Methotrexate in Linear Scleroderma: Long‐Term Efficacy in Fifty Children From a Single Pediatric Rheumatology Center

Abstract

Objective to study disease course and long‐term outcome of children with Linear Scleroderma (LiS) treated with methotrexate (MTX) since diagnosis. Methods Retrospective and cross‐sectional study including consecutive children with LiS treated with MTX for >1 year and with at least 2 years follow‐up. Disease course was analyzed by number of relapses and treatment changes. Relapse‐free survival (RFS) was examined by Kaplan‐Meier analysis comparing patients with LiS and other JLS subtypes. Disease activity and damage were assessed by Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) and thermography. Results Fifty patients with mean follow‐up 7.8 years and MTX treatment duration 3.1 years were included. Sixteen percent did not respond to first course of MTX and 16% had at least one flare. Complete remission was observed in 18.2% patients with 2‐5 years follow‐up, 80.0% by 10 years and 87.5% by >10 years. No significant difference in RFS between patients with LiS and 17 with other JLS subtypes was observed. Tissue damage was mild in 42% patients, moderate in 32% and severe in 26%. No significant correlation between severity of tissue damage and LiS subtype, disease duration, relapses and remission emerged. Treatment duration was significantly related to disease relapses (p) and severity of tissue damage (p). Conclusion Most patients with LiS treated with MTX achieve complete and long‐lasting remission. Overall aesthetic and functional sequelae are moderate, probably because tissue damage establishes early and treatment likely stabilizes it. Early diagnosis and MTX treatment as well as long‐term monitoring are crucial to improve outcome and promptly identify flares.