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An important complication of a child with juvenile idiopathic arthritis: macrophage activation syndrome

Yuvaram N V Reddy1, Navin Bhatia, Julius Xavier Scott

  • 1Sri Ramachandra Medical College and Research Institute, Chennai, Tamil Nadu, India.

BMJ Case Reports
|September 13, 2013
PubMed

Insights

A 4-year-old girl with juvenile idiopathic arthritis developed macrophage activation syndrome, a serious complication. Prompt treatment with corticosteroids led to a good recovery.

Area of Science:

  • Pediatrics
  • Rheumatology
  • Hematology

Background:

  • Juvenile idiopathic arthritis (JIA) is a chronic autoimmune disease.
  • Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatic diseases.

Observation:

  • A 4-year-old girl with a 2-year history of JIA presented with persistent fever and abdominal distension.
  • Physical examination revealed pallor, significant cervical lymphadenopathy, hepatomegaly, and splenomegaly.
  • Laboratory findings included leukocytosis, anemia, thrombocytopenia, elevated erythrocyte sedimentation rate, markedly elevated serum ferritin, and lactate dehydrogenase.

Findings:

  • Bone marrow aspirate demonstrated macrophages with ingested nuclei, indicative of hemophagocytosis.
  • The patient was diagnosed with macrophage activation syndrome secondary to JIA.
  • Treatment involved initial intravenous methylprednisolone followed by oral prednisolone.

Implications:

  • This case highlights the critical importance of recognizing MAS in pediatric patients with JIA.
  • Early diagnosis and aggressive immunosuppressive therapy are crucial for favorable outcomes in MAS.
  • MAS represents a significant challenge in managing pediatric rheumatic diseases, necessitating heightened clinical vigilance.

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