medwireNews: Patients with juvenile systemic sclerosis (SSc) experience a substantial burden of disease, and those with gastrointestinal (GI) involvement have the poorest quality of life (QoL), US researchers report.
In an analysis of data from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry for 64 children with SSc diagnosed at a median age of 10.3 years, the most commonly occurring organ manifestations were dermatologic and vascular, affecting a corresponding 93% and 92% of patients, followed by pulmonary, GI, and musculoskeletal effects, which occurred in 34–46% of patients.
The vast majority of patients had multiple affected organs, while more than a third had at least four, indicating “significant morbidity,” report the study authors in Arthritis Care & Research.
Participants with the worst patient- or caregiver-reported measures of QoL, including the Childhood Health Assessment Questionnaire and global wellbeing scores, were those with GI manifestations, whereas poorer physician-reported measures were associated with skin disease, but not GI involvement.
“This study identifies the importance of patient-centered outcome measures as an adjunct to physician measures in describing the disease burden of juvenile onset systemic sclerosis,” conclude Brandi Stevens (Riley Hospital for Children at Indiana University Health, Indianapolis) and fellow researchers.
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