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霉酚酸酯和类固醇治疗POEMS(多发性神经病、器官肿大、内分泌病、M蛋白、皮肤改变)综合征患者肾病成功稳定:一例报告

Successful stabilisation of nephropathy in a patient with POEMS (polyneuropathy, organomegaly, endocrinopathy, M-band, skin changes) syndrome on treatment with mycophenolate and steroids: a case report.

作者信息

Rosser Gareth J, Reitböck Pablo Garcia, Gray Martin C, Warwicker Paul

机构信息

Lister Renal Unit, Lister Hospital, Stevenage, SG1 4AB, UK.

出版信息

J Med Case Rep. 2010 Feb 22;4:63. doi: 10.1186/1752-1947-4-63.

Abstract

INTRODUCTION

Renal involvement in POEMS (polyneuropathy, organomegaly, endocrinopathy, M-band, skin changes) syndrome is considered to be an under-diagnosed phenomenon with no clear treatment path. The limited literature suggests steroids to be the drug of choice, although improvements are limited and usually reverse on withdrawal of the drug.

CASE PRESENTATION

A 52-year-old Caucasian woman presenting with features consistent with POEMS syndrome developed progressive renal impairment with proteinuria. Renal biopsy revealed a membranoproliferative glomerulonephritis. She was treated with relatively low dose oral mycophenolate mofetil and prednisolone which stabilised her nephropathy and neuropathy.

CONCLUSION

We describe an alternative therapeutic option in patients with this serious but poorly understood condition.

摘要

引言

POEMS(多发性神经病、器官肿大、内分泌病、M蛋白、皮肤改变)综合征中的肾脏受累被认为是一种诊断不足的现象,且没有明确的治疗途径。有限的文献表明,类固醇是首选药物,尽管改善有限,且通常在停药后会逆转。

病例介绍

一名52岁的白种女性,表现出与POEMS综合征相符的特征,出现了伴有蛋白尿的进行性肾功能损害。肾活检显示为膜增生性肾小球肾炎。她接受了相对低剂量的口服霉酚酸酯和泼尼松龙治疗,这使她的肾病和神经病变得到了稳定。

结论

我们描述了一种针对这种严重但了解不足的疾病患者的替代治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b2c3/2848064/3d72a710bb2d/1752-1947-4-63-1.jpg

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