Book chapter
109 - Systemic autoimmune rheumatic diseases in children
Rheumatology, pp.936-950
Elsevier, Eighth Edition
2023
DOI: 10.1016/B978-0-7020-8133-0.00109-7
Abstract
Systemic autoimmune rheumatic diseases in children differ from their adult counterparts in important characteristics, including presentation, course, subtypes, responses to therapy, and morbidities. Juvenile-onset systemic lupus erythematosus is a severe, relapsing, chronic multisystem disease characterized by widespread inflammation and damage to any organ. There is a marked difference in the pattern of organ involvement compared with adult-onset disease, with more severe disease in childhood. Juvenile dermatomyositis is a complex multifactorial disease, the management of which requires multidisciplinary and multispecialty care over many years. Better recognition to aid rapid diagnosis and international collaborative efforts will ultimately lead to more individualized treatment choices and improved outcomes. Sjögren’s disease in children presents with gland-specific features (typically recurrent acute on chronic parotitis and much less commonly with sicca symptoms) in approximately 50% of children with the remainder presenting with nonspecific manifestations such as arthralgias with/without arthritis, neurologic symptoms, systemic symptoms (fevers, fatigue, musculoskeletal pain). Childhood scleroderma consists of two diseases, localized scleroderma (LS) and systemic sclerosis (SSc), with both showing important differences from adult-onset disease. Juvenile LS has a worse prognosis than adult disease related to differences in subtype pattern, high prevalence of extracutaneous involvement, and longer disease duration putting them at risk for severe morbidities such as joint contractures, facial hemiatrophy, and seizures. Juvenile SSc has a lower mortality risk than adult SSc, but morbidity remains prevalent, impacting their function and growth. Caring for children and young people with these complex diseases requires an age-appropriate and multidisciplinary approach, which includes the child’s growth and developmental needs as well as those of the family.
Details
- Title: Subtitle
- 109 - Systemic autoimmune rheumatic diseases in children
- Creators
- Joyce C. ChangSuzanne C. LiScott M. LiebermanDawn M. WaheziJay J. Mehta
- Resource Type
- Book chapter
- Publication Details
- Rheumatology, pp.936-950
- Edition
- Eighth Edition
- DOI
- 10.1016/B978-0-7020-8133-0.00109-7
- Publisher
- Elsevier; Philadelphia, PA
- Language
- English
- Date published
- 2023
- Academic Unit
- Stead Family Department of Pediatrics; Rheumatology, Allergy, and Immunology
- Record Identifier
- 9984949241402771
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