Mortality of Intrathoracic Sarcoidosis in Referral vs Population-Based Settings

Reich, Jerome M.

doi:10.1378/chest.121.1.32

Cited by 130 publications

(69 citation statements)

References 11 publications

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“…All patients with sarcoidosis were staged retrospectively using a modification of the system outlined by the American Thoracic Society (ATS)/European Respiratory Society (ERS)/World Association of Sarcoidosis and Other Granulomatous Disorders joint statement as previously described [2,14]. Criteria were as follows.…”

Section: Study Design and Patient Populationmentioning

confidence: 99%

“…Criteria were as follows. Stage I: bilateral hilar lymphadenopathy without pulmonary infiltrates; stage II: bilateral hilar lymphadenopathy with pulmonary infiltrates; and stage III: pulmonary infiltrates and/or fibrosis in the absence of hilar lymphadenopathy [2,14]. In addition, we also retrospectively characterised these patients with or without an active pulmonary lymphocytic alveolitis defined by a BAL lymphocyte count .30610…”

Section: Study Design and Patient Populationmentioning

confidence: 99%

“…Based on a modification of the ATS/ERS/World Association of Sarcoidosis and Other Granulomatous Disorders joint statement staging criteria [2,14] and excluding patients with infection/colonisation, we identified 27 (38%) patients with stage I disease, 36 (50%) with stage II disease and nine (12%) with stage III disease (table 1). Using the definition of active pulmonary lymphocytic alveolitis as a BAL lymphocyte count .30610 3 lymphocytes?mL -1 , there were 27 (37%) patients with and 45 (63%) patients without an active alveolitis (table 1).…”

Section: Patient Populationmentioning

confidence: 99%

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CXCR3 ligands are augmented during the pathogenesis of pulmonary sarcoidosis

Busuttil¹,

Weigt²,

Keane³

et al. 2009

Eur Respir J

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We and other investigators have hypothesised that the CXC chemokine receptor (CXCR)3/CXCR3 ligand biological axis is involved in the formation of sarcoid lung granulomas; however, significant discrepancies in the current literature remain. In an effort to clarify previous conflicting findings, we performed the largest observational study to date of interferon-inducible ELR -(lacking the sequence glutamic acid-leucine-arginine) CXC chemokines in sarcoid bronchoalveolar fluid (BALF). BALF chemokine levels from sarcoid patients (n572) and healthy controls (n58) were measured with the ELISA method. Immunohistochemical staining was performed for CXCR3 and its ligands.BALF CXC chemokine ligand (CXCL)10 levels from sarcoid patients were not significantly increased compared with controls. BALF CXCL11 levels from sarcoid patients demonstrated a trend towards elevation; subgroup analysis by stage showed significant BALF CXCL11 elevation in stage I sarcoid patients compared with controls. BALF CXCL9 levels were elevated from sarcoid patients compared with controls. CXC11, CXCL9 and CXCR3 were expressed from epithelioid histiocytes, multinucleated giant cells and other inflammatory cells forming sarcoid lung granulomas.Our data suggest that CXCL9 and CXCL11 are important mediators in recruiting CXCR3-expressing cells. Importantly, we have made the novel observation that both lymphocytes and cells of monocyte linage express CXCR3 and are involved in the formation of sarcoid lung granulomas.

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Section: Study Design and Patient Populationmentioning

confidence: 99%

Section: Study Design and Patient Populationmentioning

confidence: 99%

Section: Patient Populationmentioning

confidence: 99%

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CXCR3 ligands are augmented during the pathogenesis of pulmonary sarcoidosis

Busuttil¹,

Weigt²,

Keane³

et al. 2009

Eur Respir J

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“…A further study that handled the long-term effects of corticosteroids is a large meta-analysis by REICH [39] that investigated the mortality of intrathoracic sarcoidosis patients in referral (2,838 cases) versus population-based settings (e.g. health maintenance organisations and government clinics in Scandinavian countries; 812 cases).…”

Section: Pulmonary Diseasementioning

confidence: 99%

Corticosteroid treatment in sarcoidosis

Grutters

Bosch²

2006

Eur Respir J

168

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At present there is no curative treatment for sarcoidosis. Immunosuppressive and/or immunomodulatory drugs can, however, be used for controlling the disease.Corticosteroids remain the mainstay of therapy. They function by suppressing the proinflammatory cytokines and chemokines that are involved in cell-mediated immune responses and granuloma formation. Only in a select group of patients is it justifiable to use these drugs, after careful evaluation of the pros and cons. Importantly, disease severity, e.g. threatened organ functions, and not disease activity itself should be the deciding factor in this process.In the case of parenchymal involvement, there is substantial evidence that corticosteroids can improve respiratory symptoms and chest radiography and lung function parameters over 6-24 months. Other generally acknowledged (empirical) criteria for systemic treatment include neurological, cardiac and sight-threatening ocular involvement and hypercalcaemia. Remarkably, despite .50 yrs of use, there is no proof of long-term (survival) benefit from corticosteroid treatment. In addition, there are still no data regarding the optimal dose and duration of corticosteroid or other immunosuppressive therapy.One of the weightiest questions remaining is whether or not these drugs can prevent scarring in patients with a fibrogenic phenotype. As new agents, including infliximab and thalidomide, enter the stage and new diagnostic tools are now available, there is clearly a momentum to design multicentric randomised controlled trials with long enough follow-up (.5 yrs) to answer this pivotal question.

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“…The clinical manifestations of sarcoidosis are highly variable and nonspecific. Although thoracic involvement occurs in more than 90% of the patients, every organ may be affected [2,3,4]. Gastrointestinal tract involvement is rare with an incidence of less than %1.…”

Section: Introductionmentioning

confidence: 99%

Gastric Sarcoidosis: An Atypical Presentation of a Rare Disease

Yanardağ¹,

Tetikkurt²,

Bilir³

et al. 2017

AJMCR

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Sarcoidosis is a multisystemic granulomatous disease characterised by the histologic evidence of granulomas in various organs. Clinically discernible gastric sarcoidosis is extremely rare, reported in less than 1% of the patients. We present a case of gastric sarcoidosis with atypicial manifestations and without current pulmonary disease. The patient was admitted for gastric fullness, anorexia, and weight loss. She had a history of migraine and nodular goiter. Family history revealed bladder carcinoma in the mother and gastric carcinoma in the uncle. Initial laboratory findings and chest x-ray were normal. Biopsies from the gastric mucosa revealed noncaseating granulomas with focal multinucleate giant cells. Six months after the patient was admitted for dry cough and fatigue. Chest x-ray and CT revealed diffuse nodules in the upper and middle lung zones. Transbronchial biopsy demonstrated noncaseiting granulomas compatible with sarcoidosis. The patient was started on mehtylprednisolone and had a significant improvement in her symptoms. This case is an unique paradigm for the exceptional presentation of sarcoidosis. Isolated extrapulmonary organ involvement without simultaneous lung disease may be the initial manifestation of sarcoidosis. Meticulous follow-up for sarcoidosis is required for patients presenting with atypical symptoms and noncaseiting granulomas in any extrapulmonary organ.

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Mortality of Intrathoracic Sarcoidosis in Referral vs Population-Based Settings

Cited by 130 publications

References 11 publications

CXCR3 ligands are augmented during the pathogenesis of pulmonary sarcoidosis

CXCR3 ligands are augmented during the pathogenesis of pulmonary sarcoidosis

Corticosteroid treatment in sarcoidosis

Gastric Sarcoidosis: An Atypical Presentation of a Rare Disease

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