摘要

成人腎病症候群致病主因常為原發性膜性腎病變 (primary membranous nephropathy)，症狀包含周邊水腫、重度蛋白尿和低白蛋白血症等。其病理機轉為腎絲球足細胞之抗原產生的體液性免疫反應，進而造成腎損傷。目前已鑑定出多種致病抗原（即生物標誌），不同於其他腎臟疾病快速進展成腎病症候群，該病程通常較緩慢，部分未經治療病人可自發性完全緩解或部分緩解。在所有腎絲球疾病中，原發性膜性腎病變在過去十幾年於病理機制和治療方面取得重大進展。本文將探討原發性膜性腎病變流行病學、病理機轉及疾病分類，並針對現行藥物治療選擇及具潛力之標靶治療藥物進行文獻回顧。

 

ABSTRACT

Primary membranous nephropathy (pMN) is the leading cause of nephrotic syndrome in adults, characterized by peripheral edema, severe proteinuria, and hypoalbuminemia. The pathogenic mechanism of pMN involves a humoral immune response to antigens in glomerular podocytes, which causes kidney injury. Several pathogenic antigens, known as biomarkers, have been identified. Unlike other renal diseases that rapidly progress to nephrotic syndrome, pMN usually has a slower clinical course; spontaneous complete or partial remission may occur in some untreated patients. In terms of glomerular diseases, significant progress has been made in recent decades in understanding the pathological mechanisms and therapeutic strategies for pMN. This research focuses on the epidemiology, pathogenesis, and disease classification, as well as an up-to-date review of current treatments and emerging targeted therapeutic agents.

 

 

Submitted for publication: 2025.03.21; Accepted for publication: 2025.08.15