İbrahim VASI, İbrahim KARADUMAN, Ömer Onur KARACA, Rahime DURAN, Rıza Can KARDAŞ, İbrahim Yahya ÇAKIR, Esma ESEROĞLU, Burak KARAKAYA, Ertuğrul Çağrı BÖLEK, Hamit KÜÇÜK, Mehmet Akif ÖZTÜRK, Abdulsamet ERDEN
Rheumatology Quarterly - 2026;4(1):31-38
Objectives: Systemic sclerosis (SSc) is a chronic autoimmune disease marked by immune dysregulation, vasculopathy, and fibrosis. Interstitial lung disease (ILD) is among its most frequent and lethal complications. Despite therapeutic advances, SSc patients continue to experience excess mortality, mainly due to ILD and pulmonary hypertension (PH). We aimed to evaluate long-term survival and identify predictors of mortality in patients with systemic sclerosis-associated ILD (SSc-ILD). Methods: We performed a retrospective single-centre cohort study at Gazi University Hospital, including patients fulfilling 1980 ACR and 2013 ACR/EULAR criteria with HRCT-confirmed ILD (2010-2025). The primary outcome was all-cause mortality, verified through the national registry. Results: Among 126 patients, 29 (23.0%) died during follow-up, corresponding to a mortality rate of 24.8/1000 person-years, ~4.5-fold higher than the national average. Survival probability was 72% at 10 years and 49% at 15 years. SSc-related deaths (34.5%) were mainly due to ILD progression and PH; non-SSc-related causes included infections (31.0%), lung cancer (17.3%), and cardiovascular events. COVID-19 pneumonia contributed to infection-related deaths. After univariable and then multivariable Cox regression analysis, reduced baseline DLCO <70% predicted was found independently associated with increased mortality risk. Conclusion: Mortality in SSc-ILD is largely driven by ILD-associated complications rather than isolated respiratory failure alone. Pulmonary hypertension and impaired DLCO emerged as the strongest prognostic markers, highlighting the importance of systematic cardiopulmonary assessment and comprehensive management strategies that extend beyond fibrosis control to include infection prevention and malignancy surveillance.
