Consider Cardiomyopathy in Subjects With Familial Partial Lipodystrophy

To the Editor:Hegele1 excellently described the presence of premature coronary artery disease in 8 out of 23 adult subjects (aged above 35 years) with familial partial lipodystrophy (FPL) carrying LMNA mutations. His intriguing observations underline the striking similarity between FPL and the metabolic syndrome (also called syndrome X). The latter is known to result in premature coronary artery disease. In FPL, the phenotype of altered fatty tissue distribution and the prominence of the skeletal muscles, especially of the lower limbs, precedes the manifestation of dyslipidemia and diabetes mellitus. Dyslipidemia in turn seems to occur many years before diabetes mellitus may occur.2 However, it is remarkable, that Hegele reported on insulin resistance in all studied subjects, although 18/23 had dyslipidemia and 12/23 had diabetes mellitus. Does hyperinsulinemia and insulin resistance, respectively, precede dyslipidemia and diabetes mellitus in this selected cohort of patients? The observed coronary artery disease in FPL carrying LMNA mutations is presumably caused by the cardiovascular risk factors, …