Fabry disease linked with increased mortality risk in transplant patients
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Following kidney transplant, patients with Fabry disease had higher mortality rates than those without the disease. However, among patients on dialysis, those with Fabry disease had better mortality rates than patients without the disease.
“Similar to those on dialysis without Fabry disease (FD), the major cause of death amongst people with FD on dialysis was cardiovascular disease suggesting that the excess mortality risk was likely secondary to FD disease progression in non-kidney tissues,” Monica S. Ng, PhD, of the department of nephrology at Princess Alexandra Hospital in Australia, and colleagues wrote.
Using the Australian and New Zealand Dialysis and Transplant registry, researchers compared the outcomes of people with and those without FD receiving kidney replacement treatment (KRT). The dialysis cohort included 35 patients with FD and 79,400 without FD.
Patients with FD had mortality rates of 9%, 26% and 43%, respectively, at 1, 3 and 5 years. Patients without FD had mortality rates of 11%, 28% and 40%. The overall primary cause of death was cardiovascular disease. Patients with FD on dialysis had higher mortality rates compared with those without FD, according to results of a univariate analysis.
Patients who smoked cigarettes, had a BMI lower than 18.5 kg/m², initiated KRT prior to enzyme replacement therapy (ERT) availability and started dialysis later experienced increased mortality risk. However, patients of a “non-white” ethnicity with a BMI greater than 25 kg/m² had lower mortality risk.
The transplant group included 20 patients with FD and 26,511 patients without FD. Mortality rates at 1, 3 and 5 years in the FD group were 10%, 15% and 20%, respectively, vs. 6%, 10% and 14%, respectively, in the group without FD.
The small sample size of patients with FD impacted the findings; FD is often underdiagnosed in KRT populations, according to the researchers.
“In multivariable analysis, KRT commencement post-ERT era was associated with superior post-transplant mortality rates, suggesting a beneficial effect of ERT therapy,” Ng and colleagues wrote. “This aligns with the findings from observational studies of transplant recipients, with FD demonstrating similar mortality rates compared to non-FD transplant recipients and improved cardiac indices compared to untreated people after ERT.”
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