Systemic onset juvenile idiopathic arthritis (also called systemic juvenile idiopathic arthritis [sJIA] and formerly called systemic onset juvenile rheumatoid arthritis or Still's disease) is a subset of juvenile idiopathic arthritis (JIA) that describes patients with fever, rash, and arthritis. It is sometimes called adult-onset Still's disease when it occurs in patients over the age of 16.
The treatment options for systemic onset juvenile idiopathic arthritis are discussed in this review. The clinical manifestations, diagnosis, complications, and prognosis of systemic onset juvenile idiopathic arthritis are discussed separately, as are pauciarthritis and polyarthritis. (See "Systemic juvenile idiopathic arthritis: Clinical manifestations and diagnosis" and "Systemic onset juvenile idiopathic arthritis: Course, prognosis, and complications" and "Pauciarticular onset juvenile idiopathic arthritis" and "Polyarticular onset juvenile idiopathic arthritis: Clinical manifestations and diagnosis" and "Classification of juvenile arthritis (JRA/JIA)" and "Treatment and prognosis of hemophagocytic lymphohistiocytosis".)
Nonsteroidal antiinflammatory drugs (NSAIDs) alone are effective for many children with systemic onset juvenile idiopathic arthritis (sJIA) . Second line agents, such as glucocorticoids or methotrexate, are used if NSAIDs are ineffective. Biologic agents, such as monoclonal antibodies to interleukin-1 (IL-1) or IL-6, are effective in reducing clinical symptoms in patients with disease refractory to conventional therapy and more limited data suggest they are also beneficial when used early in the care of ill children with systemic onset JIA (ie, instead of glucocorticoids) once the diagnosis is confirmed.
The initiation of any therapy in sJIA including NSAIDs, but probably not glucocorticoids, has been associated anecdotally with a transiently elevated risk of life-threatening macrophage activation syndrome (MAS). Accordingly, careful monitoring is essential. Since glucocorticoids represent a poor long-term therapeutic option because of associated toxicity, additional disease-modifying drugs are required in many patients to avoid the aggressive and often permanently disabling consequences of unremitting disease. Although the following discussion is an attempt to give useful guidelines, difficult cases of systemic onset JIA should be referred to an experienced pediatric rheumatologist. (See "Treatment and prognosis of hemophagocytic lymphohistiocytosis".)
The administration of live-viral vaccines and other standard childhood immunizations in patients with JIA is discussed in detail separately. (See "Pauciarticular onset juvenile idiopathic arthritis", section on 'Immunizations'.)