Introduction – Takayasu’s arteritis
Takayasu’s arteritis (TA) is a granulomatous vasculitis that affects the aorta and its branches. The disease occurs primarily in children and young adults. The incidence in Caucasians is around 3 per million, with significant female dominance (4:1).
The most common symptoms include:
- Ischemic pain in upper extremities
- Prominent tenderness over carotid arteries
- Absence of pulsation over peripheral vessels
- Loss of vision
Involvement of renal arteries often leads to hypertension while blood pressure can be difficult to measure due to stenoses in the brachial arteries. In physical examination, it is important to measure the blood pressure in both arms as there may be a discrepancy of 20-30 mmHg between the arms, as well as to check the pulse intensity.
Other symptoms include arthralgia, weight loss, and headaches. Ischemic symptoms of the brain, bowel and/or heart of a young person should raise suspicion about Takyasu’s arteritis.
Diagnosis – Takayasu’s arteritis
The diagnosis is not based on histological findings but is confirmed by non-invasive visualization methods such as magnetic resonance angiography (MRA) and ultrasound. MRA provides an opportunity to assess the degree of severity of the arteritis.
Other non-invasive methods
18F-fluorodeoxyglucose positron emission computerized tomography (18F-FDG-CT-PET), high-resolution ultrasound (US), magnetic resonance, and CT angiography (MRA and CTA) are of great importance in detecting and monitoring arterial changes. The use of these methods should be sought in each case of TA.
Treatment – Takayasu’s arteritis
High-dose prednisolone, about 60 mg/d, often has dramatic effects on symptoms. The dose of corticosteroids is gradually reduced as symptoms subside. In treatment-resistant cases, cytostatic drugs, primarily methotrexate (Ebetrex), mycophenolic acid (CellCept) or azathioprine (Imurel) are used.
Infliximab (Remicade) (3-5 mg/kg every 4-6 weeks) can be used in therapy-resistant cases for corticosteroid-reduction purposes. Tocilizumab (RoActemra) and rituximab (MabThera) have shown a positive effect in the treatment of therapy-resistant cases. A combination of vessel wall changes with high inflammatory activity is used to calculate the activity index and is recommended for follow-up of treatment effect. TA patients with low disease activity at diagnosis may experience a worsening of symptoms in a relatively limited subgroup of patients. These patient groups need qualified follow-up.
Angioplasty and endovascular interventions start to gain more space in therapy-resistant cases of Takayasu’s arteritis. Side effects associated with surgical intervention are frequent in patients where the disease activity is high.
Long-term thrombosis prophylaxis with low-dose ASA is recommended in combination with pharmacotherapy and surgical interventions.
With the treatment strategy described above, the long-term survival rate exceeds 90%.