Homocysteine and Erythrocyte Sedimentation Rate Correlate with Cerebrovascular Disease in Fabry Disease

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Abstract
Cerebrovascular disease (CVD) is a common clinical problem in Fabry disease; however, expression of this disease manifestation is not uniform and risk factors for its development are not well studied. A number of common CVD risk factors are known in the general population, and these may also play a role in the development of CVD in Fabry disease. To evaluate the potential associations between various risk factors and CVD in patients with Fabry disease. Thirty-two Fabry disease patients were studied, with 15 having evidence of CVD. T-tests were used to compare the positive and negative CVD groups and logistic regression was used to look for correlations with CVD history. CVD-positive patients were older (49.73 vs. 37.59 years, p2, p < 0.005), higher homocysteine (17.79 vs. 10.53 μmol/L, p < 0.05) and erythrocyte sedimentation rate (ESR) levels (23.8 vs. 7.64 mm/h, p < 0.001), and elevated Mainz Severity Score Index (MSSI) scores (23.8 vs. 11.8, p < 0.001). Correlations were found between age (odds ratio (OR) 1.11), DTPA glomerular filtration rate (OR 0.95), homocysteine concentration (OR 1.22), ESR (OR 1.16) and the MSSI (OR 1.19) scores with a positive CVD history (all p < 0.05). Elevated homocysteine and ESR are independent risk factors for CVD in Fabry disease. This finding adds to our ability to predict those patients with Fabry disease who are at a higher risk of developing CVD, and may be an aid in deciding which patients should have primary CVD prevention therapies.

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