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No additional Fabry-related risks in COVID-19, small study results | Severe COVID-19 risk appears to be related to immune system function

The risk of severe COVID-19 in people with Fabry disease appears to be determined by immune system function rather than the genetic disorder itself — “similar to that in the general population” — according to a small study.

“Immunosuppressive therapy in kidney transplant recipients represented the highest risk [patient] population,” the researchers wrote.

Further studies of Fabry disease and COVID-19 could reveal potential protective responses in these patients, which could be of interest for future treatments, the researchers noted.

The Little Study,”COVID-19 in Fabry disease: a prospective reference center study“, was published in Orphanet Journal of Rare Diseases.

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Fabry is a rare inherited disease that mainly affects the heart, nervous system and kidneys. There are two forms of Fabry: the classic, which typically appears in childhood or adolescence, and the late-onset form, which generally begins after age 30.

SARS-CoV-2, the virus that causes COVID-19, is primarily an infection of the lungs. But it can also affect various other organs, including the brain, heart, blood vessels, and kidneys. To reduce disease and mortality, vulnerable populations such as Fabry patients must be identified and treated early.

However, it is not known whether a SARS-CoV-2 infection poses a particular risk for Fabry patients.

Investigation of COVID-19 risk factors in Fabry patients

During the pandemic, researchers at Zurich University Hospital in Switzerland followed 104 unvaccinated people with Fabry and reported symptoms and duration of COVID-19, as well as testing for anti-SARS-CoV-2 antibodies.

“To our knowledge, this is the first clinical study that has systematically investigated SARS-CoV-2 infection [Fabry disease] patient during [the] Pandemic,” the team wrote.

The study included 24 men with classic Fabry and 12 with late-onset disease, and 49 women with classic Fabry and 19 with the late-onset form.

Thirteen (12.5%) participants were diagnosed with SARS-CoV-2 infection. Specifically, four men and six women with classic Fabry were found to have COVID-19, as were three men with late-onset disease.

Of the infected patients, five had no pre-existing damage to the heart, lungs, kidneys, or nervous system, while six had advanced Fabry involving the heart, kidney, or nervous system. Two of the participants were transplant recipients.

In all, two infected people developed severe COVID-19 that required hospitalization and oxygen supplements. Both patients were kidney transplant recipients receiving immunosuppressive therapy and also had cardiac disease.

“Our finding that kidney transplant recipients suffered the most from severe COVID-19 is of particular relevance to vaccination strategies,” the researchers write. “More specifically, the various immunosuppressive drugs are known to impair or suppress the response to vaccination through multiple mechanisms.”

The remaining eight infected people developed mild COVID-19 illness, while three showed no symptoms. Two patients experienced Fabry pain crisis and three developed long-lasting COVID-19-like signs characterized by persistent symptoms lasting four or more weeks.

None of the patients required intubation or critical care or died from COVID-19. All non-hospitalized people received treatment for symptoms such as pain and fever, if present. No COVID-related therapies were administered to hospitalized or non-hospitalized patients.

Blood samples were collected from 61 participants who were asymptomatic at the time. Five showed significant levels of anti-SARS-CoV-2 antibodies, but only two of them had COVID-19 symptoms with confirmed viral RNA in their medical records.

“In summary, the risk of severe COVID-19 is in [Fabry] Patients, like the general population, appear to be immune-driven rather than immune-driven [Fabry disease] itself,” the authors concluded. “Immunosuppression in kidney transplant recipients presented the highest risk in this population.”

“Further studies on lysosomal dysfunction in [Fabry disease] and SARS-CoV-2 infection could indicate potential protective or modified responses in these patients and could be of interest for future treatments,” the authors added.

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