Unexpected Hypotension in a Female Patient with Fabry Disease: Switching from Agalsidase α to β after Long-term ERT

Fabry disease is a rare X-linked lysosomal storage disorder. Enzyme replacement therapies (ERTs), 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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Publication Details

Journal
Internal Medicine
Published
2025-02-07
DOI
https://doi.org/10.2169/internalmedicine.4685-24
Citations
3
Primary Topic
Lysosomal Storage Disorders Research
Type
article
Field-Weighted Citation Impact
3.49
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article

Unexpected Hypotension in a Female Patient with Fabry Disease: Switching from Agalsidase α to β after Long-term ERT

Shoji Saito, Reiko Muto, Naoyuki Matsuda, Kazumitsu Sugiura et al.
3 citations
Internal Medicine
Lysosomal Storage Disorders Research
3.49
article

Unexpected Hypotension in a Female Patient with Fabry Disease: Switching from Agalsidase α to β after Long-term ERT

Shoji Saito, Reiko Muto, Naoyuki Matsuda, Kazumitsu Sugiura, Tetsuya Amano, Yuka Sato, Shoichi Maruyama, Takuya Sugiura, Noritoshi Kato, Atsushi Numaguchi, Michiko Higashi, Takayuki Katsuno, Kayaho Maeda, Fumitaka Kamiya
article en
3 citations

Abstract

Fabry disease is a rare X-linked lysosomal storage disorder. Enzyme replacement therapies (ERTs), 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.

Internal MedicineVol. 64(15)
Fujita Health University (JP), Aichi Medical University (JP), Handa City Hospital (JP), Komaki City Hospital (JP), Aichi Medical University Medical Center, Nagoya University (JP)
Gender equality
Openalex Percentile: Top 9%
Lysosomal Storage Disorders Research
3.49
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Unexpected Hypotension in a Female Patient with Fabry Disease: Switching from Agalsidase α to β after Long-term ERT — Shoji Saito, Reiko Muto, et al. · Internal Medicine (2025) | TGRS Research Map | TGRS