Macrophage activation syndrome in childhood rheumatic diseases

Angelo Ravelli, Alejandra Pringe, Clara Malattia, Ilaria Sala, Alberto Martini

Research output: Contribution to journalArticlepeer-review


Macrophage activation syndrome (MAS) is a life-threatening complication of systemic inflammatory disorders that is thought to be caused by the activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to widespread hemophagocytosis and cytokine overproduction. Recent findings in hemophagocytic lymphohistiocytosis, a disease that is clinically very similar to MAS, highlight the possible pathogenetic role of a defective function of cytotoxic lymphocytes. Prompt diagnosis is important and recently preliminary diagnostic guidelines for the syndrome have been proposed. The recognition that MAS belongs to the secondary or reactive hemophagocytic syndromes has led to propose to rename it according to the contemporary classification of histiocytic disorders. Cyclosporine A has been found to be effective in patients with corticosteroid resistant MAS.

Original languageEnglish
Pages (from-to)225-230
Number of pages6
JournalCurrent Rheumatology Reviews
Issue number3
Publication statusPublished - Aug 2007


  • Hemophagocytic lymphohistiocytosis
  • Hemophagocytic syndrome
  • Macrophage activation syndrome
  • Systemic juvenile idiopathic arthritis

ASJC Scopus subject areas

  • Rheumatology


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