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原发性膜性肾病中持续性疾病和复发的长期结局。

Long-term outcomes of persistent disease and relapse in primary membranous nephropathy.

机构信息

Manchester Institute of Nephrology and Transplantation, Central Manchester University Hospitals NHS Foundation Trust, Manchester, UK.

Department of Renal Medicine, Manchester Royal Infirmary, Oxford Road, Manchester M13 9WL, UK.

出版信息

Nephrol Dial Transplant. 2016 Dec;31(12):2108-2114. doi: 10.1093/ndt/gfv435. Epub 2016 Jan 13.

DOI:10.1093/ndt/gfv435
PMID:26769682
Abstract

BACKGROUND

Primary membranous nephropathy is associated with variable clinical course ranging from spontaneous remission to slow progression to end stage renal failure. Achieving remission confers better renal survival in primary membranous nephropathy (PMN). Longer term outcomes such as patient survival and relapse of active disease remain poorly understood.

METHODS

We performed a retrospective study of 128 consecutive adult patients diagnosed with biopsy proven PMN at a single UK centre between 1980 and 2010. These patients were followed prospectively over a median of 128 months. We assessed impact of persistent disease and relapse on Stage 5 chronic kidney disease (CKD-5) and patient survival and present longer term cumulative incidences of different end points.

RESULTS

One hundred patients achieved partial remission (PartRem) and 28 patients did not achieve remission (NoRem). Nine per cent of patients achieving first remission developed CKD-5 and 75% of those with NoRem developed CKD-5 [hazard ratio (HR) 0.07, 95% confidence interval 0.03-0.19). Relapse following PartRem occurred in 31 patients (31%) during follow-up and was significantly associated with progression to CKD-5. Progression to CKD-5 was strongly associated with death (47 versus 6%, HR 23.4; P < 0.01). Cumulative incidence at 15 years following first presentation included: death, 14%; CKD-5, 28%; and relapse 40% (in patients who achieved first remission).

CONCLUSIONS

Our data strongly suggest that mortality in PMN is seen in patients with disease progression to CKD-5. Achieving remission is strongly associated with improved renal survival after first presentation and following relapse. We suggest that patients who achieve remission should be followed up in longer term, and better strategies to help improve outcomes are needed in clinical practice.

摘要

背景

原发性膜性肾病的临床病程变化较大,可自发缓解,也可缓慢进展至终末期肾衰竭。在原发性膜性肾病(PMN)中,达到缓解可改善肾脏预后。然而,长期结局,如患者生存和疾病复发,仍知之甚少。

方法

我们对 1980 年至 2010 年间在英国单一中心经活检证实为 PMN 的 128 例连续成年患者进行了回顾性研究。这些患者在中位随访 128 个月期间接受了前瞻性随访。我们评估了持续性疾病和复发对 5 期慢性肾脏病(CKD-5)及患者生存的影响,并报告了不同终点的长期累积发生率。

结果

100 例患者达到部分缓解(PartRem),28 例患者未达到缓解(NoRem)。首次缓解后,有 9%的患者发展为 CKD-5,而无缓解的患者中有 75%发展为 CKD-5[风险比(HR)0.07,95%置信区间 0.03-0.19)]。在随访期间,31 例(31%)PartRem 缓解的患者发生复发,且与进展为 CKD-5显著相关。进展为 CKD-5 与死亡密切相关(47%对 6%,HR 23.4;P<0.01)。首次就诊后 15 年的累积发生率包括:死亡,14%;CKD-5,28%;以及复发 40%(在首次缓解的患者中)。

结论

我们的数据强烈表明,PMN 患者的死亡见于进展为 CKD-5 的患者。首次就诊和复发后达到缓解与改善肾脏预后密切相关。我们建议应在长期内对达到缓解的患者进行随访,临床实践中需要更好的策略来改善结局。

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