Takayasu's arteritis

Takayasu's arteritis' is a chronic vasculitis of unknown etiology mainly affecting the aorta, its main branches and pulmonary arteries. Involvement of the vessel wall leads to stenosis, occlusions and aneurysm. It occurs mainly in women under 40 years of age, with an endemic area in Southeast Asia. The disease was first described by Japanese ophthalmologist Mikito Takayasu.

Takayasu's arteritis in an 18-month-old child presenting with cerebral aneurysm

Takayasu's arteritis

Clinical picture

The disease typically presents in 2 stages:

1. nonspecific phase' (systemic): febrile, weight loss, fatigue, weakness, myalgia, arthralgia, Raynaud's phenomenon, recurrent infections, recurrent iritis;
2. specific phase (vascular occlusive): systolic murmurs, absence of peripheral pulsations, signs of ischemia of the affected districts, hypertension, cardiomyopathy, aortic valve involvement, etc.

Diagnosis

Physical examination

• weakening pulse/ murmur over large arteries, different values ​​of blood pressure in the upper limbs.

Laboratory

• ↑ CRP + FW, anemia with leukocytosis.

Imaging methods

• US, aortography, angiography, CT-angio, MRI (segmental narrowing of large arteries/microaneurysms of the vessel wall).

Treatment

Treatment relies on the administration of corticosteroids. In unresponsive patients, corticosteroids are combined with immunosuppressants (cyclophosphamide, methotrexate, azathioprine). Proper correction of hypertension and symptomatic treatment is also essential. Stenosis and occlusions are treated with PTA or surgery.

References

Related articles

• Systemic vasculitis
• Angioinvasive treatment of arterial occlusions and stenoses
• Giant cell arteritis

Literature used

• KLENER, P, et al. Vnitřní lékařství. 3. vydání. Praha : Galén, 2006. ISBN 80-7262-430-X.

• BANI HANI, Amjad, et al. Takayasuova arteriitida. Cor et Vasa. 2008, roč. -, vol. 50, no. 3, s. 117-122, ISSN 1803-7712.