What is the treatment for aortitis?

Treatment of Aortitis

The treatment of aortitis requires immunomodulatory therapies with corticosteroids as initial standard therapy followed by long-term nonsteroidal therapy, managed by a multidisciplinary team including rheumatologists and cardiologists. 1

Diagnosis and Initial Assessment

• Diagnosis requires:
  • Imaging studies showing homogeneous circumferential thickening of the aortic wall
  • Laboratory markers: ESR and CRP to establish baseline disease activity
  • Thoracic aorta imaging (CT angiography or MRI) to assess extent of involvement

Treatment Algorithm Based on Type of Aortitis

1. Inflammatory/Non-infectious Aortitis (Takayasu Arteritis, Giant Cell Arteritis)

First-Line Therapy:

• High-dose glucocorticoids:
  • Prednisone 40-60 mg daily (1 mg/kg/day up to 80 mg) 1, 2
  • Prolonged taper over 1-2 years to prevent relapse 1
  • Nearly half of patients will relapse during tapering 1

Second-Line/Steroid-Sparing Agents:

• Methotrexate: 20-25 mg/week as preferred first-line steroid-sparing agent 2
• Azathioprine: 2 mg/kg/day if methotrexate is not tolerated 2
• TNF inhibitors: For refractory disease 2
• Tocilizumab: For inadequate response to other therapies 2

2. Infectious Aortitis

• Appropriate antimicrobial therapy based on identified organism 3
• Surgical intervention often necessary due to delay in diagnosis 3

Monitoring Disease Activity

• Clinical assessment every 1-3 months
• Laboratory monitoring: ESR and CRP levels
• Periodic imaging (MRI, CT angiography, PET) every 3-6 months 2
• Disease progression may occur despite normal inflammatory markers 1

Surgical Considerations

• Delay surgical intervention until disease is quiescent whenever possible 2
• High risk of graft failure in the setting of active inflammation 1
• Indications for immediate intervention:
  • Coronary compromise
  • Progressive tissue/organ infarction
  • Cerebrovascular accident
  • Loss of limb viability
  • Myocardial ischemia 2
• High-dose glucocorticoids should be administered in the perioperative period 2

Adjunctive Therapies

• Antiplatelet therapy: Low-dose aspirin (75-150 mg daily) for patients with neurological symptoms 2
• Antihypertensive medications: Particularly inhibitors of the renin-angiotensin-aldosterone system 2
• Smoking cessation: Strongly encouraged as tobacco use is associated with increased mortality 1

Special Considerations for Specific Types of Aortitis

Giant Cell Arteritis

• Treatment should aim to prevent tissue ischemia and vascular damage 1
• Progression of vascular damage may occur even in patients in clinical remission 1

Takayasu Arteritis

• Revascularization for aortic stenosis or aneurysm follows the same indications as in non-inflammatory disorders 1
• Risk of anastomotic aneurysms (12% over two decades) related to aneurysms at surgery 1

Ankylosing Spondylitis

• Treatment of aortic root expansion and aortic valvular abnormalities follows standard protocols 1

Important Pitfalls to Avoid

1. Monotherapy with glucocorticoids: Increases risk of steroid-related toxicity and may provide insufficient disease control 2

2. Premature surgical intervention: Operating during active inflammation increases risk of graft failure 1

3. Inadequate monitoring: Disease progression can occur despite normal inflammatory markers 1

4. Insufficient treatment duration: Therapy typically required for 1-2 years to avoid recurrence 1

5. Overlooking comorbidities: Consider cardiovascular risk factors, osteoporosis prevention, and infection risk during immunosuppressive therapy