全身型幼年特发性关节炎临床特征及转归

目的：分析全身型幼年特发性关节炎（SoJIA）的临床特征及转归。方法：收集2010年1月至2017年1月入住温州医科大学附属第二医院育英儿童医院儿童风湿科的41例SoJIA患儿的临床、实验室及影像学资料。结果：SoJIA患儿共41例，发病年龄9个月～11.5岁，平均（7.35±3.17）岁。出现症状至确诊时间为19.5（15.0，41.5）d。起病时主要症状为发热41例（占100%），关节炎33例（占80.5%），皮疹24例（占58.5%）。关节炎发生时，以大关节累及多见，由多到少依次为膝、踝、肩、肘。起病时以单一关节受累多见，占87.9%（29例），其中5例随访过程中进展为多关节受累。起病初无关节受累8例，其中5例随访中新发关节症状，均为少关节型，在发病后125.0（116.0，372.5）d出现。实验室检查中血白细胞总数升高28例（占68.3%），CRP升高41（占100%），ESR增快38例（占95.0%），贫血18例（占43.9%），血清铁蛋白（SF）升高24例（70.6%），IgE升高25例（占65.8%），RF阳性2例（占5.9%），ANA阳性5例（占12.2%）。并发间质性肺炎1例，肺动脉高压1例。符合Ravelli 2016年标准诊断巨噬细胞活化综合征共6例。41例中规律随访29例，平均随访1 155.0（730.0，1 872.5）d，单相病程13例（占44.8%），复发1次5例（占17.2%），复发2次及以上11例（占37.9%）。经治疗后，完全缓解25例（占86.2%），部分缓解3例（占10.3%），未缓解0例，死亡1例。失访12例。复发组年龄、血CRP、SF水平、ESR、CD3+、CD4+相对值、谷氨酰转肽酶水平均明显低于未复发组。结论：SoJIA临床表现多样，约12.2%起病初无关节炎症状，随访过程中新发关节症状。现有治疗下大部分预后良好，发病年龄小、初期高水平的血小板计数、T淋巴细胞功能失调可能与SoJIA复发相关。

Clinical features and prognosis of systemic-onset juvenile idiopathic arthritis

Objective: To analyze the clinical features of children with systemic-onset juvenile idiopathic arthritis (SoJIA). Methods: Patients diagnosed with SoJIA from January 2010 to January 2017 were evaluated and data were collected for clinical analysis. Results: A total of 41 patients (28 boys and 13 girls) were included in this study. The mean age of onset was (7.35± 3.17) years old. The most common presentations were fever (100%), arthritis (80.5%), and skin rash (58.5%). Macrophage activation syndrome was found in 6 cases. Oligoarticular type of arthritis accounted for 87.9% of the affected patients. The mean period from the first day of clinical symptoms to that of developing arthritis was 220 days (545 days, 112 days, 125 days, 120 days, and 200 days respectively), and the patterns of arthritis were all oligoarticular type. The most common joints involved were the knee, ankle, shoulder and elbow. Leukocytosis and anemia accounted for 68.3%, and 43.9% respectively. The majority of inflammatory parameters, including CRP, ESR and SF were elevated. 44.8% of patients had single-phase course, 17.2% had relapsing once and 37.9% had relapsing at least twice. After therapy, 25 cases achieved complete reminssion, 12 cases failed to follow-up, and 1 case was dead. Relapsing group was significantly younger. The levels of CRP, SF, ALT, CD3+, CD4+ and GGT in the relapsing group were significantly lower. Conclusion: The clinical manifestations of SoJIA were various, 12.2% of patients did not have arthritis on disease onset, but developed arthritis during follow-up process. Most prognosis is good. The younger onset age, thrombocytosis andT lymphocyte dysfunction may indicate a relapsing course.