Tavares Anna Carolina Faria Moreira Gomes, Ferreira Gilda Aparecida, Guimarães Luciano Junqueira, Guimarães Raquel Rosa, Santos Flávia Patrícia Sena Teixeira
Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brasil.
Departamento do Aparelho Locomotor, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brasil.
Rev Bras Reumatol. 2015 Jan-Feb;55(1):79-82. doi: 10.1016/j.rbr.2014.02.007. Epub 2014 Aug 27.
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 systemic-onset juvenile idiopathic arthritis, which achieved complete remission of the disease after therapy guided by 2004-HLH protocol.
巨噬细胞活化综合征（MAS）是一种罕见且可能致命的疾病，通常与慢性风湿性疾病相关，主要是幼年特发性关节炎。它属于噬血细胞综合征的继发形式，其他病因包括淋巴增殖性疾病和感染。其最重要的临床和实验室表现为持续发热、脾肿大、出血、肝功能损害、血细胞减少、低白蛋白血症、高甘油三酯血症、低纤维蛋白原血症和高铁蛋白血症。治疗需要迅速开始，大多数病例对皮质类固醇和环孢素反应良好。爱泼斯坦-巴尔病毒被认为是许多MAS病例的可能触发因素，尤其是在使用肿瘤坏死因子（TNF）阻滞剂治疗的患者中。在这些难治性病例中，应给予依托泊苷（VP16），并联合皮质类固醇和环孢素。我们的目的是描述一例可能由EBV感染引起的MAS罕见病例，该病例发生在一名全身型幼年特发性关节炎患者身上，在2004-HLH方案指导下的治疗后疾病实现了完全缓解。
