Fabry disease and COVID-19: international expert recommendations for management based on real-world experience

• Authors: Dawn Laney, Emory University; Dominque P. Germain, Universite Paris Saclay; João Paulo Oliveira, University of Porto; Alessandro P. Burlina, St Bassiano Hospital; Gustavo Horacio Cabrera, Provincia de Buenos Aires; Geu-Ru Hong, Yonsei University; Robert J. Hopkin, University of Cincinnati; Dau-Ming Niu, Taipei Veterans General Hospital; Mark Thomas, Royal Perth Hospital; Hernán Trimarchi, British Hospital of Buenos Aires; William R. Wilcox, Emory University; Juan Manuel Politei, Fundacion Para el Estudio de Enfermedades Neurometabolicas; Alberto Ortiz, IIS-Fundacion Jimenez Diaz
• Journal Title: CLINICAL KIDNEY JOURNAL
• Volume: Volume 13, Number 6
• Publisher: OXFORD UNIV PRESS | 2020-12-01, Pages 913-925
• Type of Work: Article | Final Publisher PDF
• Publisher DOI: http://doi.org/10.1093/ckj/sfaa227
• Subjects: Biology, Virology; Health Sciences, Medicine and Surgery
• Keywords: Science & Technology; Life Sciences & Biomedicine; Urology & Nephrology; chloroquine; COVID-19; enzyme replacement therapy; Fabry disease; lysosome; pathogenesis; prevention; SARS-CoV-2; CARDIAC MANIFESTATIONS; NATURAL-HISTORY; INVOLVEMENT; INFECTIONS; DEPRESSION

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.