Abstract

The rapid spread of coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 has raised questions about Fabry disease (FD) as an independent risk factor for severe COVID-19 symptoms. Available real-world data on 22 patients from an international group of healthcare providers reveals that most patients with FD experience mild-to-moderate COVID-19 symptoms with an additional complication of Fabry pain crises and transient worsening of kidney function in some cases; however, two patients over the age of 55 years with renal or cardiac disease experienced critical COVID-19 complications. These outcomes support the theory that pre-existent tissue injury and inflammation may predispose patients with more advanced FD to a more severe course of COVID-19, while less advanced FD patients do not appear to be more susceptible than the general population. Given these observed risk factors, it is best to reinforce all recommended safety precautions for individuals with advanced FD. Diagnosis of FD should not preclude providing full therapeutic and organ support as needed for patients with FD and severe or critical COVID-19, although a FD-specific safety profile review should always be conducted prior to initiating COVID-19-specific therapies. Continued specific FD therapy with enzyme replacement therapy, chaperone therapy, dialysis, renin–angiotensin blockers or participation to clinical trials during the pandemic is recommended as FD progression will only increase susceptibility to infection. In order to compile outcome data and inform best practices, an international registry for patients affected by Fabry and infected by COVID-19 should be established.

Details

Title
Fabry disease and COVID-19: international expert recommendations for management based on real-world experience
Author
Laney, Dawn A 1 ; Germain, Dominique P 2 ; Oliveira, João Paulo 3 ; Burlina, Alessandro P 4 ; Cabrera, Gustavo Horacio 5 ; Geu-Ru Hong 6 ; Hopkin, Robert J 7 ; Dau-Ming Niu 8 ; Thomas, Mark 9 ; Trimarchi, Hernán 10 ; Wilcox, William R 1 ; Politei, Juan Manuel 11 ; Ortiz, Alberto 12 

 Division of Medical Genetics, Department of Human Genetics, Emory University School of Medicine, Atlanta, GA, USA 
 Division of Medical Genetics, University of Versailles, AP-HP Paris Saclay University, Paris, France 
 Centro Hospitalar Universitário de São João & Faculdade de Medicina da Universidade do Porto, Porto, Portugal 
 Neurology Unit, St Bassiano Hospital, Bassano del Grappa, Italy 
 Santa Maria de la Salud, San Isidro, Provincia de Buenos Aires, Argentina 
 Department of Cardiology, Yonsei University Severance Hospital, Seoul, Korea 
 Division of Human Genetics, Cincinnati Children's Hospital Medical Center, and Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA 
 Department of Pediatrics, Taipei Veterans General Hospital, Taipei, Taiwan 
 Department of Nephrology, Royal Perth Hospital, Perth, Australia 
10  Nephrology service, British Hospital, Buenos Aires, Argentina 
11  Department of Neurology, Fundacion Para el Estudio de Enfermedades Neurometabolicas (FESEN), Buenos Aires, Argentina 
12  Unidad de Dialisis, IIS-Fundacion Jimenez Diaz, School of Medicine, UAM, IRSIN and REDINREN, Madrid, Spain 
Pages
913-925
Publication year
2020
Publication date
Dec 2020
Publisher
Oxford University Press
ISSN
20488505
e-ISSN
20488513
Source type
Scholarly Journal
Language of publication
English
ProQuest document ID
3169588937
Copyright
© The Author(s) 2020. Published by Oxford University Press on behalf of ERA-EDTA. This work is published under http://creativecommons.org/licenses/by-nc/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.