Determining Disease Activity Biomarkers in Individuals With Takayasu's Arteritis

Takayasu's arteritis is a chronic inflammatory condition that affects the aorta, the largest
blood vessel in the body, and its branches. Inflammation causes segments of the vessels to
become narrowed, blocked, or even stretched, possibly resulting in aneurysms. The disease is
very rare but most commonly occurs in young Asian women. Symptoms may include arm pain with
use, decreased or absent pulses, lightheadedness or dizziness, headaches, and visual
disturbances. Many individuals with Takayasu' arteritis, however, have no apparent symptoms
despite disease activity. Additionally, current tests used to measure vessel inflammation are
considered unreliable and inconsistent. It is therefore very difficult to determine the
extent of disease activity in a person with Takayasu's arteritis. This study will use novel
scientific methods to establish new biomarkers that can be used to monitor disease activity
in individuals with Takayasu's arteritis. These biomarkers may be used to help direct
clinical care and assist in future drug development.

Study visits will occur monthly for the first year, then every 3 months thereafter for the
remainder of the study. Blood and urine collection will occur at every visit. A physical
examination, disease assessment, and review of medications will occur every 3 months;
participants may also be asked to complete various questionnaires. Diagnostic testing, which
may include chest radiograph, echocardiogram, magnetic resonance imaging, or angiography,
will occur every 6 months. Tobacco, alcohol, and drug use will be assessed on a yearly basis.
Participants may have additional study visits if a disease flare or disease-related
complications occur during the study.

Inclusion Criteria:

- Evidence of arteriogram abnormalities compatible with Takayasu's arteritis (includes
conventional dye angiography, magnetic resonance angiography, or computed tomography
angiography)

- Evidence of one of the following criteria:

1. Age at disease onset of 50 years or younger

2. Claudication of the arms or legs

3. Decreased brachial artery pulse (one or both arteries)

4. Blood pressure difference between the arms of at least 10 mm Hg

5. Bruit over the subclavian arteries or aorta

- Parental consent for children under the age of 18

Exclusion Criteria:

- Arteriographic lesions that could be entirely due to atherosclerosis

- Fibromuscular dysplasia

- Cogan's syndrome

- Behcet's disease

- Sarcoidosis

- Kawasaki disease

- Giant cell arteritis (large vessel vasculitis and at least 50 years old)

- Syphilis or other infectious forms of large vessel vasculitis