Takayasu arteritis

Alternative names:

pulseless disease

Definition:

An inflammatory condition of the aorta.

Causes, incidence, and risk factors:

The cause of Takayasu arteritis is unknown but the disease occurs almost exclusively in young women, many of whom are of Asian or African descent. The disease appears to be rheumatic or autoimmune in nature and may involve systems other than just the heart's aorta and its major branches.

Because of changes in the aorta and its branches (those that feed the head, neck, and upper limb areas) patients may have decreased or absent radial pulses (pulse in the wrist). Inflammation in the carotid arteries (supplying the brain) may cause visual problems or neurological problems. Hypertension is common and may relate to decreased blood flow to the kidneys. Rupture of affected vessels may occur following the formation of aneurysms.

Systemic complaints may include fever, rash, muscle aches (myalgia), and arthritis. Inflammation can also develop in the lining of the chest cavity (pleuritis) and the sack-like covering of the heart, the pericardium (pericarditis).

Survival in children is infrequent. Survival in adults is somewhat better.