Macrophage activation syndrome in pediatrics: 10 years data from an Indian center.

Abstract

Macrophage activation syndrome (MAS) is a dreaded complication of systemic inflammatory diseases and is most commonly seen in systemic juvenile idiopathic arthritis (sJIA). We evaluate the clinical features, laboratory findings and outcomes in pediatric MAS, assess the response to different pharmacological therapies, and finally identify possible factors associated with an unfavorable outcome. This is a retrospective analysis of data from patients diagnosed as having MAS, admitted between July 2008 and April 2018 into the Department of Pediatric Rheumatology, Institute Of Child Health Kolkata. The data noted were the clinical and laboratory features, treatment details, responses to therapy and outcomes. Thirty-one patients were diagnosed as having MAS. Primary illness was sJIA in 26 (84%), systemic lupus erythematosus in 4 (13%) and Kawasaki disease (KD) in 1 (3%). All had fever with varying degrees of multisystemic involvement. Hyperferritinemia was universally present. Pulse methylprednisolone with cyclosporine was used for treating the majority. Ten patients (32%) expired. Macrophage activation syndrome is a near fatal complication with protean manifestations and multiorgan dysfunction. Hyperferritinemia is characteristic, higher values being associated with increased mortality. Cases resistant to steroids and cyclosporine had a poor prognosis. Late presentations with multiorgan dysfunction were associated with the poorest outcomes.