title: Macrophage activation syndrome in autoimmune disease.
abstract: 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.
authors.affiliation: Division of Rheumatology, Allergy and Clinical Immunology, University of California, Davis, CA 95616, USA. megershwin@ucdavis.edu
authors.forename: Sean
authors.initials: S
authors.lastname: Deane
authors.forename: Carlo
authors.initials: C
authors.lastname: Selmi
authors.forename: Suzanne S
authors.initials: SS
authors.lastname: Teuber
authors.forename: M Eric
authors.initials: ME
authors.lastname: Gershwin
mesh_terms: D001327:Autoimmune Diseases / Q000276:immunology*; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology; D006801:Humans; D007694:Killer Cells, Natural / Q000276:immunology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis; D008262:Macrophage Activation; D055501:Macrophage Activation Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology; D012216:Rheumatic Diseases / Q000276:immunology