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; Takashi Ishida

doi:10.2169/internalmedicine.1302-22

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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, Takashi Ishida

著者情報

Hikaru Mamizu
Department of Respiratory Medicine, Niigata Prefectural Central Hospital, Japan
Takeshi Ohta
Department of Respiratory Medicine, Niigata Prefectural Central Hospital, Japan
Kensuke Yanai
Department of Respiratory Medicine, Niigata Prefectural Central Hospital, Japan
Ryo Yamazaki
Department of Respiratory Medicine, Niigata Prefectural Central Hospital, Japan
Maiko Mamizu
Department of Respiratory Medicine, Niigata Prefectural Central Hospital, Japan
Daisuke Ishikawa
Department of Respiratory Medicine, Niigata Prefectural Central Hospital, Japan
Hidenori Kawakami
Department of Respiratory Medicine, Niigata Prefectural Central Hospital, Japan
Toshiki Furukawa
Department of Respiratory Medicine, Niigata Prefectural Central Hospital, Japan
Takashi Ishida
Department of Respiratory Medicine, Niigata Prefectural Central Hospital, Japan

キーワード: eosinophilic granulomatosis with polyangiitis, IgG4-related disease, immunoglobulin G4 (IgG4), activated T cell, mepolizumab

ジャーナルオープンアクセス早期公開

論文ID: 1302-22

DOI https://doi.org/10.2169/internalmedicine.1302-22

この記事には本公開記事があります。

The final version of this article is now available: Vol. 62 (2023), No. 20 pp. 2995-3000

詳細

抄録

We 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.

責任著者(Corresponding author)

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