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Systemic juvenile idiopathic arthritis: Clinical manifestations and diagnosis

Yukiko Kimura, MD
Section Editor
Marisa Klein-Gitelman, MD, MPH
Deputy Editor
Elizabeth TePas, MD, MS


Systemic juvenile idiopathic arthritis (sJIA, formerly called Still's disease or systemic juvenile rheumatoid arthritis) is officially classified as a subset of JIA that includes patients characterized by daily quotidian fever, rash, and arthritis. It is termed "adult-onset Still's disease" when it begins in patients over the age of 16 years. sJIA accounts for approximately 10 to 20 percent of all cases of JIA. It typically affects both sexes equally and may present in children as young as one year of age or younger. (See "Juvenile idiopathic arthritis: Epidemiology and immunopathogenesis".)

The clinical manifestations and diagnosis of sJIA are reviewed here (table 1). The management, complications, and prognosis of sJIA, as well as oligoarticular, polyarticular, enthesitis-related, and psoriatic JIA, and general topics on JIA are discussed separately. (See "Systemic juvenile idiopathic arthritis: Treatment" and "Systemic juvenile idiopathic arthritis: Course, prognosis, and complications" and "Oligoarticular juvenile idiopathic arthritis" and "Polyarticular juvenile idiopathic arthritis: Clinical manifestations, diagnosis, and complications" and "Spondyloarthritis in children" and "Psoriatic juvenile idiopathic arthritis: Pathogenesis, clinical manifestations, and diagnosis" and "Psoriatic juvenile idiopathic arthritis: Management and prognosis" and "Classification of juvenile arthritis" and "Juvenile idiopathic arthritis: Epidemiology and immunopathogenesis".)


Patients with sJIA fall into the category of systemic arthritis in the 2004 International League of Associations for Rheumatology (ILAR) proposed classification of the childhood arthritides [1]. Children with this illness comprise between 10 and 20 percent of all cases of JIA. However, data suggest that this illness is a unique condition closer to the autoinflammatory family of diseases, with distinct manifestations and treatment responses that distinguish it from the other diseases categorized as JIA [2,3]. (See "Classification of juvenile arthritis" and "Periodic fever syndromes and other autoinflammatory diseases: An overview".)

This form of JIA is very distinctive but may be the most difficult to diagnose and treat for the following reasons:

Arthritis, although necessary to establish a definitive diagnosis, may not be evident early in the course of the disease [1,4].

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Literature review current through: Nov 2017. | This topic last updated: Apr 27, 2016.
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  1. Petty RE, Southwood TR, Manners P, et al. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol 2004; 31:390.
  2. Prakken B, Albani S, Martini A. Juvenile idiopathic arthritis. Lancet 2011; 377:2138.
  3. Nigrovic PA. Review: is there a window of opportunity for treatment of systemic juvenile idiopathic arthritis? Arthritis Rheumatol 2014; 66:1405.
  4. Behrens EM, Beukelman T, Gallo L, et al. Evaluation of the presentation of systemic onset juvenile rheumatoid arthritis: data from the Pennsylvania Systemic Onset Juvenile Arthritis Registry (PASOJAR). J Rheumatol 2008; 35:343.
  5. Hensinger RN, DeVito PD, Ragsdale CG. Changes in the cervical spine in juvenile rheumatoid arthritis. J Bone Joint Surg Am 1986; 68:189.
  6. Prendiville JS, Tucker LB, Cabral DA, Crawford RI. A pruritic linear urticarial rash, fever, and systemic inflammatory disease in five adolescents: adult-onset still disease or systemic juvenile idiopathic arthritis sine arthritis? Pediatr Dermatol 2004; 21:580.
  7. Parvez N, Carpenter JL. Cardiac tamponade in Still disease: a review of the literature. South Med J 2009; 102:832.
  8. Kimura Y, Weiss JE, Haroldson KL, et al. Pulmonary hypertension and other potentially fatal pulmonary complications in systemic juvenile idiopathic arthritis. Arthritis Care Res (Hoboken) 2013; 65:745.
  9. Dogra S, Gehlot A, Suri D, et al. Incomplete Kawasaki disease followed by systemic onset juvenile idiopathic arthritis- the diagnostic dilemma. Indian J Pediatr 2013; 80:783.
  10. Kumar S, Vaidyanathan B, Gayathri S, Rajam L. Systemic onset juvenile idiopathic arthritis with macrophage activation syndrome misdiagnosed as Kawasaki disease: case report and literature review. Rheumatol Int 2013; 33:1065.
  11. Binstadt BA, Levine JC, Nigrovic PA, et al. Coronary artery dilation among patients presenting with systemic-onset juvenile idiopathic arthritis. Pediatrics 2005; 116:e89.
  12. Harvey AR, Pippard MJ, Ansell BM. Microcytic anaemia in juvenile chronic arthritis. Scand J Rheumatol 1987; 16:53.
  13. Martini A, Ravelli A, Di Fuccia G, et al. Intravenous iron therapy for severe anaemia in systemic-onset juvenile chronic arthritis. Lancet 1994; 344:1052.
  14. Gorelik M, Fall N, Altaye M, et al. Follistatin-like protein 1 and the ferritin/erythrocyte sedimentation rate ratio are potential biomarkers for dysregulated gene expression and macrophage activation syndrome in systemic juvenile idiopathic arthritis. J Rheumatol 2013; 40:1191.
  15. Pelkonen P, Swanljung K, Siimes MA. Ferritinemia as an indicator of systemic disease activity in children with systemic juvenile rheumatoid arthritis. Acta Paediatr Scand 1986; 75:64.
  16. Cassidy JT, Petty RE. Juvenile rheumatoid arthritis. In: Textbook of Pediatric Rheumatology, 4th ed, Cassidy JT, Petty RE (Eds), WB Saunders Company, Philadelphia 2001. p.218.
  17. DeWitt EM, Kimura Y, Beukelman T, et al. Consensus treatment plans for new-onset systemic juvenile idiopathic arthritis. Arthritis Care Res (Hoboken) 2012; 64:1001.
  18. Cush JJ, Medsger TA Jr, Christy WC, et al. Adult-onset Still's disease. Clinical course and outcome. Arthritis Rheum 1987; 30:186.
  19. Nocton JJ, Miller LC, Tucker LB, Schaller JG. Human parvovirus B19-associated arthritis in children. J Pediatr 1993; 122:186.
  20. Cabral DA, Tucker LB. Malignancies in children who initially present with rheumatic complaints. J Pediatr 1999; 134:53.