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Management of connective tissue diseases associated interstitial lung disease: a review of the published literature

Wallace, Beth; Vummidi, Dharshan; Khanna, Dinesh

Current Opinion in Rheumatology: May 2016 - Volume 28 - Issue 3 - p 236–245
doi: 10.1097/BOR.0000000000000270

Purpose of review Interstitial lung disease (ILD), though a common and often a severe manifestation of many connective tissue diseases (CTD), is challenging to manage because of its variable presentation and the relative lack of guidelines to assist the clinician. In this review, we discuss the approach to diagnosis, treatment, and monitoring patients with CTD-associated ILD, with a focus on systemic sclerosis (SSc), rheumatoid arthritis (RA), and idiopathic inflammatory myopathy (IIM).

Recent findings High-resolution computed tomography scan and pulmonary function testing can be reliably used to diagnose ILD and monitor progression, and often to determine its likely histologic subtype and severity. In SSc-ILD, randomized controlled trials show ILD stabilization with cyclophosphamide treatment; preliminary data from another randomized controlled trial demonstrates similar findings with mycophenolate. There are no robust clinical trials supporting specific treatments for RA-ILD or IIM-ILD, but rituximab in RA-ILD, and cyclophosphamide, mycophenolate and calcineurin inhibitors in IIM-ILD show promise.

Summary Though ILD contributes substantially to morbidity and mortality in patients with CTD, there are minimal data to guide its management except in SSc-ILD.

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aDepartment of Rheumatology

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

Correspondence to Dinesh Khanna, MD, MSc, Professor of Medicine, Frederick G. Huetwell Professor of Rheumatology. Director, University of Michigan Scleroderma Program. Division of Rheumatology/Dept. of Internal Medicine, 300 North Ingalls Street, Suite 7C27, Ann Arbor, MI 48109, USA. Tel: +1 734 647 8173; fax: +1 734 763 5761; e-mail:

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