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Systemic Juvenile Idiopathic Arthritis and Adult Onset Still Disease

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Textbook of Autoinflammation

Abstract

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.

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Abbreviations

ACR:

American College of Rheumatology

AOSD:

Adult-onset Still disease

CRP:

C-reactive protein

DAMP:

Damage-associated molecular pattern

DIC:

Disseminated intravascular coagulation

DMARD:

Disease-modifying anti-rheumatic drug

ESR:

Erythrocyte sedimentation rate

FAMIN:

Fatty acid metabolism-immunity nexus

GWAS:

Genome-wide association study

HLA:

Human leukocyte antigen

HLH:

Hemophagocytic lymphohistiocyosis

HSCT:

Hematopoietic stem cell transplantation

IL:

Interleukin

IL-(X)R:

Interleukin (X) receptor

IL1ra:

IL-1 receptor antagonist

ILAR:

International League of Associations for Rheumatology

INF:

Interferon

IVIG:

Intravenous immunoglobulin

JAK:

Janus kinase

JIA:

Juvenile idiopathic arthritis

MAS:

Macrophage activation syndrome

MHC:

Major histocompatibility complex

MKD:

Mevalonate kinase deficiency

NK:

Natural killer

NSAIDs:

Non-steroid anti-inflammatory drugs

sIL-(X)R:

Soluble interleukin (X) receptor

sJIA:

Systemic juvenile idiopathic arthritis

STAT:

Signal transducer and activator of transcription

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Authors and Affiliations

  1. Division of Immunology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA

    Peter A. Nigrovic

  2. Division of Rheumatology, Allergy and Immunology, Center for Adults with Pediatric Rheumatic Illness, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

    Peter A. Nigrovic

  3. Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada

    Rayfel Schneider

Authors
  1. Peter A. Nigrovic
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  2. Rayfel Schneider
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Correspondence to Peter A. Nigrovic .

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Editors and Affiliations

  1. Pediatric Rheumatology Unit, Shaare Zedek Medical Center and Hebrew University, Jerusalem, Israel

    Philip J. Hashkes

  2. Department Paediatrics, Division of Rheumatology, The Hospital for Sick Children and University of Toronto, Toronto, ON, Canada

    Ronald M. Laxer

  3. Department of Internal Medicine, Radboudumc Expertisecenter for Immunodeficiency and Autoinflammation, Radboud University Medical Center, Nijmegen, The Netherlands

    Anna Simon

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Nigrovic, P.A., Schneider, R. (2019). Systemic Juvenile Idiopathic Arthritis and Adult Onset Still Disease. In: Hashkes, P., Laxer, R., Simon, A. (eds) Textbook of Autoinflammation. Springer, Cham. https://doi.org/10.1007/978-3-319-98605-0_32

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  • DOI: https://doi.org/10.1007/978-3-319-98605-0_32

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