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膜性肾病合并IgA肾病

Combined membranous nephropathy and IgA nephropathy.

作者信息

Stokes M B, Alpers C E

机构信息

Department of Pathology, University of Washington Medical Center, Seattle, USA.

出版信息

Am J Kidney Dis. 1998 Oct;32(4):649-56. doi: 10.1016/s0272-6386(98)70031-9.

DOI:10.1016/s0272-6386(98)70031-9
PMID:9774129
Abstract

IgA nephropathy (IgAN) and membranous nephropathy (MN) are both common renal biopsy findings that rarely have been described together in the same patient. The significance of this finding is not clear. We present the clinical and pathological data of four patients with combined MN-IgAN and discuss possible pathogenetic mechanisms. By definition, all cases showed immunodominant mesangial deposits of IgA (+/-C3) and subepithelial capillary wall deposits of IgG (+/-C3) by immunofluorescence microscopy, confirmed by electron microscopy. There were three men and one woman, whose ages ranged from 41 to 67 years (average, 51.7 years). All four presented with microscopic hematuria and proteinuria, three in the nephrotic range. Renal function was normal in three individuals, and one subject had mild renal insufficiency accompanied by long-standing hypertension. Two other patients had newly uncovered hypertension. Complement levels were normal in all subjects. One patient had a positive antinuclear antibody (ANA) test, but none had other serologic or clinical features diagnostic of lupus. None of the four individuals had any other predisposing factors for either MN or IgAN, including hepatitis B infection. All four patients had stable renal function at last determination (average follow-up, 24 months; range, 4 to 34 months), with markedly reduced proteinuria in three individuals and persistent heavy proteinuria in one. A review of the literature indicates that combined MN-IgAN is most often characterized by heavy proteinuria and stable renal function. Some cases may be related to hepatitis B infection, but in most instances the cause is unknown. The combination of these two pathological processes does not result in a particularly deleterious clinical outcome for patients.

摘要

IgA肾病(IgAN)和膜性肾病(MN)都是常见的肾活检结果,在同一患者中很少同时出现。这一发现的意义尚不清楚。我们展示了4例合并MN-IgAN患者的临床和病理数据,并讨论了可能的发病机制。根据定义,所有病例通过免疫荧光显微镜检查均显示IgA(±C3)的免疫优势性系膜沉积以及IgG(±C3)的上皮下毛细血管壁沉积,电子显微镜检查予以证实。患者中有3名男性和1名女性,年龄在41至67岁之间(平均51.7岁)。所有4例患者均表现为镜下血尿和蛋白尿,其中3例为肾病范围蛋白尿。3例患者肾功能正常,1例患者伴有长期高血压且有轻度肾功能不全。另外2例患者新发高血压。所有患者补体水平均正常。1例患者抗核抗体(ANA)检测呈阳性,但均无其他狼疮诊断的血清学或临床特征。4例患者均无MN或IgAN的其他易感因素,包括乙型肝炎感染。最后一次测定时,所有4例患者肾功能均稳定(平均随访24个月;范围4至34个月),3例患者蛋白尿明显减少,1例患者持续大量蛋白尿。文献回顾表明,MN-IgAN合并症最常见的特征是大量蛋白尿和肾功能稳定。部分病例可能与乙型肝炎感染有关,但在大多数情况下病因不明。这两种病理过程的合并对患者并未导致特别有害的临床结局。

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