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- Takuya Sugiura, Reiko Muto, Tatsuaki Amano, Fumitaka Kamiya, Yuka Sato, Kayaho Maeda, Shoji Saito, Takayuki Katsuno, Noritoshi Kato, Michiko Higashi, Atsushi Numaguchi, Naoyuki Matsuda, Kazumitsu Sugiura, and Shoichi Maruyama.
- Department of Nephrology, Nagoya University Graduate School of Medicine, Japan.
- Intern. Med. 2025 Feb 8.
AbstractFabry disease (FD) is a rare X-linked lysosomal storage disorder. Enzyme replacement therapies (ERT), such as agalsidase α and β, are available treatment options. While infusion-related reactions (IRRs) are known to occur at the initiation of ERT owing to immune responses, there is limited information on IRRs during long-term ERT. We report the case of a female patient with Fabry disease who developed unexpected hypotension after six years of stable treatment with agalsidase α, leading to a switch to agalsidase β. Continuous monitoring may be essential to identify potential IRRs in female patients with Fabry disease receiving long-term ERT.
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