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Longitudinal Adipokine and Lipid Profiles in Fabry Disease
Constantin Gatterer1, Daniela Allmer1,2, Dietrich Beitzke3
1Division of Cardiology, Department of Internal Medicine II, Medical University of Vienna, 1090 Vienna, Austria.
Insights
In Fabry disease (FD), adiponectin levels correlate with cardiac involvement, while lipid profiles remain stable over time. This suggests adipokines, not dyslipidemia, may indicate cardiac manifestations in FD patients.
Area of Science:
- Cardiology
- Endocrinology
- Genetics
Background:
- Fabry disease (FD) is a genetic lysosomal storage disorder with significant renal and cardiac complications.
- Cardiac magnetic resonance imaging (CMR) aids structural assessment, but circulating biomarkers for cardiac involvement in FD are not fully understood.
- Adipokines, such as adiponectin and leptin, are implicated in inflammation, metabolism, and fibrosis, making them potential biomarkers.
Purpose of the Study:
- To investigate the association between adiponectin and leptin levels with cardiac involvement in FD patients.
- To analyze long-term lipid trajectories in FD patients.
- To explore the relationship between these biomarkers, cardiac manifestations, and FD-specific therapy.
Main Methods:
- A longitudinal observational study of 49 FD patients with 149 visits.
- Assessed circulating adiponectin, leptin, NT-proBNP, and lipids longitudinally.
- Used multivariable linear regression to analyze associations with NT-proBNP, stratified by therapy status and sex.
Main Results:
- Adiponectin positively correlated with NT-proBNP (cardiac involvement) independently of other factors (p < 0.001).
- Higher adiponectin was linked to left ventricular hypertrophy and fibrosis on CMR (p = 0.009, p < 0.001), particularly in treated patients.
- Lipid parameters (adiponectin, leptin, triglycerides, cholesterol) remained stable long-term, regardless of therapy or sex.
Conclusions:
- Adiponectin is associated with cardiac involvement in Fabry disease.
- Conventional lipid parameters are stable over time in FD patients.
- Adipokine alterations, rather than dyslipidemia, may serve as indicators of cardiac manifestations in FD.
Abstract:
Background/Objectives: Fabry disease (FD) is a lysosomal storage disorder characterized by progressive renal and cardiac involvement and an increased burden of cardiovascular and cerebrovascular events. While cardiac magnetic resonance imaging (CMR) has significantly advanced structural assessment, circulating biomarkers reflecting disease-related cardiac manifestations remain incompletely understood. We therefore investigated adiponectin and leptin, two adipokines involved in inflammatory, metabolic, and fibrotic pathways, in relation to cardiac involvement and analyzed long-term lipid trajectories in FD. Methods: This longitudinal observational study included 49 patients with FD with 149 study visits. Circulating adiponectin, leptin, NT-proBNP, and conventional lipid parameters were assessed longitudinally and stratified by FD-specific therapy status and sex. Multivariable linear regression was performed to evaluate independent associations with log-transformed NT-proBNP values. Results: Adiponectin was positively associated with NT-proBNP, reflecting cardiac involvement, independent of age, sex, BMI, and eGFR (p < 0.001). Higher adiponectin levels were observed in patients with left ventricular hypertrophy or low T1 and those with fibrosis, detected by CMR (p = 0.009 and p < 0.001, respectively). This association was mainly seen in patients receiving FD-specific therapy, raising the question of whether this reflects underlying organ involvement or treatment effects. Leptin demonstrated weaker, inverse associations. Adiponectin, leptin, Triglycerides, total cholesterol, and HDL- and LDL-cholesterol levels remained stable over long-term follow-up, irrespective of FD-specific therapy or sex. Conclusions: In FD, adiponectin appears to be associated with cardiac involvement, and conventional lipid parameters remained unchanged over time. These findings suggest that alterations in adipokines, rather than progressive dyslipidemia, may reflect disease-related cardiac manifestations.
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