A Case of Eosinophilic Granulomatosis with Polyangiitis Similar to IgG4-related Disease

Ashikaga, Eru; Odaka, Hidesato; Asahi, Ruriko; Takahashi, Susumu; Kurokawa, Hirokazu; Enomoto, Katsuhiko

doi:10.2169/naika.110.2434

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2023

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“…However, IgG4-RD rarely develops alone. In many cases it is complicated with IgG4-RD of glandular tissues such as the pancreas, lacrimal glands, salivary glands, and parotid glands of other organs ( 10 ). In the present case, CT showed no lesions of IgG4-RD in the glandular tissues.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2023

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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.

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Section: Discussionmentioning

confidence: 99%