48例全身型幼年特发性关节炎临床分析

目的：分析全身型幼年特发性关节炎（ SOJIA）的临床特征、治疗及预后。方法收集西安市儿童医院风湿免疫科自2012年9月至2015年4月收治的48例SOJIA患儿资料，分析其临床特征、治疗方案及疗效。结果48例患儿中，男女比率1．67：1；发病年龄4月至13．5岁，平均年龄为6．3岁。主要症状为发热48例（100．0％），关节炎39例（81．3％），皮疹36例（75％），并发巨噬细胞活化综合征4例（8．3％）。实验室检查白细胞计数、血沉、超敏C反应蛋白及血清铁蛋白均明显升高，感染骨髓象为主（占76．7％）。采用个体化初始治疗后42例（87．5％）临床缓解；5例甲泼尼龙冲击治疗后临床缓解；1例合并巨噬细胞活化综合征（MAS）死亡。10例（20．8％）减药过程中病情反复加用生物制剂、环孢素A、来氟米特等治疗后临床缓解；6例（12．5％）停药后复发；4例（8．3％）失访。结论 SOJIA患儿男性略多于女性，发病年龄跨度较广。约有1／4患儿临床不伴有关节炎及皮疹，最常累及大关节。血常规、血沉、血清铁蛋白、C反应蛋白为判定临床缓解的敏感指标。对于难治性、激素依赖性SOJIA患儿适时加用生物制剂可改善其预后，减少激素副作用。

Clinical analysis of 48 cases of systemic onset juvenile idiopathic arthritis

Objectve To analyze the clinical features , therapy and prognosis of systemic onset juvenile idiopathic arthritis ( SOJIA ) . Methods Data of patients with SOJIA admitted in department of rheumatology immunology of Xi ’ an Children ’ s Hospital from September 2012 to April 2015 were collected for the analysis of clinical features , therapeutic scheme and outcomes .Results A total of 48 cases of SOJIA were hospitalized with boy to girl ratio of 1.67:1, and the average age was 6.3 years (ranged 4 months-13.5 years).The main symptoms of SOJIA included fever in 48 cases (100.0%), arthritis in 39 cases (81.3%), macular rash in 36 cases (75%), and macrophage activation syndrome in 4 cases (8.3%).For laboratory results, the evident features of SOJIA were elevated white blood counts, erythrocyte sedimentation rate , C-reactive protein level and serum ferritin .Infection of bone marrow was the majority .Forty-two cases ( 87.5%) got clinical remission after individual initial treatment , 5 cases got clinical remission after methylprednisolone pulse therapy, and 1 case with MAS died.Ten cases (20.8%) repeated illness during reducing drug , and they relieved after using biologics , cyclosporine A and leflunomide treatment .Six cases (12.5%) recurred after discontinuation of medication , 4 cases (8.3%) lost to follow-up.Conclusion SOJIA occurs more frequently in boys than girls with broad age span .Arthritis and macular rash is not necessarily present in 1/4 cases.Large joints are mostly frequently involved .Blood routine, ESR, serum ferritin and C reactive protein are the sensitive indexes of determining clinical remission .For patients with refractory or hormone dependent SOJIA , timely use of biological agents can improve their prognosis and reduce side effects of hormone .