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Review
. 2024 Nov 1;36(6):420-426.
doi: 10.1097/BOR.0000000000001049. Epub 2024 Aug 27.

Treatment of interstitial lung disease in systemic sclerosis: guidelines and new clinical trial results

Sindhu R Johnson  1 , Elana J Bernstein  2
Affiliations
Review

Treatment of interstitial lung disease in systemic sclerosis: guidelines and new clinical trial results

Sindhu R Johnson et al. Curr Opin Rheumatol. .

Abstract

Purpose of review: Interstitial lung disease (ILD) is the leading cause of death in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD). The American College of Rheumatology (ACR), in conjunction with the American College of Chest Physicians (CHEST), recently published clinical practice guidelines for the treatment of adults with systemic autoimmune rheumatic disease-associated ILD, including SSc-ILD. Herein, we summarize evidence from randomized trials evaluating the safety and efficacy of pharmacologic therapies for the treatment of SSc-ILD.

Recent findings: In this review, we present findings from recent randomized controlled trials in SSc-ILD. The pharmacologic therapies discussed include immunosuppressive medications (mycophenolate, cyclophosphamide, rituximab, and tocilizumab) and antifibrotic medications (nintedanib and pirfenidone).

Summary: Randomized trials provide an evidence base for the SSc-ILD treatment recommendations put forth in the ACR/CHEST Guidelines for the treatment of ILD in people with systemic autoimmune rheumatic diseases. These guidelines will help inform clinical practice and highlight areas in which further research is needed.

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Conflict of interest statement

Conflicts of interest

Dr Johnson has no conflicts of interest to declare. Dr. Bernstein reports consulting fees from Boehringer Ingelheim and Cabaletta and grant support from Boehringer Ingelheim and aTyr.

Figures

Figure 1.
Figure 1.
Pharmacologic options for the treatment of SSc-ILD
See this image and copyright information in PMC

References

    1. Alahmari H, Ahmad Z, Johnson SR. Environmental Risks for Systemic Sclerosis. Rheumatic diseases clinics of North America. 2022;48(4):845–60. - PubMed
    1. Distler O, Assassi S, Cottin V, et al. Predictors of progression in systemic sclerosis patients with interstitial lung disease. Eur Respir J. 2020;55(5). - PMC - PubMed
    1. Hussein H, Lee P, Chau C, Johnson SR. The effect of male sex on survival in systemic sclerosis. J Rheumatol. 2014;41(11):2193–200. - PubMed
    1. Al-Sheikh H, Ahmad Z, Johnson SR. Ethnic Variations in Systemic Sclerosis Disease Manifestations, Internal Organ Involvement, and Mortality. J Rheumatol. 2019. - PubMed

    1. Johnson SR, Bernstein EJ, Bolster MB, et al. 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Screening and Monitoring of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases. Arthritis & rheumatology. 2024.

      * This is the first collaboration between the American College of Rheumatology and the American College of Chest Physicians to provide guidelines for the screening and monitoring of SSc-ILD

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