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[POEMS综合征中的肾脏受累]

[Renal involvement in POEMS syndrome].

作者信息

Rey J P, Modesto A, Orfila C, Cozette P, Donnadieu P, Lauze I, Héran B, Bertrand J L, Camo J, Négre C

机构信息

CHG Perpignan, Toulouse.

出版信息

Nephrologie. 1994;15(6):391-4.

PMID:7862228
Abstract

We described a patient with a POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal grammapathy and Skin changes) who was found to have renal involvement with peculiar renal pathological findings. Hitherto, 17 other cases, most of them from Japan, of POEMS syndrome with renal involvement, have been published. Clinical features are variable: acute renal failure with anasarca or moderate chronic renal insufficiency with mild proteinuria. This latter presentation often passes unnoticed. There is no severe HTA, no microangiopathic hemolytic anemia. Renal biopsy shows prominent glomerular changes which are unusual and distinct from membranoproliferative glomerulonephritis (MPGN) and from glomerular thrombotic microangiopathy (TMA). Mesangial proliferation and thickening of the capillary wall with double contour evoke by light microscopy a MPGN. By immunofluorescent microscopy, no immunoglobulins or complement deposits are found. The finding of mesangiolytic lesions has led to the term of "mesangiolytic glomerulonephritis". The presence, on electron microscopy, of lucent subendothelial space could evoke TMA. But there is neither thrombi, nor arteriolar changes. We are inclined to presume that microangiopathic lesions are due to chronic injury of glomerular endothelial cells, exacerbated at outbreaks of the disease. An increased production of IL-6 could support the efficacity of corticosteroid therapy, particularly in acute clinical situations.

摘要

我们描述了一名患有POEMS综合征(多发性神经病、脏器肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变)的患者,该患者被发现存在肾脏受累且伴有特殊的肾脏病理表现。迄今为止,已发表了另外17例伴有肾脏受累的POEMS综合征病例,其中大多数来自日本。临床特征各不相同:表现为全身性水肿的急性肾衰竭或伴有轻度蛋白尿的中度慢性肾功能不全。后一种表现常常未被注意到。不存在严重高血压,也没有微血管病性溶血性贫血。肾活检显示出明显的肾小球改变,这些改变不常见,且与膜增生性肾小球肾炎(MPGN)和肾小球血栓性微血管病(TMA)不同。光镜下可见系膜增生以及毛细血管壁增厚伴双轨征,提示为MPGN。免疫荧光显微镜检查未发现免疫球蛋白或补体沉积。系膜溶解性病损的发现导致了“系膜溶解性肾小球肾炎”这一术语的出现。电镜下可见透亮的内皮下间隙,这可能提示TMA。但既没有血栓形成,也没有小动脉改变。我们倾向于推测微血管病性病变是由于肾小球内皮细胞的慢性损伤所致,在疾病发作时会加重。白细胞介素-6的产生增加可能支持皮质类固醇治疗的有效性,尤其是在急性临床情况下。

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