High Blood Press Cardiovasc Prev 2020 Dec 12;27(6):539-546. Epub 2020 Oct 12.
Division of Internal Medicine and Nephrology, Department of Life, Health and Environmental Sciences, San Salvatore Hospital, University of L'Aquila, San Salvatore Hospital, Building Delta 6, L'Aquila, Italy.
Fabry disease is a progressive, X-linked inherited lysosomal storage disorder where accumulation of glycosphingolipids increases the risk for early cardiovascular complications, including heart failure, stroke, and end stage renal disease. Besides disease-specific therapy, blood pressure (BP) control is of central importance in Fabry disease to reduce disease progression and improve prognosis. Both Fabry disease and hypertension are characterized by the activation of the innate component of the immune system, with Toll-like receptor 4 (TLR4) as a common trigger to the inflammatory cascade. Read More