What are the symptoms and treatment options for Takayasu disease?

Symptoms and Treatment of Takayasu Arteritis

Takayasu arteritis presents with two distinct phases: an acute inflammatory phase with constitutional symptoms and a chronic phase with organ-specific manifestations caused by vascular stenosis or aneurysms. 1

Clinical Manifestations

Acute Phase Symptoms

• Constitutional ("B") symptoms:
  • Weight loss
  • Fatigue
  • Night sweats
  • Anorexia
  • Malaise 1
• Elevated inflammatory markers:
  • C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) elevated in 70% of patients in acute phase 1

Chronic Phase Symptoms

• Upper extremity claudication (>50% of patients) 1
• Cerebrovascular insufficiency symptoms (50% of patients):
  • Vision loss
  • Lightheadedness
  • Stroke 1, 2
• Carotid artery pain (33% of patients) 1
• Hypertension (common, especially with renal artery involvement) 1, 3
• Diminished or absent peripheral pulses ("pulseless disease") 1
• Blood pressure differences >10 mmHg between arms 1
• Vascular bruits over aorta and major branches 1, 4
• Aortic complications:
  • Stenosis (53% of patients)
  • Aneurysm formation (23-32% of patients) 1

Diagnostic Criteria

American College of Rheumatology 1990 criteria (3 of 6 required for diagnosis, 90.5% sensitivity and 97.8% specificity):

1. Age of onset <40 years
2. Intermittent claudication
3. Diminished brachial artery pulse
4. Subclavian artery or aortic bruit
5. Systolic blood pressure variation >10 mmHg between arms
6. Angiographic evidence of aorta or aortic branch vessel stenosis 1, 4

Treatment Approach

Initial Assessment

• Thoracic aorta and branch vessel imaging (CT or MRI) to assess aneurysm or occlusive disease 1, 5
• Laboratory markers (ESR and CRP) to establish baseline disease activity 5

First-Line Therapy

• High-dose glucocorticoids (prednisone 40-60 mg daily) are the cornerstone of initial treatment for active Takayasu arteritis to reduce inflammation. 1, 5
• Methotrexate (20-25 mg/week) is the preferred first-line steroid-sparing agent 5

Disease Monitoring

• Monitor disease activity every 1-3 months with:
  • Physical examination
  • ESR and CRP levels
  • Periodic imaging (MRI, CT angiography) every 3-6 months 5

Refractory Disease Treatment

• TNF inhibitors are recommended for refractory disease 5
• Cyclophosphamide for severe refractory cases 5
• Tocilizumab may be considered for inadequate response to other therapies 5
• Azathioprine (2 mg/kg/day) as an alternative if methotrexate is not tolerated 5, 3

Surgical Intervention

• Delay surgical intervention until disease is quiescent 1, 5
• Exceptions requiring immediate intervention:
  • Coronary compromise
  • Progressive tissue/organ infarction
  • Cerebrovascular accident
  • Loss of limb viability
  • Myocardial ischemia 5
• High-dose glucocorticoids should be administered perioperatively 5
• Long-term monitoring for anastomotic aneurysms is essential (cumulative incidence of 13.8% at 20 years) 6

Adjunctive Therapy

• Antiplatelet therapy (low-dose aspirin 75-150 mg daily) for patients with neurological symptoms 5
• Anti-hypertensives, including inhibitors of the renin-angiotensin-aldosterone system, for blood pressure control 5

Special Considerations

• Pediatric cases may present with nonspecific symptoms that delay diagnosis 3, 7
• Prognosis is better with early diagnosis and treatment 2, 7
• Regular imaging surveillance is mandatory for life to detect late complications 6

Common Pitfalls

• Delayed diagnosis due to nonspecific initial symptoms 3
• Glucocorticoid monotherapy should be avoided due to increased risk of steroid-related toxicity 5
• Surgical intervention during active inflammation increases complication risk 1, 5
• Failure to monitor for anastomotic aneurysms after surgical repair 6