Systemic-onset juvenile idiopathic arthritis

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Systemic juvenile idiopathic arthritis
Other names: Systemic-onset juvenile rheumatoid arthritis, Still's Disease

Systemic juvenile idiopathic arthritis (or the juvenile onset form of Still's disease[1]) is a type of juvenile idiopathic arthritis (JIA) with extra-articular manifestations like fever and rash apart from arthritis. It was originally called systemic-onset juvenile rheumatoid arthritis or Still's disease.

Predominantly extra-articular manifestations like high fevers, rheumatic rash, enlargement of the liver and spleen, enlargement of the lymph nodes, and anemia. Other manifestations include inflammation of the pleura, inflammation of the pericardium, inflammation of the heart's muscular tissue, and inflammation of the peritoneum are also seen.[citation needed] It is sometimes called "juvenile-onset Still's disease" to distinguish it from adult-onset Still's disease. However, there is some evidence that the main difference between two conditions is the age of onset.[2]

Signs and symptoms

Systemic-onset juvenile idiopathic arthritis with salmon-colored, maculopapular rash

Systemic JIA is characterized by arthritis, fever, which typically is higher than the low-grade fever associated with polyarticular and a salmon pink rash. It accounts for 10-20% of JIA and affects males and females equally, unlike the other two subtypes of JIA, and affects adolescents. It generally involves both large and small joints. Systemic JIA can be challenging to diagnose because the fever and rash come and go. Fever can occur at the same time every day or twice a day (often in late afternoon or evening) with a spontaneous rapid return to baseline (vs. continuous fever of septic arthritis). The rash often occurs with fever. It is a discrete, salmon-pink macules of different sizes. It migrates to different locations on skin, rarely persisting in one location more than one hour. The rash is commonly seen on trunk and proximal extremities or over pressure areas.[citation needed]

Arthritis is often absent in the first weeks or even 6–8 months into the illness. Systemic JIA may have internal organ involvement such as hepatosplenomegaly, lymphadenopathy, serositis, hepatitis, or tenosynovitis.[citation needed]


The cause is unknown but it's thought to be related to environmental, genetic, and hormonal factors.[citation needed]

A polymorphism in macrophage migration inhibitory factor has been associated with this condition.[3]


Rheumatoid factor and ANA tests are generally negative in systemic JIA. Lab findings: anemia of chronic disease (can also appear in non-systemic types[4]), neutrophilia, thrombocytosis, elevated acute phase reactants (ESR, CRP, ferritin).[citation needed]


Treatment with either glucocorticoids, methotrexate, anakinra, or tocilizumab has been examined.[5] Anakinra has been shown to resolve the clinical features of the disease in 87% of patients.[6] It also induces remission in half of corticosteroid-resistant patients.[7] The results of another study were similar, with half of the patients responding to treatment with Anakinra.[8] Canakinumab, an antibody to interleukin-1 beta, is indicated for treatment in patients who respond poorly to other treatments.[9]


25% of cases progress to severe destructive arthritis.[10] In the United States, mortality is estimated at 4% [11] and in Europe, mortality is estimated at 21.7%.[12]


Still's disease is named after English physician Sir George Frederic Still (1861–1941).[13][14] It was characterized by EG Bywaters in 1971.[15][16]


  1. "Still's Disease". MedicineNet. Archived from the original on 9 June 2017. Retrieved 8 June 2017.
  2. Vastert SJ, Jamilloux Y, Quartier P, Ohlman S, Osterling Koskinen L, Kullenberg T, Franck-Larsson K, Fautrel B, de Benedetti F (2019). "Anakinra in children and adults with Still's disease". Rheumatology. 58 (Suppl 6): vi9–vi22. doi:10.1093/rheumatology/kez350. PMC 6878842. PMID 31769856.
  3. De Benedetti F, Meazza C, Vivarelli M, et al. (May 2003). "Functional and prognostic relevance of the -173 polymorphism of the macrophage migration inhibitory factor gene in systemic-onset juvenile idiopathic arthritis". Arthritis Rheum. 48 (5): 1398–407. doi:10.1002/art.10882. PMID 12746913.
  4. Archived 2022-03-14 at the Wayback Machine[bare URL PDF]
  5. DeWitt, Esi Morgan; Kimura, Yukiko; Beukelman, Timothy; Nigrovic, Peter A.; Onel, Karen; Prahalad, Sampath; Schneider, Rayfel; Stoll, Matthew L.; Angeles-Han, Sheila; Milojevic, Diana; Schikler, Kenneth N.; Vehe, Richard K.; Weiss, Jennifer E.; Weiss, Pamela; Ilowite, Norman T.; Wallace, Carol A. (1 January 2012). "Consensus treatment plans for new-onset systemic juvenile idiopathic arthritis". Arthritis Care & Research. 64 (7): 1001–10. doi:10.1002/acr.21625. PMC 3368104. PMID 22290637.
  6. Vastert, Sebastiaan J; De Jager, Wilco; Noordman, Bo; Prakken, Berent J; Wulffraat, Nico M (1 January 2012). "IL-1 receptor antagonist restores IL-18 NK cell axis in systemic JIA". Journal of Translational Medicine. 10 (Suppl 3): P45. doi:10.1186/1479-5876-10-S3-P45. PMC 3508836.
  7. Wulffraat, NM; de Jager, W; Prakken, B; Kuis, W (1 January 2008). "Early effects of Anakinra in corticosteroid naïve SOJIA patients". Pediatric Rheumatology. 6 (Suppl 1): P29. doi:10.1186/1546-0096-6-S1-P29. PMC 3334087.
  8. Gattorno, Marco; Piccini, Alessandra; Lasigliè, Denise; Tassi, Sara; Brisca, Giacomo; Carta, Sonia; Delfino, Laura; Ferlito, Francesca; Pelagatti, Maria Antonietta; Caroli, Francesco; Buoncompagni, Antonella; Viola, Stefania; Loy, Anna; Sironi, Marina; Vecchi, Annunciata; Ravelli, Angelo; Martini, Alberto; Rubartelli, Anna (1 May 2008). "The pattern of response to anti–interleukin-1 treatment distinguishes two subsets of patients with systemic-onset juvenile idiopathic arthritis". Arthritis & Rheumatism. 58 (5): 1505–1515. doi:10.1002/art.23437. PMID 18438814.
  9. Archived 2016-06-26 at the Wayback Machine[bare URL PDF]
  10. Singh-Grewal, D.; Schneider, R.; Bayer, N.; Feldman, B. M. (1 May 2006). "Predictors of disease course and remission in systemic juvenile idiopathic arthritis: Significance of early clinical and laboratory features". Arthritis & Rheumatism. 54 (5): 1595–1601. doi:10.1002/art.21774. PMID 16645998.
  11. Hoffman, F. "Background Information". Roche Group Media Relations. Archived 2016-03-04 at the Wayback Machine
  12. Davies, Rebecca; Southwood, T.; Kearsley-Fleet, L.; Lunt, M.; Hyrich, K. (2015). "Standardized Mortality Rates are Increased in Patients with Severe Juvenile Idiopathic Arthritis". Oxford Journal of Rheumatology. 54 (1): i153. Archived from the original on 2015-10-10. Retrieved 2023-09-27.
  13. synd/1773 at Who Named It?
  14. G. F. Still. A special form of joint disease met with in children. Doctoral dissertation, Cambridge, 1896.
  15. Bywaters EG (March 1971). "Still's disease in the adult". Ann. Rheum. Dis. 30 (2): 121–33. doi:10.1136/ard.30.2.121. PMC 1005739. PMID 5315135.
  16. Cimaz, R; Von, Scheven; Hofer, M (9 May 2012). "Systemic-onset juvenile idiopathic arthritis: the changing life of a rare disease" (PDF). Swiss Medical Weekly. 142: w13582. doi:10.4414/smw.2012.13582. PMID 22573189. Archived (PDF) from the original on 19 November 2023. Retrieved 27 September 2023.

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