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Interstitial Lung Disease in Systemic Sclerosis: Focus on Early Detection and Intervention

Authors Fischer A, Patel NM, Volkmann ER

Received 10 August 2019

Accepted for publication 9 November 2019

Published 9 December 2019 Volume 2019:11 Pages 283—307


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 4

Editor who approved publication: Professor Chuan-Ju Liu

Aryeh Fischer,1 Nina M Patel,2 Elizabeth R Volkmann3

1Division of Rheumatology, Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, University of Colorado School of Medicine, Denver, CO, USA; 2Division of Pulmonary, Allergy and Critical Care Medicine, Columbia University Irving Medical Center, New York, NY, USA; 3Division of Rheumatology, Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, CA, USA

Correspondence: Elizabeth R Volkmann
Division of Rheumatology, Department of Medicine, David Geffen School of Medicine, University of California, 1000 Veteran Avenue, Suite 32-59, Los Angeles, CA 90095, USA
Tel +1 310-825-2448
Fax +1 310-206-6553

Abstract: Systemic sclerosis (SSc) is a progressive and often devastating disease characterized by autoimmune dysfunction, vasculopathy, and fibrosis. Interstitial lung disease (ILD) is identified in the majority of patients with SSc and is the leading cause of SSc-related mortality. Although clinical manifestations and ILD severity vary among patients, lung function typically declines to the greatest extent during the first 3–4 years after disease onset. We aim to provide an overview of SSc-associated ILD (SSc-ILD) with a focus on current and emerging tools for early diagnosis of ILD and current and novel treatments under investigation. Early detection of ILD provides the opportunity for early therapeutic intervention, which could improve patient outcomes. Thoracic high-resolution computed tomography is the most effective method of identifying ILD in patients with SSc; it enables detection of mild lung abnormalities and plays an important role in monitoring disease progression. Cyclophosphamide and mycophenolate mofetil are the most commonly prescribed treatments for SSc-ILD. Recently, nintedanib (an antifibrotic) was approved by the Food and Drug Administration for patients with SSc-ILD; it is indicated for slowing the rate of decline in pulmonary function. However, there is a need for additional effective and well-tolerated disease-modifying therapy. Ongoing studies are evaluating other antifibrotics and novel agents. We envision that early detection of lung involvement, combined with the emergence and integration of novel therapies, will lead to improved outcomes in patients with SSc-ILD.

Keywords: systemic sclerosis, interstitial lung diseases, early diagnosis, disease progression, treatment outcome

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