Takayasu Arteritis
Description Diagnostic criteria General and supportive measures Medicine treatment Referral ICD-10 code: 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 CRITERI
Description
Diagnostic criteria
General and supportive measures
Medicine treatment
Referral
ICD-10 code: 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
EULAR/PRINTO/PRES Criteria
Angiographic abnormalities of the aorta or its main branches and pulmonary arteries (aneurysm/dilatation, narrowing, occlusion or arterial wall thickening not due to fibromuscular dysplasia).
AND
At least one of the following five:
- Pulse deficit (lost/decreased/unequal peripheral artery pulses and/or claudication induced by activity).
- Systolic blood pressure > 10 mmHg difference between any limbs.
- Bruits or thrills over the aorta and/or its major branches.
- Hypertension
- Elevated acute phase reactants.
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 Tuberculin Skin Test (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 a single daily dose.
Induction therapy
- Prednisone, oral, 2 mg/kg/day (maximum 60 mg) for maximum of 4 weeks.
- Reduce dose slowly over 12 weeks to 0.25 mg/kg on alternate days.
Continue maintenance treatment with:
- Methotrexate, oral, 10–15 mg/m2 /week. Specialist initiated.
- Maximum dose: 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.
Standard Treatment Guidelines And Essential Medicines List For South Africa, Hospital Level, Paediatrics, 2023 Edition
Chapter 12: Rheumatology And Vasculitides, 12.5 Takayasu Arteritis