Takayasu arteritisPulseless disease
Takayasu arteritis is an inflammation of the aorta and its major branches. The aorta is the artery that carries blood from the heart to the rest of the body.
The cause of Takayasu arteritis is unknown. The disease occurs mainly in children and women between the ages of 20 to 40. It is more common in people of East Asian, Indian or Mexican descent. Several genes that increase the chance of having this problem were recently found.
Takayasu arteritis appears to be an autoimmune condition. This means the body's immune system mistakenly attacks healthy tissue. The condition may also involve other organ systems.
Symptoms may include:
- Arm weakness or pain with use
- Chest pain
- Muscle or joint pain
- Skin rash
- Night sweats
- Vision changes
- Weight loss
- Decreased radial pulses (at the wrist)
- Difference in blood pressure between the 2 arms
- High blood pressure (hypertension)
Exams and Tests
Possible tests include:
- Complete blood count (CBC)
- C-reactive protein (CRP)
- Electrocardiogram (ECG)
- Erythrocyte sedimentation rate (ESR)
- Magnetic resonance angiography (MRA)
- Magnetic resonance imaging (MRI)
- Computed tomography (CT)
- X-ray of the chest
Treatment of Takayasu arteritis is difficult. However, people who have the right treatment can see positive results. It is important to identify the condition early.
Most people are first treated with high doses of steroids. Immunosuppressive drugs, such as azathioprine, mycophenolate, methotrexate, or leflunomide are often added.
Biologic agents including TNF inhibitors such as infliximab are recommended for people who do not improve with the previous treatment.
In difficult cases, tocilizumab or rituximab may be helpful.
Surgery is used to open up narrowed arteries to supply blood or open up the constriction.
This disease can be fatal without treatment. However, a combined treatment approach using medicines and surgery has lowered death rates. Adults have a better chance of survival than children.
Complications may include:
- Blood clot
- Heart attack
- Heart failure
When to Contact a Medical Professional
Call your health care provider if you have symptoms of this condition. Weak pulse, chest pain, and breathing difficulty require immediate care.
Clifford A, Hoffman GS. Recent advances in the medical management of Takayasu arteritis: an update on use of biologic therapies.Curr Opin Rheumatol. 2014;26(1): 7-15. PMID: 24225487 www.ncbi.nlm.nih.gov/pubmed/24225487.
Glebova NO, Abularrage CJ. Takayasu's disease. In: Cronenwett JL, Johnston W, eds. Rutherford's Vascular Surgery. 8th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 80.
Renauer PA, Saruhan-Direskeneli G, Coit P, et al. Identification of susceptibility loci in IL6, RPS9/LILRB3, and an intergenic locus on chromosome 21q22 in Takayasu Arteritis in a genome-wide association study. Arthritis & Rheumatology. 2015;67: 1361-1368. PMID: 25604533 www.ncbi.nlm.nih.gov/pubmed/25604533.
Review Date: 10/18/2015
Reviewed By: Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.