Systemic juvenile idiopathic arthritis: Course, prognosis, and complications
- Yukiko Kimura, MD
Yukiko Kimura, MD
- Professor of Pediatrics
- Rutgers University - New Jersey Medical School
- Division Chief of Pediatric Rheumatology
- Joseph M Sanzari Children's Hospital
- Hackensack University Medical Center
Systemic juvenile idiopathic arthritis (formerly called Still's disease or systemic-onset juvenile rheumatoid arthritis [JRA]) is a subset of juvenile idiopathic arthritis (JIA). Adult-onset Still's disease (AOSD) is probably the same or similar disease when it begins in patients ≥16 years of age. Systemic JIA is classified as a subset of JIA, but the pathophysiology is most consistent with an autoinflammatory disorder. (See "Classification of juvenile arthritis", section on 'Systemic arthritis'.)
Children with systemic JIA require close supervision and careful monitoring. Systemic complications, including infections, macrophage activation syndrome (MAS), pericarditis, pulmonary hypertension, interstitial lung disease, and other forms of internal organ involvement, are more common in this subtype of JIA than in any other and are associated with increased mortality [1-3].
The clinical course, prognosis, and complications of systemic JIA are discussed in this review. The clinical manifestations, diagnosis, and treatment of systemic JIA are discussed separately, as are oligoarticular and polyarticular JIA. (See "Systemic juvenile idiopathic arthritis: Clinical manifestations and diagnosis" and "Systemic juvenile idiopathic arthritis: Treatment" and "Classification of juvenile arthritis" and "Oligoarticular juvenile idiopathic arthritis" and "Polyarticular juvenile idiopathic arthritis: Clinical manifestations, diagnosis, and complications".)
The course of systemic JIA is highly variable, which has prompted many observers to suspect that systemic JIA is not a single entity. The typical child with systemic JIA experiences a several-month period of spiking fevers and rash, with varying degrees of arthralgia and arthritis. This is often followed by a relative quiescence of the systemic manifestations. However, there are some children who have persistently active systemic disease, which is associated with worse outcomes [2,4]. (See 'Prognosis' below.)
Generally speaking, there are three disease-course patterns: monophasic, polycyclic, and persistent [4-6]. The actual proportions of systemic JIA patients who follow these disease course patterns are unclear since all studies looking at disease course were single-center studies and the definitions of remission and inactive disease were variable.
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