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

Overview

Journal Semin Respir Crit Care Med

Specialties Critical Care
Pulmonary Medicine

Date 2024 May 13

PMID 38740369

Authors

Erica Mulcaire-Jones

Janelle Vu Pugashetti

Justin M Oldham

Dinesh Khanna

Affiliations

Soon will be listed here.

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.

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