Journal article
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 & research (2010), Vol.70(12), pp.1806-1813
12/2018
DOI: 10.1002/acr.23547
PMCID: PMC6344352
PMID: 29457372
Abstract
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.
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.
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.
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.
Details
- Title: Subtitle
- 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
- Creators
- Brandi E Stevens - Riley Hospital for Children at Indiana University Health, Indianapolis, and University of Pittsburgh Medical Center Children's Hospital of Pittsburgh, Pittsburgh, PennsylvaniaKathryn S Torok - University of Pittsburgh Medical Center Children's Hospital of Pittsburgh, Pittsburgh, PennsylvaniaSuzanne C Li - Hackensack University Medical Center, Hackensack Meridian Health, Hackensack, New JerseyNicole Hershey - University of Pittsburgh Medical Center Children's Hospital of Pittsburgh, Pittsburgh, PennsylvaniaMegan Curran - Northwestern University Feinberg School of Medicine, Chicago, IllinoisGloria C Higgins - Ohio State University and Nationwide Children's Hospital, ColumbusKatharine F Moore - University of Colorado Children's Hospital Colorado, AuroraC. Egla Rabinovich - Duke University Medical Center, Durham, North CarolinaSamuel Dodson - University of Washington, SeattleAnne M Stevens - University of Washington, Seattle
- Contributors
- Polly J Ferguson (Contributor) - University of Iowa, Stead Family Department of PediatricsChildhood Arthritis and Rheumatology Research Alliance Registry Investigators (Author)
- Resource Type
- Journal article
- Publication Details
- Arthritis care & research (2010), Vol.70(12), pp.1806-1813
- Publisher
- United States
- DOI
- 10.1002/acr.23547
- PMID
- 29457372
- PMCID
- PMC6344352
- ISSN
- 2151-464X
- eISSN
- 2151-4658
- Grant note
- RC2AR058934 / NIH HHS\r\nK 23 AR059722 / NIAMS NIH HHS\r\nK23 AR059722 / NIAMS NIH HHS\r\nRC2 AR058934 / NIAMS NIH HHS
- Language
- English
- Date published
- 12/2018
- Academic Unit
- Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Rheumatology, Allergy, and Immunology
- Record Identifier
- 9984070223702771
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