Treatment preferences in early systemic sclerosis-associated interstitial lung disease: real-world data from the Turkish Systemic Sclerosis Registry

Abstract

Background: The management of interstitial lung disease associated with systemic sclerosis (SSc-ILD) primarily relies on conventional immunosuppressives and, increasingly, biologic therapies. However, there remains an unmet need, as many patients either fail to respond adequately or experience progressive disease. Current treatment guidelines also emphasize the role of antifibrotic agents, such as nintedanib, as an option for slowing the progression of fibrotic changes in the lungs. Objectives: This study aimed to investigate preferred treatments in early SSc-ILD based on real-world data. Methods: This study included SSc patients registered in the Turkish Systemic Sclerosis Registry who met the 2013 ACR/EULAR (American College of Rheumatology/European League Against Rheumatism) SSc classification criteria and had fewer than 3 years of disease duration. Data from the initial records of patients diagnosed with ILD based on lung computed tomography (CT) findings were analyzed cross-sectionally. Results: Among the registered 251 patients with SSc, 106 patients (42%) had SSc-ILD. The mean age of the patients with SSc-ILD was 50± 13.9 years, with a female-to-male ratio of 5.6:1. Fourty-one patients (38.7%) had diffuse, 61 (57.5%) had limited skin involvement, and 4 (3.8%) had SSc sine scleroderma. Eighty-nine 89 patients (87,2%) received conventional immunosuppressives. Among the conventional immunosuppressives, mycophenolate mofetil (MMF) was the most frequently preferred agent (69.6%), followed by cyclophosphamide (CYC) (13,7%) and azathioprine (AZA) (3.9%). Among the 13 patients (12.8%) who received biological immunosuppressives, RTX was the most preferred agent (n=11). Ten patients (9.4%) received nintedinib as an antifibrotic treatment. Conclusion: Real-world data from our registry revealed that mycophenolate mofetil (MMF) was the most commonly preferred conventional immunosuppressive for early-stage SSc-ILD, aligning with current guidelines and consistent with previous reports [1]. However, the proportion of patients receiving antifibrotic therapy was lower in our registry, which may be attributed to the short disease duration. Long-term follow-up with comparative analyses of treatment responses would provide more valuable insights.