Table 1.
Conditions that mimic LVV.
| Infectious |
| Tuberculous aortitis |
| Vascular anomalies include true aneurysm formation, aortic narrowing, and pseudoaneurysm caused by hematogenous dissemination and/or vessel wall erosion |
| Syphilitic aortitis |
| Tertiary syphilis can lead to ascending aortic aneurysm, aortic valve regurgitation, and coronary ostia narrowing |
| IgG4-related disease |
| IgG4 plasma cell infiltration causes vessel wall thickening and luminal dilation of the aorta and its main branches |
| Erdheim-Chester's disease |
| Commonly associated vascular anomalies include vascular ectasia, stenoses, and periarterial thickening of the aorta and its main branches |
| Atherosclerosis |
| Degenerative aortic aneurysm |
| Central retinal artery occlusion or branch retinal artery occlusion |
| Acute monocular vision loss caused by atheromatous plaques or distal emboli mimicking arteritic ischemic optic neuropathy |
| Nonarteritic ischemic optic neuropathy |
| Monocular vision loss triggered by vascular insufficiency and disruption of the optic disc's autoregulation |
| Variable vessel vasculitides |
| Cogan's syndrome |
| Associated large-vessel vasculopathy can lead to aortic aneurysms and left-sided heart valvulitis |
| Behçet's syndrome |
| Vascular complications include aortic, pulmonary, and peripheral artery aneurysms, arterial and venous thromboses, or thromboangiitis |
| Vasculitis associated with systemic disease |
| Rheumatoid aortitis |
| Aortitis caused by long-standing untreated seropositive disease |
| Relapsing polychondritis |
| Aortitis leading to aortic aneurysms at risk of rupture and/or dissection |
| Seronegative arthritis |
| Aortitis associated with ankylosing spondylitis and peripheral spondyloarthropathies can lead to ascending aortic aneurysm, aortic root annulus dilation, and valvular regurgitation |
| Other conditions |
| Fibromuscular dysplasia |
| Often involves renal arteries but may also affect large arteries including carotid, vertebral, and intracranial arteries |
| Segmental arterial mediolysis |
| Most commonly involves medium-sized abdominal visceral arteries but may rarely affect all vessels including carotid arteries with a risk of cerebral infarction |