ACR open rheumatology 2019-Apr
Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry.
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Keywords
Abstract
Methods
This is a cross-sectional analysis of children with jLS enrolled in the CARRA Legacy Registry between May 2010 and April 2014. Descriptive statistics were used for demographic, clinical, and laboratory features. Data analysis included two-sample t test, χ2 test, Fisher's exact test, linear/logistic regression, and analysis of variance.Results
Of 381 children with jLS, 76% were female and 80% Caucasian. Mean onset age was 8.2 years, with 17% having a 2-year or greater delay to first pediatric rheumatology (PRH) visit. Linear scleroderma was the most common subtype (54%). Antinuclear antibody (ANA) positivity was associated with joint contracture (P = 0.04), muscle atrophy (P = 0.014), and extremity shortening (P = 0.007). Elevated aldolase was associated with joint contracture (P = 0.008) and elevated creatine kinase (CK) with muscle atrophy (P = 0.028) and extremity shortening (P = 0.016). Children with functional limitation (27%) had earlier first PRH visit compared with those without (P = 0.01). Poorer function correlated with muscle atrophy, joint contracture, and extremity shortening (P < 0.001). Methotrexate (97%) and corticosteroids (68%) were the most common medications used.