oa Current Allergy & Clinical Immunology - Macrophage activation syndrome : PID article

Volume 29, Issue 3
  • ISSN : 1609-3607



Macrophage activation syndrome (MAS) is an uncommon condition in which hyperinflammation and excessive cytokine release lead to excessive stimulation of T-lymphocytes and macrophages and result in a characteristic clinical picture of haematological dysfunction and multiple organ failure. This condition occurs most commonly in the context of inflammatory conditions such as systemic juvenile idiopathic arthritis (sJIA). MAS is currently classified as one of the forms of secondary haemophagocytic lymphohistiocytosis (HLH). The diagnosis of MAS is clinically important as specific therapy may be required and a missed diagnosis may lead to high mortality. Unfortunately, the diagnosis is often not easily made due to the broad range of presenting features and their overlap with other potential complications of sJIA, such as severe infection. Also, no single clinical or laboratory test is able to diagnose MAS conclusively. In this review we highlight some of the most important clinical and laboratory features, diagnostic criteria, outcomes and management of MAS.

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