Macrophage Activation Syndrome in Autoimmune Disease

作者：Deane Sean; Selmi Carlo; Teuber Suzanne S; Gershwin M Eric^*

来源：International Archives of Allergy and Immunology, 2010, 153(2): 109-120.

DOI：10.1159/000312628

• Autoimmune responses
• Autoimmunity
• Hemophagocytic syndromes
• Juvenile idiopathic arthritis
• Lymphohistiocytosis
• Macrophage activation syndrome
• Macrophages

Macrophage activation syndrome (MAS) is a phenomenon characterized by cytopenia, organ dysfunction, and coagulopathy associated with an inappropriate activation of macrophages. Current diagnostic criteria are imprecise, but the syndrome is now recognized as a form of hemophagocytic lymphohistiocytosis that is characteristically associated with autoimmune diatheses. The diagnosis of incipient MAS in patients with autoimmune disease requires a high index of suspicion, as several characteristics of the disorder may be present in the underlying condition or infectious complications associated with the treatment thereof. Proposed treatment regimens include aggressive approaches that require validation in future controlled studies. This review discusses the major aspects of the pathophysiology, diagnosis, and management of MAS with a focus on the association with autoimmune disease.

• 出版日期2010