儿童紫癜性肾炎诊治现状多中心回顾性调查分析

Multicenter investigation of diagnosis and treatment of Henoch-Schonlein purpura nephritis in childhood

目的 探讨我国住院儿童紫癜性肾炎的临床特点、病理表现及治疗现状.方法 回顾性分析全国40所医院2008年7月1日至2011年6月30日收治的新发过敏性紫癜、经临床或病理确诊为紫癜性肾炎并在调查单位接受治疗的紫癜性肾炎患儿的病历资料,并填写统一的调查表,收集相关资料,病例登记表经参研单位初步汇总后,由中华医学会儿科学分会肾脏学组最终汇总,由专门数据录入人员录入和核对,并随机抽样进行数据核实,最后对儿童紫癜性肾炎的临床特点、病理表现及治疗现状进行统计分析.结果 此次调研最终符合入选条件且病例完整可供分析者共4863例患儿,其中男2935例,女1928例,男女比例为1.52∶1,6～13岁为发病高峰.4702例（96.7％）患儿于过敏性紫癜起病6个月内确诊肾损害.临床分型最常见为血尿和蛋白尿型（2831例,58.2％）.其中1625例患儿行肾活检治疗,最常见肾活检指征为蛋白尿合并镜下血尿（1149例,70.7％）.1448例肾活检患儿提供病理分级,以Ⅲ级和Ⅱ级为多见.4863例患儿中接受激素及其他免疫抑制剂治疗者共3677例（75.6％）.最常见的治疗方案为单用激素1655例（34.0％）；单纯血尿型患儿（362例,56.2％）多未予特殊治疗,血尿和蛋白尿型患儿最常选择治疗方案仍为单用激素治疗.肾组织病理Ⅰ级和Ⅱ级以单用激素或联合雷公藤多甙治疗为主,病理Ⅲ级和Ⅳ级以激素＋环磷酰胺＋甲泼尼龙冲击治疗为主.结论 本研究调查分析了我国住院紫癜性肾炎患儿的临床诊治现状,目前多根据临床分型及参照病理分级选择治疗方案,但免疫抑制剂及非特异性治疗的选择和使用方式多样,仍有待进一步临床多中心研究结果验证并加以规范.

Objective To retrospectively investigate the current diagnosis and treatment of children with Henoch-Schonlein purpura nephritis in hospital, to survey the application of practical evidence-based guidelines in children with Henoch-Schonlein purpura nephritis. Method A nationwide survey in 40 hospitals was conducted and data of hospitalized children diagnosed as Henoch-Schonlein purpura nephritis for the first time during the period of 1st July 2008 to 30th June 2011 were analyzed. The collected information included age, gender, disease duration, clinical manifestations, relevant auxiliary examination results, renal biopsy, and treatment and so on. The data were collected and analyzed by the subspecialty group of ncphrology, Chinese Society of Pediatrics. Result There were 4863 hospitalized children with Henoch-Schonlein purpura nephritis from July 1, 2008 to June 30, 2011 in 40 hospitals. The male （ n = 2935） to female （n = 1928） ratio was 1.52： 1, the peak incidence between 6 to 13 years old. Renal impairment occurred in 96. 7% （n = 4702） with 6 months from the onset of Henoch-Schonlein purpura. The most common clinical findings were proteinuria and hematuria （2831 patients, 58. 2% ）; 1448 patients received renal biopsy, subclass m and II were the most common histological types; 3677 patients （75.6%） were treated with corticosteroids and immunosuppressants. The most common treatment scheme was corticosteroids only （ 1655 patients, 34. 0% ）. More than half of the patients （ 362 patients, 56. 2% ） with pure hematuria received no cortieosteroids or immunosuppressants. Patients with hematuria and proteinuria always received corticosteroids only （1017 patients, 35.9% ）. Corticosteroids with or without tripterygium glycosides were always given to the patients with subclass I and Ⅲ in renal biopsy. The patients with subclass Ⅲ and Ⅳ were mainly treated with combination of corticosteroids, cyclophosphamide and methylprednisolone. Conclusion The incidence of purpura nephritis has increased; the duration of renal impairment had no correlation with the gender. Compared to the female, the male patients are more likely to have proteinuria. The patients with mild proteinuria also can present with severe renal histological impairment. There was no unified treatment scheme in the immunosuppressants and non-specific drugs. Multicenter randomized controlled trials （RCTs） are needed to explore and manage the treatment of purpura nephritis.