Takayasu's arteritis

Takayasu 's arteritis is an autoimmune disease in which there is a granulomatous inflammation of the aorta and its main branches. It is related to the giant and one as this to the vasculitis. It is named after one of its first describer Mikito Takayasu (1908 ).

Epidemiology

Takayasu 's arteritis primarily affects young women under 40 years. It is a rare disease, the incidence in Western Europe is less than 1:1,000,000.

Symptoms

The symptoms can be summarized in two complexes, which can vary in strength. The first complex includes general inflammatory reactions such as fever, night sweats, weight loss and pain in the limbs and can last for years. The resulting vessel constrictions lead to the symptoms of the second complex: circulatory disorders of the extremities, dizziness, fainting, blurred vision, stroke and high blood pressure.

Pathogenesis

Takayasu arteritis preferably attacks the large arteries of the elastic type. The granulomas are formed mainly in the middle layer of the vessel wall, which is destroyed. Insertion Vernarbungsprozesse then lead to a narrowing of the vessel diameter and thus an obstacle to the blood stream. Due to the high pressure prevailing in the vessels close to the heart, it may in the long term, however, also result in the formation of aneurysms ( outgrowths of the vessel wall ).

Diagnosis

The diagnosis is based on the ACR criteria of which at least three must be present.

• Age < 40 years
• Claudication of the extremities (especially the poor )
• Attenuated pulse of the brachial artery (hence pulseless disease)
• Difference in blood pressure between the two arms > 10 mmHg ( systolic)
• Flow noise over the aorta or subclavian artery
• Changes in the arteriogram

In contrast to Wegener 's granulomatosis can be found in Takayasu 's arteritis no ANCA autoantibodies.

Therapy

The administration of glucocorticoids is still the drug therapy of choice. In recurrences also methotrexate, azathioprine or TNF- blockers are used. In rupturgefährdeten aneurysms vascular surgery may be necessary.

Forecast

The prognosis of untreated Takayasu 's arteritis is bad, but can be significantly improved by the current therapy. Larger studies from Japan indicate that two-thirds of patients can be maintained in a stable stage and serious complications occur in only one district.