Ibrahim VASI, Ibrahim KARADUMAN, Rahime DURAN, Ibrahim Yahya CAKIR, Esma ESEROGLU, Burak KARAKAYA, Busra KOKSOY, Ertugrul Cagri BOLEK, Hamit KUCUK, Berna GOKER, Nilgun Demirci YILMAZ, Mehmet Akif OZTURK, Abdulsamet ERDEN
İstanbul Kuzey Klinikleri Dergisi - 2026;13(3):263-273
OBJECTIVE: Interstitial lung disease (ILD) is a frequent and prognostically important manifestation of systemic sclerosis (SSc), with a substantial proportion of patients developing progressive pulmonary fibrosis (PPF) despite immunosuppressive treatment. The objective of the study was to compare real-world lung function trajectories and the occurrence of progressive PPF among patients with SSc-ILD receiving commonly used immunosuppressive therapies. METHODS: We conducted a retrospective single-center cohort study of adult patients with SSc-ILD receiving immunosuppressive therapy, assessing longitudinal changes in pulmonary function and PPF according to international guideline-based criteria. RESULTS: Among 80 patients with SSc-ILD, mycophenolate mofetil (MMF) was the most frequently used immunosuppressive agent across both first-line and subsequent treatment lines. Despite its extensive use, MMF was associated with relatively low rates of progressive PPF (18.2% in first-line and 25% in second/third-line use) and largely stable forced vital capacity over time, with only modest decline in diffusing capacity for carbon monoxide. Cyclophosphamide (CYC) showed transient improvements in lung function at 1 year, followed by stabilization. In contrast, first-line azathioprine (AZA) use was associated with the highest PPF proportion (55.6%). CONCLUSION: In this real-world SSc-ILD cohort, MMF emerged as the most frequently used backbone therapy after the scleroderma lung study II, with generally stable lung function trajectories observed during follow-up. Higher proportions of progressive PPF were observed among AZA-treated patients, whereas CYC was mainly used as an induction therapy in selected cases. Rituximab, administered alone or in combination with MMF, was more frequently used in patients with progressive or treatment-refractory disease and was associated with relatively low progression proportions in this cohort. These findings reflect treatment patterns and clinical trajectories observed in routine clinical practice rather than comparative treatment efficacy.
