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Updated: Nov 29, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

[New developments in systemic sclerosis-associated interstitial lung disease].

Grégoire Humair¹, Cécile Daccord¹, Catherine Beigelman-Aubry²

¹Service de pneumologie, CHUV, 1011 Lausanne.

Revue Medicale Suisse

|November 18, 2020

Summary

This summary is machine-generated.

Interstitial lung disease (ILD) is a major cause of death in systemic sclerosis (SSc), often appearing within five years of diagnosis. Emerging treatments include biological agents and antifibrotic drugs, shifting from traditional immunosuppression.

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Area of Science:

Rheumatology
Pulmonology
Immunology

Background:

Interstitial lung disease (ILD) is a significant complication and leading cause of mortality in systemic sclerosis (SSc).
ILD in SSc typically manifests within the first five years post-diagnosis.
Risk factors for SSc-ILD include anti-topoisomerase I antibodies (Scl-70) and diffuse cutaneous SSc.

Purpose of the Study:

To summarize the current understanding of systemic sclerosis-associated interstitial lung disease (SSc-ILD).
To outline the common clinical and radiological presentations of SSc-ILD.
To review the evolving treatment landscape for SSc-ILD.

Main Methods:

Literature review of SSc-ILD epidemiology, risk factors, and clinical presentations.
Analysis of current and emerging therapeutic strategies for SSc-ILD.
Synthesis of data on immunosuppressive, biological, and antifibrotic treatments.

Main Results:

Nonspecific interstitial pneumonia (NSIP) is the most frequent radio-pathological finding, followed by usual interstitial pneumonia (UIP).
Established risk factors include Scl-70 antibodies and diffuse cutaneous SSc.
Recent evidence supports the efficacy of biological agents (rituximab, tocilizumab) and antifibrotic drugs (nintedanib).

Conclusions:

SSc-ILD is a critical determinant of survival in systemic sclerosis.
Treatment paradigms are shifting towards targeted therapies beyond classical immunosuppression.
Biological agents and antifibrotics represent promising advancements in managing SSc-ILD.