人类白细胞抗原-B27阳性与附着点炎症相关关节炎患儿70例临床分析

Clinical analysis of human leucocyte antigen - B27 - positive enthesitis related arthritis in 70 children

目的探讨HLA－B27阳性与附着点炎症相关关节炎（ERA）的临床特征、辅助检查、治疗及疗效。方法回顾性分析2007年1月至2017年8月重庆医科大学附属儿童医院诊断为ERA且血清HLA－B27阳性患儿的临床特征、辅助检查、治疗及随访情况，采用SPSS 19.0软件进行数据处理。结果共纳入70例患儿，男67例，女3例；发病年龄（10.08±2.67）岁。临床特征：患儿均以外周关节炎伴或不伴附着点炎症为主要表现，24.3%（17/70例）的患儿在病程中出现中轴关节炎症状，35.7%（25/70例）出现附着点炎症表现。87.1%（61/70例）的患儿以外周关节炎起病，且患儿在病程中均出现外周关节受累表现，以膝关节受累最为显著。2例并前葡萄膜炎。Patrick试验阳性47例（67.1%），骶髂关节压痛9例（12.9%）。幼年关节炎疾病活动评分（JADAS27评分）为（20.12±8.61）分。38.6%（27/70例）的患儿有阳性家族史。辅助检查：多数患儿红细胞沉降率、C反应蛋白（CRP）及血小板升高，合并轻－中度贫血，少数白细胞计数轻度升高，血清肿瘤坏死因子－α（TNF－α）、白细胞介素（IL）－6、IL－1β、IL－10水平升高。88.6%（31/35例）的患儿有骨量下降；骨关节X线检查阳性率81.1%（43/53例），骶髂关节病变5例；关节超声检查阳性率达81.5%（44/54例）；磁共振成像（MRI）检查阳性率95.1%（58/61例），其中骶髂关节炎30例（42.9%）。治疗及转归：根据病情选择加用非甾体抗炎药（NSAIDs）、缓解病情抗风湿药物（DMARDs）、糖皮质激素、钙剂及阿法骨化醇等治疗，50.0%（35/70例）的患儿加用TNF－α拮抗剂。患儿临床症状、实验室检查指标及JADAS27评分在规范治疗后均有不同程度改善。 结论ERA临床症状缺乏特异性，诊断较困难，极易误诊。影像学技术的提高，尤其是MRI，有助于ERA早期诊断。加强对ERA的认识及管理，掌握其临床特征、免疫学及影像学特点，有助于及时诊断，合理治疗，提高患儿整体身体功能及生活质量，避免及延缓残疾发生。

ObjectiveTo investigate the clinical features, auxiliary examination, treatment and efficacy of human leucocyte antigen（HLA）-B27-positive enthesitis related arthritis （ERA）.MethodsThe clinical manifestations, auxiliary examination, treatment and follow-up of HLA-B27-positive ERA diagnosed in the Children′s Hospital of Chongqing Medical University from January 2007 to August 2017 were analyzed retrospectively.SPSS 19.0 software was used for data processing.ResultsA total of 70 children were enrolled, including 67 males and 3 females, and the average age of onset was（10.08±2.67） years old.Clinical features： all of the 70 children had peripheral arthritis with or without enthesitis, and axial arthritis symptoms appeared in 24.3% （17/70 cases） children, and 35.7% （25/70 cases） children showed enthesitis.The onset of peripheral arthritis accounted for 87.1% （61/70 cases）, and all cases showed peripheral arthritis during the course of the disease, mostly the knees.Two cases were complicated with uveitis.Forty-seven cases （67.1%） were positive in Patrick test, and 9 cases（12.9%）had sacroiliac joint tenderness.The average Juvenile Arthritis Disease Activity Score with 27 joints （JADAS27） score was 20.12±8.61.Twenty-seven cases（38.6%） had positive family history.Auxiliary examination： Erythrocyte sedimentation rate, C-reactive protein（CRP）and platelets increased in most children, and a few with leukocytosis increased slightly, nearly half of children with anemia mildly or moderately, and serum tumor necrosis factor-α（TNF-α）, interleukin （IL）-6, IL-1β, IL-10 levels increased.Eighty-eight point six percent （31/35 cases） children had decreased bone mass; the positive rate of X-ray examination was 81.1%（43/53 cases）, including 5 cases of sacroiliac joint disease; the positive rate of ultrasonography was 81.5%（44/54 cases）; the positive rate of nuclear magnetic resonance（MRI）was 95.1%（58/61 cases）, including 30 cases （42.9%） of sacroiliitis.Treatment and outcome： Nonsteroidal antiinflammatory drugs （NSAIDs）, disease modifying antirheumatic drugs （DMARDs）, glucocorticoids, calcium and alfacalcidol were added, and 50.0% （35/70 cases） patients had added TNF-α antagonist.The follow-up of all of the patients showed improvement in terms of clinical symptoms, laboratory tests and JADAS27 score after standard treatment.ConclusionsIt is difficult to diagnose but easy to misdiagnose because of the lack of specificity of ERA clinical symptoms. The improvement of imaging techniques, especially MRI, is helpful for the early diagnosis of ERA.To strengthen the understanding and management of ERA, and to grasp its clinical features, immunology and imaging features, help to make timely diagnosis and reasonable treatment, and to improve physical function and quality of life of patients, and to avoid or delay disability.