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Systemic-onset juvenile idiopathic arthritis

Disease definition

A rare pediatric rheumatological disease characterized by the variable occurrence of chronic arthritis, intermittently high spiking fever, maculopapular rash during fever episodes, hepatomegaly and/or splenomegaly, lymphadenopathy, and serositis.

ORPHA:85414

Classification level: Disorder
  • Synonym(s):
    • Still disease
    • Systemic-onset JIA
  • Prevalence: 1-9 / 100 000
  • Inheritance: Unknown or Multigenic/multifactorial 
  • Age of onset: Childhood
  • ICD-10: M08.2
  • OMIM: 604302  618795
  • UMLS: C0087031  C1384600
  • MeSH: -
  • GARD: 10966
  • MedDRA: -
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