Secondary causes of aortitis
| Diagnosis | Age at onset | Tissue pattern | Core symptoms and signs | Typical imaging features |
|---|---|---|---|---|
| IgG4-related disease | Any (typically older) | Lymphoplasmacytic | Lacrimal, salivary gland swelling, pancreatitis, retroperitoneal fibrosis most commonly C-reactive protein often normal Elevated serum IgG4, positive tissue IgG4 | More often in the abdominal than the thoracic aorta May have periaortitis or retroperitoneal fibrosis |
| Rheumatoid arthritis | Any, usually long-standing rheumatoid arthritis | Granulomatous | Small joint, symmetrical polyarthritis Usually high erythrocyte sedimentation rate and C-reactive protein, positive rheumatoid factor, positive anti-cyclic citrullinated peptide Erosions on radiographs of hands and feet | Thoracic or abdominal aorta |
| Spondyloarthritis | Any | Lymphoplasmacytic | Inflammatory back pain Usually high erythrocyte sedimentation rate and C-reactive protein Positive human leukocyte antigen B27, positive radiographs or magnetic resonance imaging of sacroiliac joint and spine | Aortic root with or without aortic insufficiency |
| Systemic lupus erythematosus | Any | Lymphoplasmacytic | Photosensitivity, rash, arthritis, nephritis Positive antinuclear antibody, extractable nuclear antigen, and anti-double-stranded DNA; low complement components 3 and 4; active urine studies | Thoracic or abdominal aorta with or without branch vessels |
| Relapsing polychondritis | Any | Mixed | Chondritis, scleritis, tracheomalacia High C-reactive protein, seronegative | Aortic root and ascending aorta |
| Cogan syndrome | Any (often younger) | Mixed | Interstitial keratitis, hearing loss, vestibular dysfunction, high C-reactive protein | Ascending aorta and arch, with or without aortic insufficiency |
| Sarcoidosis | Any | Granulomatous (well-formed nonnecrotizing granulomas) | Lung, lymph node, musculoskeletal, hematologic, central nervous system, cardiac High C-reactive protein May have high serum or urine calcium, positive lung or cardiac imaging, positive tissue biopsy | Thoracic or abdominal aorta |
| Drug exposure (granulocyte-colony stimulating factor, immune checkpoint inhibitors) | Any | Unknown (usually radiographic diagnosis) | Fever, pain in back, chest, or abdomen, high C-reactive protein, relapsing polychondritis, history of exposure Resolution of imaging changes with drug withdrawal with or without prednisone | Thoracic and abdominal aorta |
Ig = immunoglobulin
Based on information from references 10–12,20–29.