Department of Nephrology, National Center for Global Health and Medicine, Japan.
Department of Nephrology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Japan.
Intern Med. 2021 Jun 15;60(12):1963-1969. doi: 10.2169/internalmedicine.6399-20. Epub 2021 Feb 1.
The first case of eosinophilic granulomatosis with polyangiitis (EGPA) simultaneously demonstrating various clinical manifestations, including retroperitoneal fibrosis (RPF) causing hydronephrosis and membranous nephropathy (MN) leading to nephrotic syndrome, is presented. There have been no previous case reports demonstrating the simultaneous onset of these three disease categories with significant complex pathologies. This case was successfully managed by providing adequate combination therapies according to each disease category, leading to complete remission (CR) of all three diseases. In conclusion, we believe this case is extremely rare and clinically suggestive, and that these findings can be applied to a future phenotype-tailored treatment strategy for EGPA.
本文报道了首例嗜酸性肉芽肿性多血管炎（EGPA）同时表现出多种临床表现的病例，包括导致肾积水的腹膜后纤维化（RPF）和导致肾病综合征的膜性肾病（MN）。此前尚无病例报告显示这三种疾病类别同时出现并伴有严重的复杂病理。通过根据每个疾病类别提供充分的联合治疗，该病例得到了成功的管理，导致三种疾病均完全缓解（CR）。总之，我们认为这种情况极为罕见且具有临床提示意义，这些发现可应用于未来针对 EGPA 的表型定制治疗策略。
