Classification of idiopathic isolated aortitis
Inflammation of the aorta is found as an incidental histologic finding in 3% to 10% of patients undergoing aortic aneurysm surgery. The classification is based on location:
- a) Isolated idiopathic thoracic aortitis.
- • Giant cell aortitis: inflammation indistinguishable from GCA.
- • Lymphoplasmacytic infiltrate: a significant percentage (75%) of these patients has IgG4-RD.
- b) Chronic periaortitis (abdomen).
- • Idiopathic retroperitoneal fibrosis (IRPF, Ormond’s disease).
- • Inflammatory abdominal aortic aneurysm: present with abdominal/back pain (never an asymptomatic incidental finding). Elevated ESR/CRP. Associated with smoking (>75%) and family history (Northern European) of aortic aneurysm. Can be associated with retroperitoneal fibrosis and iliac artery involvement. Some of these patients have IgG4-RD. Treatment is surgical intervention and smoking cessation. Glucocorticoids and methotrexate are used if symptoms persist or associated with IgG4-RD.
- • Idiopathic isolated abdominal periaortitis: occurs without associated aneurysm or retroperitoneal fibrosis.
