Aortitis Management: Diagnostic Approach, Goals, Prognosis, and Complications
Aortitis management requires a multidisciplinary team including rheumatologists and cardiologists, with immunomodulatory therapies (corticosteroids with prolonged taper) as the initial standard therapy, followed by long-term nonsteroidal therapy. 1
Diagnostic Approach
Clinical Presentation
- Symptoms are often nonspecific, requiring high index of suspicion 2
- Systemic inflammatory response syndrome is the most common presentation (47.5% of cases) 3
- May present with chest or abdominal pain, fever, and vascular insufficiency 4
- Some patients present with vascular complications like dissection or aneurysm (10.8%) 3
Laboratory Testing
- Elevated inflammatory markers are common (median ESR 70.0 mm/h and CRP 68.0 mg/L) 3
- Acute phase reactants are typically elevated 4
Imaging
- Cross-sectional imaging is essential for diagnosis 4
- Recommended initial imaging: TTE (transthoracic echocardiography) plus either CTA (computed tomography angiography) or MRA (magnetic resonance angiography) 1
- Imaging findings include homogeneous circumferential thickening of the aortic wall 5
- Angiography remains the gold standard for confirming aortic involvement 2
- PET scanning may help assess disease activity 4
Management Approach
Medical Management
- Immunomodulatory therapy is the cornerstone of treatment 1
- Corticosteroids with prolonged taper are the initial standard therapy (used in 91.5% of cases) 1, 3
- Long-term nonsteroidal therapy follows initial steroid treatment 1
- Methotrexate is commonly used as a steroid-sparing agent (89.8% of cases) 3
- For infectious aortitis: appropriate antimicrobial therapy based on identified pathogen 2
Surgical Considerations
- Elective repair of aortitis-related aneurysm should be delayed until clinical remission of inflammation 1
- High risk of graft failure exists in the setting of active inflammation 1
- Approximately 33% of patients may require interventional procedures 6
Lifestyle Modifications
- Smoking cessation is strongly recommended due to association with increased mortality 1
- Regular cardiovascular exercise is beneficial, but avoid contact/competitive sports and isometric exercise 1
- Weight lifting should be restricted to approximately 50 lb 1
Goals of Management
- Control inflammation to prevent disease progression 1
- Prevent vascular complications (aneurysm formation, dissection, rupture) 1, 5
- Achieve clinical remission before considering surgical intervention for aneurysms 1
- Maintain long-term surveillance to monitor for disease activity and complications 1
Prognosis
- With appropriate treatment, approximately 67% of patients show improvement in disease activity 6
- About 27% show no change in disease activity despite treatment 6
- Disease progression occurs in approximately 7% of patients despite treatment 6
- Isolated aortitis has higher risk of dissection (16.6%) compared to other types (1.96%) 3
Complications
- Vascular complications develop in 48.3% of patients during disease course 3
- Specific complications include:
Special Considerations
Patient Counseling
- Patients should seek immediate medical care for any unexpected chest discomfort 1
- Medical alert bracelet indicating predisposition to aortic emergencies is recommended 1
- Family screening is important when genetic or familial causes are suspected 1
Pregnancy Considerations
- Discuss birth control methods and risks of pregnancy-related aortic complications including dissection 1
- Pregnancy may increase risk of aortic complications in certain types of aortitis 1