• Internal medicine · Oct 2023

    Case Reports

    A Case of Refractory Eosinophilic Granulomatosis with Polyangiitis Complicated with IgG4-related Disease Showing Different Treatment Responses for Each Organ.

    • Hikaru Mamizu, Takeshi Ohta, Kensuke Yanai, Ryo Yamazaki, Maiko Mamizu, Daisuke Ishikawa, Hidenori Kawakami, Toshiki Furukawa, and Takashi Ishida.
    • Department of Respiratory Medicine, Niigata Prefectural Central Hospital, Japan.
    • Intern. Med. 2023 Oct 15; 62 (20): 299530002995-3000.

    AbstractWe herein report a 75-year-old woman who presented with dyspnea and purpura. She was diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA) based on axonal damage observed in the left tibial nerve and skin and lung pathologies. Lung pathology showed IgG4-positive plasma cells, considered a complication of IgG4-related disease (IgG4-RD). Computed tomography revealed thickening of the abdominal aorta and a poor contrast area in the left kidney, which was indicative of IgG4-RD. Steroid administration improved the IgG4-RD. However, the EGPA resisted treatment; therefore, immunosuppressive drugs and mepolizumab were administered. Refractory EGPA complicated with IgG4-RD showed different treatment responses for each organ.

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