Fatal Aortic Dissection due to a Fulminant Variety of Isolated Aortitis

Abstract

Aortitis is typically a chronic, progressive disease manifestation associated with large vessel vasculitidies, most notably giant cell, Takayasu arteritis, and a newly described entity, isolated aortitis. The aortitis may lead to aneurysm formation and symptoms associated with branch vessel occlusion in these diseases, but aortic dissection is rare and usually a late complication of smoldering, incompletely treated disease. We present a case of aortitis in a previously healthy 39-year-old man who succumbed to aortic dissection hours after the onset of symptoms. No aneurysm or fibrosis was found on postmortem examination. The inflammation was characterized by disruption of the media with patchy transmural chronic and focally acute inflammatory infiltrate. We review case reports of other individuals with aortitis, who initially or very early in their course presented with aortic dissection in the absence of known rheumatic disease and most without evidence of aneurysm formation. We believe that this represents a process characterized by an aggressive vasculitis of the aorta with its own clinical features, a fulminant variety of isolated aortitis.

Relationship between intralobar pulmonary sequestration and type A aortic dissection: A case report

“…Acute aortic syndromes, like aortic dissection and rupture, can occur in such patients with aortitis[ 6 ]. Ryder et al [ 7 ] presented a case of a previously healthy 39-year-old man who succumbed to aortic dissection hours after the onset of symptoms. Aortitis was detected during the postmortem examination in this case[ 7 ].…”

“…Ryder et al [ 7 ] presented a case of a previously healthy 39-year-old man who succumbed to aortic dissection hours after the onset of symptoms. Aortitis was detected during the postmortem examination in this case[ 7 ]. They also identified a cohort of patients who presented with a subtype of isolated inflammatory aortitis, which was characterized by aggressive vasculitis with acute inflammatory infiltration.…”