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Pulmonology meets rheumatology in sarcoidosis: a review on the therapeutic approach

Drent, Marjoleina,b; Cremers, Johanna P.b; Jansen, Tim L.b,c

Current Opinion in Rheumatology: May 2014 - Volume 26 - Issue 3 - p 276–284
doi: 10.1097/BOR.0000000000000052
CLINICAL THERAPEUTICS: Edited by Cees G.M. Kallenberg
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Purpose of review Sarcoidosis is a systemic disease characterized by the formation of granulomas in various organs, mainly lungs and lymphatic system. Joint, muscle, and bone involvement is also rather common. Recent studies on its pathogenesis and therapeutic management are reviewed here.

Recent findings The pathogenesis of sarcoidosis is not fully elucidated. An exaggerated granulomatous reaction after exposure to unidentified antigens in genetically susceptible individuals evokes a clinical situation which we call sarcoidosis. No firm guidelines exist on whether, when, and how treatment should be started. Treatment is dependent on the presentation and the distribution, extensiveness and severity of sarcoidosis. Treatment of Löfgren's triad-related symptoms starts with NSAIDs; in other more extensive manifestations of sarcoidosis, the initiating dosage of glucocorticosteroids is approximately 20 mg daily. In terms of evidence-based treatment for sarcoidosis, only a few randomized controlled trials have been done. There is no cure for chronic sarcoidosis, and treatment only changes the granulomatous process and its clinical consequences.

Summary Identified associations of certain polymorphisms with severity of the disease and treatment response suggest future research questions as well as finding the cause(s) of sarcoidosis, and the elucidation of relevant biomarkers and new efficient treatments. Between 20 and 70% of patients need systemic therapy. The increased awareness of long-term side-effects of glucocorticosteroids and the emergence of new drugs have changed the treatment of sarcoidosis. Alternative or additional options to corticosteroids should be assessed.

Video abstract http://links.lww.com/COR/A13

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aDepartment of Toxicology, Faculty of Health, Medicine and Life Sciences, Maastricht University, Maastricht

binterstitial lung diseases care team (ild care team), Department of Respiratory Medicine, Hospital Gelderse Vallei, Ede

cDepartment of Rheumatology, University Medical Centre St Radboud, Nijmegen, The Netherlands

Correspondence to Professor Marjolein Drent, MD, PhD, Interstitial Lung Diseases Care Team (ILD Care Team), Department of Respiratory Medicine, Hospital Gelderse Vallei (ZGV), P.O. Box 18, 6720 AA Bennekom, The Netherlands. Fax: +31 84 2234007; e-mail: drentm@zgv.nl

Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Website (www.co-rheumatology.com).

© 2014 Lippincott Williams & Wilkins, Inc.