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Objective  Localized scleroderma ( LS ) is a chronic inflammatory and fibrosing skin disorder. We present baseline data on the juvenile  LS  ( jLS ) cohort from the Childhood Arthritis and Rheumatology Research Alliance ( CARRA ) Legacy Registry, a multicenter observational registry of pediatric rheumatologic disorders.    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, χ 2  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.    Conclusion  Children with  jLS  without joint limitation are referred later, highlighting the insidious onset and need for educating referring providers. Poorer function correlated with muscle atrophy, joint contracture, and limb shortening.  ANA  positivity and elevated  CK  or aldolase were associated with muscle atrophy, joint contracture, and/or limb shortening, suggesting predictors of muscle involvement.

Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry