Abstract
BACKGROUND: Localized scleroderma (LS) is an autoimmune condition of the skin and underlying tissue. Active or recurring disease can lead to cumulative tissue damage, especially in paediatric-onset disease. OBJECTIVES: To highlight the rate of relapse of LS activity in a cohort of paediatric patients and to evaluate for potential clinical and laboratory predictors of disease relapse. METHODS: Clinical and laboratory data were gathered prospectively. Patients were categorized as experiencing relapse or not, and clinical and laboratory parameters were compared. A logistic regression was fit to predict odds of relapse while controlling for multiple predictors. A subgroup of patients was also evaluated to determine the average time from treatment completion to relapse. RESULTS: Seventy-seven patients were followed for the identified study duration of > 2 years and had achieved disease remission, with 35 (45%) experiencing LS relapse. Patients who were older at disease onset, antinuclear antibody (ANA) positive and without an extracutaneous manifestation (ECM) were more likely to relapse. All three variables remained significant in the multivariable logistic regression model. Results of the subgroup mirrored the larger sample. The average time between treatment completion and relapse was 21 months. CONCLUSIONS: Assessment of patients with LS experiencing a relapse of disease activity has shown older age of initial LS onset and ANA positivity to be potential markers for risk of relapse. Patients meeting these parameters may require greater clinical vigilance. The presence of one or more ECM may be protective. Clinicians treating patients with LS should provide significant long-term follow-up to monitor for relapse.