Macrophage activation syndrome in juvenile systemic lupus erythematosus: An under-recognized complication?

A. Pringe, L. Trail, N. Ruperto, A. Buoncompagni, A. Loy, L. Breda, A. Martini, Angelo Ravelli

Research output: Contribution to journalArticlepeer-review


Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatic diseases that is thought to be caused by the activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to widespread haemophagocytosis and cytokine overproduction. It is seen most commonly in systemic juvenile idiopathic arthritis, but is increasingly recognized also in juvenile systemic lupus erythematosus (J-SLE). Recognition of MAS in patients with J-SLE is often challenging because it may mimic the clinical features of the underlying disease or be confused with an infectious complication. This review summarizes the characteristics of patients with J-SLE-associated MAS reported in the literature or seen by the authors and analyses the distinctive clinical, diagnostic and therapeutic issues that the occurrence of MAS may raise in patients with J-SLE.

Original languageEnglish
Pages (from-to)587-592
Number of pages6
Issue number8
Publication statusPublished - 2007


  • Childhood
  • Juvenile-onset systemic lupus erythematosus
  • Macrophage activations syndrome
  • Paediatrics
  • Reactive haemophagocytic syndrome

ASJC Scopus subject areas

  • Immunology
  • Rheumatology


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