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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
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
6-methylprednisolone 'mini-pulses': a new modality of glucocorticoid treatment in systemic onset juvenile chronic arthritis.
Picco P, Gattorno M, Buoncompagni A, Pistoia V, Borrone C
Scand J Rheumatol 1996;25(1):24-7. doi: 10.3109/03009749609082663. PMID: 8774551

Diagnosis

Lymphadenopathy in a patient with systemic onset juvenile chronic arthritis.
Munro R, Porter DR, Sturrock RD
Ann Rheum Dis 1998 Sep;57(9):513-7. doi: 10.1136/ard.57.9.513. PMID: 9849308Free 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
Bartonella henselae infection mimicking systemic onset juvenile chronic arthritis in a 2 1/2-year-old girl.
Hayem F, Chacar S, Hayem G
J Rheumatol 1996 Jul;23(7):1263-5. PMID: 8823702
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
Antiphospholipid antibody associated thrombosis in juvenile chronic arthritis.
Caporali R, Ravelli A, Ramenghi B, Montecucco C, Martini A
Arch Dis Child 1992 Nov;67(11):1384-5. doi: 10.1136/adc.67.11.1384. PMID: 1471893Free PMC Article

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
Lymphadenopathy in a patient with systemic onset juvenile chronic arthritis.
Munro R, Porter DR, Sturrock RD
Ann Rheum Dis 1998 Sep;57(9):513-7. doi: 10.1136/ard.57.9.513. PMID: 9849308Free PMC Article
6-methylprednisolone 'mini-pulses': a new modality of glucocorticoid treatment in systemic onset juvenile chronic arthritis.
Picco P, Gattorno M, Buoncompagni A, Pistoia V, Borrone C
Scand J Rheumatol 1996;25(1):24-7. doi: 10.3109/03009749609082663. PMID: 8774551
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
Antiphospholipid antibody associated thrombosis in juvenile chronic arthritis.
Caporali R, Ravelli A, Ramenghi B, Montecucco C, Martini A
Arch Dis Child 1992 Nov;67(11):1384-5. doi: 10.1136/adc.67.11.1384. PMID: 1471893Free PMC Article

Prognosis

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
Major histocompatibility complex markers and disease heterogeneity in one hundred eight patients with systemic onset juvenile chronic arthritis.
Desaymard C, Kaplan C, Fournier C, Manigne P, Hayem F, Kahn MF, Prieur AM
Rev Rhum Engl Ed 1996 Jan;63(1):9-16. PMID: 9064118
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
Increased frequency of DR4 in systemic onset juvenile chronic arthritis.
Bedford PA, Ansell BM, Hall PJ, Woo P
Clin Exp Rheumatol 1992 Mar-Apr;10(2):189-93. PMID: 1505114

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
6-methylprednisolone 'mini-pulses': a new modality of glucocorticoid treatment in systemic onset juvenile chronic arthritis.
Picco P, Gattorno M, Buoncompagni A, Pistoia V, Borrone C
Scand J Rheumatol 1996;25(1):24-7. doi: 10.3109/03009749609082663. PMID: 8774551
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
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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