Elevated Ambulatory Blood Pressure Measurements Are Associated With a Progressive Form of Fabry Disease

Abstract

Background and objectivesPublished data on hypertension incidence and management in Anderson-Fabry disease are scanty and it remains to be shown how much an elevated blood pressure contributes to the patient’s organ damage. Therefore, we have assessed blood pressure values and their correlations with clinical findings in a cohort of Fabry patients.MethodsBetween January 2015 and May 2019, all adult Fabry patients (n = 32; n = 24 females, n = 8 males) referred to our institute were enrolled. Data regarding hypertension were obtained by ambulatory blood pressure monitoring, home self-monitoring and repeated office measurements. Organ involvement and hypertension risk factors were also evaluated.ResultsThe ambulatory monitoring revealed elevated blood pressure in 18.75% (n = 6) of the Fabry population and 50% of this group was diagnosed with masked hypertension. All these patients were females and they presented a lower glomerular filtration rate and a more advanced cardiac hypertrophy compared with normotensive subjects. They were mostly (66.7%) affected by a progressive form of the disease while the majority of the normotensives (84.6%) were stable. No correlation was found between the category of GLA mutation and the development of hypertension.ConclusionNewly detected hypertension can be found in a restricted portion of stable Fabry patients while it becomes more prevalent among clinically progressive cases. The use of the ambulatory blood pressure monitoring is of paramount importance to reveal masked hypertension which can contribute to the progressive worsening of the organ damage. Therefore, a proper diagnosis and therapy of hypertension may improve the outcome of Fabry patients.

Fabry Disease: Screening and Analysis of the Associated Clinical Manifestations in Patients Attending Dialysis and Nephrology Clinics in Durban, South Africa

“…They concluded that a decrease in heart rate variability, rather than an increase in blood pressure variability, might be an early indicator of FD (22). Another study conducted by Rossi et al also demonstrated a lower prevalence of hypertension in patients with ESKD, pre-renal, and patients with transplants (23). In our study, there was a signi cance (p = < 0.001) between hypertension and patients with alpha-Gal-A levels < 500pg/ml when the univariate analysis was employed.…”

Blood Pressure Variability in Fabry Disease Patients

“…Schiffman et al [26] conducted a study involving 279 male and 168 female FD patients and reported that nearly 80% of both males and females with CKD at baseline were hypertensive, while only about 50% of those with a baseline eGFR ≥60 mL/min/1.73 m 2 were hypertensive. In a recent study by Rossi et al [29], ambulatory monitoring showed elevated BP in 6 (18.75%) of 31 FD patients, and masked hypertension was present in 3 of them. The hypertensive patients had a lower GFR in comparison with normotensive subjects.…”