Letter to the Editor

Macrophage Activation Syndrome Associated with Systemic Onset Juvenile Rheumatoid Arthritis

Authors: Antonio G. Tristano, MD, MSC

Abstract

Coagulation abnormalities in systemic onset juvenile rheumatoid arthritis (JRA) could be due to two different pathogenic mechanisms; one caused by vasculitis and the other induced by macrophage activation syndrome (MAS). MAS is now widely recognized as a severe, potentially life-threatening complication of systemic onset JRA; therefore, early diagnosis and immediate treatment are indispensable for a good outcome. In patients with systemic onset JRA, MAS needs to be differentiated from an acute exacerbation of the underlying disease through clinical and laboratory findings, including pancytopenia, persistent fever, coagulation abnormalities, bleeding, liver and neurologic abnormalities, and phagocytosis of bone marrow-derived elements (hematophagocytic histiocytes).

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