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Systemic onset juvenile idiopathic arthritis with macrophage activation syndrome misdiagnosed as Kawasaki disease: case report and literature review

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Abstract

Patients with systemic onset juvenile idiopathic arthritis (SoJIA) are rarely known to develop coronary artery dilatation. The American heart association (AHA) statement on evaluation of suspected Kawasaki disease (KD) would lead some SoJIA patients (particularly in the early stages of the disease) to be inaccurately classified as KD. In addition to the institution of inappropriate therapy with IVIG, misdiagnosis as KD can delay definitive treatment for these SoJIA patients who probably have a worse predicted outcome. We present a 6-year-old male patient with SoJIA who was initially classified as incomplete KD. The child developed life-threatening macrophage activation syndrome (MAS). Previous literature regarding coronary dilatation in SoJIA is also reviewed.

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Fig. 1

Abbreviations

AHA:

American Heart Association

DIC:

Disseminated intravascular coagulation

ECHO:

Echocardiography

HLH:

Hemophagocytic Lymphohistiocytosis

IVIG:

Intravenous immunoglobulin

KD:

Kawasaki disease

LAD:

Left anterior descending coronary artery

LMCA:

Left main coronary artery

MAS:

Macrophage activation syndrome

RCA:

Right coronary artery

SoJIA:

Systemic onset Juvenile idiopathic arthritis

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Authors and Affiliations

  1. Pediatric Rheumatology Clinic, Department of Pediatrics, Amrita Institute of Medical Sciences, AIMS Hospital, Ponekarra P.O., Cochin, 682041, Kerala, India

    Sharath Kumar

  2. Department of Pediatric Cardiology, Amrita Institute of Medical Sciences, Cochin, Kerala, India

    Balu Vaidyanathan

  3. Department of Pediatrics, Amrita Institute of Medical Sciences, Cochin, Kerala, India

    S. Gayathri & L. Rajam

Authors
  1. Sharath Kumar
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  2. Balu Vaidyanathan
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  3. S. Gayathri
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  4. L. Rajam
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Correspondence to Sharath Kumar.

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Kumar, S., Vaidyanathan, B., Gayathri, S. et al. Systemic onset juvenile idiopathic arthritis with macrophage activation syndrome misdiagnosed as Kawasaki disease: case report and literature review. Rheumatol Int 33, 1065–1069 (2013). https://doi.org/10.1007/s00296-010-1650-8

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  • DOI: https://doi.org/10.1007/s00296-010-1650-8

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