Macrophage activation syndrome in a patient with systemic juvenile idiopathic arthritis

Abstract

r e v b r a s r e u m a t o l . 2 0 1 5;5 5(1):79-82 REVISTA BRASILEIRA DE REUMATOLOGIA w w w . r e u m a t o l o g i a . c o m . b r lymphohistiocytosis Epstein-Barr virus Systemic-onset juvenile idiopathic arthritis HLH-04 treatment protocol a b s t r a c t Machrophage activation syndrome (MAS) is a rare and potentially fatal disease, commonly associated with chronic rheumatic diseases, mainly juvenile idiopathic arthritis. It is included in the group of secondary forms of haemophagocytic syndrome, and other causes are lymphoproliferative diseases and infections. Its most important clinical and laboratorial manifestations are non-remitting fever, splenomegaly, bleeding, impairment of liver function, cytopenias, hypoalbuminemia, hypertriglyceridemia, hypofibrinogenemia and hyperferritinemia. The treatment needs to be started quickly, and the majority of cases have a good response with corticosteroids and cyclosporine. The Epstein-Barr virus is described as a possible trigger for many cases of MAS, especially in these patients in treatment with tumor necrosis factor (TNF) blockers. In these refractory cases, etoposide (VP16)should be administered, associated with corticosteroids and cyclosporine. Our objective is to describe a rare case of MAS probably due to EBV infection in a subject with systemiconset juvenile idiopathic arthritis, which achieved complete remission of the disease after therapy guided by 2004-HLH protocol.

Macrophage Activation Syndrome secondary to Systemic Juvenile Idiopathic Arthritis: A Case Report

“…For instance, a case of MAS was described secondary to Epstein-Barr virus (EBV) infection, in a patient with SoJIA, confirmed by typical clinical and laboratory manifestations, myelogram and positive serology against EBV. 5 However, MAS can occur without any identifiable triggers as was probably seen in our case. EBV and tuberculosis, are some of the common infections associated with MAS, which were excluded in our patient by appropriate tests.…”