Ⅴ型狼疮性肾炎的远期预后

Long-term outcome of patients with membranous lupus nephropathy

目的:回顾性分析Ⅴ型狼疮性肾炎(LN)的远期预后及影响预后的因素。方法:临床符合系统性红斑狼疮,经肾活检诊断为Ⅴ型LN(ISN/RPS2003肾脏病理分型标准)的患者100例[女性90例,男性10例,平均年龄(32±9)岁]。通过Kaplan-Meier法估算人肾存活率,并建立COX风险模型,分析影响预后的危险因素。结果:100例平均随访(77.6±56)个月(中位时间59.5个月)。共2例死亡,5年、10年人存活率均为98%,5年、10年肾存活率分别为96.1%、92.7%。肾小管间质严重损害(RR66.514)、治疗初大量蛋白尿(RR19.159)和持续不缓解(RR9.834)是影响肾存活率的独立危险因素。进入终末期肾衰6例中3例治疗初即有严重肾小管间质病变,2例随访中发生转型(1例转为Ⅴ型伴Ⅳ型,1例转为Ⅴ型伴Ⅲ型),1例CSA中毒。并发症:包括感染(13%),血栓(3%),股骨头坏死(3%),糖尿病(4%)和肿瘤(1%)。结论:Ⅴ型LN10年人肾存活率达98%和93%,严重肾小管间质病变、大量蛋白尿和持续不缓解是Ⅴ型LN进展为ESRF的危险因素。

Objective：To analyze the long-term outcome of patients with membranous lupus nephropathy （MLN） and prognostic factors. Methodology：One hundred Chinese patients （female 90 and male 10 with a mean age 32 ± 9 years） suffered from systemic lupus erythematosus （SLE） and biopsy-proven MLN （ISN/RPS2003 classification criteria） were enrolled in this retrospective study. The patient survival and renal survival was estimated by Kaplan-Meier method, and Cox proportional hazards regression analysis was used to assess the risk factors associated with end stage renal failure （ESRF）. Results：All patients were followed （77.6 ± 56） months. Eventually, two patients died, ten years patient survival was 98%, 5 and 10 years renal survival were 96. 1% and 92.7% respectively. It was found that severe tubular-interstitial lesion （RR 66. 514） , nephrotic range proteinuria （RR 19. 159） and continuous without remission （RR 9. 834） were independent risk factors to ESRF. Three of 6 patients who reached ESRF showed severe tubular-interstitial lesion on initial biopsy. Twenty-one patients underwent rebiopsy after （44 ±42） months＇follow-up, eight （38.1%） of them （ MLN with class IV in 5, MLN with class Ⅲ in 2 and class VI in 1 ） had transformed their histological classifications on repeat renal biopsy, and three （37. 5% ） of them developed ESRF. The adverse effects included infection （13%）, thrombosis （3%）, avascalar necrosis （3%）, diabetes mellitus （4%） and skin cancer （1% ）. Conclusion ：The rate of patient and renal survival was high in this group of patients with MLN. The severe tubular-interstitial lesions, nephrotic range proteinuria, and continuous without remission were high risks to develop ESRF.