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膜性肾病的诊断与自然病程。

Diagnosis and natural course of membranous nephropathy.

作者信息

Glassock Richard J

机构信息

Geffen School of Medicine at UCLA, Los Angeles, CA 92677, USA.

出版信息

Semin Nephrol. 2003 Jul;23(4):324-32. doi: 10.1016/s0270-9295(03)00049-4.

Abstract

Membranous nephropathy is a relatively common glomerular disease found to underlie both nonnephrotic and nephrotic proteinuria. In adults, about 75% of cases are primary (idiopathic) and 25% are secondary to a wide variety of causes, including neoplasia, infections, autoimmunity, and drugs. Presenting features are not distinctive enough to permit a diagnosis without a renal biopsy examination. Serologic studies are normal in the idiopathic disorder. The morphologic features are characteristic and include gradual thickening of the capillary wall caused by the in situ deposition of immune complexes accompanied by new basement membrane synthesis. The natural history of the untreated disorder is variable. Spontaneous remissions (complete and partial) of proteinuria, usually accompanied by stable renal function, eventually occur in 40% to 50% of patients and the remainder slowly progress to end-stage renal disease (ESRD) or die of complications or from unrelated disease after 5 to 15 years. Factors associated with a progressive course include older age at onset, male gender, persisting hypertension, hyperlipidemia and/or hypoalbuminemia, reduced renal function at discovery, persisting nephrotic range glomerular proteinuria, concomitant tubular proteinuria, and advanced glomerular damage with chronic tubulointerstitial fibrosis.

摘要

膜性肾病是一种相对常见的肾小球疾病,是非肾病性和肾病性蛋白尿的基础病因。在成年人中,约75%的病例为原发性(特发性),25%继发于多种病因,包括肿瘤、感染、自身免疫和药物。临床表现不够特异,若无肾活检检查则无法确诊。特发性疾病的血清学检查结果正常。其形态学特征具有特异性,包括免疫复合物原位沉积导致毛细血管壁逐渐增厚,并伴有新的基底膜合成。未经治疗的疾病自然病程多变。蛋白尿自发缓解(完全缓解和部分缓解),通常伴有稳定的肾功能,最终发生在40%至50%的患者中,其余患者在5至15年后缓慢进展至终末期肾病(ESRD)或死于并发症或其他无关疾病。与疾病进展相关的因素包括发病时年龄较大、男性、持续性高血压、高脂血症和/或低白蛋白血症、发现时肾功能减退、持续性肾病范围的肾小球蛋白尿、合并肾小管蛋白尿以及伴有慢性肾小管间质纤维化的严重肾小球损伤。

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