Tristano Antonio G
Department of Internal Medicine, Dr. Domingo Luciani Hospital, Caracas, Venezuela.
Med Sci Monit. 2008 Mar;14(3):RA27-36.
Macrophage activation syndrome (MAS) or hemophagocytic syndrome is a severe complication of chronic rheumatic diseases especially in systemic-onset juvenile rheumatoid arthritis (JRA). Although the cause of MAS is unknown, dysregulation of macrophage-lymphocyte interactions with subsequent increases in the levels of both T cell-derived and macrophage-derived cytokines could be involved in this syndrome, leading to an intense systemic inflammatory reaction, which accounts for the main clinical picture. Patients usually present with an acute febrile illness, hepatosplenomegaly, lymphadenopathy, cutaneous and mucosal bleeding, pancytopenia, and central nervous system, cardiac, and renal involvement. Treatment of MAS in patients with rheumatic diseases has not been standardized yet, but it commonly includes a variety of agents such as high-dose corticosteroids, cyclosporine, cyclophosphamide, etoposide, and intravenous immunoglobulin (IVIG). This article reviews the current literature about the pathogenesis, clinical manifestation, diagnosis, and treatment of this severe complication associated with rheumatic diseases.
巨噬细胞活化综合征（MAS）或噬血细胞综合征是慢性风湿性疾病的一种严重并发症，尤其在全身型幼年类风湿关节炎（JRA）中。尽管MAS的病因尚不清楚，但巨噬细胞与淋巴细胞相互作用失调，随后T细胞衍生和巨噬细胞衍生细胞因子水平升高，可能与该综合征有关，导致强烈的全身炎症反应，这构成了主要的临床表现。患者通常表现为急性发热性疾病、肝脾肿大、淋巴结病、皮肤和黏膜出血、全血细胞减少以及中枢神经系统、心脏和肾脏受累。风湿性疾病患者MAS的治疗尚未标准化，但通常包括多种药物，如大剂量皮质类固醇、环孢素、环磷酰胺、依托泊苷和静脉注射免疫球蛋白（IVIG）。本文综述了有关这种与风湿性疾病相关的严重并发症的发病机制、临床表现、诊断和治疗的当前文献。
