Reducing agalsidase beta infusion time in Fabry patients: low incidence of antibody formation and infusion-associated reactions in an Italian multicenter study

  • Published on 09/02/2024
  •  Reading time: 5 min.

Mignani Renzo 1, Americo Claudio 2, Aucella Filippo 3, Battaglia Yuri 4, Cianci Vittoria 5, Sapuppo Annamaria 6, Lanzillo Chiara 7, Pennacchiotti Fabio 8, Tartaglia Luciano 9, Marchi Giacomo 10, Pieruzzi Federico 11,12

1 https://ror.org/01111rn36 Nephrology, Dialysis and Transplantation, IRCCS S. Orsola Hospital University of Bologna Bologna Italy
2 Nephrology and Dialysis Unit Pierantoni Hospital Forlì Italy
3 Nephrology Unit Ospedale Casa Sollievo della Sofferenza San Giovanni Rotondo, Foggia Italy
4 Nephrology and Dialysis Unit St. Anna University Hospital Ferrara Italy
5 Regional Epilepsy Centre Great Metropolitan Hospital Reggio Calabria Italy
6 https://ror.org/03a64bh57 Pediatric Clinic, Department of Clinical and Experimental Medicine University of Catania Catania Italy
7 https://ror.org/04zhd1705 Division of Cardiology Policlinico Casilino Rome Italy
8 Nephrology Unit Manduria Hospital Taranto Italy
9 https://ror.org/01xtv3204 Nephrology Dialysis and Transplantation Unit, Department of Medical and Surgical Sciences University of Foggia Foggia Italy
10 https://ror.org/039bp8j42 MetabERN Referral Center for Lysosomal Storage Disorders, Internal Medicine Unit University of Verona Verona Italy
11 Clinical Nephrology, School of Medicine and Surgery University of Milano Bicocca Italy
12 Nephrology and Dialysis Unit Fondazione IRCCS San Gerardo dei Tintori Monza Italy

Abstract

Background Fabry disease is a rare progressive X-linked lysosomal storage disease caused by mutations in the GLA gene that encodes α-galactosidase A. Agalsidase beta is a recombinant enzyme replacement therapy authorized in Europe at a standard dose of 1.0 mg/kg intravenously every other week at an initial infusion rate of ≤ 0.25 mg/min until patient tolerance is established, after which the infusion rate may be increased gradually. However,...

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