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Recent progress in systemic sclerosis-interstitial lung disease

Castelino, Flavia V.a; Dellaripa, Paul F.b

Current Opinion in Rheumatology: November 2018 - Volume 30 - Issue 6 - p 570–575
doi: 10.1097/BOR.0000000000000544

Purpose of review Systemic sclerosis is a systemic autoimmune disorder wherein interstitial lung disease (ILD) is the major source or morbidity and mortality. Research into mechanisms of fibrosis and its intersection with autoimmunity, specifically lung fibrosis, has accelerated and been applied to autoimmune disorders such as scleroderma. This review highlights important emerging insights and treatment trials.

Recent findings The important elements of this review focus on the challenges faced in identifying patients not only who develop lung disease but who are at a higher risk for progression given the heterogeneous natural history of ILD in scleroderma. Risk assessment scoring models using radiographic and physiologic parameters are highlighted and recent and ongoing clinical trials in scleroderma ILD are discussed.

Summary The implications of much of this ongoing work is a potential paradigm shift in our ability to identify those patients at risk for progression, and to offer novel therapies that can limit the progression of inflammatory and fibrotic lung disease in this challenging group of patients.

aDivision of Rheumatology, Massachusetts General Hospital

bDivision of Rheumatology, Brigham and Women's Hospital, Boston, Massachusetts, USA

Correspondence to Paul F. Dellaripa, MD, Brigham and Women's Hospital, Division of Rheumatology, Hale Building for Transformative Medicine, Floor 3, 75 Francis Street, Boston, MA 02115, USA. E-mail:

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