We report a 64-year-old caucasian woman diagnosed with membranous nephropathy secondary to alpha-1 antitrypsin deficiency (AATD). AATD is a rare autosomal codominant genetic disorder. Its clinical manifestations are mostly observed in the lungs, with early-onset emphysema. Nephropathy due to AATD is still very rare and only a few cohort studies have been reported. It has been recognised that alpha-1 antitrypsin has a protective role in the kidneys which enhances the possibility of development of kidney failure, such as nephrotic syndrome, in cases of AATD. Further clinical investigation is needed to understand the relationship between the development of nephropathy, namely membranous nephropathy, and AATD.

中文翻译：

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继发于 α-1 抗胰蛋白酶缺乏症的肾病综合征

我们报告了一名 64 岁的白人女性，她被诊断患有继发于 alpha-1 抗胰蛋白酶缺乏症 (AATD) 的膜性肾病。AATD 是一种罕见的常染色体显性遗传病。其临床表现多见于肺部，伴有早发性肺气肿。由 AATD 引起的肾病仍然非常罕见，仅报道了少数队列研究。已经认识到，α-1 抗胰蛋白酶在肾脏中具有保护作用，在 AATD 的情况下，这会增加肾衰竭（例如肾病综合征）发展的可能性。需要进一步的临床研究来了解肾病（即膜性肾病）的发展与 AATD 之间的关系。