上海单中心101例儿童狼疮性肾炎的长期随访分析

Long-term follow-up of 101 cases with pediatric lupus nephritis in a single center in Shanghai

目的分析儿童狼疮性肾炎（LN）的预后及其影响因素。方法对101例LN的患儿进行回顾性分析。分为肾活检组与未肾活检组；病理分型包括A组（I＋Ⅱ型），B组（Ⅲ＋Ⅳ型），C组（V型）3组；诱导期药物环磷酰胺（CTX）组与霉酚酸酯（MMF）组；临床结局缓解组（完全缓解和部分缓解）和无效组（治疗无效和死亡）。用药不依从定义为诱导期CTX冲击间期大于45d或疗程不足，或MMF或其他免疫抑制剂自行停止服用1周以上。用SPSS11．0软件中的Life—Tables分析累积生存率。结果（1）LN患儿3年和5年的累积生存率为93．59％和87．80％；3年和5年的肾脏累积生存率为100％和91．12％。（2）单因素分析显示诱导期疾病缓解与是否肾活检、不同病理类型、不同药物诱导、治疗是否依从和发病时蛋白尿量5个因素有关；维持期疾病缓解与前4个因素有关。（3）多因素分析显示维持期与疾病缓解相关的主要因素为治疗是否依从（χ2=9．818，P=0．002）。用药依从性差主要发生在未行肾活检组（χ2=9．569，P=0．002）。（4）Ⅲ和Ⅳ型LN中，无用药不依从发生，MMF和CTX在此组中疗效无明显差异（P=0．405）。结论LN儿童疾病缓解的主要影响因素为免疫抑制剂的用药依从性。未行肾活检组患儿疾病缓解率低可能与病理类型不确定及用药依从性差有关。在Ⅲ和Ⅳ型LN中，MMF和CTX的诱导缓解疗效无差异。对尿液检查轻度异常的LN患儿应坚持行肾活检病理检查，可指导治疗、改善预后。

Objective To deeply understand prognosis of pediatric cases with lupus nephritis （LN） treated in our hospital and analyze the prognostic factors. Method One hundred and one patients were enrolled, who were diagnosed as lupus nephritis in our hospital during the period from Jan. 1996 to Dec. 2007. Clinical data were retrospectively analyzed; the observation was ended on 31st Dec. 2009. Patients were divided into renal biopsy group and non renal biopsy group ; group A （ type Ⅲ ＋ Ⅱ LN） , group B （ type Ⅲ ＋ Ⅳ LN） and group C （ type V LN） ; CTX group （cyelophosphamide） and MMF group （ myeophenolate mofetil） ; remission group （complete remission and partial remission） and ineffective group （treatment failure and death）. Medication non-compliance means （ 1 ） the interval of CTX pulse was more than 45 days or treatment course less than 6 times; （2） patients discontinued MMF or other immunosuppressant on themselves more than a week ago. SPSS 11.0 software Life-Tables method was used to analyze cumulative survival rates. Result （1） Three and five years＇ patient survival rates were 93.59% and 87.80% respectively. Three and five years＇ kidney survival rates were 100% and 91.12% respectively. （2） Univariate analysis showed that induction remission were related to five factors, including whether received renal biopsy （ χ2 = 9. 023, P = 0. 003 ）, different pathological types （χ2 = 9. 437, P = 0. 009 ）, different induction drug （ χ2 = 4. 610,P =0. 032）, treatment compliance （ χ2 = 18.716,P =0. 000） and proteinuria amount （χ2 = 8. 013 ,P =0. 046） , and maintenance remission were related to the former four factors （χ2 = 10.209,P=0.001;χ2 = 7.757,P =0.021;χ2 = 4.206,P =0.04;χ2 = 24.571,P =0.000）. （3） Multivariate analysis showed that maintenance remission was mainly related to medication-compliance （χ2 = 9. 818,P =0. 002）. Poor medication compliance mainly occurred in non renal biopsy group （χ2 = 9. 569,P = 0. 002）. （4） In renal biopsy group, 15 cases showed a small amount proteinuria, 4 of them were proved as severe pathological type LN （2 eases type Ⅲ, 1 case type Ⅳ and 1 ease type Ⅴ ）. （5） In group B, no medication non-compliance occurred, and the efficacy of MMF and CTX had no significant differenee （P = 0. 405）. Conclusion The main affecting factor of remission rate was medication compliance. In type m and IV lupus nephritis, the efficacy of MMF and CTX were no significant difference. The poor outcome of non-renal biopsy group may be due to unelear pathological classification and poor medication compliance. We strongly reeommebnd that SLE patients with mild abnormal results of urinalysis should receive renal biopsy.