Premium
A large coronary aneurysm and its probable precursor lesions in a patient with autosomal dominant polycystic kidney disease: An implication for the process of aneurysmogenesis
Author(s) -
Ohara Kentaro,
Kimura Tokuhiro,
Karasawa Takaaki,
Tokuyama Hirobumi,
Wakino Shu,
Hayashi Koichi,
Itoh Hiroshi,
Okada Yasunori
Publication year - 2012
Publication title -
pathology international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
eISSN - 1440-1827
pISSN - 1320-5463
DOI - 10.1111/pin.12007
Subject(s) - autosomal dominant polycystic kidney disease , medicine , autopsy , fibromuscular dysplasia , aneurysm , asymptomatic , pathology , polycystic kidney disease , lesion , renal artery , cardiology , kidney , cyst , disease , radiology
Coronary artery aneurysms are rare complications of autosomal dominant polycystic kidney disease (ADPKD), and their pathogenesis remains poorly understood. We report an autopsy case of a 64‐year‐old ADPKD patient with an asymptomatic, large (4 cm in diameter) saccular aneurysm arising from the left circumflex (LCX) branch of the coronary artery with only mild atherosclerotic changes. Autopsy also revealed small, focal defects of media with or without microaneurysm formation in the LCX, mesenteric and renal arteries, and a fibromuscular dysplasia‐like lesion with microaneurysm in the common iliac artery. Since polycystin‐1 and ‐2 are expressed in arterial smooth‐muscle cells, these findings imply that abnormal polycystin expression in ADPKD initially causes the focal medial defects, some of which might later progress to microaneurysms and then overt aneurysms. To the best of our knowledge, this is the first description of the pathologic findings of an ADPKD‐associated coronary aneurysm and its probable precursor lesions in arteries.