Ramanan Athimalaipet V, Rosenblum Norman D, Feldman Brian M, Laxer Ronald M, Schneider Rayfel
Department of Pediatrics, Health Policy Management and Evaluation, and Public Health Sciences, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
J Rheumatol. 2004 Oct;31(10):2068-70.
Systemic-onset juvenile rheumatoid arthritis (SoJRA) constitutes about 10-20% of all JRA. However more than two-thirds of the mortality seen in JRA patients is accounted for by SoJRA. Macrophage activation syndrome (MAS), which can also be considered as a form of secondary hemophagocytic lymphohistiocytosis, is a major cause of morbidity and mortality in children with SoJRA. MAS is characterized by persistent high fever, pancytopenia, mild to serious derangements of liver cell function, encephalopathy, and disseminated intravascular coagulation. Renal involvement in MAS is a rarely recognized feature. In 2 recently reported case series of MAS in SoJRA, renal involvement appeared to be associated with poor prognosis. We describe 3 children with SoJRA who had renal involvement complicating MAS and had a favorable outcome.
全身型幼年类风湿性关节炎（SoJRA）约占所有幼年类风湿性关节炎（JRA）的10%-20%。然而，JRA患者中超过三分之二的死亡是由SoJRA导致的。巨噬细胞活化综合征（MAS），也可被视为继发性噬血细胞性淋巴组织细胞增生症的一种形式，是SoJRA患儿发病和死亡的主要原因。MAS的特征为持续高热、全血细胞减少、肝细胞功能轻至重度紊乱、脑病及弥散性血管内凝血。MAS累及肾脏是一个很少被认识到的特征。在最近报道的2个SoJRA的MAS病例系列中，肾脏受累似乎与预后不良有关。我们描述了3例SoJRA患儿，他们的肾脏受累使MAS复杂化，但预后良好。
