Review Article

A Review of Primary Vasculitis Mimickers Based on the Chapel Hill Consensus Classification

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