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Macrophage activation syndrome in 13 children with systemic-onset juvenile idiopathic arthritis

Hua-Song Zeng1, Xiao-Yan Xiong, Yan-Dan Wei

  • 1Department of Allergy, Immunology and Rheumatology, Guangzhou Children's Hospital, Guangzhou 510120, China. huasongz@gmail.com

World Journal of Pediatrics : WJP
|July 30, 2008
PubMed

Insights

Macrophage activation syndrome (MAS) is a severe complication of systemic-onset juvenile idiopathic arthritis (SoJIA). Early recognition and treatment of MAS in children are crucial for reducing mortality rates.

Area of Science:

  • Pediatric Rheumatology
  • Immunology
  • Hematology

Background:

  • Macrophage activation syndrome (MAS) is a life-threatening condition associated with chronic rheumatic diseases, particularly systemic-onset juvenile idiopathic arthritis (SoJIA) in children.
  • MAS presents with diverse clinical and laboratory findings, often lacking specific characteristics, making early diagnosis challenging.

Purpose of the Study:

  • To analyze the clinical and laboratory features of SoJIA patients diagnosed with MAS.
  • To identify key characteristics that aid in the early recognition and management of MAS in pediatric patients.

Main Methods:

  • Retrospective analysis of clinical and laboratory data from 13 SoJIA patients with MAS.
  • Data collected from January 2003 to October 2007.

Main Results:

  • The study included 13 patients (9 boys, 4 girls) aged 5 months to 12 years.
  • Common manifestations included persistent fever, anemia, arthritis, hepatosplenomegaly, lymphadenopathy, liver dysfunction, abnormal lipid metabolism, and bone marrow hemophagocytosis.
  • Severe complications such as acute respiratory distress syndrome and multi-organ failure occurred, with a mortality rate of 23% (3 out of 13 patients).
  • Perforin A91V gene analysis in 6 patients showed normal results.
  • Glucocorticoids, immunosuppressive therapy, and plasmapheresis (in one severe case) demonstrated effectiveness.

Conclusions:

  • MAS is a critical complication of juvenile idiopathic arthritis (JIA), especially the systemic-onset form.
  • Prompt diagnosis and timely intervention are essential to improve outcomes and reduce mortality in pediatric MAS patients.
Abstract

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