Takayasu arteritis

Takayasu arteritis is a primary vasculitis of large-caliber arteries (aorta and its branches, pulmonary arteries), characterized by giant cell granulomatous inflammation.

Its etiology is unknown, but it is significantly associated with autoimmune diseases and states of hypercoagulability.

It is a rare disease, predominantly found in Asia (Japan, India, Korea, Thailand, and Indonesia), Mexico, South America, and Africa. Its incidence is approximately 1 to 2 cases per million inhabitants per year in Western countries. It primarily affects young women (age around 30, with a ratio of 2 to 24 women for every man, depending on the series).

Clinical Presentation

The disease typically progresses through two phases: systemic and vascular. However, the systemic phase is not always present, and both phases can coexist.

Systemic Phase

• Nonspecific symptoms that generally do not lead to a diagnosis: fever, arthralgia, myalgia.
• More suggestive symptoms: episcleritis, erythema nodosum.

Vascular Phase (due to stenosis formation and potential aneurysms)

• Peripheral Vascular Manifestations
  • Claudication on exertion (more common in the upper limbs than in the lower limbs).
  • Raynaud's phenomenon.
  • Absent peripheral pulse.
  • Vascular murmur.
  • Blood pressure asymmetry.

• Neurological Manifestations:
  • Headaches, dizziness, transient ischemic attacks (rarely fully developed strokes), subclavian steal syndrome, loss of consciousness.
  • Rare seizures.
  • Very suggestive in the pre-ischemic stage: carotidynia (pain along the carotid arteries).

• Ocular Manifestations:
  • Ischemic retinopathy: rare in Western countries.

• Hypertension (30-70% of cases) (due to renovascular issues, etc.):
  • !!! The main factor for poor prognosis!!!

• Cardiac Manifestations:
  • Coronary involvement (10%, with angina in 70% of cases).
  • Valvular involvement (10% with aortic insufficiency, other valvular issues are very rare).
  • Giant cell myocarditis… rarely symptomatic.
  • Heart failure (occurs in up to 33% of cases).

• Pulmonary Artery Involvement (7% symptomatic):
  • Presentation of pulmonary hypertension: dyspnea worsens on exertion, hemoptysis, syncope.

• Digestive Manifestations
  • Frequent celiac-mesenteric involvement, but rarely symptomatic (nausea, diarrhea, pain).

• Renal Manifestations:
  • Rare: proteinuria, nephrotic syndrome, hematuria, renal failure, renal amyloidosis, glomerulonephritis.

• Cutaneous Manifestations (5-30%):
  • Erythema nodosum, subcutaneous nodules, sometimes necrotic, often ulcerated.

Associated Diseases

• 1 to 3% of cases are associated with Crohn’s disease (100 times more common than in the general population!).
• Spondyloarthropathies.
• Controversial associations with SLE, sarcoidosis, Wegener’s disease.

Additional Tests

Biology:

• No specific findings. Inflammatory syndrome in 70% (ESR = marker of disease activity). Possible ANA/Rose-Latex.

CT Angiography and Aortography:

• Reference exams to grade the extent and type of involvement. Segmental lesions: stenoses (± proximal aneurysms).

Color Doppler Ultrasound:

• Equivalent sensitivity except for the thoracic aorta and pulmonary arteries.

Diagnosis

Ishikawa-Sharma Criteria (1995): Probable Takayasu arteritis if there is an association of: 2 major criteria / 1 major + 2 minor criteria / 4 minor criteria:

Major Criteria:

• Stenosis/occlusion of the left subclavian artery on arteriography.
• Stenosis/occlusion of the right subclavian artery on arteriography.
• Presence of at least one of the following symptoms for at least one month: intermittent claudication, absent pulse, blood pressure asymmetry, fever, neck pain, visual disturbances, dizziness, dyspnea, palpitations.

Minor Criteria:

• ESR > 20.
• Carotidynia.
• Arm BP > 140/90 mmHg and popliteal BP > 160/90 mmHg.
• Heart murmur and aortic insufficiency.
• Pulmonary artery lesion on arteriography.
• Stenosis/occlusion of the carotid arteries on arteriography.
• Stenosis/occlusion of the brachiocephalic trunk on arteriography.
• Thoracic aorta lesion on arteriography.
• Abdominal aorta lesion on arteriography.
• Coronary artery lesion before age 30 without dyslipidemia/diabetes.

Therapeutic Management - Treatments

There is not enough data to have a clear prognosis for the disease, and treatments vary widely. The occurrence of hypertension or cardiac/renal involvement is, however, a negative prognostic factor.

Medical Treatment

• First-line: Prednisone 1 mg/kg for 1-3 months, tapering over 1 year.
• In case of failure: immunosuppressants (azathioprine, cyclophosphamide, methotrexate), attempt withdrawal after one year.

~50% relapse rate at 5 years.

Percutaneous Angioplasty

Mainly used for renal arteries in case of renovascular hypertension. 80% success rate.

Surgical Treatment

Only for symptomatic stenoses when percutaneous angioplasty is contraindicated. Followed by corticosteroid therapy to prevent graft occlusion.

Bibliography

EMC, Traité de médecine AKOS, Elsevier, 2018

Jameson JL et al., Harrison's Principles of Internal Medicine, 20th edition, McGraw Hill Higher Education, 2018