• Internal medicine · Jun 2021

    The First Case of Eosinophilic Granulomatosis with Polyangiitis Simultaneously Demonstrating Various Clinical Manifestations with Retroperitoneal Fibrosis and Membranous Nephropathy.

    • Isao Kondo, Yohei Arai, Emi Sakamoto, Daisuke Katagiri, and Fumihiko Hinoshita.
    • Department of Nephrology, National Center for Global Health and Medicine, Japan.
    • Intern. Med. 2021 Jun 15; 60 (12): 1963-1969.

    AbstractThe 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.

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