Primary Membranous Nephropathy
Primary Membranous Nephropathy
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原发性膜性肾病
DOI：
10.2215/cjn.11761116
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发表时间：
2017-06-01
影响因子：
9.8
通讯作者：
Couser, William G.
中科院分区：
文献类型：
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作者：
Couser, William G.
膜性肾病（MN）是一种独特的肾小球病变，是非糖尿病白人成人特发性肾病综合征的最常见原因。约 80% 的病例是肾脏局限性的（原发性 MN、PMN），20% 的病例与其他全身性疾病或暴露相关（继发性 MN）。本综述仅关注 PMN。大多数 PMN 病例具有针对足细胞膜抗原 PLA2R 的循环 IgG4 自身抗体 (70%)，活检证据 PLA2R 染色表明尽管血清抗体水平呈阴性 (15%) 或血清抗 THSD7A (3%-5%)，但近期有免疫疾病活动。剩下的 10% 没有明显的抗 PLA2R/THSd7A 抗体或抗原，可能患有 PMN，可能继发于不同的、尚未鉴定的抗足细胞抗体。现在大量的临床和实验数据表明这些抗体具有致病性。临床上，80%的PMN患者表现为肾病综合征，20%表现为非肾病性蛋白尿。如果不进行治疗，大约三分之一的人会自发缓解，尤其是那些抗 PLA2R 水平缺失或较低的患者，三分之一的人在 10 年内进展为 ESRD，其余的则发展为非进展性 CKD。在不再检测到循环抗 PLA2R/THSD7A 抗体（免疫缓解）后，蛋白尿可能持续数月。所有 PMN 患者从诊断之时起就应接受支持性护理，以尽量减少蛋白质排泄。诊断时抗 PLA2R/THSD7A 水平升高且蛋白尿 >3.5 g/d 的患者，以及未能将蛋白尿降低至
Membranous nephropathy (MN) is a unique glomerular lesion that is the most common cause of idiopathic nephrotic syndrome in nondiabetic white adults. About 80% of cases are renal limited (primary MN, PMN) and 20% are associated with other systemic diseases or exposures (secondary MN). This review focuses only on PMN. Most cases of PMN have circulating IgG4 autoantibody to the podocyte membrane antigen PLA2R (70%), biopsy evidence PLA2R staining indicating recent immunologic disease activity despite negative serum antibody levels (15%), or serum anti-THSD7A (3%-5%). The remaining 10% without demonstrable anti-PLA2R/THSd7A antibody or antigen likely have PMN probably secondary to a different, still unidentified, anti-podocyte antibody. Considerable clinical and experimental data now suggests these antibodies are pathogenic. Clinically, 80% of patients with PMN present with nephrotic syndrome and 20% with non-nephrotic proteinuria. Untreated, about one third undergo spontaneous remission, especially those with absent or low anti-PLA2R levels, one-third progress to ESRD over 10 years, and the remainder develop nonprogressive CKD. Proteinuria can persist for months after circulating anti-PLA2R/THSD7A antibody is no longer detectable (immunologic remission). All patients with PMN should be treated with supportive care from the time of diagnosis to minimize protein excretion. Patients with elevated anti-PLA2R/THSD7A levels and proteinuria >3.5 g/d at diagnosis, and those who fail to reduce proteinuria to