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微小病变型肾病中的系膜IgA沉积:不同疾病实体的巧合还是微小病变型疾病的变异?

Mesangial IgA deposition in minimal change nephrotic syndrome: coincidence of different entities or variant of minimal change disease?

作者信息

Westhoff T H, Waldherr R, Loddenkemper C, Ries W, Zidek W, van der Giet M

机构信息

Charite-Campus Benjamin Franklin, Medical Clinic IV, Nephrology, Hindenburgdamm 30, Berlin, Germany.

出版信息

Clin Nephrol. 2006 Mar;65(3):203-7. doi: 10.5414/cnp65203.

Abstract

BACKGROUND

Mesangial deposition of IgA (MCA) is a very rare finding in minimal change disease and has previously been considered a pure coincidence. In the U.S. and Europe only anecdotal case reports exist. To date, there has been no consensus on nomenclature and categorization of this entity. We describe 2 cases of MCA with analogue histological findings but relevant differences in clinical presentation, and we discuss the clinical implications of mesangial IgA deposition in minimal change nephrotic syndrome.

PATIENTS

A 47-year-old female was admitted to hospital with nephrotic syndrome, microscopic hematuria, arterial hypertension and slight impairment of renal function 3 weeks after an unspecific upper airway infection. A 42-year-old male presented with nephrotic syndrome, microscopic hematuria, normotension and normal renal function. Both of the nephrotic syndromes were steroid-responsive and steroid-dependent.

FINDINGS

The clinical presentation of the male patient was consistent with the features of minimal change glomerulopathy, whereas the female patient combined signs of minimal change disease and IgA nephropathy. Light microscopy revealed mesangial IgA immune deposits and slight mesangial hypercellularity. Electron microscopic studies of MCA patients disclose diffuse effacement of glomerular foot processes.

CONCLUSION

Our cases and a review of the literature indicate that the histological diagnosis of MCA may comprise different pathogenetic entities. From the clinical point of view, MCA has to be regarded as a minimal change nephrotic syndrome with symptomatic or asymptomatic mesangial IgA deposition. IgA deposition constitutes a risk factor for impairment of renal function and indicates a frequently relapsing course.

摘要

背景

IgA系膜沉积(MCA)在微小病变病中是非常罕见的发现,此前一直被认为是纯粹的巧合。在美国和欧洲,仅有零星的病例报告。迄今为止,对于该实体的命名和分类尚未达成共识。我们描述了2例具有类似组织学表现但临床表现存在相关差异的MCA病例,并讨论了系膜IgA沉积在微小病变肾病综合征中的临床意义。

患者

一名47岁女性在非特异性上呼吸道感染3周后因肾病综合征、镜下血尿、动脉高血压和肾功能轻度损害入院。一名42岁男性表现为肾病综合征、镜下血尿、血压正常和肾功能正常。这两种肾病综合征均对类固醇有反应且依赖类固醇。

发现

男性患者的临床表现与微小病变性肾小球病的特征相符,而女性患者合并了微小病变病和IgA肾病的体征。光镜检查显示系膜IgA免疫沉积和轻度系膜细胞增多。对MCA患者的电镜研究揭示肾小球足突弥漫性消失。

结论

我们的病例及文献回顾表明,MCA的组织学诊断可能包括不同的致病实体。从临床角度来看,MCA必须被视为伴有有症状或无症状系膜IgA沉积的微小病变肾病综合征。IgA沉积构成肾功能损害的危险因素,并提示疾病频繁复发的病程。

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