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

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.