Macrophage Activation Syndrome in a Child with Systemic Juvenile Rheumatoid Arthritis

Abstract

Macrophage activation syndrome (MAS) is a rare and potentially fatal complication of rheumaticdisorders in children; we describe a 14-month old boy in whom MAS developed as first presentation ofsystemic juvenile rheumatoid arthritis (S-JRA). He suffered from fever, bleeding tendency from mouth,gastro-intestinal tract, ecchymosis & site of injections followed by bilateral wrist swellings. Physicalexamination revealed cervical lymphadenopathy & hepatosplenomegaly. Laboratory findings wereabnormal liver enzymes, coagulopathies resembling disseminated intravascular coagulation, anemia,thrombocytopenia and abnormal C.S.F. findings.Up to the best of my knowledge this could be the first MAS associated with S-JRA in Iraq.Key words: Macrophage Activation Syndrome, Arthritis, Juvenile Rheumatoid