Prediction of disease relapse in a cohort of paediatric patients with localized scleroderma.

Autor: Kurzinski, K.L., Zigler, C.K., Torok, K.S.
Předmět:
Zdroj: British Journal of Dermatology; May2019, Vol. 180 Issue 5, p1183-1189, 7p
Abstrakt: Summary: 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. What's already known about this topic? Localized scleroderma (LS) is marked by periods of disease inflammation (activity) that can yield cutaneous and connective tissue damage, which can lead to significant physical and psychological disability.Some paediatric patients with LS experience a relapse of disease activity after initially achieving disease remission. What does this study add? There was a high rate of relapse (nearly 50%) in a cohort of paediatric patients with LS. Positive antinuclear antibody and older age at onset were associated with relapse.Medication noncompliance with methotrexate and corticosteroids can contribute to relapse.Clinician awareness of the high rate of recurrence in LS will help shift the general approach to this condition as a chronic autoimmune condition, recognizing recurrence and reinitiating treatment more promptly. Linked Comment: Pain. Br J Dermatol 2019; 180:990–991. Respond to this article [ABSTRACT FROM AUTHOR]
Databáze: Complementary Index