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Nat Rev Dis Primers. 2019 Jul 4;5(1):45. doi: 10.1038/s41572-019-0096-x.

Sarcoidosis.

Author information

1
Respiratory Medicine Division, Department of Medicine Solna, and Center for Molecular Medicine (CMM), Karolinska Institutet; and Respiratory Medicine, Theme Inflammation and Infection, Karolinska University Hospital, Stockholm, Sweden. Johan.Grunewald@ki.se.
2
Interstitial Lung Diseases Center of Excellence, Department of Pulmonology, St Antonius Hospital, Nieuwegein, Netherlands; and Division of Heart and Lungs, University Medical Center Utrecht, Utrecht, Netherlands.
3
Clinical Epidemiology Division, Department of Medicine Solna, Karolinska Institutet, Stockholm, Sweden.
4
Scleroderma and Sarcoidosis Patient Care and Research Center, University Medical Center Comprehensive Pulmonary Hypertension Center, Tulane University School of Medicine, New Orleans, LA, USA.
5
Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
6
Department of Pneumology, Medical Center University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.

Erratum in

Abstract

Sarcoidosis is an inflammatory disorder of unknown cause that is characterized by granuloma formation in affected organs, most often in the lungs. Patients frequently suffer from cough, shortness of breath, chest pain and pronounced fatigue and are at risk of developing lung fibrosis or irreversible damage to other organs. The disease develops in genetically predisposed individuals with exposure to an as-yet unknown antigen. Genetic factors affect not only the risk of developing sarcoidosis but also the disease course, which is highly variable and difficult to predict. The typical T cell accumulation, local T cell immune response and granuloma formation in the lungs indicate that the inflammatory response in sarcoidosis is induced by specific antigens, possibly including self-antigens, which is consistent with an autoimmune involvement. Diagnosis can be challenging for clinicians because of the potential for almost any organ to be affected. As the aetiology of sarcoidosis is unknown, no specific treatment and no pathognomic markers exist. Thus, improved biomarkers to determine disease activity and to identify patients at risk of developing fibrosis are needed. Corticosteroids still constitute the first-line treatment, but new treatment strategies, including those targeting quality-of-life issues, are being evaluated and should yield appropriate, personalized and more effective treatments.

PMID:
31273209
DOI:
10.1038/s41572-019-0096-x

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