, Robert M; Becich, Michael; Hochheiser, Harry S; Kaminski, Naftali; Wisniewski, Stephen R; Gibson, Kevin F

    2015-10-01

    Sarcoidosis is a systemic disease characterized by noncaseating granulomatous inflammation with tremendous clinical heterogeneity and uncertain pathobiology and lacking in clinically useful biomarkers. The Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis (GRADS) study is an observational cohort study designed to explore the role of the lung microbiome and genome in these two diseases. This article describes the design and rationale for the GRADS study sarcoidosis protocol. The study addresses the hypothesis that distinct patterns in the lung microbiome are characteristic of sarcoidosis phenotypes and are reflected in changes in systemic inflammatory responses as measured by peripheral blood changes in gene transcription. The goal is to enroll 400 participants, with a minimum of 35 in each of 9 clinical phenotype subgroups prioritized by their clinical relevance to understanding of the pathobiology and clinical heterogeneity of sarcoidosis. Participants with a confirmed diagnosis of sarcoidosis undergo a baseline visit with self-administered questionnaires, chest computed tomography, pulmonary function tests, and blood and urine testing. A research or clinical bronchoscopy with a research bronchoalveolar lavage will be performed to obtain samples for genomic and microbiome analyses. Comparisons will be made by blood genomic analysis and with clinical phenotypic variables. A 6-month follow-up visit is planned to assess each participant's clinical course. By the use of an integrative approach to the analysis of the microbiome and genome in selected clinical phenotypes, the GRADS study is powerfully positioned to inform and direct studies on the pathobiology of sarcoidosis, identify diagnostic or prognostic biomarkers, and provide novel molecular phenotypes that could lead to improved personalized approaches to therapy for sarcoidosis.

  10. Efficacy of /sup 67/Ga-scintigraphy in predicting the diagnostic yield of transbronchial lung biopsy in pulmonary sarcoidosis

    SciTech Connect

    Ackart, R.S.; Munzel, T.L.; Rodriguez, J.J.; Donlan, C.J.; Klayton, R.J.; Foreman, D.R.

    1982-07-01

    Nineteen consecutive patients with clinically suspected sarcoidosis underwent /sup 67/Ga-scintigraphy prior to transbronchial lung biopsy (TBLB) to determine if /sup 67/Ga uptake in lung parenchyma would increase the diagnostic yield of the biopsy procedure. Biopsies were obtained from the areas showing parenchymal uptake on the /sup 67/Ga scan in 13 of the 19 patients. In the six patients not demonstrating uptake of /sup 67/Ga in the lung parenchyma, biopsies were obtained at random from the right lower lobe. There was no correlation between /sup 67/Ga uptake in hilar nodes or pulmonary parenchyma tissue and the diagnostic yield from TBLB. Researchers conclude that /sup 67/Ga scanning is neither efficacious nor cost-effective in predicting the diagnostic yield of TBLB in sarcoidosis.

  11. Intra-cardiac distribution of late gadolinium enhancement in cardiac sarcoidosis and dilated cardiomyopathy

    PubMed Central

    Sano, Makoto; Satoh, Hiroshi; Suwa, Kenichiro; Saotome, Masao; Urushida, Tsuyoshi; Katoh, Hideki; Hayashi, Hideharu; Saitoh, Takeji

    2016-01-01

    Cardiac involvement of sarcoid lesions is diagnosed by myocardial biopsy which is frequently false-negative, and patients with cardiac sarcoidosis (CS) who have impaired left ventricular (LV) systolic function are sometimes diagnosed with dilated cardiomyopathy (DCM). Late gadolinium enhancement (LE) in magnetic resonance imaging is now a critical finding in diagnosing CS, and the novel Japanese guideline considers myocardial LE to be a major criterion of CS. This article describes the value of LE in patients with CS who have impaired LV systolic function, particularly the diagnostic and clinical significance of LE distribution in comparison with DCM. LE existed at all LV segments and myocardial layers in patients with CS, whereas it was localized predominantly in the midwall of basal to mid septum in those with DCM. Transmural (nodular), circumferential, and subepicardial and subendocardial LE distribution were highly specific in patients with CS, whereas the prevalence of striated midwall LE were high both in patients with CS and with DCM. Since sarcoidosis patients with LE have higher incidences of heart failure symptoms, ventricular tachyarrhythmia and sudden cardiac death, the analyses of extent and distribution of LE are crucial in early diagnosis and therapeutic approach for patients with CS. PMID:27721933

  12. Digestive-tract sarcoidosis: French nationwide case-control study of 25 cases.

    PubMed

    Ghrenassia, Etienne; Mekinian, Arsene; Chapelon-Albric, Catherine; Levy, Pierre; Cosnes, Jacques; Sève, Pascal; Lefèvre, Guillaume; Dhôte, Robin; Launay, David; Prendki, Virginie; Morell-Dubois, Sandrine; Sadoun, Danielle; Mehdaoui, Anas; Soussan, Michael; Bourrier, Anne; Ricard, Laure; Benamouzig, Robert; Valeyre, Dominique; Fain, Olivier

    2016-07-01

    Digestive tract sarcoidosis (DTS) is rare and case-series are lacking. In this retrospective case-control study, we aimed to compare the characteristics, outcome, and treatment of patients with DTS, nondigestive tract sarcoidosis (NDTS), and Crohn disease.We included cases of confirmed sarcoidosis, symptomatic digestive tract involvement, and noncaseating granuloma in any digestive tract. Each case was compared with 2 controls with sarcoidoisis without digestive tract involvement and 4 with Crohn disease.We compared 25 cases of DTS to 50 controls with NDTS and 100 controls with Crohn disease. The major digestive clinical features were abdominal pain (56%), weight loss (52%), nausea/vomiting (48%), diarrhea (32%), and digestive bleeding (28%). On endoscopy of DTS, macroscopic lesions were observed in the esophagus (9%), stomach (78%), duodenum (9%), colon, (25%) and rectum (19%). As compared with NDTS, DTS was associated with weight loss (odds ratio [OR] 5.8; 95% confidence interval [CI] 1.44-23.3) and the absence of thoracic adenopathy (OR 5.0; 95% CI 1.03-25). As compared with Crohn disease, DTS was associated with Afro-Caribbean origin (OR 27; 95% CI 3.6-204) and the absence of ileum or colon macroscopic lesions (OR 62.5; 95% CI 10.3-500). On the last follow-up, patients with DTS showed no need for surgery (versus 31% for patients with Crohn disease; P = 0.0013), and clinical digestive remission was frequent (76% vs. 35% for patients with Crohn disease; P = 0.0002).The differential diagnosis with Crohn disease could be an issue with DTS. Nevertheless, the 2 diseases often have different clinical presentation and outcome. PMID:27442665

  13. Single Lesion of Sarcoidosis Presenting as Cicatricial Alopecia: A Rare Report from India

    PubMed Central

    Ghosh, Aparajita; Sengupta, Sujata; Coondoo, Arijit; Gharami, Ramesh Chandra

    2014-01-01

    Sarcoidosis affects different systems of the body including the skin where it can affect various cutaneous sites. Among these sites, the scalp is a very unusual location for lesions of sarcoidosis. Sarcoidosis of the scalp can very rarely be accompanied by cicatricial alopecia. We report here a rare case of sarcoidosis of scalp with cicatricial alopecia. To the best of our knowledge, this is the first such report from India. PMID:25191039

  14. Prevalence of cardiac sarcoidosis in white population: a case-control study: Proposal for a novel risk index based on commonly available tests.

    PubMed

    Martusewicz-Boros, Magdalena M; Boros, Piotr W; Wiatr, Elżbieta; Zych, Jacek; Piotrowska-Kownacka, Dorota; Roszkowski-Śliż, Kazimierz

    2016-08-01

    Cardiac sarcoidosis (CS) is a life-threatening and underdiagnosed manifestation of the disease, which requires a complicated and expensive diagnostic pathway. There is a need for simple tool for practitioners to determine the risk of CS without access to specialized equipment.The aim of study was to determine the prevalence of CS in a group of patients diagnosed with or followed up because of sarcoidosis. A secondary objective was the search for factors associated with heart involvement.We performed a prospective case-control study (screening analysis) in consecutive sarcoidosis patients collected from October 2012 to September 2015. Cardiac magnetic resonance (CMR) imaging was performed to confirm or exclude cardiac involvement in all patients. The study was conducted in a hospital-based referral center for patients with sarcoidosis and other interstitial lung diseases.Analysis was performed in a group of 201 patients (all white) with biopsy-proven sarcoidosis, mean age 41.4 ± 10.2, 121 of them (60.2%) males. Four patients with previously recognized cardiac diseases, which make CMR imaging for CS inconclusive, were not included.Cardiac involvement was detected by CMR in 49 patients (24.4%). Factors associated with an increased risk of CS (univariate analyses) included male sex (odds ratio [OR]: 2.5; 1.21-5.16, P = 0.01), cardiac-related symptoms (OR: 3.53; 1.81-6.89, P = 0.0002), extrathoracic sarcoidosis (OR: 3.48; 1.77-6.84, P = 0.0003), elevated serum NT-proBNP (OR: 3.82; 1.55-9.42, P = 0.004), any electrocardiography abnormality (OR: 5.38; 2.48-11.67, P = 0.0001), and contemporary radiological progression sarcoidosis in the lungs (OR: 2.98; 1.52-5.84, P = 0.001). Abnormalities in echocardiography and Holter ECG were also risk factors, but not significant in multivariate analyses. A CS Risk Index was developed using a multivariate model to predict CS, achieving an accuracy of 82%, sensitivity of 50%, specificity of 94%, and

  15. /sup 67/Ga citrate scanning and serum angiotensin converting enzyme levels in sarcoidosis

    SciTech Connect

    Gupta, R.G.; Bekerman, C.; Sicilian, L.; Oparil, S.; Pinsky, S.M.; Szidon, J.P.

    1982-09-01

    /sup 67/Ga citrate scans and serum angiotensin converting enzyme (ACE) levels were obtained in 54 patients with sarcoidosis and analyzed in relation to clinical manifestations. /sup 67/Ga scans were abnormal in 97% of patients with clinically active disease (n . 30) and in 71% of patients with inactive disease (n . 24). Serum ACE levels were abnormally high (2 standard deviations above the control mean) in 73% of patients with clinically active disease and in 54% of patients with inactive disease. Serum ACE levels correlated significantly with /sup 67/Ga uptake score (r..436; p less than .005). The frequency of abnormal /sup 67/Ga scans and elevated serum ACE levels suggests that inflammatory activity with little or no clinical expression is common in sarcoidosis. Abnormal /sup 67/Ga scans were highly sensitive (97%) but had poor specificity (29%) to clinical disease activity. The accuracy of negative prediction of clinical activity by normal scans (87%) was better than the accuracy of positive prediction of clinical activity by abnormal scans (63%). /sup 67/Ga scans can be used to support the clinical indentification of inactive sacoidosis.

  16. Right Ventricular Cardiomyopathy Meeting the Arrhythmogenic Right Ventricular Dysplasia Revised Criteria? Don't Forget Sarcoidosis!

    PubMed Central

    Vasaturo, Sabina; Ploeg, David E.; Buitrago, Guadalupe; Zeppenfeld, Katja; Veselic-Charvat, Maud

    2015-01-01

    A 53-year-old woman was referred for ventricular fibrillation with resuscitation. A CT-angiography showed signs of a right ventricular enlargement without obvious cause. A cardiac MRI demonstrated a dilated and hypokinetic right ventricle with extensive late gadolinium enhancement. Arrhythmogenic right ventricular dysplasia (ARVD) was suspected according to the "revised ARVD task force criteria". An endomyocardial biopsy was inconclusive. The patient developed purulent pericarditis after epicardial ablation therapy and died of toxic shock syndrome. The post-mortem pathologic examination demonstrated sarcoidosis involving the heart, lungs, and thyroid gland. PMID:25995699

  17. Sarcoidosis: correlation of HRCT findings with results of pulmonary function tests and serum angiotensin-converting enzyme assay.

    PubMed

    Mimori, Y

    1998-01-01

    We examined correlations between findings on chest high resolution computed tomography (HRCT), pulmonary function and values of serum angiotensin converting enzyme (ACE) in 25 patients with sarcoidosis. The most frequent CT features were small nodular opacities. The small nodules, representing the confluence of epithelioid granulomas, are strongly correlated with peribronchovascular, perilobular, and centrilobular lesions, where there is an abundance of lymphatic plexus. This strongly suggests the importance of the lymph vessels in the pathogenesis of sarcoidosis. The pulmonary functions tests showed obstructive defects in 6 and mixed-type defects in 2 of the 25 patients. Furthermore, an elevation of V50/V25 ratio suggesting small-airway disease was detected in many patients who showed normal values of FEV1.0% and %VC. This fact indicates that small-airway disease was manifested earlier in sarcoidosis patients. Statistically significant negative correlations were found between visual score and %VC, %FVC, FEV1.0%, %TLC, and %DLco, but there was no significant correlation between visual score and serum ACE. ACE is derived from granuloma-forming epithelioid cells, and the activity of ACE decreased rapidly in mature granulomas. Epithelioid cells in the mature granulomas which can be recognized on HRCT scan have stopped or are about to stop the release of ACE. In this study, serum ACE activity was found to be elevated and correlated with %V25 and V50/V25 at an early stage of the disease. The results of this study provide meaningful insights into the process of sarcoidosis in lung.

  18. Utility of endoscopic ultrasound-guided transbronchial fine-needle cytology in the diagnosis of sarcoidosis: A Saudi experience

    PubMed Central

    Raddaoui, Emad; Alhamad, Esam H.; Zaidi, Shaesta Naseem; Arafah, Maha; AlHabeeb, Fatmah Fahad

    2014-01-01

    Background: Endoscopic ultrasound-guided transbronchial fine-needle aspiration (EBUS-TFNA) is a minimally invasive technique for diagnosis of mediastinal masses/lesions. Although most studies have reported the utility of EBUS-TFNA in malignancy, its use has been extended to the benign conditions as well. Objective: The present study focused on utility of EBUS in contributing to reach the final diagnosis of sarcoidosis. Design: From May 2010 to December 2013, 19 of 80 patients who underwent EBUS-TFNA at one center for radiologically suspicious lesions for sarcoidosis, and with no definite histological diagnosis, were included in this retrospective study. Results: When the histological diagnosis was taken as the gold standard, the sensitivity of EBUS-TFNA was 84.2% and specificity 100% with the positive predictive value of 100. The combined diagnostic sensitivity of EBUS-TFNA and transbronchial lung biopsy was 100%. Conclusion: EBUS is a valuable, minimally invasive diagnostic modality to support the diagnosis of sarcoidosis in appropriate clinical setting, after conventional work-up – particularly if patients have suspicious radiological findings. This minimally invasive procedure helps in providing a final diagnosis without exposing the patient to the risk of complications from more invasive procedures. PMID:25558271

  19. The Effect Of Pixel Size On The Detection Rate Of Early Pulmonary Sarcoidosis In Digital Chest Radiographic Systems

    NASA Astrophysics Data System (ADS)

    MacMahon, Heber; Vyborny, Carl; Powell, Gregory; Doi, Kunio; Metz, Charles E.

    1984-08-01

    In digital radiography the pixel size used determines the potential spatial resolution of the system. The need for spatial resolution varies depending on the subject matter imaged. In many areas, including the chest, the minimum spatial resolution requirements have not been determined. Sarcoidosis is a disease which frequently causes subtle interstitial infiltrates in the lungs. As the initial step in an investigation designed to determine the minimum pixel size required in digital chest radiographic systems, we have studied 1 mm pixel digitized images on patients with early pulmonary sarcoidosis. The results of this preliminary study suggest that neither mild interstitial pulmonary infiltrates nor other abnormalities such as pneumothoraces may be detected reliably with 1 mm pixel digital images.

  20. Immunogenetics of Disease-Causing Inflammation in Sarcoidosis.

    PubMed

    Grunewald, Johan; Spagnolo, Paolo; Wahlström, Jan; Eklund, Anders

    2015-08-01

    Sarcoidosis is a systemic inflammatory disorder characterised by tissue infiltration by mononuclear phagocytes and lymphocytes with associated non-caseating granuloma formation. Originally described as a disorder of the skin, sarcoidosis can involve any organ with wide-ranging clinical manifestations and disease course. Recent studies have provided new insights into the mechanisms involved in disease pathobiology, and we now know that sarcoidosis has a clear genetic basis largely involving human leukocyte antigen (HLA) genes. In contrast to Mendelian-monogenic disorders--which are generally due to specific and relatively rare mutations often leading to a single amino acid change in an encoded protein--sarcoidosis results from genetic variations relatively common in the general population and involving multiple genes, each contributing an effect of varying magnitude. However, an individual may have the necessary genetic profile and yet the disease will not develop unless an environmental or infectious factor is encountered. Genetics appears also to contribute to the huge variability in clinical phenotype and disease behaviour. Moreover, it has been established that sarcoidosis granulomatous inflammation is a highly polarized T helper 1 immune response that starts with an antigenic stimulus followed by T cell activation via a classic HLA class II-mediated pathway. A complex network of lymphocytes, macrophages, and cytokines is pivotal in the orchestration and evolution of the granulomatous process. Despite these advances, the aetiology of sarcoidosis remains elusive and its pathogenesis incompletely understood. As such, there is an urgent need for a better understanding of disease pathogenesis, which hopefully will translate into the development of truly effective therapies. PMID:25791751

  1. Immunogenetics of Disease-Causing Inflammation in Sarcoidosis.

    PubMed

    Grunewald, Johan; Spagnolo, Paolo; Wahlström, Jan; Eklund, Anders

    2015-08-01

    Sarcoidosis is a systemic inflammatory disorder characterised by tissue infiltration by mononuclear phagocytes and lymphocytes with associated non-caseating granuloma formation. Originally described as a disorder of the skin, sarcoidosis can involve any organ with wide-ranging clinical manifestations and disease course. Recent studies have provided new insights into the mechanisms involved in disease pathobiology, and we now know that sarcoidosis has a clear genetic basis largely involving human leukocyte antigen (HLA) genes. In contrast to Mendelian-monogenic disorders--which are generally due to specific and relatively rare mutations often leading to a single amino acid change in an encoded protein--sarcoidosis results from genetic variations relatively common in the general population and involving multiple genes, each contributing an effect of varying magnitude. However, an individual may have the necessary genetic profile and yet the disease will not develop unless an environmental or infectious factor is encountered. Genetics appears also to contribute to the huge variability in clinical phenotype and disease behaviour. Moreover, it has been established that sarcoidosis granulomatous inflammation is a highly polarized T helper 1 immune response that starts with an antigenic stimulus followed by T cell activation via a classic HLA class II-mediated pathway. A complex network of lymphocytes, macrophages, and cytokines is pivotal in the orchestration and evolution of the granulomatous process. Despite these advances, the aetiology of sarcoidosis remains elusive and its pathogenesis incompletely understood. As such, there is an urgent need for a better understanding of disease pathogenesis, which hopefully will translate into the development of truly effective therapies.

  2. Radioisotope bone scanning in a case of sarcoidosis

    SciTech Connect

    Cinti, D.C.; Hawkins, H.B.; Slavin, J.D. Jr.

    1985-03-01

    The application of radioisotope scanning to osseous involvement from systemic sarcoidosis has been infrequently described in the scientific literature. Most commonly, the small bones of the hands and feet are affected if sarcoidosis involves the skeleton. Nonetheless, there are also occasional manifestations of sarcoid in the skull, long bones, and vertebral bodies. This paper describes a case of sarcoid involving the lung parenchyma with multiple lesions in the skull and ribs demonstrated by bone scanning with Tc-99m MDP. Following treatment with steroids, the bone scan showed complete resolution of the rib lesions and almost complete resolution of the lesions in the calvarium.

  3. Early-onset sarcoidosis mimicking refractory cutaneous histiocytosis.

    PubMed

    Ohga, Shouichi; Ichino, Kiyomi; Urabe, Kazunori; Ishimura, Masataka; Takada, Hidetoshi; Nishikomori, Ryuta; Furue, Masutaka; Hara, Toshiro

    2008-03-01

    A 10-year-old female was diagnosed as having early-onset sarcoidosis (EOS) after a prolonged skin disease. A granuloma emerged on the face at age 2 and massive lesions extended to the rest of the body. Repeated biopsies indicated histiocytic proliferation. At age 7, fever, disseminated macular eruptions, and multinucleated giant cells in the bone marrow prompted vinblastine and prednisolone therapy. Five months after stopping therapy, hypercalcemic crisis occurred along with fever, cytopenias, and interferon-gamma-nemia indicating a macrophage activation syndrome. A biopsy of nodules confirmed the diagnosis of sarcoidosis. The atypical EOS should be differentiated from histiocytosis.

  4. Cutaneous Sarcoidosis: Preamble of a Paucisymptomatic Systemic Disease.

    PubMed

    Ramírez Huaranga, Marco Aurelio; Ramos Rodríguez, Claudia Carolina; de la Rocha Vedia, Iris Violeta; García Arpa, Mónica; Murillo Lázaro, Cristina; Bellón Guardia, María

    2015-01-01

    Sarcoidosis is a systemic granulomatous disease with a wide range of clinical manifestations. Skin involvement is an early, frequent and accessible location for a histopathological study. Several risk factors have been described to determine the likelihood of systemic involvement of an apparent cutaneous condition. Early diagnosis and systemic treatment could prevent future complications. A series of three cases is presented in which the initial diagnosis was cutaneous sarcoidosis, but it was actually the first manifestation of a systemic disease. A review of this topic is also presented. PMID:25823568

  5. In vitro gallium-67 lung index for the evaluation of sarcoidosis

    SciTech Connect

    Braude, A.C.; Cohen, R.; Rahmani, R.; Hornstein, A.; Klein, M.; Meindok, H.O.; Chamberlain, D.W.; Rebuck, A.S.

    1984-11-01

    In the evaluation of the active alveolitis of pulmonary sarcoidosis, both the proportional lymphocyte count obtained by bronchoalveolar lavage and state of activation of the alveolar macrophage by gallium scanning are required. We injected 6 mCi (200 MBq) of /sup 67/Ga intravenously on 24 occasions in 13 patients with biopsy-proved sarcoidosis. Forty-eight hours later, patients were scanned with a rectilinear scanner and the generated scintigrams were evaluated using the NIH index. Seventy-two hours after injection, bronchoalveolar lavage was performed, and venous blood was sampled. The harvested lavage fluid was analyzed for absolute and proportional cell counts, and radioactivity was measured in blood and BAL fluid. An in vitro /sup 67/Ga index was generated and expressed as counts/100,000 alveolar macrophages/ml blood (mean, 0.0146 +/- 0.0087 SD). There was a significant relationship between the in vitro index and proportional lymphocyte BAL counts (r . 0.79; p less than 0.002) that was comparable to that obtained using the NIH index (r . 0.74; p less than 0.005). These data suggest that the in vitro index might offer a more objective assessment of /sup 67/Ga uptake by the lung, but this would require validation against clinical parameters in a prospective study.

  6. Assessment of Cardiac Sarcoidosis with Advanced Imaging Modalities

    PubMed Central

    Akasaka, Takashi

    2014-01-01

    Sarcoidosis is a chronic systemic disease of unknown etiology that is characterized by the presence of noncaseating epithelioid granulomas, usually in multiple organs. Several studies have shown that sarcoidosis might be the result of an exaggerated granulomatous reaction after exposure to unidentified antigens in genetically susceptible individuals. Cardiac involvement may occur and lead to an adverse outcome: the heart mechanics will be affected and that causes ventricular failure, and the cardiac electrical system will be disrupted and lead to third degree atrioventricular block, malignant ventricular tachycardia, and sudden cardiac death. Thus, early diagnosis and treatment of this potentially devastating disease is critically important. However, sensitive and accurate imaging modalities have not been established. Recent studies have demonstrated the promising potential of cardiac magnetic resonance imaging (MRI) and 18F-fluoro-2-deoxyglucose positron emission tomography (18F-FDG PET) in the diagnosis and assessment of cardiac sarcoidosis (CS). In this review, we discuss the epidemiology, etiology, histological findings, and clinical features of sarcoidosis. We also introduce advanced imaging including 18F-FDG PET and cardiac MRI as more reliable diagnostic modalities for CS. PMID:25250336

  7. Association between exposure to crystalline silica and risk of sarcoidosis

    PubMed Central

    Rafnsson, V.; Ingimarsson, O.; Hjalmarsson, I.; Gunnarsdottir, H.

    1998-01-01

    OBJECTIVES: The possibility of an association between exposure to silica and autoimmune diseases has recently come under discussion. In the following case-referent study, a cohort exposed to diatomaceous earth and cristobalite provided an opportunity to evaluate such an exposure with reference to sarcoidosis. METHODS: The inhabitants of a district served by a single healthcare centre and a hospital formed the study base. A diatomaceous earth plant is located in this community and the medical institutions are responsible for primary and secondary health care of the population. Cases of sarcoidosis were identified from the hospital records according to certain clinical, radiological, and histological criteria. Referents were selected randomly from the population of the district. Information on exposure to crystalline silica, cristobalite, was obtained by record linkage of the cases and referents with a file which included all present and past workers at the diatomaceous earth plant and those who had worked at loading vessels with the product from the plant. RESULTS: Eight cases of sarcoidosis were found, six of which were in the exposed group. Of the 70 referents, 13 were exposed. The odds ratio (95% confidence interval) was 13.2 (2.0 to 140.9). CONCLUSION: The odds ratios were high and there were some indications of a dose-response relation which will hopefully encourage further studies. To our knowledge this is the first study to indicate a relation between sarcoidosis and exposure to the crystalline silica, cristobalite.   PMID:9930085

  8. Multicentric Castleman's disease developing during follow-up of sarcoidosis.

    PubMed

    Sawata, Tetsuro; Bando, Masashi; Nakayama, Masayuki; Mato, Naoko; Takemura, Tamiko; Sugiyama, Yukihiko

    2016-07-01

    Pulmonary sarcoidosis is reported to have complication of lymphoproliferative disease such as malignant lymphoma, but the complication of multicentric Castleman's disease (MCD) is rarely reported. In our case of a 60-year-old woman, bilateral hilar lymphadenopathy was noted in her chest X-ray. We performed a transbronchial lung biopsy. She was diagnosed as having pulmonary sarcoidosis (Stage II). The shadow on chest X-ray disappeared without treatment. However, after 8 years, swelling of the mediastinal and abdominal lymph node, thickened bronchovascular bundle, and multiple nodular shadows were identified, and a thoracoscopic lung biopsy was performed. Based on the histopathological findings and elevated serum interleukin-6 level (75.7 pg/mL), she was diagnosed with pulmonary sarcoidosis complicated by MCD. When a change in chest X-ray findings are found during monitoring of pulmonary sarcoidosis, it is important to proceed with a thoracoscopic lung biopsy, because of the possibility of the rare complication of MCD. PMID:27512568

  9. Respiratory clearance of 99mTc-DTPA and pulmonary involvement in sarcoidosis

    SciTech Connect

    Dusser, D.J.; Collignon, M.A.; Stanislas-Leguern, G.; Barritault, L.G.; Chretien, J.; Huchon, G.J.

    1986-09-01

    To investigate the relationships between the respiratory epithelial clearance of micronic aerosolized /sup 99m/Tc-DTPA (RC-DTPA) and pulmonary function, serum angiotensin-converting enzyme (SACE), and lymphocytic alveolitis in patients with sarcoidosis, RC-DTPA was measured in 49 nonsmokers with pulmonary sarcoidosis and 38 normal nonsmokers. Pulmonary involvement was evaluated on chest roentgenograms (type O = normal, type I = hilar adenopathies, type II = hilar adenopathies associated with parenchymal shadows, type III = parenchymal shadows without adenopathy) and by pulmonary function tests. Serum angiotensin-converting enzyme was determined, and a bronchoalveolar lavage was performed for alveolar lymphocyte differential counting (Ly%). RC-DTPA was increased (greater than or equal to 1.96%/min) in 12 of 31 patients with type II or III involvement but was normal in all 18 patients with type O or I involvement (p = 0.002). Patients with increased RC-DTPA had low FVC, TLC, FEV1, and resting Pao2 (p less than 0.05); resting and exercise AaPo2 were increased (p less than 0.05), but RC-DTPA correlated negatively with FEV1 (p less than 0.01), Pao2 at rest (p less than 0.005), and DLCO (p less than 0.05) and positively with resting and exercise AaPO2 (p less than 0.01). In patients with increased RC-DTPA (42 +/- 17%), Ly% did not differ from Ly% in patients with normal RC-DTPA (34 +/- 16%). SACE was increased in patients with increased RC-DTPA (56 +/- 26 U/ml versus 38 +/- 16 U/ml; p = 0.007) and correlated positively with RC-DTPA (p less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

  10. Localization of propionibacterium acnes in granulomas supports a possible etiologic link between sarcoidosis and the bacterium.

    PubMed

    Negi, Mariko; Takemura, Tamiko; Guzman, Josune; Uchida, Keisuke; Furukawa, Asuka; Suzuki, Yoshimi; Iida, Tadatsune; Ishige, Ikuo; Minami, Junko; Yamada, Tetsuo; Kawachi, Hiroshi; Costabel, Ulrich; Eishi, Yoshinobu

    2012-09-01

    Sarcoidosis likely results from the exposure of a genetically susceptible subject to an environmental agent, possibly an infectious one. Mycobacterial and propionibacterial organisms are the most commonly implicated potential etiologic agents. Propionibacterium acnes is the only microorganism, however, found in sarcoid lesions by bacterial culture. To evaluate the pathogenic role of this indigenous bacterium, we screened for the bacterium in sarcoid and non-sarcoid tissues using immunohistochemical methods with novel P. acnes-specific monoclonal antibodies that react with cell-membrane-bound lipoteichoic acid (PAB antibody) and ribosome-bound trigger-factor protein (TIG antibody). We examined formalin-fixed and paraffin-embedded samples of lungs and lymph nodes from 196 patients with sarcoidosis, and corresponding control samples from 275 patients with non-sarcoidosis diseases. The samples were mostly from Japanese patients, with 64 lymph node samples from German patients. Immunohistochemistry with PAB antibody revealed small round bodies within sarcoid granulomas in 20/27 (74%) video-assisted thoracic surgery lung samples, 24/50 (48%) transbronchial lung biopsy samples, 71/81 (88%) Japanese lymph node samples, and 34/38 (89%) German lymph node samples. PAB antibody did not react with non-sarcoid granulomas in any of the 45 tuberculosis samples or the 34 samples with sarcoid reaction. In nongranulomatous areas, small round bodies detected by PAB antibody were found in alveolar macrophages of lungs and paracortical macrophages of lymph nodes from many sarcoid and some non-sarcoid patients. Large-spheroidal acid-fast bodies, Hamazaki-Wesenberg bodies, which were found in 50% of sarcoid and 15% of non-sarcoid lymph node samples, reacted with both PAB and TIG antibodies. Electron microscopy revealed that these Hamazaki-Wesenberg bodies had a single bacterial structure and lacked a cell wall with occasional protrusions from the body. The high frequency and specificity

  11. Systemic sarcoidosis with hypercalcaemia, hypothalamic-pituitary dysfunction and thyroid involvement.

    PubMed

    Alsahwi, Nassib; Blavo, Delali; Karanchi, Harsha

    2016-01-01

    Sarcoidosis is a multisystem granulomatous disorder. The lungs are the principal organs affected, however, extrapulmonary involvement including disorders of the pituitary and thyroid glands has been reported but presentation with multiple endocrine manifestations is rare. We report the case of a 36-year-old African-American woman who presented with hypercalcaemia, abnormal thyroid function studies and secondary amenorrhoea. On workup including laboratory, radiological testing and biopsy she was diagnosed with sarcoidosis with multi-organ involvement. Endocrine manifestations included non-parathyroid hormone mediated hypercalcaemia related to sarcoidosis, thyroid involvement with sarcoidosis and hypothalamic-pituitary involvement with a sellar and suprasellar mass associated with secondary adrenal insufficiency, secondary hypogonadism, growth hormone deficiency and secondary hypothyroidism. We report to the best of our knowledge the first case of simultaneous multiple endocrine manifestations of sarcoidosis that included hypercalcaemia, hypopituitarism and sarcoidosis of the thyroid gland. PMID:27495178

  12. CUTANEOUS SARCOIDOSIS - A GREAT MASQUERADER: A REPORT OF THREE INTERESTING CASES

    PubMed Central

    Reddy, Raghunatha R; Shashi Kumar, B M; Harish, M R

    2011-01-01

    It is a known fact that cutaneous sarcoidosis is a great imitator in dermatology. We report three cases of cutaneous sarcoidosis without systemic involvement and with varied dermatologic presentation with regard to age and morphology. Lesions mimicked various common dermatologic conditions, causing great confusion for the diagnosis and posing problems for management. Awareness of these varied morphologic presentations is essential for the early diagnosis and management of the master mimicker - cutaneous sarcoidosis. PMID:22121281

  13. Correlation Network Analysis Reveals Relationships between MicroRNAs, Transcription Factor T-bet, and Deregulated Cytokine/Chemokine-Receptor Network in Pulmonary Sarcoidosis

    PubMed Central

    Dyskova, Tereza; Fillerova, Regina; Novosad, Tomas; Kudelka, Milos; Zurkova, Monika; Gajdos, Petr; Kolek, Vitezslav; Kriegova, Eva

    2015-01-01

    Sarcoidosis is an inflammatory granulomatous disease with unknown etiology driven by cytokines and chemokines. There is limited information regarding the regulation of cytokine/chemokine-receptor network in bronchoalveolar lavage (BAL) cells in pulmonary sarcoidosis, suggesting contribution of miRNAs and transcription factors. We therefore investigated gene expression of 25 inflammation-related miRNAs, 27 cytokines/chemokines/receptors, and a Th1-transcription factor T-bet in unseparated BAL cells obtained from 48 sarcoidosis patients and 14 control subjects using quantitative RT-PCR. We then examined both miRNA-mRNA expressions to enrich relevant relationships. This first study on miRNAs in sarcoid BAL cells detected deregulation of miR-146a, miR-150, miR-202, miR-204, and miR-222 expression comparing to controls. Subanalysis revealed higher number of miR-155, let-7c transcripts in progressing (n = 20) comparing to regressing (n = 28) disease as assessed by 2-year follow-up. Correlation network analysis revealed relationships between microRNAs, transcription factor T-bet, and deregulated cytokine/chemokine-receptor network in sarcoid BAL cells. Furthermore, T-bet showed more pronounced regulatory capability to sarcoidosis-associated cytokines/chemokines/receptors than miRNAs, which may function rather as “fine-tuners” of cytokine/chemokine expression. Our correlation network study implies contribution of both microRNAs and Th1-transcription factor T-bet to the regulation of cytokine/chemokine-receptor network in BAL cells in sarcoidosis. Functional studies are needed to confirm biological relevance of the obtained relationships. PMID:26696750

  14. Detecting Cardiac Sarcoidosis with a Right Atrial Mass Using Transthoracic Echocardiography.

    PubMed

    Takahashi, Yusuke; Izumi, Chisato; Miyake, Makoto; Nakajima, Seiko; Nishimura, Shunsuke; Kuroda, Maiko; Yoshikawa, Yusuke; Amano, Masashi; Hayama, Yukiko; Imamura, Sari; Onishi, Naoaki; Tamaki, Yodo; Enomoto, Soichiro; Tamura, Toshihiro; Kondo, Hirokazu; Kaitani, Kazuaki; Nakagawa, Yoshihisa

    2016-01-01

    An asymptomatic 40-year-old woman with a first-degree atrioventricular block presented a right atrial mass in transthoracic echocardiograms. Transesophageal echocardiograms showed abnormally thickened tissue on the interatrial septum, which extended around the aortic annulus. Multimodality examinations demonstrated lesions in the heart, lungs, liver, and spleen, suggesting sarcoidosis. She was diagnosed with cardiac sarcoidosis after we detected granulomas in a lung specimen. A right atrial mass shrunk following steroid therapy. We should therefore consider the possibility of cardiac sarcoidosis when we see wall thickening and a mass echo in the atrium. These signs may point to an early-phase lesion of cardiac sarcoidosis.

  15. Systemic and lung protein changes in sarcoidosis. Lymphocyte counts, gallium uptake values, and serum angiotensin-converting enzyme levels may reflect different aspects of disease activity

    SciTech Connect

    Check, I.J.; Kidd, M.R.; Staton, G.W. Jr.

    1986-01-01

    BAL lymphocyte percentages, quantitated gallium-67 lung uptake, and SACE levels have all been proposed as measures of disease activity in sarcoidosis. We analyzed 32 paired sera and BAL fluids from sarcoidosis patients by high-resolution agarose electrophoresis to look for protein changes characteristic of systemic or local inflammation and compared the results with those from the above tests. Nine patients (group 1) had serum inflammatory protein changes and increased total protein, albumin, beta 1-globulin (transferrin), and gamma-globulin levels in fluid recovered by BAL. Thirteen patients (group 2) had normal protein levels in sera but abnormal protein levels in BAL specimens. Ten patients (group 3) had normal protein levels in sera and in BAL specimens. Patients in groups 1 and 2 had a disproportionate increase in beta 1-globulin (transferrin) and gamma-globulin levels in their BAL specimens. The BAL lymphocyte percentage changes paralleled the BAL protein level changes, suggesting relationships among the immunoregulatory role of these cells, increased local immunoglobulin synthesis, and the pathogenesis of altered alveolar permeability. Gallium-67 uptake was highest in patients with serum inflammatory protein changes. Thus, systemic inflammation may facilitate pulmonary gallium-67 uptake, possibly by changes in BAL fluid or serum transferrin saturation and/or kinetics. SACE levels showed no relationship to changes in the levels of serum or BAL proteins. These data suggest that the various proposed measures of disease activity reflect different aspects of inflammation in sarcoidosis.

  16. Resolution of Cutaneous Sarcoidosis Following Topical Application of Ganoderma lucidum (Reishi Mushroom).

    PubMed

    Saylam Kurtipek, Gulcan; Ataseven, Arzu; Kurtipek, Ercan; Kucukosmanoglu, İlknur; Toksoz, Mustafa Rasid

    2016-03-01

    Ganoderma lucidum (reishi mushroom) has been used in traditional Chinese and Japanese medicine as a herbal remedy for over 2000 years. Studies have shown that G. lucidum has anti-allergic, anti-oxidant, anti-tumor, anti-viral, and anti-inflammatory properties. A review of the literature revealed that there were no studies examining the use of G. lucidum for the treatment of skin diseases. Here, we report the case of a 44-year-old male patient who used soap enriched with G. lucidum and goat's milk for 3 days in treating annular cutaneous sarcoidosis. The patient showed almost complete regression of the lesions. PMID:26868374

  17. Fulminant musculoskeletal and neurologic sarcoidosis: case report and literature update.

    PubMed

    Sweeney, Ashley; Hammer, Richard; Evenski, Andrea; Crim, Julia

    2016-11-01

    We report a case of fulminant sarcoidosis in a 28-year-old man presenting with skin nodules, multifocal small and large joint arthralgias, and blurred vision. Characteristic bone, soft tissue, articular, and CNS findings were evident on multimodality imaging. Bony abnormalities included near-complete destruction of a distal phalanx, "lace-like" lucent lesions, erosive arthritis, lytic lesions with and without sclerotic margins, and bone marrow replacement visible only on MRI. The extent of bony disease at time of presentation was unusual. We review the widely varying reported prevalence of imaging findings of bony sarcoidosis in the literature, and discuss reasons for this variability. We found that musculoskeletal findings at US and MRI were less specific than radiographic and CT findings, but were useful in quantifying extent of disease.

  18. Fulminant musculoskeletal and neurologic sarcoidosis: case report and literature update.

    PubMed

    Sweeney, Ashley; Hammer, Richard; Evenski, Andrea; Crim, Julia

    2016-11-01

    We report a case of fulminant sarcoidosis in a 28-year-old man presenting with skin nodules, multifocal small and large joint arthralgias, and blurred vision. Characteristic bone, soft tissue, articular, and CNS findings were evident on multimodality imaging. Bony abnormalities included near-complete destruction of a distal phalanx, "lace-like" lucent lesions, erosive arthritis, lytic lesions with and without sclerotic margins, and bone marrow replacement visible only on MRI. The extent of bony disease at time of presentation was unusual. We review the widely varying reported prevalence of imaging findings of bony sarcoidosis in the literature, and discuss reasons for this variability. We found that musculoskeletal findings at US and MRI were less specific than radiographic and CT findings, but were useful in quantifying extent of disease. PMID:27596753

  19. The "mystery" of cutaneous sarcoidosis: facts and controversies.

    PubMed

    Tchernev, G; Cardoso, J C; Chokoeva, A A; Verma, S B; Tana, C; Ananiev, J; Gulubova, M; Philipov, S; Kanazawa, N; Nenoff, P; Lotti, T; Wollina, U

    2014-01-01

    The reason why the cutaneous form of sarcoidosis is well known in the literature is because of its spectrum of manifestations granting it the fame of a Great Imitator. The mystery shrouding the pathogenesis of this rare cutaneous disease is still there (in spite of the fundamental progress of the various diagnostic methods in current day medicine). The production of the morphological substrate - the epithelioid cell granuloma - which is considered to be characteristic of skin sarcoidosis, could, however, also be the end result of a reaction to i) various specific infectious agents such as Leishmaniasis cutis, coccidioidomycosis, etc., ii) certain residual bacterial or other mycobacterial antigens which, at the moment of setting the diagnosis are - by definition - non-infectious but still immunogenic, as well as iii) different tumor antigens in lesional tissue or other location. Often, differentiating between sarcodiosis and a sarcoid-like reaction, based on the updated criteria for cutaneous sarcoidosis, is problematic to downright impossible. A future characterization of the genetic signature of the two conditions, as well as the implementation of additional mandatory panels for i) the identification of certain infectious or ii) non-infectious but immunogenic and iii) tumor antigens in the epithelioid cell granuloma (or in another location in the organism), could be a considerable contribution to the process of differentiating between the two above-mentioned conditions. This will create conditions for greater accuracy when setting the subsequent therapeutic approaches.

  20. Sudden unexpected death due to severe pulmonary and cardiac sarcoidosis.

    PubMed

    Ginelliová, Alžbeta; Farkaš, Daniel; Farkašová Iannaccone, Silvia; Vyhnálková, Vlasta

    2016-09-01

    In this paper we report the autopsy findings of a 57 year old woman who died unexpectedly at home. She had been complaining of shortness of breath, episodes of dry coughing, and nausea. Her past medical and social history was unremarkable. She had no previous history of any viral or bacterial disease and no history of oncological disorders. Autopsy revealed multiple grayish-white nodular lesions in the pleura and epicardial fat and areas resembling fibrosis on the cut surface of the anterior and posterior wall of the left ventricle and interventricular septum. Histological examination of the lungs and heart revealed multiple well-formed noncaseating epithelioid cell granulomas with multinucleated giant cells. Death was attributed to myocardial ischemia due to vasculitis of intramural coronary artery branches associated with sarcoidosis. Sarcoidosis is a multisystemic disease of unknown etiology characterized by the formation of noncaseating epithelioid cell granulomas in the affected organs and tissues. The diagnosis of sarcoidosis in this case was established when other causes of granulomatous disease such as tuberculosis, berylliosis, hypersensitivity pneumonitis, and giant cell myocarditis had been reasonably excluded. PMID:27379608

  1. Two Cases of Heerfordt's Syndrome: A Rare Manifestation of Sarcoidosis

    PubMed Central

    Furuta, Yasushi; Fukuda, Satoshi

    2016-01-01

    Heerfordt's syndrome is a rare manifestation of sarcoidosis characterized by the presence of facial nerve palsy, parotid gland enlargement, anterior uveitis, and low grade fever. Two cases of Heerfordt's syndrome and a literature review are presented. Case  1. A 53-year-old man presented with swelling of his right eyelid, right facial nerve palsy, and swelling of his right parotid gland. A biopsy specimen from the swollen eyelid indicated sarcoidosis and he was diagnosed with incomplete Heerfordt's syndrome based on the absence of uveitis. His symptoms were improved by corticosteroid therapy. Case  2. A 55-year-old woman presented with left facial nerve palsy, bilateral hearing loss, and swelling of her bilateral parotid glands. She had been previously diagnosed with uveitis and bilateral hilar lymphadenopathy. Although no histological confirmation was performed, she was diagnosed with complete Heerfordt's syndrome on the basis of her clinical symptoms. Swelling of the bilateral parotid glands and left facial nerve palsy were improved immediately by corticosteroid therapy. Sarcoidosis is a relatively uncommon disease for the otolaryngologist. However, the otolaryngologist may encounter Heerfordt's syndrome as this syndrome presents with facial nerve palsy and swelling of the parotid gland. Therefore, we otolaryngologists should diagnose and treat Heerfordt's syndrome appropriately in cooperation with pneumologists and ophthalmologists. PMID:26885424

  2. Assessing the activity of sarcoidosis: quantitative /sup 67/Ga-citrate imaging

    SciTech Connect

    Fajman, W.A.; Greenwald, L.V.; Staton, G.; Check, I.J.; Pine, J.; Gilman, M.; Scheidt, K.A.; McClees, E.C.

    1984-04-01

    Three different methods of quantitating /sup 67/Ga-citrate lung images - a visual index, a computer-assisted index, and the total-lung-to-background ratio - were compared in 71 studies of patients with biopsy-proven sarcoidosis. Fifty consecutive cases were analyzed independently by two different observers using all three methods. In these studies, each index was correlated with the cell differential in the bronchoalveolar lavage fluid. The total-lung-to-background ratio proved to be the simplest to perform; correlated best with the original visual index and the percentage of lymphocytes obtained in bronchoalveolar lavage fluid. Sensitivity for detecting active disease was 84% compared with 64% and 58% for the visual and computer-assisted indices, respectively, with no sacrifice in specificity.

  3. Hypercalcemia, Anemia, and Acute Kidney Injury: A Rare Presentation of Sarcoidosis

    PubMed Central

    Sharma, Neeraj; Tariq, Hassan; Uday, Kalpana; Skaradinskiy, Yevgeniy; Niazi, Masooma; Chilimuri, Sridhar

    2015-01-01

    We discuss a case of a 61-year-old woman who presented with substernal chest pain. She was found to have elevated calcium levels, anemia, and acute kidney injury. The hypercalcemia persisted despite therapy with fluids and bisphosphonates. She was found to have nonparathyroid hormone (PTH) mediated hypercalcemia. The chest X-ray did not reveal any pathology. Our Initial impression was likely underlying hematologic malignancy such as lymphoma or multiple myeloma. A bone marrow biopsy was performed that revealed nonnecrotizing granulomatous inflammation. Further workup revealed elevated vitamin 1,25 dihydroxy level, beta-two microglobulin level, and ACE levels. Noncontrast computed tomography (CT) scan of chest showed bilateral apical bronchiectasis, but did not show any lymphadenopathy or evidence of malignancy. Subsequently, a fiber optic bronchoscopy with transbronchial biopsy showed nonnecrotizing granulomatous inflammation consistent with sarcoidosis. After initiating glucocorticoid therapy, the patient's hypercalcemia improved and her kidney function returned to baseline. PMID:26199627

  4. Sarcoidal granulomas in the mediastinal lymph nodes after treatment for marginal zone lymphoma of the esophagus: report of a case with review of the concept of the sarcoidosis-lymphoma syndrome

    PubMed Central

    Ishida, Mitsuaki; Hodohara, Keiko; Furuya, Aya; Okuno, Hiroko; Yoshii, Miyuki; Horinouchi, Akiko; Shirakawa, Ayaka; Iwai, Muneo; Kagotani, Akiko; Yoshida, Takashi

    2014-01-01

    Patients with sarcoidosis have a high risk of development of malignant lymphoma, and this association was coined the term “sarcoidosis-lymphoma syndrome”. Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is a distinct clinicopathological entity, and the stomach is the most common site. The occurrence of this type of lymphoma in the esophagus is extremely rare. In this report, we describe the first documented case of sarcoidal granulomas in the mediastinal lymph nodes after treatment for MALT lymphoma of the esophagus. A 60-year-old Japanese female was found to have a submucosal tumor in the esophagus. Histopathological study revealed proliferation of small- to medium-sized lymphoid cells with convoluted nuclei, and immunohistochemically, these lymphoid cells were diffusely positive for CD20, bcl-2, and MUM1. R-CHOP therapy was performed, which led to tumor remission. Two years later, swelling of the mediastinal lymph nodes was detected. Histopathological study of the lymph nodes revealed presence of variably-sized epithelioid granulomas without caseating necrosis but no malignant lymphoma was noted. Sarcoidal granulomas can be observed in patients with malignant tumors including malignant lymphoma and carcinoma without history of systemic sarcoidosis. It is important to recognize that systemic sarcoidosis and sarcoidal reaction without evidence of systemic disease can occur after development of malignant lymphoma, therefore, sarcoidal reaction must be included in the differential diagnostic consideration of recurrent malignant lymphoma. PMID:25120829

  5. Gene expression profiles in granuloma tissue reveal novel diagnostic markers in sarcoidosis.

    PubMed

    Christophi, George P; Caza, Tiffany; Curtiss, Christopher; Gumber, Divya; Massa, Paul T; Landas, Steve K

    2014-06-01

    Sarcoidosis is an immune-mediated multisystem disease characterized by the formation of non-caseating granulomas. The pathogenesis of sarcoidosis is unclear, with proposed infectious or environmental antigens triggering an aberrant immune response in susceptible hosts. Multiple pro-inflammatory signaling pathways have been implicated in mediating macrophage activation and granuloma formation in sarcoidosis, including IFN-γ/STAT-1, IL-6/STAT-3, and NF-κB. It is difficult to distinguish sarcoidosis from other granulomatous diseases or assess disease severity and treatment response with histopathology alone. Therefore, development of improved diagnostic tools is imperative. Herein, we describe an efficient and reliable technique to classify granulomatous disease through selected gene expression and identify novel genes and cytokine pathways contributing to the pathogenesis of sarcoidosis. We quantified the expression of twenty selected mRNAs extracted from formalin-fixed paraffin embedded (FFPE) tissue (n = 38) of normal lung, suture granulomas, sarcoid granulomas, and fungal granulomas. Utilizing quantitative real-time RT-PCR we analyzed the expression of several genes, including IL-6, COX-2, MCP-1, IFN-γ, T-bet, IRF-1, Nox2, IL-33, and eotaxin-1 and revealed differential regulation between suture, sarcoidosis, and fungal granulomas. This is the first study demonstrating that quantification of target gene expression in FFPE tissue biopsies is a potentially effective diagnostic and research tool in sarcoidosis.

  6. The influence of T cell receptor and cytokine genes on sarcoidosis susceptibility in African Americans.

    PubMed

    Rybicki, B A; Maliarik, M J; Malvitz, E; Sheffer, R G; Major, M; Popovich, J; Iannuzzi, M C

    1999-09-01

    The pathogenesis of sarcoidosis, a multisystem granulomatous disorder, is mediated through immunoregulatory pathways. While sarcoidosis clusters in families, inherited risk factors remain undefined. In search of possible sarcoidosis susceptibility genes, we examined anonymous polymorphic genetic markers tightly linked to six different candidate gene regions on chromosomes 2q13, 5q31, 6p23-25, 7p14-15, 14q11 and 22q11. These candidate regions contain T cell receptor, interleukin (IL) and interferon regulatory factor (IRF) genes. Our study population consisted of 105 African-American sarcoidosis cases and 95 unrelated healthy controls. The allelic frequency distribution of two out of the six markers, IL-1 alpha marker (p = 0.010) on 2q13 and the F13A marker (p = 0.0006) on 6p23-25, was statistically significantly different in cases compared with controls. The two alleles most strongly associated with sarcoidosis were IL-1 alpha*137 (Odds Ratio (OR) = 2.60; 95% confidence interval (CI) = 1.36-4.98) and F13A*188 (OR = 2.42; 95% CI = 1.37-4.30). Individuals that had both of these alleles were at a six-fold increased risk for sarcoidosis (OR = 6.19; 95% CI = 2.54-15.10). Restricting the analysis to cases with at least one first or second-degree relative affected with sarcoidosis increased the OR to 15.38. IL-1 levels are elevated in sarcoidosis and the F13A marker is tightly linked to a gene that codes for a newly identified interferon regulatory factor protein (IRF-4), which is thought to play a role in T cell effector functions. Our results suggest genetic susceptibility to sarcoidosis may be conferred by more than one immune-related gene that act synergistically on disease risk.

  7. Role of a functional polymorphism in the F2R gene promoter in sarcoidosis.

    PubMed

    Platé, Manuela; Lawson, Phillippa J; Hill, Michael R; Marshall, Richard P; Booth, Helen L; Kumari, Meena; Laurent, Geoffrey J; Hurst, John R; Chambers, Rachel C

    2015-11-01

    Sarcoidosis is a multisystem granulomatous disease of unknown aetiology characterized by increased inflammation, and results from gene-environment interactions. Proteinase-activated receptor-1 mediates the interplay between coagulation and inflammation. The rs2227744G > A promoter single nucleotide polymorphism has been linked to inflammation, cardiovascular disease and chronic obstructive pulmonary disease exacerbations. Using a case-control study (184 cases with sarcoidosis and 368 controls), we show that the rs2227744A allele significantly associates with protection from sarcoidosis (P = 0.003, OR = 0.68 (0.52-0.88)).

  8. Role of a functional polymorphism in the F2R gene promoter in sarcoidosis

    PubMed Central

    Platé, Manuela; Lawson, Phillippa J.; Hill, Michael R.; Marshall, Richard P.; Booth, Helen L.; Kumari, Meena; Laurent, Geoffrey J.; Hurst, John R.

    2015-01-01

    Sarcoidosis is a multisystem granulomatous disease of unknown aetiology characterized by increased inflammation, and results from gene–environment interactions. Proteinase‐activated receptor‐1 mediates the interplay between coagulation and inflammation. The rs2227744G > A promoter single nucleotide polymorphism has been linked to inflammation, cardiovascular disease and chronic obstructive pulmonary disease exacerbations. Using a case‐control study (184 cases with sarcoidosis and 368 controls), we show that the rs2227744A allele significantly associates with protection from sarcoidosis (P = 0.003, OR = 0.68 (0.52–0.88)). PMID:26278396

  9. MRI and FDG PET/CT imaging manifestations of cardiac sarcoidosis.

    PubMed

    Lu, Yang; Sweiss, Nadera J

    2015-12-01

    A 52-year-old man had biopsy-proven sarcoidosis of mediastinal lymph nodes. Cardiac sarcoidosis was confirmed on cardiac MRI with typical imaging features as delayed gadolinium enhancement. Follow-up FDG PET/CT with a 3-day pretest diet modification showed suppression of overall myocardial uptake of FDG but with multifocal abnormal FDG uptake in the myocardium regions corresponding to the previous MRI findings. Additional noncardiac active sarcoidosis involving multiple organ and lymph nodes were also visualized on FDG PET/CT. PMID:26544904

  10. Sarcoidosis on tattoos: a review of the literature from 1939 to 2011.

    PubMed

    Kluger, N

    2013-08-01

    Sarcoidosis is an autoimmune disease of unknown etiology characterized by the presence of non-caseating epithelioid cell granulomas in multiple organs. Cutaneous sarcoidosis occurs in approximately 25% of the cases. Sarcoid reactions on old scars, traumatized skin sites and around embedded foreign material have long been observed. For the past 70 years, sarcoidal granulomas on tattoos and permanent make-up have also been documented. Granulomatous and sarcoidal tattoo reactions may be the first and sometimes only cutaneous manifestation of systemic sarcoidosis. This review summarizes the currently available data on this topic and discusses the issues related to the diagnosis, management and physiopathogeny of sarcoidal reactions on tattoos.

  11. Multiple atypical bone involvement in sarcoidosis.

    PubMed

    Mañá, J; Segarra, M I; Casas, R; Mairal, L; Fernández-Nogués, F

    1993-02-01

    We describe a patient with right paratracheal and left hilar lymph nodes detected by chance on chest radiography that followed a spontaneous remission. However, a few months later she complained of multiple scalp nodules. Skull roentgenogram and computerized tomogram scan showed multiple osteolytic lesions with increased uptake in bone and gallium scans. Lytic lesions were also detected in her right mandibular bone and right clavicle. Noncaseating granulomas were demonstrated in skull and cervical lymph node biopsies. PMID:8474084

  12. Multiple bilateral pulmonary nodules masquerading as pulmonary metastasis; a case of nodular sarcoidosis

    PubMed Central

    Jafari, Mostafa; Farrokh, Donya; Mohammadpanah, Najmeh

    2016-01-01

    Sarcoidosis is a multi-system inflammatory disorder of unknown etiology that is manifested by the presence of non-caseating granulomas. Multiple pulmonary nodules are rare presentations of sarcoidosis. We report a case of nodular sarcoidosis in a young male of Middle-East origin who had initially presented with bilateral painful ankle edema. His chest X-ray showed multiple bilateral pulmonary nodules. A high resolution computed tomography scan of the chest demonstrated multiple pulmonary nodular lesions and also mediastinal and hilar lymphadenopathy. Subsequent biopsies revealed non-necrotizing granuloma with multi-nucleated giant cells indicative of sarcoidosis. An appropriate work-up was done to confirm the true nature of the nodules and facilitate treatment. PMID:27757192

  13. A rare case of eyelid sarcoidosis presenting as an orbital mass.

    PubMed

    Gaspar, Balan Louis; Gupta, Kirti; Singh, Usha

    2016-03-01

    Intraorbital sarcoidosis presenting externally as a solitary eyelid mass has been described in the literature as isolated case reports. We describe a rare case of asymptomatic sarcoidosis with orbital mass as the presenting feature in a young woman. The lesion was excised with the clinical possibility of a thrombosed varix. On histology, the lesion was characterized by numerous nonnecrotizing epithelioid cell granulomas with several multinucleated giant cells containing abundant asteroid bodies and oxalate crystals. No tubercular bacilli were detected. A diagnosis of sarcoidosis was rendered and on further clinical work-up, she was detected to have hilar lymphadenopathy. Sarcoidosis should be considered in the differential diagnosis of orbital mass as it could be the initial manifestation of the disease process. PMID:27146941

  14. A rare case of eyelid sarcoidosis presenting as an orbital mass

    PubMed Central

    Gaspar, Balan Louis; Gupta, Kirti; Singh, Usha

    2016-01-01

    Intraorbital sarcoidosis presenting externally as a solitary eyelid mass has been described in the literature as isolated case reports. We describe a rare case of asymptomatic sarcoidosis with orbital mass as the presenting feature in a young woman. The lesion was excised with the clinical possibility of a thrombosed varix. On histology, the lesion was characterized by numerous nonnecrotizing epithelioid cell granulomas with several multinucleated giant cells containing abundant asteroid bodies and oxalate crystals. No tubercular bacilli were detected. A diagnosis of sarcoidosis was rendered and on further clinical work-up, she was detected to have hilar lymphadenopathy. Sarcoidosis should be considered in the differential diagnosis of orbital mass as it could be the initial manifestation of the disease process. PMID:27146941

  15. Oral Lesion as the first Clinical Presentation in Sarcoidosis: A Case Report.

    PubMed

    Al-Azri, Abdul Rahman S; Logan, Richard M; Goss, Alastair N

    2012-05-01

    Sarcoidosis is a common multi-system disease characterized histolopathologically by the formation of non-caseating granulomas in the affected tissues. The oral involvement of sarcoidosis is relatively rare with less than 70 reported cases in literature while an oral lesion as the initial presenting sign is even less common. Oral lesions of sarcoidosis may mimic the lesions of other serious systemic diseases including Crohn's disease and tuberculosis as well as lesions localized to the orofacial region such as orofacial granulomatosis. This report presents a case of non-progressive sarcoidosis where the initial presenting symptom was a lesion in the buccal vestibule attached to the gingivae. A brief review of the pathology and clinical features is also presented.

  16. Cardiac sarcoidosis demonstrated by Tl-201 and Ga-67 SPECT imaging

    SciTech Connect

    Taki, J.; Nakajima, K.; Bunko, H.; Ohguchi, M.; Tonami, N.; Hisada, K. )

    1990-09-01

    Ga-67 and Tl-201 SPECT was performed to evaluate cardiac sarcoidosis in a 15-year-old boy. Tl-201 SPECT imaging showed decreased uptake in the inferior to lateral wall and Ga-67 accumulation in the area of decreased Tl-201 uptake. These findings suggested cardiac sarcoidosis, and cardiac biopsy confirmed this diagnosis. After corticosteroid therapy, myocardial uptake of Ga-67 disappeared and myocardial TI-201 uptake became more homogeneous.

  17. Development of a sarcoidosis murine lung granuloma model using Mycobacterium superoxide dismutase A peptide.

    PubMed

    Swaisgood, Carmen M; Oswald-Richter, Kyra; Moeller, Stephen D; Klemenc, Jennifer M; Ruple, Lisa M; Farver, Carol F; Drake, John M; Culver, Daniel A; Drake, Wonder P

    2011-02-01

    Sarcoidosis is characterized by noncaseating granulomas containing CD4(+) T cells with a Th1 immunophenotype. Although the causative antigens remain unknown, independent studies noted molecular and immunologic evidence of mycobacterial virulence factors in sarcoidosis specimens. A major limiting factor in discovering new insights into the pathogenesis of sarcoidosis is the lack of an animal model. Using a distinct superoxide dismutase A peptide (sodA) associated with sarcoidosis granulomas, we developed a pulmonary model of sarcoidosis granulomatous inflammation. Mice were sensitized by a subcutaneous injection of sodA, incorporated in incomplete Freund's adjuvant (IFA). Control subjects consisted of mice with no sensitization (ConNS), sensitized with IFA only (ConIFA), or with Schistosoma mansoni eggs. Fourteen days later, sensitized mice were challenged by tail-vein injection of naked beads, covalently coupled to sodA peptides or to schistosome egg antigens (SEA). Histologic analysis revealed hilar lymphadenopathy and noncaseating granulomas in the lungs of sodA-treated or SEA-treated mice. Flow cytometry of bronchoalveolar lavage (BAL) demonstrated CD4(+) T-cell responses against sodA peptide in the sodA-sensitized mice only. Cytometric bead analysis revealed significant differences in IL-2 and IFN-γ secretion in the BAL fluid of sodA-treated mice, compared with mice that received SEA or naked beads (P = 0.008, Wilcoxon rank sum test). ConNS and ConIFA mice demonstrated no significant formation of granuloma, and no Th1 immunophenotype. The use of microbial peptides distinct for sarcoidosis reveals a histologic and immunologic profile in the murine model that correlates well with those profiles noted in human sarcoidosis, providing the framework to investigate the molecular basis for the progression or resolution of sarcoidosis. PMID:20348207

  18. Combined pulmonary involvement in hereditary lysozyme amyloidosis with associated pulmonary sarcoidosis: a case report.

    PubMed

    McCarthy, Cormac; Deegan, Alexander P; Garvey, John F; McDonnell, Timothy J

    2013-01-01

    Sarcoidosis is a multisystem inflammatory disorder of unknown cause which can affect any organ system. Autosomal dominant lysozyme amyloidosis is a very rare form of hereditary amyloidosis. The Arg64 variant is extraordinarily rare with each family showing a particular pattern of organ involvement, however while Sicca syndrome, gastrointestinal involvement and renal failure are common, lymph node involvement is very rare. In this case report we describe the first reported case of sarcoidosis in association with hereditary lysozyme amyloidosis. PMID:24351625

  19. Mediastinal sarcoidosis mimicking lymph malignancy recurrence after anti-neoplastic therapy.

    PubMed

    El Hammoumi, Massine; El Marjany, Mohamed; Moussaoui, Driss; Doudouh, Aberahim; Mansouri, Hamid; Kabiri, El Hassane

    2015-07-01

    The aim of our work is to promote the awareness about the development of sarcoidosis after antineoplastic therapy in order to avoid diagnostic errors with FDG-PET/CT findings. We report the observation of three women with breast, cervix and stomach treated cancers who developed a sarcoidosis after the end of anti-neoplastic therapy. The utility of FDG-PET/CT is in pinpointing the organs candidates for diagnostic biopsy and not distinguishing between the malignancy and granulomatous or inflammatory diseases.

  20. Orofacial presentations of sarcoidosis--a case series and review of the literature.

    PubMed

    Poate, T W J; Sharma, R; Moutasim, K A; Escudier, M P; Warnakulasuriya, S

    2008-10-25

    Sarcoidosis is a multi-system disease of unknown aetiology characterised by the presence of non-caseating granulomas, the lungs and lymph nodes being the most affected sites. Orofacial manifestations of the condition are increasingly recognised, with several recent case reports where the initial presentation of the disease is in the region. Here, we report six cases of orofacial sarcoidosis which help to illustrate the wide spectrum of the condition.

  1. Meta-analytical association between angiotensin-converting enzyme gene polymorphisms and sarcoidosis risk.

    PubMed

    Zhu, R; Bi, L Q; Kong, H; Tilley, S L; Wang, H; Xie, W P

    2015-01-01

    Previous reports identified an association between sarcoidosis and an insertion/deletion (I/D) polymorphism in angiotensin-converting enzyme. Our meta-analysis of articles published between March 1996 and June 2013 identified studies in the PubMed, EMBASE, and the China National Knowledge Infrastructure databases. We examined whether angiotensin-converting enzyme polymorphisms influence sarcoidosis susceptibility. The strength of the association between I/D polymorphisms and sarcoidosis risk was measured based on the odds ratio and 95% confidence interval. Analysis was based on recessive and dominant models. Ethnic subgroup analysis from 18 articles (1882 cases and 3066 controls) showed that DD homozygote carriers were at a slightly increased risk of sarcoidosis compared with II homozygotes and DI heterozygotes (P = 0.03). Comparison of DD plus DI vs II revealed no significant association with sarcoidosis in group and ethnic subgroup analysis. We found that the I/D polymorphism in the angiotensin-converting enzyme gene was not associated with a major risk of sarcoidosis. PMID:25966127

  2. Unsuspected exposure to beryllium: potential implications for sarcoidosis diagnoses.

    PubMed

    Laczniak, Andrew N; Gross, Nathan A; Fuortes, Laurence J; Field, R William

    2014-07-21

    Exposure to Beryllium (Be) can cause sensitization (BeS) and chronic beryllium disease (CBD) in some individuals.  Even relatively low exposures may be sufficient to generate an asymptomatic, or in some cases a symptomatic, immune response. Since the clinical presentation of CBD is similar to that of sarcoidosis, it is helpful to have information on exposure to beryllium in order to reduce misdiagnosis. The purpose of this pilot study is to explore the occurrence of Be surface deposits at worksites with little or no previous reported use of commercially available Be products.  The workplaces chosen for this study represent a convenience sample of businesses in eastern Iowa. One hundred thirty-six surface dust samples were collected from 27 businesses for analysis of Be. The results were then divided into categories by the amount of detected Be according to U.S. Department of Energy guidelines as described in 10 CFR 850.30 and 10 CFR 850.31. Overall, at least one of the samples at 78% of the work sites tested contained deposited Be above the analytical limit of quantitation (0.035 µg beryllium per sample).  Beryllium was detected in 46% of the samples collected. Twelve percent of the samples exceeded 0.2 µg/100 cm² and 4% of the samples exceeded a Be concentration of 3 µg/100 cm². The findings from this study suggest that there may be a wider range and greater number of work environments that have the potential for Be exposure than has been documented previously.  These findings could have implications for the accurate diagnosis of sarcoidosis.

  3. The BTNL2 Gene and Sarcoidosis Susceptibility in African Americans and Whites

    PubMed Central

    Rybicki, Benjamin A.; Walewski, José L.; Maliarik, Mary J.; Kian, Hamed; Iannuzzi, Michael C.

    2005-01-01

    The BTNL2 gene is a member of the B7 receptor family that probably functions as a T-cell costimulatory molecule. It resides in the class II major histocompatibility complex (MHC) region of chromosome 6p and has recently been associated with sarcoidosis susceptibility in a white German population. We sought to replicate the BTNL2 association in an African American family-based study population (n=219 nuclear families) and two case-control populations—one African American (n=295 pairs) and one white (n=366 pairs). Ten SNPs were detected within a 490-bp region spanning exon/intron 5 of BTNL2. Haplotype variation within this region was significantly associated with sarcoidosis in all three study populations but more so in whites (P=.0006) than in the African American case-control (P=.02) or family-based (P=.03) samples. The previously reported BTNL2 SNP with the strongest sarcoidosis association, rs2076530, was also the SNP with the strongest association in our white population (P<.0001). The A allele of rs2076530 results in a premature exon-splice site and increases risk for sarcoidosis (odds ratio=2.03; 95% confidence interval 1.32–3.12). Although rs2076530 was not associated with sarcoidosis in either African American sample, a three-locus haplotype that included rs2076530 was associated with sarcoidosis across all three study samples. Multivariable logistic regression analyses showed that BTNL2 effects are independent of human leukocyte antigen class II genes in whites but may interact antagonistically in African Americans. Our results underscore the complexity of genetic risk for sarcoidosis emanating from the MHC region. PMID:16080124

  4. European survey on the usefulness of /sup 67/Ga lung scans in assessing sarcoidosis. Experience in 14 research centers in seven different countries

    SciTech Connect

    Rizzato, G.; Blasi, A.

    1986-01-01

    Fifty-eight contributors from 12 European and 2 American sarcoidosis centers have collaborated in a survey to define many questions concerning the use of /sup 67/Ga lung scan in sarcoidosis. The new quantitative scoring methods based on digital evaluation seem better in detecting lung activity. In 20.1% of untreated patients, the /sup 67/Ga lung scan appeared to be the only noninvasive method with which clinical activity could be detected. /sup 67/Ga scans may be useful in guiding lung biopsy and in choosing pulmonary segments for BAL. Of 382 patients studied during follow-up (154 patients with three to nine scans at intervals of 2 to 12 months), the /sup 67/Ga scan was far more sensitive than chest radiography, both in detecting improvement and in foreseeing relapses. Steroid therapy appears to suppress ACE levels more than /sup 67/Ga uptake, and /sup 67/Ga uptake more than the alveolitis detectable by BAL. Gallium-67 uptake rebounds to positivity occur in about 40% of patients after steroid discontinuation and in about 20% of patients after steroid reduction to daily doses of 10 mg or less of prednisone. The /sup 67/Ga dose of 1.5 mCi seems appropriate for clinical purposes and is recommended for the subjective scoring method in order to reduce the cost and the radiation burden.

  5. Prediction of therapeutic response in steroid-treated pulmonary sarcoidosis. Evaluation of clinical parameters, bronchoalveolar lavage, gallium-67 lung scanning, and serum angiotensin-converting enzyme levels

    SciTech Connect

    Hollinger, W.M.; Staton, G.W. Jr.; Fajman, W.A.; Gilman, M.J.; Pine, J.R.; Check, I.J.

    1985-07-01

    To find a pretreatment predictor of steroid responsiveness in pulmonary sarcoidosis the authors studied 21 patients before and after steroid treatment by clinical evaluation, pulmonary function tests, bronchoalveolar lavage (BAL), gallium-67 lung scan, and serum angiotensin-converting enzyme (SACE) level. Although clinical score, forced vital capacity (FVC), BAL percent lymphocytes (% lymphs), quantitated gallium-67 lung uptake, and SACE levels all improved with therapy, only the pretreatment BAL % lymphs correlated with the improvement in FVC (r = 0.47, p less than 0.05). Pretreatment BAL % lymphs of greater than or equal to 35% predicted improvement in FVC of 10/11 patients, whereas among 10 patients with BAL % lymphs less than 35%, 5 patients improved and 5 deteriorated. Clinical score, pulmonary function parameters, quantitated gallium-67 lung uptake, and SACE level used alone, in combination with BAL % lymphs or in combination with each other, did not improve this predictive value. The authors conclude that steroid therapy improves a number of clinical and laboratory parameters in sarcoidosis, but only the pretreatment BAL % lymphs are useful in predicting therapeutic responsiveness.

  6. [Cardiac sarcoidosis: seven keypoints to remind in order to avoid misdiagnosis].

    PubMed

    Porretta, Alessandra Pia; Van der Gucht, Axel; Bisch, Laurence; Mitsakis, Periklis; Pomoni, Anastasia; Allenbach, Gilles; Lalonde, Marie Nicod; Schaefer, Niklaus; Buss, Guillaume; Prior, John O; Pruvot, Étienne

    2016-05-25

    Early diagnosis of cardiac sarcoidosis remains difficult in the absence of specific symptoms. The evolution and prognosis of the disease are strongly correlated to an early and appropriate treatment. The multi-modality assessment based on cardiac MRI and positron emission tomography associated with computed tomography (PET/CT) has significantly improved the detection of cardiac sarcoidosis over the last two decades. These approaches appear as useful and suitable imaging strategy for the early diagnosis, the assessment of the disease extent as well as the management and therapeutic follow-up. This article is a didactic review on cardiac sarcoidosis, with a special focus on recent diagnostic and therapeutic modalities, prognosis and interest of imaging techniques.

  7. A rare manifestation of a multisystemic disease: a case of vocal cord palsy secondary to sarcoidosis.

    PubMed

    Mastan, Saleem; Advani, Rajeev; Stobbs, Nicola; Kumar, Nirmal

    2015-04-26

    We describe a rare case of recurrent laryngeal nerve neuritis secondary to sarcoidosis. A 40-year-old woman presented with persistent dysphonia. This was her first episode of dysphonia with no reports of laryngeal trauma. Fibre-optic laryngoscopy revealed a normal nasal passage, nasopharynx and pharynx. The supraglottic structures were all unremarkable; however, inspection of the true vocal cords revealed a left vocal cord palsy that was identified as being in a paramedian position. Radiological investigation showed mediastinal adenopathy that measured up to 20 mm in the short axis diameter. Histological examination showed granulomatous lymphadenitis of the lymph node with a central area of sclerosis surrounded by discrete, non-caseating granuloma. Stains for acid-fast bacilli were negative. The morphological features were suggestive of sarcoidosis. The lymphadenopathy distribution and size did not suggest left recurrent laryngeal nerve compression, giving a subsequent diagnosis of recurrent laryngeal nerve neuritis secondary to sarcoidosis.

  8. Tl-201 uptake in bone and soft tissue involvement of sarcoidosis.

    PubMed

    Degirmenci, B; Mavi, A; Ozkal, S; Itil, O; Havitcioglu, H; Ozaksoy, D; Yilmaz, M; Kaya, G C

    2001-06-01

    The authors describe a 38-year-old man who was referred to the nuclear medicine department because of pain and swelling of his fingers in both hands. Tc-99m MDP and Tl-201 scans were performed to evaluate the lesions. A Tc-99m MDP bone scan showed hyperemia and increased uptake in the lesions. A Tl-201 scan showed marked uptake in both early and delayed images in the lesions of his fingers. Bone biopsy and histologic examination confirmed sarcoidosis. This case indicates that Tl-201 uptake can be seen in bone lesions resulting from sarcoidosis. PMID:11353294

  9. An unusual cause of widespread lytic bone lesions caused by sarcoidosis.

    PubMed

    Mehrotra, Pallavi; Muller, Michelle; Higgins, Bernard; Zammit-Maempel, Ivan

    2011-01-01

    We present a case of a 59 year old asymptomatic lady who was found to have incidental findings of pulmonary, osseous and hepatic involvement with sarcoidosis. The osseous lesions were lytic and involved unusual sites such as the vertebrae and skull base. The initial clinical concern had been of multiple myeloma or disseminated metastases. Biopsy of material obtained following mediastinoscopy revealed chronic, non-necrotising granulomatous lymphadenopathy indicative of sarcoidosis. Cases such as this could greatly benefit from multidisciplinary team discussion particularly when the clinical picture is not typical of malignancy. PMID:22470811

  10. Oxygen desaturation during a 6-minute walk test as a predictor of maximal exercise-induced gas exchange abnormalities in sarcoidosis

    PubMed Central

    Chenivesse, Cecile; Boulanger, Sarah; Langlois, Carole; Wemeau-Stervinou, Lidwine; Perez, Thierry

    2016-01-01

    Background Common tests for evaluating gas exchange impairment have different strengths and weaknesses. Alveolar-to-arterial oxygen pressure difference (AaDO2) at peak exercise is a sensitive indicator but it cannot be measured repeatedly. Diffusing capacity of the lung for carbon monoxide (DLco) is measured at rest and may be too insensitive to predict the effects of exercise on gas exchange impairment. Oxygen desaturation during a 6-minute walk test (∆SpO2-6MWT) can be measured repeatedly, but its value in sarcoidosis is unknown. Here, we evaluated the ability of ∆SpO2-6MWT and DLco to predict gas exchange impairment during exercise in sarcoidosis. Methods This retrospective study of 130 subjects with sarcoidosis investigated the relationship between DLco, ∆SpO2-6MWT, and peak AaDO2 using correlation tests, inter-test reliability analyses, and predictive values. For the analyses of inter-test reliability and predictive values, DLco, peak AaDO2, and ∆SpO2-6MWT were considered as binary variables (normal/abnormal) according to previously defined thresholds. Results Correlation coefficients between DLco, ∆SpO2-6MWT, and peak AaDO2 were intermediate (0.53–0.67, P<0.0003) and Kappa coefficients were low (0.21–0.42, P=0.0003–0.02). DLco predicted (I) increased peak AaDO2 with a positive predictive value (PPV) of 66% and a negative predictive value (NPV) of 78% and (II) increased ∆SpO2-6MWT with a PPV at 36% and an NPV at 88%. Normal DLco was a good predictor of the absence of severe desaturation during the 6MWT (94% NPV) and at peak exercise during cardiopulmonary exercise test (CPET) (100% NPV). ∆SpO2-6MWT predicted peak AaDO2 increase with a PPV of 74% and an NPV of 60%. Conclusions In a large population of sarcoidosis patients, neither ∆SpO2-6MWT nor DLco was a good predictor of increased peak AaDO2. In contrast, normal DLco was a good predictor of the absence of severe desaturation during the 6MWT and at peak exercise during CPET.

  11. Oxygen desaturation during a 6-minute walk test as a predictor of maximal exercise-induced gas exchange abnormalities in sarcoidosis

    PubMed Central

    Chenivesse, Cecile; Boulanger, Sarah; Langlois, Carole; Wemeau-Stervinou, Lidwine; Perez, Thierry

    2016-01-01

    Background Common tests for evaluating gas exchange impairment have different strengths and weaknesses. Alveolar-to-arterial oxygen pressure difference (AaDO2) at peak exercise is a sensitive indicator but it cannot be measured repeatedly. Diffusing capacity of the lung for carbon monoxide (DLco) is measured at rest and may be too insensitive to predict the effects of exercise on gas exchange impairment. Oxygen desaturation during a 6-minute walk test (∆SpO2-6MWT) can be measured repeatedly, but its value in sarcoidosis is unknown. Here, we evaluated the ability of ∆SpO2-6MWT and DLco to predict gas exchange impairment during exercise in sarcoidosis. Methods This retrospective study of 130 subjects with sarcoidosis investigated the relationship between DLco, ∆SpO2-6MWT, and peak AaDO2 using correlation tests, inter-test reliability analyses, and predictive values. For the analyses of inter-test reliability and predictive values, DLco, peak AaDO2, and ∆SpO2-6MWT were considered as binary variables (normal/abnormal) according to previously defined thresholds. Results Correlation coefficients between DLco, ∆SpO2-6MWT, and peak AaDO2 were intermediate (0.53–0.67, P<0.0003) and Kappa coefficients were low (0.21–0.42, P=0.0003–0.02). DLco predicted (I) increased peak AaDO2 with a positive predictive value (PPV) of 66% and a negative predictive value (NPV) of 78% and (II) increased ∆SpO2-6MWT with a PPV at 36% and an NPV at 88%. Normal DLco was a good predictor of the absence of severe desaturation during the 6MWT (94% NPV) and at peak exercise during cardiopulmonary exercise test (CPET) (100% NPV). ∆SpO2-6MWT predicted peak AaDO2 increase with a PPV of 74% and an NPV of 60%. Conclusions In a large population of sarcoidosis patients, neither ∆SpO2-6MWT nor DLco was a good predictor of increased peak AaDO2. In contrast, normal DLco was a good predictor of the absence of severe desaturation during the 6MWT and at peak exercise during CPET. PMID

  12. Superoxide dismutase A antigens derived from molecular analysis of sarcoidosis granulomas elicit systemic Th-1 immune responses

    PubMed Central

    Allen, Shannon S; Evans, Whitney; Carlisle, James; Hajizadeh, Rana; Nadaf, Michele; Shepherd, Bryan E; Pride, David T; Johnson, Joyce E; Drake, Wonder P

    2008-01-01

    Background Sarcoidosis is an idiopathic granulomatous disease with pathologic and immunologic features similar to tuberculosis. Routine histologic staining and culture fail to identify infectious agents. An alternative means for investigating a role of infectious agents in human pathogenesis involves molecular analysis of pathologic tissues for microbial nucleic acids, as well as recognition of microbial antigens by the host immune system. Molecular analysis for superoxide dismutase A (sodA) allows speciation of mycobacteria. SodA is an abundantly secreted virulence factor that generates cellular immune responses in infected hosts. The purpose of this study is to investigate if target antigens of the sarcoidosis immune response can be identified by molecular analysis of sarcoidosis granulomas. Methods We detected sodA amplicons in 12 of 17 sarcoidosis specimens, compared to 2 of 16 controls (p = 0.001, two-tailed Fisher's exact test), and 3 of 3 tuberculosis specimens (p = 0.54). Analysis of the amplicons revealed sequences identical to M. tuberculosis (MTB) complex, as well as sequences which were genetically divergent. Using peripheral blood mononuclear cells (PBMC) from 12 of the 17 sarcoidosis subjects, we performed enzyme-linked immunospot assay (ELISPOT) to assess for immune recognition of MTB sodA peptides, along with PBMC from 26 PPD- healthy volunteers, and 11 latent tuberculosis subjects. Results Six of 12 sarcoidosis subjects recognized the sodA peptides, compared to one of 26 PPD- controls (p = 0.002), and 6/11 PPD+ subjects (p = .68). Overall, 10 of the 12 sarcoidosis subjects from whom we obtained PBMC and archival tissue possessed molecular or immunologic evidence for sodA. Conclusion Dual molecular and immunologic analysis increases the ability to find infectious antigens. The detection of Th-1 immune responses to sodA peptides derived from molecular analysis of sarcoidosis granulomas reveals that these are among the target antigens contributing to

  13. Sudden cardiac death due to coronary artery dissection as a complication of cardiac sarcoidosis.

    PubMed

    Kanaroglou, Savas; Nair, Vidhya; Fernandes, John R

    2015-01-01

    Spontaneous coronary artery dissection (SCAD) is a rare cause of acute coronary syndrome (ACS), cardiac tamponade and sudden cardiac death that typically affects young women in the postpartum period. Rarely, it can be caused by systemic inflammatory conditions such as sarcoidosis. Sarcoidosis is an inflammatory disease most often affecting the lung and lymph nodes that can sometimes affect the heart. The authors report a case of sudden cardiac death caused by SCAD in the context of undiagnosed and subclinical cardiac sarcoidosis. The decedent was a 47-year-old male with a relatively innocuous past medical history. He was found dead in bed. At autopsy, there was a lethal hemopericardium resulting in cardiac tamponade. Gross examination of the heart revealed dissection of the posterior descending coronary branch of the right coronary artery. Histologically, the coronary artery showed acute and organizing dissection with evidence of vasculitis. A chronic inflammatory infiltrate consisting of lymphocytes, histiocytes, eosinophils and giant cells was seen. Sections of the myocardium showed myocarditis with a nonnecrotizing granuloma. The death was attributed to cardiac tamponade secondary to SCAD in the context of systemic sarcoidosis. The presented case demonstrates two concurrent rare pathologies and highlights the importance of considering SCAD in cases of sudden cardiac death at autopsy.

  14. [Bioptic investigations of intrathoracic sarcoidosis in occupationally injured persons (author's transl)].

    PubMed

    Burilkov, T; Kolev, K

    1977-07-01

    Diagnostics of intrathoracic sarcoidosis in people with an occupational injury or an annoyance of respiratory organs are frequently connected with additional difficulties. The first roentgenological manifestations of a silicosis caused during the period of dry drilling especially in ore metal mining, are frequently bilaterally increased shadows of the hilum without any worth mentioning findings in the parenchyma. The radiograph of diffuse restiform and speckled shadows in people exposed to dust without a sufficient load or in younger age always demands a search for sarcoidosis. Granulomatoses of the lungs caused by organic dusts, berillium and other things may develop difficulties not only in the interpretation of clinical data but also in the bioptic findings for instance after an endoscopic antalgic biopsy of the bronchomucosa. The authors summarize their experiences with the application of bioptic methods (skin biopsy, according to the Kveim-test, scalenobiopsy, explorative thoracotomy) in workers suspected to suffer from a sarcoidosis, who--as to their profession--had been influenced by noxious substances in respect to their lungs. Two typical cases are briefly discussed. The signification of the synopsis of clinico roentgenological, industriohygienical and bioptical data is stressed especially in case of a suspicion on the combination of pneumoconiosis and sarcoidosis.

  15. Cardiac sarcoidosis mimicking hypertrophic cardiomyopathy: clinical utility of radionuclide imaging for differential diagnosis.

    PubMed

    Yazaki, Y; Isobe, M; Hayasaka, M; Tanaka, M; Fujii, T; Sekiguchi, M

    1998-06-01

    A 62-year-old woman with skin sarcoidosis was admitted to our hospital to ascertain whether she had cardiac involvement. Although she displayed no cardiac signs or symptoms, the electrocardiogram showed first-degree atrioventricular block, right bundle branch block with left anterior fascicular block, and giant negative T waves in the V3 lead. Echocardiography revealed marked hypertrophy localized in the basal portion of the interventricular septum (IVS) without systolic dysfunction, mimicking hypertrophic cardiomyopathy (HCM). Exercise thallium-201 myocardial imaging revealed redistribution in the anteroseptal region. Both gallium-67 (67Ga) and technetium-99m pyrophosphate (99mTc-PYP) scintigraphy revealed abnormal uptake in the myocardium. These findings disappeared after 2 months of steroid treatment. Reports of cardiac sarcoidosis mimicking HCM are rare. However, hypertrophy in the basal portion of the IVS is an important sign of early cardiac involvement in sarcoidosis. 67Ga and 99mTc-PYP scintigraphy were useful and necessary to differentiate this type of cardiac sarcoidosis from HCM.

  16. Topical Pimecrolimus as a New Optional Treatment in Cutaneous Sarcoidosis of Lichenoid Type

    PubMed Central

    Tammaro, Antonella; Abruzzese, Claudia; Narcisi, Alessandra; Cortesi, Giorgia; Parisella, Francesca Romana; Di Russo, Pier Paolo; De Marco, Gabriella; Persechino, Severino

    2014-01-01

    We report the case of cutaneous sarcoidosis of lichenoid type successfully treated with pimecrolimus. For the first time in the literature, we propose the use of this topical calcineurin inhibitor for the treatment of the cases refractory to common therapy regimens. PMID:24826355

  17. Ga-67 positivity in sarcoidosis of the skin with coincident thyroid uptake of uncertain etiology

    SciTech Connect

    Moreno, A.J.; Brown, J.M.; Salinas, J.A.; Feaster, B.L.; Brown, T.J.

    1984-03-01

    Gallium-67 citrate scintigraphy of a 26-year-old woman with systemic sarcoidosis demonstrated abnormal radiotracer uptake within multiple biopsy-proven sarcoidal cutaneous lesions and within both lobes of the thyroid gland. The etiology of the thyroidal uptake of the Ga-67 was uncertain but it may represent sarcoidal involvement of the gland.

  18. Sarcoidosis and multiple sclerosis: systemic toxicity associated with the use of interferon-beta therapy.

    PubMed

    Carbonelli, C; Montepietra, S; Caruso, A; Cavazza, A; Feo, C; Menzella, F; Motti, L; Zucchi, L

    2012-03-01

    Sarcoidosis is a multi-systemic inflammatory disease of unknown origin characterized by the presence of noncaseating epitheloid cell granulomas in multiple organs. Diagnosis is made on the basis of a compatible clinical-radiological scenario and the histological demonstration of the typical granulomas in the affected tissues. Interferons are immuno-modulators that have been used in a wide range of diseases, including hepatitis C virus infection, multiple sclerosis, and multiple myeloma and other types of tumours, including leukemia, lymphomas, Kaposi's sarcoma, and melanoma. Interferon-alpha-induced sarcoidosis has been reported repeatedly and there are two reports in the literature of cases of pulmonary sarcoidosis treated with interferon-1b therapy: one for advanced renal cell carcinoma and the other for multiple myeloma. A 35-year-old man on chronic immune-modulant Interferon-1b-based therapy for multiple sclerosis presented to the Neurology Unit with mild dyspnoea, dry cough, and transient pain to right upper abdomen. Lungs, spleen, liver, and almost all lymphnode stations of abdomen and mediastinum were clearly involved on ultrasound examination, chest X-ray, and computed tomography. A transbronchial biopsy showed non-caseating granuloma on histopathologic evaluation of the lungs. To the best of our knowledge, this is the first report of a chronic multisystemic sarcoidosis that was associated with interferon-beta treatment.

  19. Dual Inhibition of Rip2 and IRAK1/4 Regulates IL-1β and IL-6 in Sarcoidosis Alveolar Macrophages and Peripheral Blood Mononuclear Cells.

    PubMed

    Talreja, Jaya; Talwar, Harvinder; Ahmad, Nisar; Rastogi, Ruchi; Samavati, Lobelia

    2016-08-15

    Sarcoidosis is a multisystem granulomatous disease of unknown etiology that primarily affects the lungs. Our previous work indicates that activation of p38 plays a pivotal role in sarcoidosis inflammatory response. Therefore, we investigated the upstream kinase responsible for activation of p38 in sarcoidosis alveolar macrophages (AMs) and PBMCs. We identified that sustained p38 phosphorylation in sarcoidosis AMs and PBMCs is associated with active MAPK kinase 4 but not with MAPK kinase 3/6. Additionally, we found that sarcoidosis AMs exhibit a higher expression of IRAK1, IRAK-M, and receptor interacting protein 2 (Rip2). Surprisingly, ex vivo treatment of sarcoidosis AMs or PBMCs with IRAK1/4 inhibitor led to a significant increase in IL-1β mRNA expression both spontaneously and in response to TLR2 ligand. However, a combination of Rip2 and IRAK-1/4 inhibitors significantly decreased both IL-1β and IL-6 production in sarcoidosis PBMCs and moderately in AMs. Importantly, a combination of Rip2 and IRAK-1/4 inhibitors led to decreased IFN-γ and IL-6 and decreased percentage of activated CD4(+)CD25(+) cells in PBMCs. These data suggest that in sarcoidosis, both pathways, namely IRAK and Rip2, are deregulated. Targeted modulation of Rip2 and IRAK pathways may prove to be a novel treatment for sarcoidosis. PMID:27402699

  20. [67Ga scintigraphy as a therapeutic marker for spinal cord and muscular sarcoidosis: A case report].

    PubMed

    Yamada, Shinichiro; Nakanishi, Hirotaka; Hirayama, Masaaki; Katsuno, Masahisa; Sobue, Gen

    2015-01-01

    A 70-year-old woman was admitted to our hospital because of the right limb pain and gait disturbance. (67)Ga scintigraphy showed an increased uptake in the spinal cord, mediastinal lymph node and right tibialis anterior muscle. Based on the histopathological findings of epithelioid cell granuloma in endobronchial ultrasound-guided transbronchial needle aspiration of lymph node, she was diagnosed as having probable spinal cord/muscular sarcoidosis. After she was treated with oral prednisolone, her limb pain and gait disturbance improved. Furthermore, uptake in (67)Ga scintigraphy was reduced after the treatment. In conclusion, (67)Ga scintigraphy is useful not only for diagnosis, but also for estimating the efficiency of the treatment for sarcoidosis involving multiple organs such as the spinal cord and skeletal muscle.

  1. Beryllium-induced lung disease exhibits expression profiles similar to sarcoidosis.

    PubMed

    Li, Li; Silveira, Lori J; Hamzeh, Nabeel; Gillespie, May; Mroz, Peggy M; Mayer, Annyce S; Fingerlin, Tasha E; Maier, Lisa A

    2016-06-01

    A subset of beryllium-exposed workers develop beryllium sensitisation (BeS) which precedes chronic beryllium disease (CBD). We conducted an in-depth analysis of differentially expressed candidate genes in CBD.We performed Affymetrix GeneChip 1.0 ST array analysis on peripheral blood mononuclear cells (PBMCs) from 10 CBD, 10 BeS and 10 beryllium-exposed, nondiseased controls stimulated with BeSO4 or medium. The differentially expressed genes were validated by high-throughput real-time PCR in this group and in an additional group of cases and nonexposed controls. The functional roles of the top candidate genes in CBD were assessed using a pharmacological inhibitor. CBD gene expression data were compared with whole blood and lung tissue in sarcoidosis from the Gene Expression Omnibus.We confirmed almost 450 genes that were significantly differentially expressed between CBD and controls. The top enrichment of genes was for JAK (Janus kinase)-STAT (signal transducer and activator of transcription) signalling. A JAK2 inhibitor significantly decreased tumour necrosis factor-α and interferon-γ production. Furthermore, we found 287 differentially expressed genes overlapped in CBD/sarcoidosis. The top shared pathways included cytokine-cytokine receptor interactions, and Toll-like receptor, chemokine and JAK-STAT signalling pathways.We show that PBMCs demonstrate differentially expressed gene profiles relevant to the immunnopathogenesis of CBD. CBD and sarcoidosis share similar differential expression of pathogenic genes and pathways. PMID:27103383

  2. Beryllium-induced lung disease exhibits expression profiles similar to sarcoidosis.

    PubMed

    Li, Li; Silveira, Lori J; Hamzeh, Nabeel; Gillespie, May; Mroz, Peggy M; Mayer, Annyce S; Fingerlin, Tasha E; Maier, Lisa A

    2016-06-01

    A subset of beryllium-exposed workers develop beryllium sensitisation (BeS) which precedes chronic beryllium disease (CBD). We conducted an in-depth analysis of differentially expressed candidate genes in CBD.We performed Affymetrix GeneChip 1.0 ST array analysis on peripheral blood mononuclear cells (PBMCs) from 10 CBD, 10 BeS and 10 beryllium-exposed, nondiseased controls stimulated with BeSO4 or medium. The differentially expressed genes were validated by high-throughput real-time PCR in this group and in an additional group of cases and nonexposed controls. The functional roles of the top candidate genes in CBD were assessed using a pharmacological inhibitor. CBD gene expression data were compared with whole blood and lung tissue in sarcoidosis from the Gene Expression Omnibus.We confirmed almost 450 genes that were significantly differentially expressed between CBD and controls. The top enrichment of genes was for JAK (Janus kinase)-STAT (signal transducer and activator of transcription) signalling. A JAK2 inhibitor significantly decreased tumour necrosis factor-α and interferon-γ production. Furthermore, we found 287 differentially expressed genes overlapped in CBD/sarcoidosis. The top shared pathways included cytokine-cytokine receptor interactions, and Toll-like receptor, chemokine and JAK-STAT signalling pathways.We show that PBMCs demonstrate differentially expressed gene profiles relevant to the immunnopathogenesis of CBD. CBD and sarcoidosis share similar differential expression of pathogenic genes and pathways.

  3. Validity of mini-dose gallium for objective quantification of alveolitis in pulmonary sarcoidosis

    SciTech Connect

    Vitti, R.; Maurer, A.H.; Siegel, J.A.; Fewell, J.W.; Malmud, L.S.

    1985-05-01

    Patents (pts) with pulmonary sarcoidosis will often have frequent gallium (Ga) studies to assess the response of alveolitis to corticosteroid therapy. Since many pts are young and the cumulative radiation burden significant the authors have evaluated the ability of computer based Ga lung imaging to reliably quantify Ga activity using a 0.5 mCi dose of Ga. Twelve pts with biopsy proven pulmonary sarcoidosis received 0.5 mCi of Ga-67 citrate intravenously and had a posterior view of the chest including a portion of the liver obtained at 24 hrs post injection. At 24 hrs the pts received an additional 2.5 mCi and were then imaged at 48 and 72 hrs. A gallium score (GS) was computer generated as follows: regions of interest (ROI's) were manually created for the lungs, liver and background. Following a 9 point spatial smooth and background subtraction, the images were scaled so that the liver activity was assigned an arbitrary value of 400 counts per pixel. The resulting average counts per pixel for the lungs were then summed for the GS. Using a paired T-test there was only a borderline significant difference (0.05sarcoidosis who commonly have serial studies.

  4. Multisystemic diseases and ethnicity: a focus on lupus erythematosus, systemic sclerosis, sarcoidosis and Behçet disease.

    PubMed

    Petit, A; Dadzie, O E

    2013-10-01

    We present an overview of the association between ethnicity and the clinical and epidemiological aspects of four multisystemic diseases: lupus erythematosus, systemic sclerosis, sarcoidosis and Behçet disease. In particular, we highlight observed ethnic differences in cutaneous manifestations of these diseases. This article should help guide clinical management, as well as serve to highlight future areas for research.

  5. Autoinflammatory granulomatous diseases: from Blau syndrome and early-onset sarcoidosis to NOD2-mediated disease and Crohn's disease

    PubMed Central

    Caso, Francesco; Galozzi, Paola; Costa, Luisa; Sfriso, Paolo; Cantarini, Luca; Punzi, Leonardo

    2015-01-01

    The recent identification of genetic mutations leading to dysfunction of inflammatory and apoptotic pathways, has allowed to characterise a group of diseases, recognised as monogenic autoinflammatory syndromes. Among those, Blau syndrome (BS) and early-onset sarcoidosis (EOS) have been identified as familial and sporadic phenotypes of the same non-caseating granulomatous form. Both the diseases are caused by mutations in the CARD15/NOD2 gene, encoding the cytosolic NOD2 protein, one of the key molecules in the regulation of innate immunity. Clinical onset is typically located in the first years of life and phenotype is characterised by simultaneous or less articular, cutaneous and ocular non-caseating granulomatous inflammation, which can be variably associated with a heterogeneous systemic spectrum. The CARD15/NOD2 gene has also been identified as one of the genes linked to susceptibility to Crohn's disease (CD), a common polygenic inflammatory granulomatous bowel disease. The heightened nuclear factor-κB activity, found in the intestinal tissue of patients affected by CD, has probably a genetic cause related to several CARD15/NOD2 polymorphisms. Other substitutions in the CARD15/NOD2 gene have also been found in a recently described disorder, called NOD2-associated autoinflammatory disease, which shares several clinical characteristics with BS and EOS. This review attempts to describe these diseases on the basis of the most recent evidences. We described genetic and clinical aspects, mainly focusing on BS and EOS, the most representative diseases of autoinflammatory granulomatous diseases, with the ultimate purpose to expand their knowledge. PMID:26509073

  6. [Case of sarcoidosis with squamous cell carcinoma which originated from solitary bronchial papilloma].

    PubMed

    Urushiyama, Hirokazu; Yamauchi, Yasuhiro; Suzuki, Satoshi; Sunohara, Mitsuhiro; Kouyama, Tadashi; Ohishi, Nobuya; Fukami, Takeshi; Nakajima, Jun; Ushiku, Tetsuo; Oota, Satoshi; Fukayama, Masashi; Nagase, Takahide

    2010-11-01

    A 60-year-old man was given a clinical diagnosis of sarcoidosis, with enlarged mediastinal and hilar lymphadenopathy by chest CT, high levels of angiotensin-converting enzyme, and gallium scintigraphy findings. After 2 years follow-up, chest CT showed that only the right superior lobe bronchial lymph node had enlarged, occluding the right B1 bronchus, but other enlarged lymph nodes had not changed in size. We performed bronchoscopy to evaluate the occlusion of the right B1 bronchus, and recognized a polypoid lesion. Transbronchial tumor biopsy specimens revealed squamous cell lung carcinoma. A right upper lobectomy and drainage of the hilar and mediastinal lymph regions were performed. Histopathological examination revealed the coexistence of squamous cell carcinoma with many non-caseating epithelioid cell granulomas in all hilar and mediastinal drainage lymph nodes, but no metastasis. Non-caseating epithelioid cell granulomas were also seen in the lung interstitium. Histopathological examination suggested that the squamous cell carcinoma originated from a solitary bronchial papilloma. A diagnosis of lung cancer complicated with sarcoidosis was difficult by clinical imaging alone, including FDG-PET/CT. This suggests the importance of bronchoscopic examination, if a clinical course of the disease appears to be different from the usual course. This was a rare case of squamous cell carcinoma which originated from a solitary bronchial papilloma.

  7. Amyloïdosis, sarcoidosis and systemic lupus erythematosus.

    PubMed

    Rezgui, Amel; Hassine, Imene Ben; Karmani, Monia; Fredj, Fatma Ben; Laouani, Chadia

    2016-01-01

    The occurrence of renal and multiple organ Amyloïdosis is currently considered exceptional in the course of systemic lupus erythematosus. We report a case of a concomitant SLE and Amyloïdosis in a 57 year old female patient with hypothyroidism history, who presented with erythema nodosum, fever, arthralgia and sicca syndrome. Biological findings showed an inflammatory syndrome, renal failure, proteinuria (1g / 24h), positive auto antibodies and anti DNA. Lung radiology revealed medistinal lymphadenopathy, pleural nodules, ground glass infiltrates and pleuritis. Bronchial biopsy showed non specific inflammation. The salivary gland biopsy showed amyloïd deposits. This case report reminds us that lupus and Amyloïdosis association, although exceptional remains possible. The occurrence of Lofgren syndrome in this situation make the originality of this report. PMID:27583087

  8. [Analysis of mediastinal lymphadenopathy in sarcoidosis with transesophageal ultrasonic endoscopy; influences on cardiovascular system].

    PubMed

    Dambara, T; Ueki, J; Aiba, M; Tamaki, S; Saito, H; Matsuda, K; Nukiwa, T; Kira, S

    1989-01-01

    We studied influences of mediastinal lymphadenopathy on cardiovascular system in nine cases of sarcoidosis with transesophageal ultrasonic endoscopy. Chest x-ray films revealed bilateral hilar lymphadenopathy in all cases, whose diagnoses were made histologically with biopsies of peripheral lung through flexible fiber optic bronchoscope and/or of lymph nodes. These findings were typical in this disease. Many compartments of cardiovascular system in mediastinal and hilar regions were visible with this technique in all subjects. And enlarged lymph nodes changed dimensions in these compartments, especially in main pulmonary arteries, left atria, pulmonary veins and aorta. Furthermore, these phenomena were seen in azygos vein and superior caval vein in some cases. It is suggested that these lymph nodes may influence hemodynamics in above-mentioned compartments of cardiovascular system, if this phenomenon deteriorates more progressively. PMID:2657139

  9. Comparison of gallium-67 scanning, bronchoalveolar lavage, and serum angiotensin-converting enzyme levels in pulmonary sarcoidosis. Predicting response to therapy

    SciTech Connect

    Baughman, R.P.; Fernandez, M.; Bosken, C.H.; Mantil, J.; Hurtubise, P.

    1984-05-01

    Patients with active pulmonary sarcoidosis underwent bronchoalveolar lavage, gallium scan, and serum angiotensin-converting enzyme (ACE) level determination prior to treatment with corticosteroids. Pulmonary function was tested before and after therapy. Increase in vital capacity after treatment ranged from 40 to 1,030 ml; 12 of the 16 patients studied had an increase of more than 200 ml. There was a close correlation between the percentage uptake of gallium scan and the increase of the vital capacity after therapy (r . 0.95, p less than 0.01). There was no relationship between the percentage of lymphocytes obtained on lavage and the changes in vital capacity with therapy (r . 0.05). There was a positive correlation between the changes in vital capacity and the ratio of T4(+):T8(+)lymphocytes (r . 0.62, p less than 0.05) and number of T4 (+) lymphocytes (r . 0.92, p less than 0.01) in the bronchoalveolar fluid. There was a low correlation between the pretreatment ACE level and the change in vital capacity (r . 0.368, p greater than 0.05).

  10. The indirect costs of systemic autoimmune diseases, systemic lupus erythematosus, systemic sclerosis and sarcoidosis: a summary of 2012 real-life data from the Social Insurance Institution in Poland.

    PubMed

    Kawalec, Paweł P; Malinowski, Krzysztof P

    2015-01-01

    Systemic lupus erythematosus, systemic sclerosis and sarcoidosis are three different autoimmune systemic diseases that generate a significant burden to society due to treatment costs and also those caused by a work disability or absenteeism among patients. Relevant 2012 data referring to the three components of absenteeism produced by autoimmune systemic diseases, sick leave, short-term and long-term work disability, were obtained from the Social Insurance Institution in Poland (PSII). By applying the Human Capital Approach using gross domestic product per capita, gross value added per worker and gross income per worker in 2012, total indirect costs for the diseases were calculated. All costs were presented in euros and were valid for 2012. The PSII recorded 1600 patients with systemic lupus erythematosus, 500 patients with systemic sclerosis and 2700 patients with sarcoidosis in the 2012 - total indirect costs were as high as 7,260,595, 2,268,571 and 4,027,575 EUR, respectively. Costs were estimated using gross domestic product per capita; 17,485,412, 5,463,312 and 9,699,455 EUR, accordingly, calculated using gross value added per worker and 5,346,933, 1,670,648 and 2,966,034 EUR estimated using gross income per worker, respectively. Considering only data on absenteeism gathered by the PSII we can conclude that the three autoimmune systemic diseases bore great indirect costs. Their social burden for Poland could be even greater when considering presenteeism as well as other components of absenteeism such as loss of unpaid work, a gray economy or loss of leisure time. PMID:26153642

  11. The indirect costs of systemic autoimmune diseases, systemic lupus erythematosus, systemic sclerosis and sarcoidosis: a summary of 2012 real-life data from the Social Insurance Institution in Poland.

    PubMed

    Kawalec, Paweł P; Malinowski, Krzysztof P

    2015-01-01

    Systemic lupus erythematosus, systemic sclerosis and sarcoidosis are three different autoimmune systemic diseases that generate a significant burden to society due to treatment costs and also those caused by a work disability or absenteeism among patients. Relevant 2012 data referring to the three components of absenteeism produced by autoimmune systemic diseases, sick leave, short-term and long-term work disability, were obtained from the Social Insurance Institution in Poland (PSII). By applying the Human Capital Approach using gross domestic product per capita, gross value added per worker and gross income per worker in 2012, total indirect costs for the diseases were calculated. All costs were presented in euros and were valid for 2012. The PSII recorded 1600 patients with systemic lupus erythematosus, 500 patients with systemic sclerosis and 2700 patients with sarcoidosis in the 2012 - total indirect costs were as high as 7,260,595, 2,268,571 and 4,027,575 EUR, respectively. Costs were estimated using gross domestic product per capita; 17,485,412, 5,463,312 and 9,699,455 EUR, accordingly, calculated using gross value added per worker and 5,346,933, 1,670,648 and 2,966,034 EUR estimated using gross income per worker, respectively. Considering only data on absenteeism gathered by the PSII we can conclude that the three autoimmune systemic diseases bore great indirect costs. Their social burden for Poland could be even greater when considering presenteeism as well as other components of absenteeism such as loss of unpaid work, a gray economy or loss of leisure time.

  12. A Rare Case of Isolated Cerebral Sarcoidosis Presenting as Suprasellar Mass Lesion with Salt-Wasting Hypopituitarism

    PubMed Central

    Krenzlin, H.; Jussen, D.; Musahl, C.; Scheil-Bertram, S.; Wernecke, K.; Horn, P.

    2015-01-01

    Background Sarcoidosis is a systemic disorder of unknown origin characterized by noncaseating granulomas. Clinical symptoms due to central nervous system (CNS) involvement occur in 5 to 7% of all cases; subclinical involvement is more frequent. Sole CNS involvement is very rare. Case Report A 25-year-old man presented with increasing polyuria and polydipsia over 8 weeks. Magnetic resonance imaging (MRI) revealed a supra- and infra-chiasmatic pre-thalamic mass lesion 1.0 × 1.4 × 1.4cm in diameter. Microsurgical biopsy verified a necrotizing noncaseating epithelioid cell tumor indicative for neurosarcoidosis. All symptoms dissolved within 3 months under stringent corticoid therapy. Conclusion Intracranial mass lesions as the primary and only manifestation of neuronal sarcoidosis are rare. Because conservative treatment is safe and effective, surgery is limited to biopsy and the alleviation of pressure-related symptoms to preserve neurologic function. PMID:26251792

  13. A Rare Case of Isolated Cerebral Sarcoidosis Presenting as Suprasellar Mass Lesion with Salt-Wasting Hypopituitarism.

    PubMed

    Krenzlin, H; Jussen, D; Musahl, C; Scheil-Bertram, S; Wernecke, K; Horn, P

    2015-07-01

    Background Sarcoidosis is a systemic disorder of unknown origin characterized by noncaseating granulomas. Clinical symptoms due to central nervous system (CNS) involvement occur in 5 to 7% of all cases; subclinical involvement is more frequent. Sole CNS involvement is very rare. Case Report A 25-year-old man presented with increasing polyuria and polydipsia over 8 weeks. Magnetic resonance imaging (MRI) revealed a supra- and infra-chiasmatic pre-thalamic mass lesion 1.0 × 1.4 × 1.4cm in diameter. Microsurgical biopsy verified a necrotizing noncaseating epithelioid cell tumor indicative for neurosarcoidosis. All symptoms dissolved within 3 months under stringent corticoid therapy. Conclusion Intracranial mass lesions as the primary and only manifestation of neuronal sarcoidosis are rare. Because conservative treatment is safe and effective, surgery is limited to biopsy and the alleviation of pressure-related symptoms to preserve neurologic function.

  14. PAR-2, IL-4R, TGF-β and TNF-α in bronchoalveolar lavage distinguishes extrinsic allergic alveolitis from sarcoidosis

    PubMed Central

    MATĚJ, RADOSLAV; SMĚTÁKOVÁ, MAGDALENA; VAŠÁKOVÁ, MARTINA; NOVÁKOVÁ, JANA; ŠTERCLOVÁ, MARTINA; KUKAL, JAROMÍR; OLEJÁR, TOMÁŠ

    2014-01-01

    Sarcoidosis (SARC) and extrinsic allergic alveolitis (EAA) share certain markers, making a differential diagnosis difficult even with histopathological investigation. In lung tissue, proteinase-activated receptor-2 (PAR-2) is primarily investigated with regard to epithelial and inflammatory perspectives. Varying levels of certain chemokines can be a useful tool for distinguishing EAA and SARC. Thus, in the present study, differences in the levels of transforming growth factor (TGF)-β1, tumor necrosis factor (TNF)-α, interleukin-4 receptor (IL-4R) and PAR-2 in bronchoalveolar lavage fluid (BALF) were compared, using an ELISA method, between 14 patients with EAA and six patients with SARC. Statistically significant higher levels of IL-4R, PAR-2 and the PAR-2/TGF-β1 and PAR-2/TNF-α ratios were observed in EAA patients as compared with SARC patients. Furthermore, the ratios of TNF-α/total protein, TGF-β1/PAR-2 and TNF-α/PAR-2 were significantly lower in EAA patients than in SARC patients. The results indicated a higher detection of PAR-2 in EAA samples in association with TNF-α and TGF-β levels. As EAA and PAR-2 in parallel belong to the Th2-mediated pathway, the results significantly indicated an association between this receptor and etiology. In addition, the results indicated that SARC is predominantly a granulomatous inflammatory disease, thus, higher levels of TNF-α are observed. Therefore, the detection of PAR-2 and investigated chemokines in BALF may serve as a useful tool in the differential diagnosis between EAA and SARC. PMID:25009615

  15. A Case of Sarcoidosis with Unusual Radiographic Findings that Developed 5 Years after Silicone Augmentation Mammoplasty Complicated by Miliary Tuberculosis during Corticosteroid Treatment

    PubMed Central

    Miyashita, Tomoko; Yoshioka, Katsunobu; Nakamura, Tomoyuki; Yamagami, Keiko

    2011-01-01

    A 54-year-old woman with a past history of silicone augmentation mammoplasty was admitted with fever and dyspnea with diffuse interstitial shadows on computed tomography (CT). Although radiological findings were atypical, we diagnosed sarcoidosis by laboratory, microbiological, and bronchoalveolar lavage fluid analysis. Corticosteroids ameliorated the condition, but she had recurrent of fever and CT revealed miliary nodules while interstitial shadows disappeared. Liver biopsy showed that noncaseating granuloma and Ziehl-Neelsen stain was positive. We diagnosed miliary tuberculosis which developed during corticosteroid therapy. Antituberculotic therapy resulted in favorable outcome. Possibility exists that onset of sarcoidosis was induced by mammoplasty, namely, human adjuvant disease. PMID:22937422

  16. Endobronchial Ultrasound-guided Transbronchial Needle Aspiration versus Standard Bronchoscopic Modalities for Diagnosis of Sarcoidosis: A Meta-analysis

    PubMed Central

    Hu, Li-Xing; Chen, Ru-Xuan; Huang, Hui; Shao, Chi; Wang, Ping; Liu, Yong-Zhe; Xu, Zuo-Jun

    2016-01-01

    Background: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is an effective technique used to precisely detect enlarged mediastinal lymph nodes. The efficacy of EBUS-TBNA versus standard modalities for the diagnosis of sarcoidosis remains to be elucidated. In this meta-analysis, we compared the efficacies of these methods. Methods: We searched PubMed, Embase, The Cochrane Library, Wanfang, Cpvip, CNKI, and the bibliographies of the relevant references. We analyzed the data obtained with Revman 5.2 (Nordic Cochrane Center, Copenhagen, Denmark) and Stata 12.0 software (Stata Corporation, College Station, TX, USA). The Mantel-Haenszel method was used to calculate the pooled odds ratio (OR) and 95% confidence intervals (CIs). Results: Sixteen studies with a total of 1823 participants met the inclusion criteria, and data were extracted regarding the diagnostic yield of each approach. The ORs for EBUS-TBNA versus transbronchial lung biopsy (TBLB) for the diagnosis of sarcoidosis ranged from 0.26 to 126.58, and the pooled OR was 5.89 (95% CI, 2.20–15.79, P = 0.0004). These findings indicated that EBUS-TBNA provided a much higher diagnostic yield than TBLB. The pooled OR for EBUS-TBNA + TBLB + endobronchial biopsy (EBB) versus TBNA + TBLB + EBB was 1.54 (95% CI, 0.61–3.93, P = 0.36), implying that there was no significant difference between their diagnostic yields. However, clinical heterogeneity was reflected in the nature of the studies and in the operative variables. Conclusions: The results of this meta-analysis suggest that EBUS-TBNA + TBLB + EBB could be used for the diagnosis of sarcoidosis, if available. At medical centers without EBUS-TBNA, TBNA + TBLB + EBB could be used instead. PMID:27364799

  17. Granulomatous dysimmune reactions (sarcoidosis, granuloma annulare, and others) on differently injured skin areas.

    PubMed

    Lo Schiavo, Ada; Ruocco, Eleonora; Gambardella, Alessio; O'Leary, Ryan E; Gee, Sarah

    2014-01-01

    Granulomatous disorders are chronic cell-mediated immune responses histologically characterized by collections of macrophages, epithelioid cells, and multinucleated giant cells. This disease spectrum often has an infectious origin, but sometimes neither an infective agent nor an inciting antigenic stimulus can be identified. The skin may be a preferential target for these disorders, especially in the areas that have been damaged by various forms of skin injury (eg, herpetic infections, trauma, thermal or solar burns, vaccinations, tattoos). These damaged skin sites frame the new concept of an immunocompromised cutaneous district (ICD), which defines a skin area with acquired immune dysregulation that can pave the way for the local onset of opportunistic disorders, such as infections, tumors, and granulomatous disorders. Sarcoidosis, granuloma annulare (GA), and forms of granulomatous vasculitis, such as Churg-Strauss syndrome (CSS) and Wegener's granulomatosis (WG), are the most common granulomatous disorders that occur in an ICD and may share common pathogenic mechanisms. Recent studies have found clinical and pathologic overlapping features across noninfectious granulomas. Although no unifying etiology exists, the development of granulomatous processes in the ICD has often been reported and the literature contains various hypotheses to explain it: (1) overactive immune response in a previously injured region with or without loss of immune tolerance; (2) overall reduced immune response; (3) retention of an exogeneous antigen or foreign body; (4) altered neural signaling; and (5) a combination of all the aforementioned processes. T helper cells, T regulatory cells, and macrophages, as well as a number of antigenic proteins, have been identified as potential contributing factors. In addition, a genetic predisposition and an intact systemic immune system are both instrumental for the persistence of local granuloma formation in the ICD. PMID:25160106

  18. Asbestos bodies in bronchoalveolar lavage fluid. A study of 20 asbestos-exposed individuals and comparison to patients with other chronic interstitial lung diseases

    SciTech Connect

    Roggli, V.L.; Piantadosi, C.A.; Bell, D.Y.

    1986-09-01

    We studied the asbestos body (AB) content of bronchoalveolar lavage fluid from 20 patients with a history of occupational asbestos exposure, 31 patients with sarcoidosis and 5 patients with idiopathic pulmonary fibrosis. The cellular lavage pellet was digested in sodium hypochlorite and filtered onto Nuclepore filters for AB quantification by light microscopy. ABs were found in 15 of 20 asbestos-exposed individuals, 9 of 31 sarcoidosis cases and 2 of 5 patients with idiopathic pulmonary fibrosis. There was a statistically significant difference in the number of ABs per million cells recovered or per milliliter of recovered lavage fluid in the asbestos-exposed group as compared to the other categories of chronic interstitial lung disease. The highest levels occurred in patients with asbestosis. Large numbers of asbestos bodies in the lavage fluid (greater than 1 AB/10(6) cells) were indicative of considerable occupational asbestos exposure, whereas occasional bodies were a nonspecific finding.

  19. Rationale and Design of the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis Study. Alpha-1 Protocol.

    PubMed

    Strange, Charlie; Senior, Robert M; Sciurba, Frank; O'Neal, Scott; Morris, Alison; Wisniewski, Stephen R; Bowler, Russell; Hochheiser, Harry S; Becich, Michael J; Zhang, Yingze; Leader, Joseph K; Methé, Barbara A; Kaminski, Naftali; Sandhaus, Robert A

    2015-10-01

    Severe deficiency of alpha-1 antitrypsin has a highly variable clinical presentation. The Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis α1 Study is a prospective, multicenter, cross-sectional study of adults older than age 35 years with PiZZ or PiMZ alpha-1 antitrypsin genotypes. It is designed to better understand if microbial factors influence this heterogeneity. Clinical symptoms, pulmonary function testing, computed chest tomography, exercise capacity, and bronchoalveolar lavage (BAL) will be used to define chronic obstructive pulmonary disease (COPD) phenotypes that can be studied with an integrated systems biology approach that includes plasma proteomics; mouth, BAL, and stool microbiome and virome analysis; and blood microRNA and blood mononuclear cell RNA and DNA profiling. We will rely on global genome, transcriptome, proteome, and metabolome datasets. Matched cohorts of PiZZ participants on or off alpha-1 antitrypsin augmentation therapy, PiMZ participants not on augmentation therapy, and control participants from the Subpopulations and Intermediate Outcome Measures in COPD Study who match on FEV1 and age will be compared. In the primary analysis, we will determine if the PiZZ individuals on augmentation therapy have a difference in lower respiratory tract microbes identified compared with matched PiZZ individuals who are not on augmentation therapy. By characterizing the microbiome in alpha-1 antitrypsin deficiency (AATD), we hope to define new phenotypes of COPD that explain some of the diversity of clinical presentations. As a unique genetic cause of COPD, AATD may inform typical COPD pathogenesis, and better understanding of it may illuminate the complex interplay between environment and genetics. Although the biologic approaches are hypothesis generating, the results may lead to development of novel biomarkers, better understanding of COPD phenotypes, and development of novel diagnostic and therapeutic trials in AATD and COPD

  20. Rationale and Design of the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis Study. Alpha-1 Protocol

    PubMed Central

    Senior, Robert M.; Sciurba, Frank; O’Neal, Scott; Morris, Alison; Wisniewski, Stephen R.; Bowler, Russell; Hochheiser, Harry S.; Becich, Michael J.; Zhang, Yingze; Leader, Joseph K.; Methé, Barbara A.; Kaminski, Naftali; Sandhaus, Robert A.

    2015-01-01

    Severe deficiency of alpha-1 antitrypsin has a highly variable clinical presentation. The Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis α1 Study is a prospective, multicenter, cross-sectional study of adults older than age 35 years with PiZZ or PiMZ alpha-1 antitrypsin genotypes. It is designed to better understand if microbial factors influence this heterogeneity. Clinical symptoms, pulmonary function testing, computed chest tomography, exercise capacity, and bronchoalveolar lavage (BAL) will be used to define chronic obstructive pulmonary disease (COPD) phenotypes that can be studied with an integrated systems biology approach that includes plasma proteomics; mouth, BAL, and stool microbiome and virome analysis; and blood microRNA and blood mononuclear cell RNA and DNA profiling. We will rely on global genome, transcriptome, proteome, and metabolome datasets. Matched cohorts of PiZZ participants on or off alpha-1 antitrypsin augmentation therapy, PiMZ participants not on augmentation therapy, and control participants from the Subpopulations and Intermediate Outcome Measures in COPD Study who match on FEV1 and age will be compared. In the primary analysis, we will determine if the PiZZ individuals on augmentation therapy have a difference in lower respiratory tract microbes identified compared with matched PiZZ individuals who are not on augmentation therapy. By characterizing the microbiome in alpha-1 antitrypsin deficiency (AATD), we hope to define new phenotypes of COPD that explain some of the diversity of clinical presentations. As a unique genetic cause of COPD, AATD may inform typical COPD pathogenesis, and better understanding of it may illuminate the complex interplay between environment and genetics. Although the biologic approaches are hypothesis generating, the results may lead to development of novel biomarkers, better understanding of COPD phenotypes, and development of novel diagnostic and therapeutic trials in AATD and COPD

  1. Rationale and Design of the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis Study. Alpha-1 Protocol.

    PubMed

    Strange, Charlie; Senior, Robert M; Sciurba, Frank; O'Neal, Scott; Morris, Alison; Wisniewski, Stephen R; Bowler, Russell; Hochheiser, Harry S; Becich, Michael J; Zhang, Yingze; Leader, Joseph K; Methé, Barbara A; Kaminski, Naftali; Sandhaus, Robert A

    2015-10-01

    Severe deficiency of alpha-1 antitrypsin has a highly variable clinical presentation. The Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis α1 Study is a prospective, multicenter, cross-sectional study of adults older than age 35 years with PiZZ or PiMZ alpha-1 antitrypsin genotypes. It is designed to better understand if microbial factors influence this heterogeneity. Clinical symptoms, pulmonary function testing, computed chest tomography, exercise capacity, and bronchoalveolar lavage (BAL) will be used to define chronic obstructive pulmonary disease (COPD) phenotypes that can be studied with an integrated systems biology approach that includes plasma proteomics; mouth, BAL, and stool microbiome and virome analysis; and blood microRNA and blood mononuclear cell RNA and DNA profiling. We will rely on global genome, transcriptome, proteome, and metabolome datasets. Matched cohorts of PiZZ participants on or off alpha-1 antitrypsin augmentation therapy, PiMZ participants not on augmentation therapy, and control participants from the Subpopulations and Intermediate Outcome Measures in COPD Study who match on FEV1 and age will be compared. In the primary analysis, we will determine if the PiZZ individuals on augmentation therapy have a difference in lower respiratory tract microbes identified compared with matched PiZZ individuals who are not on augmentation therapy. By characterizing the microbiome in alpha-1 antitrypsin deficiency (AATD), we hope to define new phenotypes of COPD that explain some of the diversity of clinical presentations. As a unique genetic cause of COPD, AATD may inform typical COPD pathogenesis, and better understanding of it may illuminate the complex interplay between environment and genetics. Although the biologic approaches are hypothesis generating, the results may lead to development of novel biomarkers, better understanding of COPD phenotypes, and development of novel diagnostic and therapeutic trials in AATD and COPD

  2. From the definition of silicosis at the 1930 Johannesburg conference to the blurred boundaries between pneumoconioses, sarcoidosis, and pulmonary alveolar proteinosis (PAP).

    PubMed

    Vincent, Michel; Chemarin, Cecile; Cavalin, Catherine; Catinon, Mikael; Rosental, Paul-André

    2015-11-01

    The 1930 International Labour Office Conference on silicosis in Johannesburg identified silicosis by setting a medicolegal framework to its nosology: as with other occupational illnesses, its medical content was fixed under economic pressure. This article follows a reading of all the proceedings of this conference (debates and reports of experts) to examine their potential impact on the etiology and nosology of other diseases, specifically sarcoidosis and pulmonary alveolar proteinosis (PAP), "idiopathic" diseases in which inorganic particles may be involved. We propose renewed study of the role of inorganic particles in these diseases. To do this, we propose to mobilize detection means such as mineralogical analysis and electron microscopy and in depth interviewing that are currently seldom used in France, in order to establish diagnosis and the potential occupational and environmental origin of these diseases.

  3. Off-Label Uses of Anti-TNF Therapy in Three Frequent Disorders: Behçet's Disease, Sarcoidosis, and Noninfectious Uveitis

    PubMed Central

    Sánchez-Cano, Daniel; Callejas-Rubio, José Luis; Ruiz-Villaverde, Ricardo; Ríos-Fernández, Raquel; Ortego-Centeno, Norberto

    2013-01-01

    Tumoral necrosis factor α plays a central role in both the inflammatory response and that of the immune system. Thus, its blockade with the so-called anti-TNF agents (infliximab, etanercept, adalimumab, certolizumab pegol, and golimumab) has turned into the most important tool in the management of a variety of disorders, such as rheumatoid arthritis, spondyloarthropatties, inflammatory bowel disease, and psoriasis. Nonetheless, theoretically, some other autoimmune disorders may benefit from these agents. Our aim is to review these off-label uses of anti-TNF blockers in three common conditions: Behçet's disease, sarcoidosis, and noninfectious uveitis. Due to the insufficient number of adequate clinical trials and consequently to their lower prevalence compared to other immune disorders, this review is mainly based on case reports and case series. PMID:23983404

  4. [Pulmonary infiltrates in a 19 year old patient with dysuria and hypercalcemia].

    PubMed

    Schnaitmann, R; Hack, M; Gläser, F; Schütze, B; Tsegai, E; Kleber, G

    2004-08-01

    A 19 year old patient presented with the typical constellation of sarcoidosis. In the presence of indefinable pulmonary infiltrates, hypercalcemia, raised angiotensin converting enzyme and even evidence of giant and epitheloid cell granulomas, cocaine abuse should be considered. Chronic inhalative cocaine abuse can cause foreign body associated granulomatosis of the lung and other organs. It is important to establish this differential diagnosis by confidential interview and systematic polarisation microscopy to detect foreign material in tissues: unnecessary therapies with potential side effects should be avoided and drug weaning with rehabilitation of the patient should be initiated. However the potential for rapid progressive respiratory failure should not be underestimated.

  5. Learn About Sarcoidosis

    MedlinePlus

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  6. Clinical Evaluation of Red Eyes in Pediatric Patients.

    PubMed

    Beal, Casey; Giordano, Beverly

    2016-01-01

    Patients with the primary symptom of a red eye are commonly seen in pediatric primary care clinics. The differential diagnoses of a red eye are broad, but with a succinct history and physical examination, the diagnosis can be readily identified in many patients. Identifying conditions that threaten vision and understanding the urgency of referral to an ophthalmologist is paramount. Some systemic diseases such as leukemia, sarcoidosis, and juvenile idiopathic arthritis can present with the chief symptom of a red eye. Finally, trauma, ranging from mild to severe, often precipitates an office visit with a red eye, and thus understanding the signs that raise concern for a ruptured globe is essential. In the primary care setting, with a focused history, a few simple examination techniques, and an appreciation of the differential diagnosis, one can feel confident in managing patients with acute red eyes. PMID:26948259

  7. Common variable immunodeficiency with coexisting central nervous system sarcoidosis: case report and literature review with implications for diagnosis and pathogenesis.

    PubMed

    Dziadzio, Magdalena; Hortobágyi, Tibor; Kidd, Desmond; Chee, Ronnie

    2011-11-30

    We describe a patient with a history of longstanding primary generalised epilepsy, on anticonvulsant therapy, who presented with fever, headache, worsening seizures and hallucinations. Among various investigations, the patient had high CSF protein and ACE levels, leptomeningeal nodular enhancement on MRI brain and non-caseating granulomas in the brain and meninges on the biopsy. The patient was diagnosed with neurosarcoidosis. Subsequently, he was found to be panhypogammaglobulinaemic and was diagnosed with probable common variable immunodeficiency (CVID). The coexistence of common variable immunodeficiency and neurosarcoidosis is rare. Typically, non-caseating granulomas in CVID patients are localised in the lymphatic tissue and solid organs. To our knowledge, there are only five reports of the granulomas of the central nervous system (CNS) in CVID. We discuss the diagnostic difficulties in this case and review the literature. PMID:22611619

  8. The devil is in the detail: Acute Guillain–Barré syndrome camouflaged as neurosarcoidosis in a critically ill patient admitted to an Intensive Care Unit

    PubMed Central

    Sarada, Pooja Prathapan; Sundararajan, Krishnaswamy

    2016-01-01

    Guillain–Barré syndrome (GBS) is an acute demyelinating polyneuropathy, usually evoked by antecedent infection. Sarcoidosis is a multisystem chronic granulomatous disorder with neurological involvement occurring in a minority. We present a case of a 43-year-old Caucasian man who presented with acute ascending polyradiculoneuropathy with a recent diagnosis of pulmonary sarcoidosis. The absence of acute flaccid paralysis excluded a clinical diagnosis of GBS in the first instance. Subsequently, a rapid onset of proximal weakness with multi-organ failure led to the diagnosis of GBS, which necessitated intravenous immunoglobulin and plasmapheresis to which the patient responded adequately, and he was subsequently discharged home. Neurosarcoidosis often masquerades as other disorders, leading to a diagnostic dilemma; also, the occurrence of a GBS-like clinical phenotype secondary to neurosarcoidosis may make diagnosing coexisting GBS a therapeutic challenge. This article not only serves to exemplify the rare association of neurosarcoidosis with GBS but also highlights the need for a high index of clinical suspicion for GBS and accurate history taking in any patient who may present with rapidly progressing weakness to an Intensive Care Unit. PMID:27303139

  9. Invasive pulmonary aspergillosis presenting as thoracic mass causing obstruction of the superior and inferior vena cava in an immunocompetent patient: a diagnostic dilemma.

    PubMed

    Bunkar, Moti lal; Takhar, Rajendra Prasad; Arya, Savita

    2015-01-01

    Invasive pulmonary aspergillosis (IPA) is a disease of immunocompromised patients, but its prevalence is increasing in immunocompetent persons. Patients usually present with non-specific symptoms, sometimes consistent with bronchopneumonia. We discuss the case of a 19-year-old boy who presented with constitutional respiratory symptoms along with signs of obstruction of the superior and inferior vena cava. A chest radiograph was suggestive of a thoracic mass raising suspicion of bronchogenic carcinoma/ benign mass, sarcoidosis and tuberculosis, but a diagnosis of invasive aspergillosis was established. The patient showed excellent clinico-radiological improvement with administration of voriconazole. Invasive pulmonary aspergillosis may also present with atypical findings and should be considered in differentials when investigating a case even if the patient does not have a risk factor. PMID:26506978

  10. Autoimmune central diabetes insipidus in a patient with ureaplasma urealyticum infection and review on new triggers of immune response.

    PubMed

    Murdaca, Giuseppe; Russo, Rodolfo; Spanò, Francesca; Ferone, Diego; Albertelli, Manuela; Schenone, Angelo; Contatore, Miriam; Guastalla, Andrea; De Bellis, Annamaria; Garibotto, Giacomo; Puppo, Francesco

    2015-12-01

    Diabetes insipidus is a disease in which large volumes of dilute urine (polyuria) are excreted due to vasopressin (AVP) deficiency [central diabetes insipidus (CDI)] or to AVP resistance (nephrogenic diabetes insipidus). In the majority of patients, the occurrence of CDI is related to the destruction or degeneration of neurons of the hypothalamic supraoptic and paraventricular nuclei. The most common and well recognized causes include local inflammatory or autoimmune diseases, vascular disorders, Langerhans cell histiocytosis (LCH), sarcoidosis, tumors such as germinoma/craniopharyngioma or metastases, traumatic brain injuries, intracranial surgery, and midline cerebral and cranial malformations. Here we have the opportunity to describe an unusual case of female patient who developed autoimmune CDI following ureaplasma urealyticum infection and to review the literature on this uncommon feature. Moreover, we also discussed the potential mechanisms by which ureaplasma urealyticum might favor the development of autoimmune CDI. PMID:26331225

  11. Autoimmune central diabetes insipidus in a patient with ureaplasma urealyticum infection and review on new triggers of immune response.

    PubMed

    Murdaca, Giuseppe; Russo, Rodolfo; Spanò, Francesca; Ferone, Diego; Albertelli, Manuela; Schenone, Angelo; Contatore, Miriam; Guastalla, Andrea; De Bellis, Annamaria; Garibotto, Giacomo; Puppo, Francesco

    2015-12-01

    Diabetes insipidus is a disease in which large volumes of dilute urine (polyuria) are excreted due to vasopressin (AVP) deficiency [central diabetes insipidus (CDI)] or to AVP resistance (nephrogenic diabetes insipidus). In the majority of patients, the occurrence of CDI is related to the destruction or degeneration of neurons of the hypothalamic supraoptic and paraventricular nuclei. The most common and well recognized causes include local inflammatory or autoimmune diseases, vascular disorders, Langerhans cell histiocytosis (LCH), sarcoidosis, tumors such as germinoma/craniopharyngioma or metastases, traumatic brain injuries, intracranial surgery, and midline cerebral and cranial malformations. Here we have the opportunity to describe an unusual case of female patient who developed autoimmune CDI following ureaplasma urealyticum infection and to review the literature on this uncommon feature. Moreover, we also discussed the potential mechanisms by which ureaplasma urealyticum might favor the development of autoimmune CDI.

  12. Adalimumab-induced acute interstitial lung disease in a patient with rheumatoid arthritis*

    PubMed Central

    Dias, Olívia Meira; Pereira, Daniel Antunes Silva; Baldi, Bruno Guedes; Costa, André Nathan; Athanazio, Rodrigo Abensur; Kairalla, Ronaldo Adib; Carvalho, Carlos Roberto Ribeiro

    2014-01-01

    The use of immunobiological agents for the treatment of autoimmune diseases is increasing in medical practice. Anti-TNF therapies have been increasingly used in refractory autoimmune diseases, especially rheumatoid arthritis, with promising results. However, the use of such therapies has been associated with an increased risk of developing other autoimmune diseases. In addition, the use of anti-TNF agents can cause pulmonary complications, such as reactivation of mycobacterial and fungal infections, as well as sarcoidosis and other interstitial lung diseases (ILDs). There is evidence of an association between ILD and the use of anti-TNF agents, etanercept and infliximab in particular. Adalimumab is the newest drug in this class, and some authors have suggested that its use might induce or exacerbate preexisting ILDs. In this study, we report the first case of acute ILD secondary to the use of adalimumab in Brazil, in a patient with rheumatoid arthritis and without a history of ILD. PMID:24626274

  13. Utility of microbiological testing of thoracic lymph nodes sampled by endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) in patients with mediastinal lymphadenopathy.

    PubMed

    Harris, Rebecca M; Arnaout, Ramy; Koziel, Henry; Folch, Erik; Majid, Adnan; Kirby, James E

    2016-02-01

    Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) represents a minimally invasive technique to sample peribronchial and mediastinal lymph nodes for diagnosis of cancer, lymphoma, or sarcoidosis. However, the value of EBUS-TBNA in diagnosis of respiratory infections has not been well explored. Here, microbiologic testing data for EBUS-TBNA samples collected from 82 patients over a 30-month period were retrospectively reviewed. No organisms were identified on Gram, acid-fast, or fungal stains. Bacterial cultures were positive in 52% of samples; however, all but 1 culture were considered contaminants. Mycobacterial cultures yielded Mycobacterium avium-intracellulare not identified in a concurrent bronchoalveolar lavage sample in 1 patient. Fungal cultures were negative. Overall, routine microbiologic tests on EBUS-TBNA samples do not appear sufficiently sensitive to rule out infectious causes of adenopathy. High clinical suspicion for infection may require modification of sampling techniques or more sensitive detection methods.

  14. Risk of Band Keratopathy in Patients with End-Stage Renal Disease

    PubMed Central

    Weng, Shih-Feng; Jan, Ren-Long; Chang, Chun; Wang, Jhi-Joung; Su, Shih-Bin; Huang, Chien-Cheng; Tseng, Sung-Huei; Chang, Yuh-Shin

    2016-01-01

    This study is a retrospective, nationwide, matched cohort study to investigate the risk of band keratopathy following end-stage renal disease (ESRD). The study cohort included 94,039 ESRD on-dialysis patients identified by the International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM), code 585 and registered between January 2000 to December 2009 at the Taiwan National Health Insurance Research Database. An age- and sex-matched control group comprised 94,039 patients selected from the Taiwan Longitudinal Health Insurance Database 2000. Information for each patient was collected from the index date until December 2011. In total, 230 ESRD patients and 26 controls had band keratopathy (P < 0.0001) during the follow-up period, indicating a significantly elevated risk of band keratopathy in the ESRD patients compared with controls (incidence rate ratio = 12.21, 95% confidence interval [CI] = 8.14–18.32). After adjustment for potential confounders including sarcoidosis, hyperparathyroidism, iridocyclitis, and phthisis bulbi, ESRD patients were 11.56 times more likely to develop band keratopathy in the full cohort (adjusted HR = 11.56, 95% CI = 7.70–17.35). In conclusion, ESRD increases the risk of band keratopathy. Close interdisciplinary collaboration between nephrologists and ophthalmologists is important to deal with band keratopathy following ESRD and prevent visual acuity impairments. PMID:27346848

  15. Risk of Band Keratopathy in Patients with End-Stage Renal Disease.

    PubMed

    Weng, Shih-Feng; Jan, Ren-Long; Chang, Chun; Wang, Jhi-Joung; Su, Shih-Bin; Huang, Chien-Cheng; Tseng, Sung-Huei; Chang, Yuh-Shin

    2016-01-01

    This study is a retrospective, nationwide, matched cohort study to investigate the risk of band keratopathy following end-stage renal disease (ESRD). The study cohort included 94,039 ESRD on-dialysis patients identified by the International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM), code 585 and registered between January 2000 to December 2009 at the Taiwan National Health Insurance Research Database. An age- and sex-matched control group comprised 94,039 patients selected from the Taiwan Longitudinal Health Insurance Database 2000. Information for each patient was collected from the index date until December 2011. In total, 230 ESRD patients and 26 controls had band keratopathy (P < 0.0001) during the follow-up period, indicating a significantly elevated risk of band keratopathy in the ESRD patients compared with controls (incidence rate ratio = 12.21, 95% confidence interval [CI] = 8.14-18.32). After adjustment for potential confounders including sarcoidosis, hyperparathyroidism, iridocyclitis, and phthisis bulbi, ESRD patients were 11.56 times more likely to develop band keratopathy in the full cohort (adjusted HR = 11.56, 95% CI = 7.70-17.35). In conclusion, ESRD increases the risk of band keratopathy. Close interdisciplinary collaboration between nephrologists and ophthalmologists is important to deal with band keratopathy following ESRD and prevent visual acuity impairments. PMID:27346848

  16. Do thallium myocardial perfusion scan abnormalities predict survival in sarcoid patients without cardiac symptoms

    SciTech Connect

    Kinney, E.L.; Caldwell, J.W. )

    1990-07-01

    Whereas the total mortality rate for sarcoidosis is 0.2 per 100,000, the prognosis, when the heart is involved, is very much worse. The authors used the difference in mortality rate to infer whether thallium 201 myocardial perfusion scan abnormalities correspond to myocardial sarcoid by making the simplifying assumption that if they do, then patients with abnormal scans will be found to have a death rate similar to patients with sarcoid heart disease. The authors therefore analyzed complete survival data on 52 sarcoid patients without cardiac symptoms an average of eighty-nine months after they had been scanned as part of a protocol. By use of survival analysis (the Cox proportional hazards model), the only variable that was significantly associated with survival was age. The patients' scan pattern, treatment status, gender, and race were not significantly related to survival. The authors conclude that thallium myocardial perfusion scans cannot reliably be used to diagnose sarcoid heart disease in sarcoid patients without cardiac symptoms.

  17. Erythromycin prophylaxis for Legionnaire's disease in immunosuppressed patients in a contaminated hospital environment.

    PubMed

    Vereerstraeten, P; Stolear, J C; Schoutens-Serruys, E; Maes, N; Thys, J P; Liesnard, C; Rost, F; Kinnaert, P; Toussaint, C

    1986-01-01

    Between January 1 and June 30, 1983, immunosuppressive drugs were administered in 20 renal transplant recipients undergoing 23 rejection episodes and in 3 patients with renal failure secondary to systemic disease. Legionella pneumophila, serogroup 1, pneumonia was diagnosed on 12/26 (47%) occasions. In an attempt to decrease this high rate, a program of erythromycin prophylaxis was instituted for every new patient who received immunosuppressive chemotherapy until eradication of the organism from the water supply could be realized. From July 1, 1983 to April 30, 1984, erythromycin prophylaxis (1.5-3 g/day by mouth) was administered during 39 episodes of high-dose immunosuppression (20 kidney graft recipients and 4 patients with systemic diseases); no cases of Legionnaire's disease were recorded. During the same period, erythromycin prophylaxis was withheld from 9 other high-dose immunosuppression episodes (7 kidney graft recipients and one patient with sarcoidosis); 5 cases of Legionnaire's disease occurred (56%) in this group. We conclude that erythromycin effectively protects immunocompromised patients in an environment contaminated with L pneumophila.

  18. Off-label use of TNF-alpha inhibitors in a dermatological university department: retrospective evaluation of 118 patients.

    PubMed

    Sand, Freja Lærke; Thomsen, Simon Francis

    2015-01-01

    Tumor necrosis factor-alpha (TNF)-alpha inhibitors are licensed for patients with severe refractory psoriasis and psoriatic arthritis. However, TNF-alpha inhibitors have also been used off-label for various recalcitrant mucocutaneous diseases. This study aimed to evaluate the efficacy and safety of TNF-alpha inhibitors used for off-label dermatological indications. We retrospectively evaluated patient records of 118 patients treated off-label with TNF-alpha inhibitors in a dermatological university department. Patients presented with severe aphthous stomatitis/genital aphthous lesions (26), chronic urticaria (25), hidradenitis suppurativa (29), acne conglobata (11), dissecting cellulitis of the scalp (two), orofacial granulomatosis (four), sarcoidosis (four), granuloma annulare (two), granulomatous rosacea (one), granuloma faciale (one), subcorneal pustulosis (one), pyoderma gangrenosum (four), Sweet's syndrome (four), Well's syndrome (one), benign familial pemphigus (one), lichen planus (one), and folliculitis decalvans (one). A significant number of these patients went into remission during therapy with TNF-alpha inhibitors. A total of 11 patients (9%) experienced severe adverse effects during therapy. Off-label therapy with TNF-alpha inhibitors may be considered for selected patients with severe recalcitrant mucocutaneous diseases. The risk of severe adverse effects signals that a thorough benefit-risk assessment should be performed before initiating off-label treatment with TNF-alpha inhibitors for these conditions. PMID:25731720

  19. An Unusual Cause of a Solitary Lung Cavity.

    PubMed

    Yap, Vanessa; Salerno, Edward; Datta, Debapriya

    2016-01-01

    Sarcoidosis is a systemic granulomatous disease of unknown etiology. Pulmonary involvement is common, but lung nodules in sarcoidosis are uncommon, and solitary nodules that cavitate are extremely rare. Nodular sarcoidosis is usually found in young, healthy, predominantly female individuals. These lesions need to be differentiated from multiple other conditions, including neoplasms and granulomatous infections. A thorough workup for other etiologies of cavitary lung lesions is required for diagnosis. Despite an ominous presentation, nodular cavitary sarcoidosis portends a favorable outcome. We report a patient who presented with a solitary cavitary nodular lung lesion that was diagnosed as sarcoidosis after extensive workup. PMID:26882788

  20. Google-driven search for big data in autoimmune geoepidemiology: analysis of 394,827 patients with systemic autoimmune diseases.

    PubMed

    Ramos-Casals, Manuel; Brito-Zerón, Pilar; Kostov, Belchin; Sisó-Almirall, Antoni; Bosch, Xavier; Buss, David; Trilla, Antoni; Stone, John H; Khamashta, Munther A; Shoenfeld, Yehuda

    2015-08-01

    Systemic autoimmune diseases (SADs) are a significant cause of morbidity and mortality worldwide, although their epidemiological profile varies significantly country by country. We explored the potential of the Google search engine to collect and merge large series (>1000 patients) of SADs reported in the Pubmed library, with the aim of obtaining a high-definition geoepidemiological picture of each disease. We collected data from 394,827 patients with SADs. Analysis showed a predominance of medical vs. administrative databases (74% vs. 26%), public health system vs. health insurance resources (88% vs. 12%) and patient-based vs. population-based designs (82% vs. 18%). The most unbalanced gender ratio was found in primary Sjögren syndrome (pSS), with nearly 10 females affected per 1 male, followed by systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and antiphospholipid syndrome (APS) (ratio of nearly 5:1). Each disease predominantly affects a specific age group: children (Kawasaki disease, primary immunodeficiencies and Schonlein-Henoch disease), young people (SLE Behçet disease and sarcoidosis), middle-aged people (SSc, vasculitis and pSS) and the elderly (amyloidosis, polymyalgia rheumatica, and giant cell arteritis). We found significant differences in the geographical distribution of studies for each disease, and a higher frequency of the three SADs with available data (SLE, inflammatory myopathies and Kawasaki disease) in African-American patients. Using a "big data" approach enabled hitherto unseen connections in SADs to emerge. PMID:25842074

  1. Google-driven search for big data in autoimmune geoepidemiology: analysis of 394,827 patients with systemic autoimmune diseases.

    PubMed

    Ramos-Casals, Manuel; Brito-Zerón, Pilar; Kostov, Belchin; Sisó-Almirall, Antoni; Bosch, Xavier; Buss, David; Trilla, Antoni; Stone, John H; Khamashta, Munther A; Shoenfeld, Yehuda

    2015-08-01

    Systemic autoimmune diseases (SADs) are a significant cause of morbidity and mortality worldwide, although their epidemiological profile varies significantly country by country. We explored the potential of the Google search engine to collect and merge large series (>1000 patients) of SADs reported in the Pubmed library, with the aim of obtaining a high-definition geoepidemiological picture of each disease. We collected data from 394,827 patients with SADs. Analysis showed a predominance of medical vs. administrative databases (74% vs. 26%), public health system vs. health insurance resources (88% vs. 12%) and patient-based vs. population-based designs (82% vs. 18%). The most unbalanced gender ratio was found in primary Sjögren syndrome (pSS), with nearly 10 females affected per 1 male, followed by systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and antiphospholipid syndrome (APS) (ratio of nearly 5:1). Each disease predominantly affects a specific age group: children (Kawasaki disease, primary immunodeficiencies and Schonlein-Henoch disease), young people (SLE Behçet disease and sarcoidosis), middle-aged people (SSc, vasculitis and pSS) and the elderly (amyloidosis, polymyalgia rheumatica, and giant cell arteritis). We found significant differences in the geographical distribution of studies for each disease, and a higher frequency of the three SADs with available data (SLE, inflammatory myopathies and Kawasaki disease) in African-American patients. Using a "big data" approach enabled hitherto unseen connections in SADs to emerge.

  2. Impact of {sup 18}F-Fluorodeoxyglucose Positron Emission Tomography Before and After Definitive Radiation Therapy in Patients With Apparently Solitary Plasmacytoma

    SciTech Connect

    Kim, Paul J.; Hicks, Rodney J.; Wirth, Andrew; Ryan, Gail; Seymour, John F.; Prince, H. Miles

    2009-07-01

    Purpose: To evaluate the impact of {sup 18}F-fluorodeoxyglucose positron emission tomography (FDG-PET) on management of patients with apparently isolated plasmacytoma. Methods and Materials: Twenty-one patients with apparently solitary plasmacytoma who underwent FDG-PET for staging or restaging were identified from a central PET database. They were either candidates for or had received definitive radiation therapy (RT). Results: Seventeen patients had initial staging scans for bone (n = 11) or soft tissue (n = 6) plasmacytomas, and 11 had PET scans after RT. Only 1 of 14 known untreated sites of plasmacytoma was not identified on staging PET (lesion sensitivity = 93%). Three plasmacytomas were excised before PET. Staging PET influenced management in 6 of 17 patients (35%) by showing multiple myeloma (n = 1), discouraging RT after complete resection (n = 1), excluding plasmacytoma at a second site (n = 1), by increasing RT fields (n = 2), or by suggesting sarcoidosis (n = 1). Fifteen of 17 patients with initial staging PET scans received definitive RT. Restaging PET scans after RT showed complete metabolic response in 8 of 11 cases and progressive disease in 2. Two patients with either no response or partial metabolic response had late responses. Staging sestamibi and PET scans were concordant in five of six occasions (one sestamibi scan was false negative). Conclusions: FDG-PET has value for staging and RT planning in plasmacytoma and potentially could have a role in response-assessment after RT. Slow resolution of FDG uptake posttreatment does not necessarily imply an adverse prognosis.

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

    PubMed Central

    Rennard, S I; Crystal, R G

    1982-01-01

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

  4. Gallium-67 imaging in muscular sarcoidosis

    SciTech Connect

    Edan, G.; Bourguet, P.; Delaval, P.; Herry, J.Y.

    1984-07-01

    A case is presented of sarcoid myopathy in which radiogallium was seen to accumulate in the sites of muscle involvement. Uptake of the radiotracer disappeared following institution of corticosteroid therapy. The exceptional nature of this case contrasts with the high frequency of biopsy evidence of sarcoid granulomas in muscle. Gallium-67 imaging can be used to determine the extent of muscle involvement and, through evaluation of uptake intensity, the degree of disease activity before and after treatment.

  5. Gallium-67 imaging in muscular sarcoidosis

    SciTech Connect

    Edan, G.; Bourguet, P.; Delaval, P.; Herry, J.Y.

    1984-07-01

    A case is presented of sarcoid myopathy in which radiogallium was seen to accumulate in the sites of muscle involvement. Uptake of the radiotracer disappeared following institution of corticosteroid therapy. The exceptional nature of this case contrasts with the high frequency of biopsy evidence of sarcoid muscle disease but is consistent with the rarity of clinical evidence of sarcoid granulomas in muscle. Gallium-67 imaging can be used to determine the extent of muscle involvement and, through evaluation of uptake intensity, the degree of disease activity before and after treatment.

  6. Uveitis in DiGeorge syndrome: a case of autoimmune ocular inflammation in a patient with deletion 22q11.2

    PubMed Central

    Gottlieb, Chloe; Li, Zhuqing; Uzel, Gulbu; Nussenblatt, Robert B; Sen, H Nida

    2010-01-01

    Purpose Del22q11.2, also known as DiGeorge syndrome, has a spectrum of ocular, facial and systemic features. Despite features of T cell dysfunction, infection and autoimmunity (including juvenile idiopathic arthritis), uveitis has not been described in patients with DiGeorge syndrome. Methods We describe a case of a 25-year-old male with bilateral granulomatous panuveitis who after initial investigation and treatment for an infectious cause was determined to have autoimmune-related uveitis with evidence on clinical, laboratory and imaging assessments suggestive of ocular sarcoidosis. Results The patient was found to have a normal T cell count and T cell proliferative response that was compared to a control patient, and phenotypes determined by flow cytometry were normal. However, the CD4/CD8 ratio in this patient was slightly lower than normal and the number of CD28 negative T cells, in both CD4 and CD8 populations, were significantly higher than a control. Conclusions The significance of these T cell abnormalities is unknown in the context of this patient’s uveitis but is suggestive of a role in autoimmunity, which is a known phenomenon in del22q11.2 syndrome, although autoimmune-related uveitis is not a previously described feature. PMID:20141355

  7. The value of histopathological diagnosis in the elderly patients with granulomatous dermatoses. Case series.

    PubMed

    Badea, Mihail Alexandru; Buicu, Corneliu Florin; Sin, Anca Ileana; Cotoi, Ovidiu Simion; Badea, Iudita Maria; Chiotoroiu, Andreea Luciana; Morariu, Silviu Horia

    2016-01-01

    Granulomatous inflammations are a particular type of chronic septic or aseptic inflammation, in which infectious or non-infectious agents are difficult to eliminate by the immune system. These are type IV hypersensitivity reactions mediated by pre-sensitized T-lymphocytes cells CD4+ and CD8+ lymphocytes. Disorders included in this category are: tuberculosis, leprosy, syphilis, sarcoidosis, type I diabetes, multiple sclerosis, Crohn's disease and rheumatoid arthritis. At cutaneous level, this pattern of granulomatous reaction is characterized by a chronic inflammation with formation of granulomas consisting of a variable number of histiocytes, multinucleated giant cells and lymphocytes. Granulomatous dermatoses should be differentiated from other primary or secondary lesions affecting the skin such as inflammation or tumors. Often granulomatous dermatoses can be confused with other skin disorders, both clinically and histological. Histopathology examination can add important information and clarify the diagnosis. This paper presents a series of three clinical cases of granulomatous skin occurring in the elderly patients confirmed at histology examination. Clinical and histology criteria were analyzed, along with specific differential diagnosis, based on data from the literature. PMID:27516029

  8. Adalimumab (TNF α Inhibitor) Therapy Exacerbates IgA Glomerulonephritis Acute Renal Injury and Induces Lupus Autoantibodies in a Psoriasis Patient.

    PubMed

    Wei, S S; Sinniah, R

    2013-01-01

    Adalimumab (Humira) is a tumour necrosis factor α (TNF α ) inhibitor that is approved for the treatment of rheumatoid arthritis, psoriasis, psoriatic arthritis, Crohn's disease, ankylosing spondylitis, and juvenile idiopathic arthritis (Sullivan and Preda (2009), Klinkhoff (2004), and Medicare Australia). Use of TNF α inhibitors is associated with the induction of autoimmunity (systemic lupus erythematosus, vasculitis, and sarcoidosis or sarcoid-like granulomas) (Ramos-Casals et al. (2010)). We report a patient with extensive psoriasis presenting with renal failure and seropositive lupus markers without classical lupus nephritis after 18 months treatment with adalimumab. He has renal biopsy proven IgA nephritis instead. Renal biopsy is the key diagnostic tool in patients presenting with adalimumab induced nephritis and renal failure. He made a remarkable recovery after adalimumab cessation and steroid treatment. To our knowledge, this is a unique case of a psoriasis patient presenting with seropositive lupus markers without classical lupus nephritis renal failure and had renal biopsy proven IgA glomerulonephritis after receiving adalimumab.

  9. Patient satisfaction.

    PubMed

    Prakash, Bhanu

    2010-09-01

    Patient satisfaction is an important and commonly used indicator for measuring the quality in health care. Patient satisfaction affects clinical outcomes, patient retention, and medical malpractice claims. It affects the timely, efficient, and patient-centered delivery of quality health care. Patient satisfaction is thus a proxy but a very effective indicator to measure the success of doctors and hospitals. This article discusses as to how to ensure patient satisfaction in dermatological practice. PMID:21430827

  10. Patient satisfaction.

    PubMed

    Prakash, Bhanu

    2010-09-01

    Patient satisfaction is an important and commonly used indicator for measuring the quality in health care. Patient satisfaction affects clinical outcomes, patient retention, and medical malpractice claims. It affects the timely, efficient, and patient-centered delivery of quality health care. Patient satisfaction is thus a proxy but a very effective indicator to measure the success of doctors and hospitals. This article discusses as to how to ensure patient satisfaction in dermatological practice.

  11. Renal infarction due to polyarteritis nodosa in a patient with angioimmunoblastic T-cell lymphoma: a case report and a brief review of the literature.

    PubMed

    Ambrosio, Maria Raffaella; Rocca, Bruno Jim; Ginori, Alessandro; Onorati, Monica; Fabbri, Alberto; Carmellini, Mario; Lazzi, Stefano; Tripodi, Sergio

    2012-05-08

    Angioimmunoblastic T-cell lymphoma is one of the most common subtypes of peripheral T-cell lymphoma (15-20% of all cases), accounting for approximately 1-2% of all non-Hodgkin lymphomas. It often presents autoimmune phenomena including hemolytic anemia, thrombocytopenia, glomerulonephrities and circulating immune complexes. Polyarteritis nodosa is an autoimmune disease characterized by necrotizing vasculitis of medium vessels, which rarely develops in association with hematological malignant disorders. Herein we report the case of a 40-year-old man who underwent lymph node biopsy in the suspicious of sarcoidosis. On the basis of histological and immunohistochemical findings, the diagnosis of angioimmunoblastic T-cell lymphoma was performed. The patient was successfully treated with cytarabine-based regimen for 6 cycles. Three months after the initial diagnosis of angioimmunoblastic T-cell lymphoma, a whole body computed tomography showed a lesion in the lower pole of the left kidney. Renal cell carcinoma was suspected, thus a nephrectomy was carried out. The histological findings were compatible with polyarteritis nodosa. To the best of our knowledge, the association between polyarteritis nodosa and angioimmunoblastic T-cell lymphoma has been described only once. This relation may be secondary to the induction of an autoimmune phenomenon by the lymphoma with the formation of circulating immune complexes, leading to vessels walls injury. A careful evaluation is needed in the management of angioimmunoblastic T-cell lymphoma patients with signs of renal failure in order to avoid delay of treatment and organ damage.

  12. Multiple pulmonary micronodules in a patient with Crohn's disease

    PubMed Central

    Abbas, S A; Syed, Y I; Dushianthan, A; Barry, S

    2010-01-01

    Sarcoidosis is a terrific mimicker and can present in many different ways. The case of a middle-aged woman with Crohn's disease who attended the gastroenterology clinic for routine follow-up is presented. She had dry cough and breathlessness for a few weeks. A chest x-ray showed scattered multiple bilateral pulmonary micronodules. This finding on her chest x-ray posed a diagnostic challenge, especially in view of the fact that she was previously treated with immunosuppressants to control her Crohn's disease. A diagnosis of sarcoidosis was established by various tests including lung biopsy, which showed non-caseating granulomas. Within a few weeks of beginning systemic steroid treatment, improvement was noticed in symptoms and lung function tests. PMID:22767662

  13. Multiple pulmonary micronodules in a patient with Crohn's disease.

    PubMed

    Abbas, S A; Syed, Y I; Dushianthan, A; Barry, S

    2010-01-01

    Sarcoidosis is a terrific mimicker and can present in many different ways. The case of a middle-aged woman with Crohn's disease who attended the gastroenterology clinic for routine follow-up is presented. She had dry cough and breathlessness for a few weeks. A chest x-ray showed scattered multiple bilateral pulmonary micronodules. This finding on her chest x-ray posed a diagnostic challenge, especially in view of the fact that she was previously treated with immunosuppressants to control her Crohn's disease. A diagnosis of sarcoidosis was established by various tests including lung biopsy, which showed non-caseating granulomas. Within a few weeks of beginning systemic steroid treatment, improvement was noticed in symptoms and lung function tests. PMID:22767662

  14. Bronchoalveolar lavage cellular analyses in conjunction with high-resolution computed tomography imaging as a diagnostic intervention for patients with suspected interstitial lung disease

    PubMed Central

    Chockalingam, Ammaiyappan; Duraiswamy, Ranganathan; Jagadeesan, Madhavan

    2016-01-01

    Background: Bronchoalveolar lavage (BAL) has gained acceptance for diagnosis of Interstitial lung disease (ILD). The advent of high-resolution computed tomography (HRCT) has reduced the clinical utility of BAL. This work has utilized the recommendations of the American Thoracic Society (ATS) to optimize BAL and the findings have been associated with clinical examination and HRCT to precisely narrow down the cause of ILD. Materials and Methods: BAL was performed on ILD suspects at the target site chosen based on HRCT. The procedure, transport, processing, and analysis of BAL fluid were performed as per the ATS guidelines. The clinical data, HRCT findings and BAL report were used to narrow down the diagnosis of ILD. The statistical analysis was performed to assess the significance. Results: The BAL procedure was optimized as per the recommendations of the ATS. In a cohort of 50 patients, Idiopathic pulmonary fibrosis, (8) hypersensitivity pneumonitis, (17) connective tissue disorder, (9) sarcoidosis, (3) pneumoconiosis, (5) acute respiratory distress syndrome, (2) eosinophilic lung disease (2) and lymphangitic carcinomatosa, (2) aspiration bronchiolitis (1) and pulmonary histiocytosis (1) were diagnosed. Statistically significant variation in differential counts was found in different ILDs. The different ILDs were classified based on the criteria described by the ATS. Clinical Significance: BAL along with clinical and HRCT findings improved the diagnostic accuracy by incorporating, the acute or chronic nature of the disease and the cause for acute exacerbation, which helped in the better management of ILDs. PMID:27185993

  15. Patient Injuries?

    PubMed

    2015-01-01

    An injured patient may be the last thing dentists want to think about. However, in reality, patients can be injured during dental treatment or as the result of an incident such as a slip and fall in the office. Treatment-related injuries can run the gamut and include burns, lacerations, swallowed objects and allergic reactions, according to The Dentists Insurance Company.

  16. Patient Empowerment

    MedlinePlus

    ... cancer patient organization. Ask for this help. Your Responsibilities Keep Good Records Get in the habit of ... responsible for your follow-up. You should take responsibility for getting a follow-up scheduled and for ...

  17. Adverse Events Associated with Immune Checkpoint Blockade in Patients with Cancer: A Systematic Review of Case Reports

    PubMed Central

    Abdel-Wahab, Noha; Shah, Mohsin; Suarez-Almazor, Maria E.

    2016-01-01

    Background Three checkpoint inhibitor drugs have been approved by the US Food and Drug Administration for use in specific types of cancers. While the results are promising, severe immunotherapy-related adverse events (irAEs) have been reported. Objectives To conduct a systematic review of case reports describing the occurrence of irAEs in patients with cancer following checkpoint blockade therapy, primarily to identify potentially unrecognized or unusual clinical findings and toxicity. Data Sources We searched Medline, EMBASE, Web of Science, PubMed ePubs, and Cochrane CENTRAL with no restriction through August 2015. Study Selection Studies reporting cases of cancer develop irAEs following treatment with anti CTLA-4 (ipilimumab) or anti PD-1 (nivolumab or pembrolizumab) antibodies were included. Data Extraction We extracted data on patient characteristics, irAEs characteristics, how irAEs were managed, and their outcomes. Data Synthesis 191 publications met inclusion criteria, reporting on 251 cases. Most patients had metastatic melanoma (95.6%), and the majority were treated with ipilimumab (93.2%). Autoimmune colitis, hepatitis, endocrinopathies, and cutaneous irAEs were the most frequently reported irAEs in ipilimumab treated patients. A broad spectrum of toxicities were reported for almost every body system. Moreover, well-defined diseases such as sarcoidosis, polyarthritis, polymyalgia rheumatica/arteritis, lupus, celiac disease, dermatomyositis, and Vogt-Koyanagi-like syndrome were reported. The most frequent irAEs reported with anti-PD1 agents were dermatitis for pembrolizumab, and thyroid disease and pneumonitis for nivolumab. Complete resolution of adverse events occurred in most cases. However, persistent irAEs and death were reported, mainly in patients treated with ipilimumab. Limitations Our study is limited by information available in the original reports. Conclusions Evidence from case reports shows that cancer patients develop irAEs following

  18. Patient Monitoring

    NASA Technical Reports Server (NTRS)

    1978-01-01

    In photo above, the electrocardiogram of a hospitalized patient is being transmitted by telemetry. Widely employed in space operations, telemetry is a process wherein instrument data is converted to electrical signals and sent to a receiver where the signals are reconverted to usable information. In this instance, heart readings are picked up by the electrode attached to the patient's body and delivered by wire to the small box shown, which is a telemetry transmitter. The signals are relayed wirelessly to the console in the background, which converts them to EKG data. The data is displayed visually and recorded on a printout; at the same time, it is transmitted to a central control station (upper photo) where a nurse can monitor the condition of several patients simultaneously. The Patient Monitoring System was developed by SCI Systems, Inc., Huntsville, Alabama, in conjunction with Abbott Medical Electronics, Houston, Texas. In developing the system, SCI drew upon its extensive experience as a NASA contractor. The company applied telemetry technology developed for the Saturn launch vehicle and the Apollo spacecraft; instrumentation technology developed for heart, blood pressure and sleep monitoring of astronauts aboard NASA's Skylab long duration space station; and communications technology developed for the Space Shuttle.

  19. Patient Safety

    MedlinePlus

    You can help prevent medical errors by being an active member of your health care team. Research shows that patients who are more involved with their ... get better results. To reduce the risk of medical errors, you can Ask questions if you have doubts ...

  20. Refugee patients.

    PubMed

    Valeras, Aimee Burke

    2016-06-01

    This short story focuses on refugee patients. Family members talk about the horrific struggles of civil war, refugee camps, and promises of US resettlement. Their harsh reception into a brutal world includes unemployment, food scarcity, and isolation. (PsycINFO Database Record PMID:27270253