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Systemic onset juvenile chronic arthritis

MedGen UID:
730498
•Concept ID:
C1384600
•
Disease or Syndrome
Synonyms: Juvenile arthritis with systemic onset; Systemic Juvenile Idiopathic Arthritis; Systemic juvenile idiopathic arthritis; Systemic Onset Juvenile Idiopathic Arthritis; Systemic onset juvenile rheumatoid arthritis
SNOMED CT: Systemic onset juvenile rheumatoid arthritis (201796004); Systemic juvenile idiopathic arthritis (201796004); Systemic onset juvenile chronic arthritis (201796004); Juvenile arthritis with systemic onset (201796004)

Definition

A category of juvenile idiopathic arthritis defined by the presence of arthritis and high fevers, and accompanied by at least 2 of the following systemic features: lymphadenopathy, organomegaly, rash, or serositis. Macrophage activation syndrome is a well known complication. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSystemic onset juvenile chronic arthritis

Professional guidelines

PubMed

The use of intravenous pulsed methylprednisolone in the treatment of systemic-onset juvenile chronic arthritis.
Adebajo AO, Hall MA
Br J Rheumatol 1998 Nov;37(11):1240-2. doi: 10.1093/rheumatology/37.11.1240. PMID: 9851278
Early treatment of systemic onset juvenile chronic arthritis with low-dose cyclosporin A.
Gattorno M, Buoncompagni A, Faraci M, Pistoia V, Picco P
Clin Exp Rheumatol 1995 May-Jun;13(3):409-10. PMID: 7554576
Treatment of refractory juvenile chronic arthritis by monoclonal CD4 antibodies: a pilot study in two children.
Horneff G, Dirksen U, Schulze-Koops H, Emmrich F, Wahn V
Ann Rheum Dis 1995 Oct;54(10):846-9. doi: 10.1136/ard.54.10.846. PMID: 7492226Free PMC Article

Recent clinical studies

Etiology

Systemic onset juvenile chronic arthritis, polyarticular pattern and hip involvement as markers for a bad prognosis.
Modesto C, Woo P, García-Consuegra J, Merino R, García-Granero M, Arnal C, Prieur AM
Clin Exp Rheumatol 2001 Mar-Apr;19(2):211-7. PMID: 11326488
A possible role for soluble IL-6 receptor in the pathogenesis of systemic onset juvenile chronic arthritis.
Keul R, Heinrich PC, Müller-newen G, Muller K, Woo P
Cytokine 1998 Sep;10(9):729-34. doi: 10.1006/cyto.1997.0343. PMID: 9770335
Correlates of disablement in systemic onset juvenile chronic arthritis. A cross sectional study.
van der Net J, Kuis W, Prakken AB, Lukkassen I, Sinnema G, Hutter AP, Brackel HJ, de Wilde EJ, Helders PJ
Scand J Rheumatol 1997;26(3):188-96. doi: 10.3109/03009749709065680. PMID: 9225874
Factors associated with response to methotrexate in systemic-onset juvenile chronic arthritis.
Ravelli A, Ramenghi B, Di Fuccia G, Ruperto N, Zonta L, Martini A
Acta Paediatr 1994 Apr;83(4):428-32. doi: 10.1111/j.1651-2227.1994.tb18135.x. PMID: 8025404
Slow-acting antirheumatic drug use in systemic onset juvenile chronic arthritis.
Manners PJ, Ansell BM
Pediatrics 1986 Jan;77(1):99-103. PMID: 3940364

Diagnosis

Systemic onset juvenile chronic arthritis (JCA) in a Nigerian boy--a case report.
Oguntona AS, Adelowo O
Niger J Med 2008 Jan-Mar;17(1):112-4. doi: 10.4314/njm.v17i1.37369. PMID: 18390147
Macrophage activation syndrome in children with systemic-onset juvenile chronic arthritis.
Kounami S, Yoshiyama M, Nakayama K, Okuda M, Okuda S, Aoyagi N, Yoshikawa N
Acta Haematol 2005;113(2):124-9. doi: 10.1159/000083450. PMID: 15802891
Systemic onset juvenile chronic arthritis, polyarticular pattern and hip involvement as markers for a bad prognosis.
Modesto C, Woo P, García-Consuegra J, Merino R, García-Granero M, Arnal C, Prieur AM
Clin Exp Rheumatol 2001 Mar-Apr;19(2):211-7. PMID: 11326488
Thrombocytopenia in systemic-onset juvenile chronic arthritis: report of two cases with unusual bone marrow features.
Lin SJ, Jaing TH
Clin Rheumatol 1999;18(3):241-3. doi: 10.1007/s100670050092. PMID: 11206351
Correlates of disablement in systemic onset juvenile chronic arthritis. A cross sectional study.
van der Net J, Kuis W, Prakken AB, Lukkassen I, Sinnema G, Hutter AP, Brackel HJ, de Wilde EJ, Helders PJ
Scand J Rheumatol 1997;26(3):188-96. doi: 10.3109/03009749709065680. PMID: 9225874

Therapy

Macrophage activation syndrome in children with systemic-onset juvenile chronic arthritis.
Kounami S, Yoshiyama M, Nakayama K, Okuda M, Okuda S, Aoyagi N, Yoshikawa N
Acta Haematol 2005;113(2):124-9. doi: 10.1159/000083450. PMID: 15802891
Thrombocytopenia in systemic-onset juvenile chronic arthritis: report of two cases with unusual bone marrow features.
Lin SJ, Jaing TH
Clin Rheumatol 1999;18(3):241-3. doi: 10.1007/s100670050092. PMID: 11206351
Treatment of refractory juvenile chronic arthritis by monoclonal CD4 antibodies: a pilot study in two children.
Horneff G, Dirksen U, Schulze-Koops H, Emmrich F, Wahn V
Ann Rheum Dis 1995 Oct;54(10):846-9. doi: 10.1136/ard.54.10.846. PMID: 7492226Free PMC Article
Intravenous iron therapy for severe anaemia in systemic-onset juvenile chronic arthritis.
Martini A, Ravelli A, Di Fuccia G, Rosti V, Cazzola M, Barosi G
Lancet 1994 Oct 15;344(8929):1052-4. doi: 10.1016/s0140-6736(94)91710-8. PMID: 7864944
Low-dose methotrexate in systemic onset juvenile chronic arthritis.
Speckmaier M, Findeisen J, Woo P, Hall A, Sills JA, Price T, Hollingworth P, Craft A, Ansell BM
Clin Exp Rheumatol 1989 Nov-Dec;7(6):647-50. PMID: 2612084

Prognosis

Reactive haemophagocytic syndrome in children with inflammatory disorders. A retrospective study of 24 patients.
Stéphan JL, Koné-Paut I, Galambrun C, Mouy R, Bader-Meunier B, Prieur AM
Rheumatology (Oxford) 2001 Nov;40(11):1285-92. doi: 10.1093/rheumatology/40.11.1285. PMID: 11709613
Systemic onset juvenile chronic arthritis, polyarticular pattern and hip involvement as markers for a bad prognosis.
Modesto C, Woo P, García-Consuegra J, Merino R, García-Granero M, Arnal C, Prieur AM
Clin Exp Rheumatol 2001 Mar-Apr;19(2):211-7. PMID: 11326488
Thrombocytopenia in systemic-onset juvenile chronic arthritis: report of two cases with unusual bone marrow features.
Lin SJ, Jaing TH
Clin Rheumatol 1999;18(3):241-3. doi: 10.1007/s100670050092. PMID: 11206351
Treatment of refractory juvenile chronic arthritis by monoclonal CD4 antibodies: a pilot study in two children.
Horneff G, Dirksen U, Schulze-Koops H, Emmrich F, Wahn V
Ann Rheum Dis 1995 Oct;54(10):846-9. doi: 10.1136/ard.54.10.846. PMID: 7492226Free PMC Article
Factors associated with response to methotrexate in systemic-onset juvenile chronic arthritis.
Ravelli A, Ramenghi B, Di Fuccia G, Ruperto N, Zonta L, Martini A
Acta Paediatr 1994 Apr;83(4):428-32. doi: 10.1111/j.1651-2227.1994.tb18135.x. PMID: 8025404

Clinical prediction guides

IL-6 in autoimmune disease and chronic inflammatory proliferative disease.
Ishihara K, Hirano T
Cytokine Growth Factor Rev 2002 Aug-Oct;13(4-5):357-68. doi: 10.1016/s1359-6101(02)00027-8. PMID: 12220549
Systemic onset juvenile chronic arthritis, polyarticular pattern and hip involvement as markers for a bad prognosis.
Modesto C, Woo P, García-Consuegra J, Merino R, García-Granero M, Arnal C, Prieur AM
Clin Exp Rheumatol 2001 Mar-Apr;19(2):211-7. PMID: 11326488
Treatment of refractory juvenile chronic arthritis by monoclonal CD4 antibodies: a pilot study in two children.
Horneff G, Dirksen U, Schulze-Koops H, Emmrich F, Wahn V
Ann Rheum Dis 1995 Oct;54(10):846-9. doi: 10.1136/ard.54.10.846. PMID: 7492226Free PMC Article
Factors associated with response to methotrexate in systemic-onset juvenile chronic arthritis.
Ravelli A, Ramenghi B, Di Fuccia G, Ruperto N, Zonta L, Martini A
Acta Paediatr 1994 Apr;83(4):428-32. doi: 10.1111/j.1651-2227.1994.tb18135.x. PMID: 8025404
Soluble interleukin-2 receptors in children with juvenile chronic arthritis.
Fassbender K, Michels H, Vogt P, Aeschlimann A, Müller W
Scand J Rheumatol 1992;21(3):120-3. doi: 10.3109/03009749209095082. PMID: 1376493

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