Systemic Juvenile Idiopathic Arthritis and Adult Onset Still Disease

  • Peter A. NigrovicEmail author
  • Rayfel Schneider


Systemic juvenile idiopathic arthritis (sJIA) and adult onset Still disease (AOSD) are characterized by the triad of fever, rash and arthritis, together with high-grade systemic inflammation and other manifestations such as serositis, lymphadenopathy and hepatosplenomegaly. Patients exhibit considerable heterogeneity in disease severity and course, with some experiencing episodes of life-threatening inflammation termed macrophage activation syndrome. Pathophysiologic investigation into sJIA/AOSD implicates dysregulated immune control, with potential roles for both innate and adaptive immune cells, and for cytokines including interleukin (IL)-1β, IL-6, IL-18 and interferon (IFN)-γ. Therapeutic intervention with antagonists of IL-1 and IL-6 provide important clinical benefit for many patients.


Systemic juvenile idiopathic arthritis Adult-onset Still disease Fever Arthritis Interleukin Inflammasomes Macrophage activation syndrome Anakinra Rilonacept Canakinumab Tocilizumab 



American College of Rheumatology


Adult-onset Still disease


C-reactive protein


Damage-associated molecular pattern


Disseminated intravascular coagulation


Disease-modifying anti-rheumatic drug


Erythrocyte sedimentation rate


Fatty acid metabolism-immunity nexus


Genome-wide association study


Human leukocyte antigen


Hemophagocytic lymphohistiocyosis


Hematopoietic stem cell transplantation




Interleukin (X) receptor


IL-1 receptor antagonist


International League of Associations for Rheumatology




Intravenous immunoglobulin


Janus kinase


Juvenile idiopathic arthritis


Macrophage activation syndrome


Major histocompatibility complex


Mevalonate kinase deficiency


Natural killer


Non-steroid anti-inflammatory drugs


Soluble interleukin (X) receptor


Systemic juvenile idiopathic arthritis


Signal transducer and activator of transcription


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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Division of ImmunologyBoston Children’s Hospital, Harvard Medical SchoolBostonUSA
  2. 2.Division of Rheumatology, Allergy and ImmunologyCenter for Adults with Pediatric Rheumatic Illness, Brigham and Women’s Hospital, Harvard Medical SchoolBostonUSA
  3. 3.Department of PaediatricsThe Hospital for Sick Children, University of TorontoTorontoCanada

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