Takayasu arteritis

M31.4

DESCRIPTION

Takayasu arteritis is a chronic inflammatory disease involving large vessels, including the aorta and its main branches and the pulmonary vasculature. Lesions are typically segmental – obliterative and aneurysmal. Symptoms reflect end organ ischaemia.

DIAGNOSTIC CRITERIA

Clinical

Angiographic abnormalities of the aorta or its main branches plus at least one of the following:

  • Hypertension with no obvious kidney disease.
  • BP difference in limbs >10 mm Hg.
  • Decrease in peripheral arterial pulses/absent pulses.
  • Vascular bruits, particularly over aorta or main branches, carotids, subclavian, abdominal vessels.

May be associated with:

  • Congestive cardiac failure associated with aortic regurgitation/dilated cardiomyopathy/hypertension.
  • Neurologic signs secondary to hypertension/ischaemia.
  • Any signs of unexplained inflammatory activity.
  • Strongly positive TST.
  • Discrepancy in kidney sizes.

Investigations

  • C-reactive protein.
  • ESR.
  • Plasma renin.
  • Serum urea, creatinine and electrolytes.
  • TST.
  • Electrocardiography.
  • Chest X-ray.

GENERAL AND SUPPORTIVE MEASURES

MEDICINE TREATMENT

Treat hypertension See Hypertension, acute severe .

Consider TB treatment if tuberculosis cannot be conclusively excluded.

  • Aspirin soluble, oral, 5 mg/kg/day as single daily dose.

Induction therapy

  • Prednisone, oral, 2 mg/kg/day for maximum of 4 weeks.
    • Reduce dose slowly over 12 weeks to 0.25 mg/kg on alternate days.

LoEII [2]

Continue maintenance treatment with:

  • Methotrexate, oral, 10–15 mg/m2 /week. Specialist initiated
    • Maximum 25 mg/week.

PLUS

  • Folic acid, oral, 5 mg weekly (on the day after methotrexate) for the duration of the treatment.

REFERRAL

Specialist referral

  • All patients for confirmation of diagnosis with conventional angiography or magnetic resonance imaging angiography.
  • Poor response to initial therapy.