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Prevalence of uncontrolled hypertension in patients with Fabry disease

Julia Kleinert1, François Dehout, Andreas Schwarting

  • 1Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna, Vienna, Austria.

American Journal of Hypertension
|August 1, 2006
PubMed

Insights

Fabry disease patients have a high prevalence of uncontrolled hypertension. Enzyme replacement therapy showed a significant decrease in blood pressure (BP) over two years, highlighting the need for improved BP management.

Area of Science:

  • Nephrology
  • Cardiology
  • Genetics

Background:

  • Fabry disease is a rare X-linked disorder caused by alpha-galactosidase A deficiency.
  • This deficiency leads to severe renal, cardiac, and cerebrovascular complications.
  • Elevated blood pressure (BP) is a critical factor in these outcomes.

Purpose of the Study:

  • To determine the prevalence of uncontrolled hypertension in Fabry disease patients.
  • To investigate the impact of enzyme replacement therapy (ERT) on BP in this population.

Main Methods:

  • Analysis of uncontrolled hypertension (systolic BP ≥130 mm Hg or diastolic BP ≥80 mm Hg) in 391 Fabry disease patients from the Fabry Outcome Survey (FOS).
  • Assessment of BP trends during a 2-year course of ERT.

Main Results:

  • Uncontrolled hypertension was observed in 57% of men and 47% of women.
  • Patients with CKD stages 1, 2, and 3 showed varying median BP levels.
  • A significant reduction in both systolic and diastolic BP was noted after 2 years of ERT.

Conclusions:

  • Fabry disease is associated with a high prevalence of uncontrolled hypertension.
  • Improved BP control and renoprotection strategies are crucial for managing Fabry disease patients.
Abstract

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