Objective To evaluate the baseline clinical characteristics of juvenile systemic sclerosis
(SSc) patients in the international juvenile SSc inception cohort, and to compare
these characteristics between the classically defined juvenile diffuse cutaneous SSc
(dcSSc) and limited cutaneous SSc (lcSSc) subtypes and among those with overlap features.
Methods A cross-sectional study was performed using baseline visit data. Information
on demographic characteristics, organ system evaluation, treatment, and patient- and
physician-reported outcomes was extracted and summary statistics applied. Comparisons
between juvenile dcSSc and lcSSc subtypes and patients with and without overlap features
were performed using chi-square and Mann-Whitney U tests. Results At data extraction,
150 juvenile SSc patients were enrolled across 42 centers; 83% were White, 80% were
female, juvenile dcSSc predominated (72%), and 17% of the cohort had overlap features.
Significant differences were found between juvenile dcSSc and juvenile lcSSc regarding
modified Rodnan skin thickness score, the presence of Gottron's papules, digital tip
ulceration, results of the 6-minute walk test, and composite pulmonary and cardiac
involvement. All of these were more frequent in dcSSc except for cardiac involvement.
Juvenile dcSSc patients had significantly worse scores for physician-rated disease
activity and damage. A significantly higher occurrence of Gottron's papules and musculoskeletal
and composite pulmonary involvement, and a significantly lower frequency of Raynaud's
phenomenon, were seen in those with overlap features. Conclusion Results from a large
international juvenile SSc cohort demonstrate significant differences between juvenile
dcSSc and juvenile lcSSc patients, including more globally severe disease and increased
frequency of interstitial lung disease in juvenile dcSSc patients, while those with
lcSSc have more frequent cardiac involvement. Those with overlap features had an unexpected
higher frequency of interstitial lung disease.
