Phenotyping by persistent inflammation in systemic sclerosis associated interstitial lung disease: a EUSTAR database...

Area of Science:

• Rheumatology
• Pulmonology
• Immunology

Background:

• Systemic sclerosis (SSc) is a complex autoimmune disease.
• Interstitial lung disease (SSc-ILD) is a common and serious complication of SSc.
• Inflammation plays a critical role in SSc pathogenesis and progression.

Purpose of the Study:

• To evaluate the prognostic value of phenotyping SSc and SSc-ILD patients based on inflammation levels.
• To characterize disease trajectories in SSc and SSc-ILD stratified by inflammatory status and immunosuppressive treatment.

Main Methods:

• Utilized data from the European Scleroderma Trials and Research (EUSTAR) cohort.
• Classified patients into persistent inflammatory, intermediate, and non-inflammatory phenotypes based on C-reactive protein (CRP) levels.
• Employed Cox regression for mortality risk analysis and mixed-effects models for FVC and DLCO trajectory analysis.

Main Results:

• Patients with SSc-ILD and a persistent inflammatory phenotype faced a 6.7-fold higher 5-year mortality risk compared to non-inflammatory phenotypes.
• In the inflammatory phenotype, forced vital capacity (FVC) declined without treatment but stabilized with immunosuppressive therapy.
• FVC declined in the non-inflammatory phenotype, with a more pronounced decline observed in patients receiving immunosuppressive treatment.

Conclusions:

• Phenotyping by persistent inflammation offers significant prognostic insights in SSc and SSc-ILD, independent of other clinical factors.
• Early immunosuppressive treatment is recommended for SSc-ILD patients exhibiting persistent inflammation.
• Inflammation-based phenotyping can guide treatment decisions and improve outcomes in SSc-ILD.