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(Circulation. 2008;117:242-252.)
© 2008 American Heart Association, Inc.

Aortic Diseases

Aortic Aneurysms

An Immune Disease With a Strong Genetic Component

Helena Kuivaniemi, MD, PhD; Chris D. Platsoucas, PhD; M. David Tilson, III, MD

From the Center for Molecular Medicine and Genetics and Department of Surgery, Wayne State University School of Medicine, Detroit, Mich (H.K.); Department of Microbiology and Immunology, Temple University School of Medicine, Philadelphia, Pa (C.D.P.); and St Luke’s/Roosevelt Hospital Center, Continuum Health Partners, and Columbia University, New York, NY (M.D.T.).

Correspondence to Helena Kuivaniemi, MD, PhD, Center for Molecular Medicine and Genetics, Wayne State University School of Medicine, 540 E Canfield Ave, Detroit, MI 48201. E-mail kuivan@sanger.med.wayne.edu

Key Words: aorta • aneurysm • genetics • immune system • immunology

An extract of the first 250 words of the full text is provided, because this article has no abstract.

	Introduction

 
In the United States, aortic aneurysms are the 13th leading cause of death.^1,2 Approximately 15 000 individuals die every year because of the rupture of aortic aneurysms. On the basis of autopsy studies, it has been estimated that 1% to 2% of the population harbor aneurysms in their aorta, with up to 10% prevalence in older age groups.^1,2 Most aortic aneurysms go undetected until rupture, and the mortality from ruptured aneurysms is as high as 90%.^1,2

Along the length of the aorta, significant heterogeneity occurs in the distribution of aneurysm disease. The prevalence of abdominal aortic aneurysms (AAAs) located in the infrarenal section of the aorta is at least 3 times higher than that of thoracic aortic aneurysms and dissections (TAADs).^1,2 In TAADs, 50% involve the ascending aorta, 10% the arch, and 40% the descending thoracic aorta.^1,2 Only 25% of patients with TAAD have a concomitant AAA, and multisegmental disease is found in only 10% of cases.^1,2

There are also other differences between TAAD and AAA: (1) Age at onset for TAAD (65 years) is 10 years earlier than for AAA (75 years), and (2) AAAs are predominantly a disease of white men, with a 6:1 male-to-female ratio, whereas TAADs occur only slightly more frequently in men (1.7:1). Additional differences can be found in the pathobiology of these aneurysms. AAAs are characterized by signs of local chronic inflammation of the aortic wall, decrease in the number of smooth muscle cells in the aortic media layer, and fragmentation of the extracellular . . . [Full Text of this Article]

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