Primary vasculitic causes of aortitis
| Diagnosis | Age at onset | Tissue pattern | Core symptoms and signs | Typical imaging features |
|---|---|---|---|---|
| Large-vessel vasculitis | ||||
| Giant cell arteritis | > 50 years | Granulomatous | Cranial or limb ischemia Constitutional symptoms High erythrocyte sedimentation rate and C-reactive protein Positive temporal artery biopsy Positive large-vessel imaging | More often in the thoracic than the abdominal aorta, and often branch vessel involvement |
| Takayasu arteritis | < 60 years | Granulomatous | Cranial or limb ischemia Constitutional symptoms with or without high erythrocyte sedimentation rate and C-reactive protein Positive large-vessel imaging | More often in the thoracic than the abdominal aorta, and usually branch vessel involvement |
| Clinically isolated aortitis | Any | Most often granulomatous, but can be any pattern | No constitutional symptoms or symptoms of systemic vasculitis or autoimmune rheumatic disease Normal C-reactive protein and erythrocyte sedimentation rate, negative serology | Most often in the thoracic aorta Can involve other segments No branch vessel involvement |
| Small-vessel or medium-vessel vasculitis | ||||
| Granulomatosis with polyangiitis | Any | Granulomatous, may see neutrophils and necrosis | Ear, nose, throat, lung, renal vasculitis most commonly, with or without involvement of skin, joints, nerves High erythrocyte sedimentation rate and C-reactive protein, positive antineutrophil cytoplasmic antibody, active urinalysis, positive lung and ear, nose, and throat imaging, positive tissue biopsy | Thoracic or abdominal aorta with or without branch vessels |
| Eosinophilic granulomatosis with polyangiitis | Any | Granulomatous, may see eosinophils | Allergic rhinitis, asthma, mononeuritis multiplex, myocarditis, skin vasculitis most commonly Peripheral eosinophilia, active urinalysis, high erythrocyte sedimentation rate and C-reactive protein, positive lung imaging Positive antineutrophil cytoplasmic antibody in 50% of cases, positive tissue biopsy | Thoracic or abdominal aorta with or without branch vessels |
| Behçet syndrome | Any | Mixed | Oral or genital ulcers, uveitis, pathergy High C-reactive protein Seronegative | Thoracic or abdominal aorta Pulmonary artery aneurysms |
Based on information from references 4–8,10–19.