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A nderson-Fabry disease (AFD) is an X-linked lysosomal storage disease caused by a loss-of-function mutation in the lysosomal enzyme α-galactosidase A (GLA) gene, resulting in progressive intracellular accumulation of neutral glycosphingolipids, particularly globotriaosylceramid (Gb3), in different tissues including skin, kidney, central and peripheral nervous systems, and the heart.1 Patients with AFD suffer from various cardiac complications, including hypertrophic cardiomyopathy, valvular diseases, conduction defects and arrhythmias, as well as coronary artery stenosis, caused by Gb3 accumulation in vascular endothelial and smooth muscle cells (VSMCs) (Figure);2,3 however, the prevalence of coronary spastic angina (CSA) has not been clearly demonstrated among AFD patients previously. In this issue of the Journal, Kitani et al4 describe the clinical presentations and results of intracoronary acetylcholine provocation test5 in 9 consecutive patients with AFD. They found a high prevalence (8/9 patients) of acetylcholine-induced CSA without coronary artery stenosis.4 It should be noted that all 9 patients complained of angina at rest,4 which suggests possible self-selection bias to undergo cardiac assessment, and therefore, raises prior probability of a positive diagnosis of CSA even among consecutive AFD patients. Nonetheless, this report is the first to describe a high prevalence of CSA in a series of Japanese patients with AFD. Interestingly, 6 of the 8 acetylcholine-positive patients were female in this study,4 in contrast to the fact that as low as 13% of Japanese CSA patients are female.5 However, the significance of either sex or ethnicity in the prevalence of CSA was indeterminate in this study.

Unexpected, But Reasonable Association Between Anderson-Fabry Disease and Coronary Vasospasm