Takayasu arteritis

Classification according to ICD-10
M31.4	Aortic arch syndrome (Takayasu syndrome)
ICD-10 online (WHO version 2019)

The Takayasu's arteritis is an autoimmune disease , which is a granulomatous inflammation of the aorta comes and its main branches. It is related to giant cell arteritis and, like this, is one of the (primary) vasculitides . It is named after one of its first descriptors, Mikito Takayasu (1908).

Epidemiology

Takayasu's arteritis mainly affects young women under the age of 40. It is a rare disease, the incidence in Western Europe is below 1 in 1,000,000.

Symptoms

The symptoms can be summarized in two complexes, which can be of different severity. The first complex includes general inflammatory reactions such as fever , night sweats, weight loss, and body aches and can last for years. The resulting vascular constrictions lead to the symptoms of the second complex: circulatory disorders of the extremities, dizziness, fainting fits, visual disturbances, stroke and high blood pressure .

Pathogenesis

Takayasu's arteritis preferentially affects the large elastic-type arteries . The granulomas form mainly in the middle layer of the vessel wall, which is thereby destroyed. The onset of scarring then leads to a narrowing of the vessel diameter and thus to an obstacle to the blood flow. Due to the high pressure that prevails in the vessels close to the heart, aneurysms (bulges in the vessel wall) can also form in the long term .

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 arms)
• weakened pulse of the brachial arteries (hence also pulseless disease )
• Blood pressure difference between both arms> 10 mmHg (systolic)
• Flow sounds over the aorta or subclavian artery
• Changes in the arteriogram

In contrast to granulomatosis with polyangiitis found in Takayasu's arteritis not ANCA - autoantibodies .

therapy

The administration of glucocorticoids is still the drug therapy of choice. Methotrexate , azathioprine or TNF blockers are also used for relapses . Vascular surgery may be necessary for aneurysms at risk of rupture .

forecast

The prognosis for untreated Takayasu arteritis is poor, but current therapy can significantly improve it. Larger studies from Japan show that two-thirds of patients can be kept in a stable stage and only a quarter have serious complications.

literature

• M. Vanoli et al: Takayasu's arteritis: a changing disease. In: J Nephrol. 2001 Nov-Dec; 14 (6), pp. 497-505. PMID 11783606
• F. Numano: The story of Takayasu arteritis . In: Rheumatology 2002; 41, pp. 103-106. PMID 11792888

Individual evidence

1. ^ JH Klippel (Ed.): Primer on the Rheumatic Diseases, 12th Edition. Arthritis Foundation, Atlanta, USA 2001.
2. ^ WP Arend et al.: The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. In: Arthritis Rheum . 33 (1990), p. 1129.
3. ↑ R. Moriwaki, F. Numano: Takayasu arteritis: follow-up studies for 20 years. In: Heart Vessels Suppl. 1992; 7, pp. 138-145. PMID 1360960

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !