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Management of systemic sclerosis-associated interstitial lung disease

Roofeh, Davida; Jaafar, Saraa; Vummidi, Dharshanb; Khanna, Dinesha

Current Opinion in Rheumatology: May 2019 - Volume 31 - Issue 3 - p 241–249
doi: 10.1097/BOR.0000000000000592

Purpose of review To review the recently published data and provide a practical overview for management of systemic sclerosis-interstitial lung disease (SSc-ILD).

Recent findings Published evidence shows considerable practitioner variability in screening patients for ILD. Recent published data support use of cyclophosphamide or mycophenolate mofetil as first-line treatment of SSc-ILD. For patients not responding to first-line therapies, consideration is given to rituximab as rescue therapy. Recent trials of hematopoietic autologous stem cell transplantation have demonstrated benefit in patients with progressive SSc-ILD. Antifibrotic agents are approved in idiopathic pulmonary fibrosis; studies with antifibrotics are underway for SSc-ILD.

Summary The specter of rapidly progressive lung disease requires clinicians to risk stratify patients according to known predictors for progression and rigorously monitor for symptoms and advancing disease. The abovementioned therapies promise improved efficacy and favorable side-effect profiles compared to cyclophosphamide.

aDepartment of Internal Medicine, Division of Rheumatology, Scleroderma Program

bDepartment of Radiology, University of Michigan, Ann Arbor, Michigan, USA

Correspondence to Dinesh Khanna, MD, MSc, Department of Internal Medicine, Division of Rheumatology, 300 North Ingalls St., Suite 7C27, Ann Arbor, MI 48109-5422, USA. Tel: +1 734 763 7182; fax: +1 734 936 3695; e-mail:

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