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Semin Respir Crit Care Med 2024; 45(03): 435-448
DOI: 10.1055/s-0044-1786155
Review Article

Novel Therapeutic Approaches in Connective Tissue Disease-Associated Interstitial Lung Disease

Erica Mulcaire-Jones
1   Division of Rheumatology, Internal Medicine, University of Michigan, Ann Arbor, Michigan
,
Janelle Vu Pugashetti
2   Division of Pulmonary and Critical Care Medicine, Internal Medicine, University of Michigan, Ann Arbor, Michigan
,
Justin M. Oldham
2   Division of Pulmonary and Critical Care Medicine, Internal Medicine, University of Michigan, Ann Arbor, Michigan
,
Dinesh Khanna
1   Division of Rheumatology, Internal Medicine, University of Michigan, Ann Arbor, Michigan
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Abstract

Connective tissue diseases (CTD) comprise a group of autoimmune diseases that can affect multiple organs in the body including the lungs. The most common form of pulmonary involvement is interstitial lung disease (ILD). CTD-associated ILD (CTD-ILD) can take one of several courses including nonprogressive, chronically progressive, or rapidly progressive. Chronically and rapidly progressive patterns are associated with increased mortality. Limited randomized controlled trial data are available for treatment of CTD-ILD, with most data coming from systemic sclerosis-related ILD. The current first-line treatment for all CTD-ILD is immunosuppression with consideration of antifibrotics, stem cell transplant, and lung transplant in progressive disease. In this article, we review data for ILD treatment options in systemic sclerosis, rheumatoid arthritis, myositis, and primary Sjögren's syndrome-related ILDs.

Keywords

connective tissue disease - interstitial lung disease - rheumatoid arthritis - systemic sclerosis - idiopathic inflammatory myositis - primary Sjögren's syndrome - treatment


Publication History

Article published online:
13 May 2024

© 2024. Thieme. All rights reserved.

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