COVID-19 in Fabry disease: A reference center prospective study

Orphanet J Rare Dis. 2022 Jun 28;17(1):250. doi: 10.1186/s13023-022-02386-7.

PMID: 35765080

Christina Bothou, Lanja Saleh, Arnold von Eckardstein

Highlights: In this study, it was investigated whether coronavirus infection poses a particular risk for Fabry patients.

Abstract

Background: To avoid a rise in mortality during the coronavirus disease-19 (COVID-19) pandemic, vulnerable groups must be identified. A rare X-linked lysosomal storage condition called Fabry disease (FD) causes early strokes, cardiomyopathy, pneumonopathy, and chronic kidney disease (CKD). If SARS-CoV-2 infection poses a special risk to people with FD, little is known about this.

Methods: 104 individuals with FD were regularly followed who were unvaccinated during the pandemic (02.2020–03.2021). Serum antibody titers against SARS-CoV-2 were assessed in 61/104 individuals, and SARS-CoV-2 PCR testing was done in cases of symptomatic patients or in cases when other family members tested positive. Patients or the treating physician documented the symptoms and duration of COVID-19.

Results: There were no hospitalizations for intensive care units or fatalities. 13/104 (12.5%) were diagnosed with SARS-CoV-2 infection (16.7% (4/24) of the men and 12.2% (6/49) of the women with the classic phenotype, and 25% (3/12) of the men, and 0% (0/8) of the women with the later-onset phenotype). Of those, 2/13 (15.4%) patients-both kidney transplant recipients-developed severe COVID-19, were hospitalized, and required a high-flow oxygen mask. The remainder were either asymptomatic (3/13, 23.1%) or only experienced minor COVID-19 symptoms (8/13, 61.5%). 2 out of 13 patients (15.4%) had Fabry pain crises, while 3 out of 13 (23.1%) had long COVID-19-like symptoms.

Conclusions: Similar to the general population, the immune system rather than FD itself appears to be the primary driver of the risk for severe COVID-19 in FD patients. The greatest risk in this category was immunosuppression in kidney transplant patients.

Keywords: Coronavirus disease 2019 (COVID-19), Fabry disease, SARS-CoV-2 Antibody Titer