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COVID-19 in Fabry disease: a reference center prospective study
Christina Bothou1, Lanja Saleh2, Arnold von Eckardstein2
1Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ) and University of Zurich (UZH), Rämistrasse 100, 8091, Zurich, Switzerland.
Insights
Fabry disease patients experienced COVID-19 at a rate of 12.5%, with kidney transplant recipients facing severe illness. Overall risk appears linked to immune status, not Fabry disease itself.
Area of Science:
- Rare diseases
- Infectious diseases
- Genetics
Background:
- Fabry disease (FD) is an X-linked lysosomal storage disorder associated with chronic kidney disease, cardiomyopathy, pneumonopathy, and strokes.
- The impact of SARS-CoV-2 infection on FD patients remains largely unknown, necessitating identification of at-risk populations during the COVID-19 pandemic.
Purpose of the Study:
- To investigate the risk and clinical outcomes of SARS-CoV-2 infection in unvaccinated patients with Fabry disease.
- To identify factors contributing to severe COVID-19 in the FD population.
Main Methods:
- A cohort of 104 unvaccinated FD patients was monitored between February 2020 and March 2021.
- Serum antibody titers against SARS-CoV-2 were measured in 61 patients, and PCR testing was performed for symptomatic individuals or those with family exposure.
- Clinical data on COVID-19 symptoms, duration, and severity were collected from patients and physicians.
Main Results:
- 12.5% (13/104) of FD patients were diagnosed with SARS-CoV-2 infection.
- Two kidney transplant recipients experienced severe COVID-19, requiring hospitalization and high-flow oxygen.
- The majority of infected patients had mild (61.5%) or asymptomatic (23.1%) disease; 15.4% reported Fabry pain crises and 23.1% experienced long COVID-19 symptoms.
Conclusions:
- The risk of severe COVID-19 in FD patients appears primarily influenced by immune system status, similar to the general population.
- Immunosuppression, particularly in kidney transplant recipients, represents the most significant risk factor for severe COVID-19 in this cohort.
Background:
During the coronavirus disease-19 (COVID-19) pandemic, vulnerable populations must be identified to prevent increased mortality. Fabry disease (FD) is a rare X-linked lysosomal storage disorder leading to chronic kidney disease (CKD), cardiomyopathy, pneumonopathy and premature strokes. Little is known whether SARS-CoV-2 infection bears a particular risk for FD patients.
Methods:
During pandemic (02.2020-03.2021) we have regularly followed 104 unvaccinated FD patients. In 61/104, titre of serum antibodies against SARS-CoV-2 were measured and SARS-CoV-2 PCR test was performed in symptomatic patients or in case of positivity of other family members. The symptoms and duration of COVID-19 were reported by the patients or the treating physician.
Results:
No deaths or intensive care unit hospitalizations occurred. 13/104 (12.5%) were diagnosed with SARS-CoV-2 infection (16.7% (4/24) men 12.2% (6/49) women of classic phenotype, 25% (3/12) of the men and 0% (0/8) of the women of 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 rest either developed mild COVID-19 manifestations (8/13, 61.5%) or were asymptomatic (3/13, 23.1%). 2/13 (15.4%) of the patients experienced Fabry pain crisis and 3/13 (23.1%) long COVID-19 like symptoms.
Conclusions:
Similar to the general population, in FD patients the risk for severe COVID-19 seems to be driven by the immune system rather than by FD itself. Immunosuppression in kidney transplant recipients represented the highest risk in this population.
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