ArticleJournal of scleroderma and related disorders2026
Predictor factors of successful treatment response in interstitial lung disease-related systemic sclerosis.
Article in Journal of scleroderma and related disorders, 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
Background: Systemic sclerosis (SSc) is a multi-organ autoimmune disease presenting interstitial lung disease (ILD) as the most common internal organ manifestation as it can be found in 25-90% of radiological imaging even without any symptoms. ILD in SSc (ILD-SSc) causes decreased mortality and morbidity, but the effectiveness of mycophenolate immunosuppression treatment reduces ILD progression by 75.5%. The risk of progression is still fairly high during immunosuppression, signifying the need to assess predictor factors for the success of mycophenolate treatment in patients with ILD-SSc. Objective: This study aims to investigate the incidence of successful mycophenolate treatment in ILD-SSc and predictor factors for the success based on real-world experience. Methods: A retrospective cohort design was applied in this study using outpatient medical record data of patients with extensive ILD-SSc from January 2021 to December 2024. Predictor model was developed from the results of multivariate analysis using linear regression followed by performance and internal validity tests. Results: A total of 119 patients with ILD-SSc who met the inclusion criteria were selected as study subjects. The incidence of successful mycophenolate treatment was 68.1%. Predictor factors influencing treatment success in ILD-SSc were obtained from multivariate analysis, such as younger age at onset of ILD-SSc has higher risk ratio for successfull treatment response (RR 2.327; 95% CI 1.510 to 3.586; p<0.001), type limited SSc (RR 0.389; 95% CI 0.251 to 0.602; p<0.001) and extent of ILD at initial diagnosis (RR 2.230; 95% CI 1.356 to 3.670; p=0.002). Predictor score model for mycophenolate treatment success with these three factors had an area under the curve performance of 0.671 (95% CI 0.567 to 0.776; p=0.001) and good calibration based on the Hosmer-Lemeshow test (p=0.576). Conclusion: The success rate of mycophenolate treatment in ILD-SSc at Dr. Cipto Mangunkusumo National Hospital (RSUPN) has comparable results with the reports in other countries. Factors such as age at onset of ILD-SSc diagnosis, extent of ILD and type of SSc are independent predictors of mycophenolate treatment success.
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