Connective Tissue Disease Associated Interstitial Lung Disease

Scott M Matson; M Kristen Demoruelle

doi:10.1016/j.rdc.2024.03.001

Connective Tissue Disease Associated Interstitial Lung Disease

Rheum Dis Clin North Am. 2024 Aug;50(3):423-438. doi: 10.1016/j.rdc.2024.03.001.

Authors

Scott M Matson¹, M Kristen Demoruelle²

Affiliations

¹ Division of Pulmonary, Critical Care and Sleep Medicine, University of Kansas School of Medicine, 3901 Rainbow boulevard, Mailstop 3007, Kansas City, KS 66160, USA.
² Division of Rheumatology, University of Colorado School of Medicine, 1775 Aurora Court, Mail Stop B-115, Aurora, CO 80045, USA. Electronic address: [email protected].

PMID: 38942578
DOI: 10.1016/j.rdc.2024.03.001

Abstract

Connective tissue disease associated interstitial lung disease (CTD-ILD) is a heterogenous collection of conditions with a diverse spectrum of interstitial lung disease (ILD) manifestations. Currently, clinical practice of lung-directed immunosuppression in CTD-ILD is supported by several randomized, placebo-controlled trials (RCTs) in patients with scleroderma and several observational, retrospective studies in other autoimmune conditions. However, given the harm of immunosuppression in idiopathic pulmonary fibrosis, there is an urgent need for RCTs of immunosuppression and antifibrotic agents in fibrotic CTD-ILD populations as well as the study of intervention in patients with subclinical CTD-ILD.

Keywords: Antifibrotic; Autoimmunity; Connective tissue disease; Immunosuppression; Interstitial lung disease; Subclinical interstitial lung disease.

Publication types

Review

MeSH terms

Connective Tissue Diseases* / complications
Humans
Immunosuppressive Agents / therapeutic use
Lung Diseases, Interstitial* / diagnosis
Lung Diseases, Interstitial* / etiology
Scleroderma, Systemic / complications

Substances

Immunosuppressive Agents