Pathologic Findings in Progeria: Myocardial Fibrosis and Lipofuscin Pigment

Abstract

The pathologic findings in two patients with progeria are described. The two patients had several features in common: the history of congestive heart failure preceding death, the pathologic findings of interstitial and focal myocardial fibrosis and necrosis, and intracellular accumulation of lipofuscin pigment in many organs. One patient also had chronic glomerulonephritis. A common belief has been that the cardiac lesion in progeria is typical myocardial infarction. However, even in the absence of significant coronary artery disease, focal myocardial fibrosis has been noted in previous cases as well as in the patients in this report, and, therefore, it is clear that both types of lesions occur in progeria. Finally, lipofuscin accumulation has not been noted previously in progeria. This finding lends support to the concept of progeria as a syndrome of premature aging.