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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
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
Thymopentin in the treatment of juvenile chronic arthritis.
Bardare M, Corona F, Ogliari MT, Cohen E
Clin Exp Rheumatol 1990 Jan-Feb;8(1):89-93. PMID: 1971780

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
The effect of novel polymorphisms in the interleukin-6 (IL-6) gene on IL-6 transcription and plasma IL-6 levels, and an association with systemic-onset juvenile chronic arthritis.
Fishman D, Faulds G, Jeffery R, Mohamed-Ali V, Yudkin JS, Humphries S, Woo P
J Clin Invest 1998 Oct 1;102(7):1369-76. doi: 10.1172/JCI2629. PMID: 9769329Free PMC Article
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
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

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
Juvenile chronic arthritis and Wegener's granulomatosis.
Wedderburn LR, Kwan JT, Thompson PW, Rudge SR
Br J Rheumatol 1992 Feb;31(2):121-3. doi: 10.1093/rheumatology/31.2.121. PMID: 1737227

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
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
Slow-acting antirheumatic drug use in systemic onset juvenile chronic arthritis.
Manners PJ, Ansell BM
Pediatrics 1986 Jan;77(1):99-103. PMID: 3940364

Prognosis

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
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
Defective iron supply for erythropoiesis and adequate endogenous erythropoietin production in the anemia associated with systemic-onset juvenile chronic arthritis.
Cazzola M, Ponchio L, de Benedetti F, Ravelli A, Rosti V, Beguin Y, Invernizzi R, Barosi G, Martini A
Blood 1996 Jun 1;87(11):4824-30. PMID: 8639855
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
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
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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