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

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, considere...

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Bibliographic Details
Published in:Internal Medicine 2023, pp.1302-22
Main Authors: Mamizu, Hikaru, Ohta, Takeshi, Yanai, Kensuke, Yamazaki, Ryo, Mamizu, Maiko, Ishikawa, Daisuke, Kawakami, Hidenori, Furukawa, Toshiki, Ishida, Takashi
Format: Article
Language:English
Subjects:
activated T cell
eosinophilic granulomatosis with polyangiitis
IgG4-related disease
immunoglobulin G4 (IgG4)
mepolizumab
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Summary: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.
ISSN:0918-2918
1349-7235
DOI:10.2169/internalmedicine.1302-22