Macrophage Activation Syndrome as the Initial Manifestation of Systemic Lupus Erythematosus in a 7-Year-Old Girl - A Case Report

Macrophage activation syndrome (MAS) is a potentially fatal complication of several chronic rheumatic diseases. Although it occurs most commonly with systemic onset juvenile idiopathic arthritis (SoJIA), it may also occur in association with systemic lupus erythematosus (SLE), Kawasaki disease, and adult onset Still’s disease. It is usually triggered by infections or due to medication modification. Here we report a 7-year-old girl who presented simultaneously with features of MAS and SLE and responded with pulse methylprednisolone followed by oral prednisolone and pulse cyclophosphamide.