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

被引：0

作者：

Sugiura, Takuya ^{[1
]}

Muto, Reiko ^{[1
,2
]}

Amano, Tatsuaki ^{[3
]}

Kamiya, Fumitaka ^{[4
]}

Sato, Yuka ^{[1
]}

Maeda, Kayaho ^{[1
]}

Saito, Shoji ^{[1
]}

Katsuno, Takayuki ^{[5
]}

Kato, Noritoshi ^{[1
]}

Higashi, Michiko ^{[6
]}

Numaguchi, Atsushi ^{[6
]}

Matsuda, Naoyuki ^{[6
]}

Sugiura, Kazumitsu ^{[7
]}

Maruyama, Shoichi ^{[1
]}

机构：

[1] Nagoya Univ, Grad Sch Med, Dept Nephrol, Nagoya, Japan

[2] Nagoya Univ, Grad Sch Med, Dept Renal Replacement Therapy, Nagoya, Japan

[3] Handa City Hosp, Dept Nephrol, Handa, Japan

[4] Komaki City Hosp, Dept Nephrol, Komaki, Japan

[5] Aichi Med Univ, Med Ctr, Dept Nephrol & Rheumatol, Nagakute, Japan

[6] Nagoya Univ, Grad Sch Med, Dept Emergencyand Crit Care Med, Nagoya, Japan

[7] Fujita Hlth Univ, Sch Med, Dept Dermatol, Toyoake, Japan

来源：

INTERNAL MEDICINE | 2025年

关键词：

Fabry disease; Female; enzyme replacement therapy; infusion-related reactions; ENZYME REPLACEMENT THERAPY; PLASMA GLOBOTRIAOSYLSPHINGOSINE LEVELS;

D O I：

10.2169/internalmedicine.4685-24

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Fabry disease (FD) is a rare X-linked lysosomal storage disorder. Enzyme replacement therapies (ERT), such as agalsidase alpha and beta, 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 alpha, leading to a switch to agalsidase beta. Continuous monitoring may be essential to identify potential IRRs in female patients with Fabry disease receiving long-term ERT.

引用

页数：6

共 50 条

[31] Successful desensitization of a patient with Fabry disease with agalsidase beta (Fabrazyme) anaphylaxis after omalizumab pretreatment
DuBuske, Ilona
Schmidlin, Kristin
Bernstein, Jonathan A.
ANNALS OF ALLERGY ASTHMA & IMMUNOLOGY, 2021, 126 (01) : 96 - 98
[32] Dose-dependent impact of ERT on neutralizing anti-drug antibodies and long-term outcomes in Fabry disease
Lenders, Malte
Neusser, Leon Paul
Rudnicki, Michael
Nordbeck, Peter
Canaan-Kuehl, Sima
Nowak, Albina
Cybulla, Markus
Schmitz, Boris
Lukas, Jan
Wanner, Christoph
Brand, Stefan-Martin
Brand, Eva
MOLECULAR GENETICS AND METABOLISM, 2019, 126 (02) : S92 - S93
[33] Renal and secondary outcomes 1 year after switching from agalsidase alfa to pegunigalsidase alfa for Fabry disease (the BRIDGE study)
Hughes, Deralynn
Dostalova, Gabriela
Nicholls, Kathy
West, Michael
Tondel, Camilla
Jovanovic, Ana
Giraldo, Pilar
Vujkovac, Bojan
Hiwot, Tarekegn
Almon, Einat
Alon, Sari
Szlaifer, Mali
Chertkoff, Raul
Linhart, Ales
MOLECULAR GENETICS AND METABOLISM, 2021, 132 : S107 - S108
[34] Enzyme Replacement Therapy (ERT) with agalsidase site leads to Regression of Left Ventricular Hypertrophy in Male and Female Patients with Anderson Fabry Disease (AFD).
Kampmann, C
Baehner, FA
Martin, C
Wiethoff, CM
Whybra, C
Miebach, E
Ries, M
Beck, M
AMERICAN JOURNAL OF HUMAN GENETICS, 2002, 71 (04) : 580 - 580
[35] Female Fabry disease: significant improvement of Fabry disease-related gastrointestinal symptoms in a large cohort of female patients treated with agalsidase beta: data from the Fabry Registry
Wilcox, William
Feldt-Rasmussen, Ulla
Martins, Ana Maria
Ortiz, Alberto
Weidemann, Frank
Lemay, Roberta
Hopkin, Robert
MOLECULAR GENETICS AND METABOLISM, 2014, 111 (02) : S114 - S115
[36] Clinical course of 13 patients with Fabry disease switching from agalsidase-α to agalsidase-β and who were followed for 36 months
Tsuboi, Kazuya
Yamamoto, Hiroshi
MOLECULAR GENETICS AND METABOLISM, 2013, 108 (02) : S93 - S93
[37] Long-term safety and efficacy of agalsidase beta in Japanese patients with Fabry disease: aggregate data from two post-authorization safety studies
Tsurumi, Mina
Suzuki, Shinya
Hokugo, Jiro
Ueda, Kazuo
EXPERT OPINION ON DRUG SAFETY, 2021, 20 (05) : 589 - 601
[38] The utility of the FIPI score in predicting long-term clinical outcomes in patients with Fabry disease receiving enzyme replacement therapy with agalsidase alfa
Mac Lochlainn, Dylan J.
McKechnie, Douglas G. J.
Mehta, Atul B.
Hughes, Derralynn A.
MOLECULAR GENETICS AND METABOLISM, 2018, 123 (02) : 154 - 158
[39] Neuropathologic findings in Fabry's disease patient after enzyme replacement therapy with agalsidase beta.
Rapckwicz, A
Abu-Asab, M
Tsokos, M
Schiffman, R
Quezado, M
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY, 2005, 64 (05): : 452 - 452
[40] UNEVENTFUL PREGNANCY OUTCOME AFTER ENZYME REPLACEMENT THERAPY WITH AGALSIDASE BETA IN A PATIENT HETEROZYGOUS FOR FABRY DISEASE
Germain, D. P.
Tran, T. -C.
Richalet, B.
Benistan, K.
CLINICAL THERAPEUTICS, 2009, 31 : S43 - S43

← 1 2 3 4 5 →