ArticleNorthern clinics of Istanbul2026
Real-world immunosuppressive treatment patterns and disease progression in systemic sclerosis-associated interstitial lung disease.
Article in Northern clinics of Istanbul, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
objectiveInterstitial 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.
methodsWe 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.
resultsAmong 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%).
conclusionIn 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.
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