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Clinical Characteristics and Factors Associated With Disability and Impaired Quality of Life in Children With Juvenile Systemic Sclerosis: Results From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry. Arthritis Care Res (Hoboken) 2018 12;70(12):1806-1813



Pubmed ID


Pubmed Central ID




Scopus ID

2-s2.0-85050078257   5 Citations


OBJECTIVE: To investigate clinical manifestations of juvenile systemic sclerosis (SSc; scleroderma), including disease characteristics and patient quality of life, using the multinational Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry.

METHODS: Patients with juvenile SSc were prospectively enrolled between 2010 and 2013. The diagnosis of juvenile SSc was determined by the enrolling pediatric rheumatologist, with the requirement for disease onset prior to age 18 years. Collected data included demographics, disease characteristics, medication exposure, and quality of life metrics.

RESULTS: In total, 64 patients with juvenile SSc were enrolled a median of 3.6 years after disease onset, which occurred at a median age of 10.3 years. The most common organ manifestations were dermatologic and vascular, followed by musculoskeletal, gastrointestinal, and pulmonary; in 38% of patients, ≥4 organ systems were affected. Patients with juvenile SSc had significantly more disability at enrollment compared with CARRA Legacy Registry patients with juvenile idiopathic arthritis, dermatomyositis, or systemic lupus erythematosus. Although physician-reported measures correlated most closely with arthritis, dermatologic manifestations, and pulmonary manifestations, poor patient-reported measures were associated with gastrointestinal involvement. During >50 person-years of follow-up, most organ manifestations remained stable, and no mortality or development of new solid organ involvement after enrollment was reported.

CONCLUSION: In the first multicenter prospective cohort of patients with juvenile SSc in North America, the disease burden was high: multiorgan manifestations were common, and functional disability was greater than that observed in patients with other childhood-onset rheumatic diseases. Gastrointestinal involvement had the greatest impact on quality of life.

Author List

Stevens BE, Torok KS, Li SC, Hershey N, Curran M, Higgins GC, Moore KF, Egla Rabinovich C, Dodson S, Stevens AM, Childhood Arthritis and Rheumatology Research Alliance Registry Investigators


Judyann C. Olson MD Associate Professor in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Age of Onset
Cost of Illness
Disability Evaluation
North America
Patient Reported Outcome Measures
Predictive Value of Tests
Prospective Studies
Quality of Life
Risk Factors
Scleroderma, Systemic
Time Factors
jenkins-FCD Prod-480 9a4deaf152b0b06dd18151814fff2e18f6c05280