幼年特发性关节炎全身型并发巨噬细胞活化综合征13例临床分析

目的对13例幼年特发性关节炎全身型（SOJIA）合并巨噬细胞活化综合征（MAS）患儿临床资料进行回顾性分析,以期提高临床认识。方法回顾性分析13例SOJIA合并MAS患儿可能触发因素、临床表现、实验室检查结果以及治疗和预后。结果90例SOJIA患儿并发MAS13例（14．4％）,男10例,女3例。全部患儿处于SOJIA活动状态;3例可能为药物触发;8例MAS发生前存在感染。全部患儿均持续高热;肝大12例（92．3％）;凝血功能障碍10例（76．9％）;神经精神系统功能障碍8例（61．5％）。MAS发生时全部患儿白细胞（WBC）、血小板（PLT）和红细胞沉降率（ESR）较发生前显著下降;5例（62．5％）血清铁蛋白（SF）≥10000μg／L;8例（72．7％）血纤维蛋白原（Fib）≤2．5g／L;9例（69．2％）甘油三酯（TG）≥2．5mmol／L。结论MAS是SOJIA严重而致命的并发症,原发SOJIA活动性、药物以及感染是MAS的重要触发因素。肝脏显著增大、出血倾向和神经精神系统功能障碍是MAS最具鉴别意义的临床指标。血WBC、PIJT和ESR较基础值急剧下降、SF急剧升高、Fib减少以及TG升高是MAS重要的临床实验室指标。早期有力的干预治疗决定MAS的预后。

Macrophage activation syndrome in children with systemic onset juvenile idiopathic arthritis: analysis of 13 patients

OBJECTIVE: Macrophage activation syndrome (MAS) is a rare but life-threatening complication in children with rheumatic diseases, particularly systemic-onset juvenile idiopathic arthritis (SOJIA). Because of the potential fatality of this condition, prompt recognition and immediate therapeutic intervention are important. This study reviewed the data of MAS in 13 cases with SOJIA. METHODS: Retrospective review was performed on the precipitating events, clinical manifestations, laboratory data, treatment, and outcome of macrophage activation syndrome in 13 children with SOJIA seen from 1996 to 2005. RESULTS: Over the past 10 years the unit has had 90 new patients with SOJIA. Thirteen of those patients (14.4%) developed MAS during the course of their primary SOJIA, of whom ten were male. All patients were noted to have active SOJIA prior to developing MAS; 3 patients had medications, which were considered as trigger factors; 8 had infections prior to MAS, in two of them the infections were possible triggers. All the patients had high grade fever; 12 cases (92.3%) had hepatomegaly; 10 patients (76.9%) had coagulopathy, and eight patients (61.5%) had central nervous system dysfunction. The counts of platelet, white blood cells and the mean erythrocyte sedimentation rate fell dramatically in all patients; hyperferritinemia was identified in 8 patients, in 5 of whom serum ferritin (SF) was >or= 10,000 microg/L; in 8 (72.7%) of 11 cases fibrinogen was or= 2.5 mmol/L in 9 (69.2%) of 13 cases. CONCLUSION: MAS is a rare and potentially fatal complication of children with SOJIA. Primary disease activity, medications and infections preceding MAS were all important triggers. The strongest clinical discriminators were hepatomegaly, hemorrhages and central nervous system dysfunction. The strongest laboratory tests were decreased counts of platelet and white blood cells, decreased ESR and fibrinogen, dramatically increased SF and TG. It calls for the immediate treatments, particularly with cyclosporin A, which are often effective.