Takayasu Arteritis

Description

  • Takayasu arteritis (TA) is a type of systemic vasculitis that targets the large blood vessels of the heart. The main artery leaving the heart, called the aorta, and the other major blood vessels branching from the aorta, become inflamed or swollen. Initial inflammation includes the thickening of the artery wall. As the wall thickens, the space inside the vessel narrows and blood flow to some of the organs of the body becomes limited. If the vessels are inflamed for a long time, they can become damaged to the point of occlusion (blockage of blood flow through the vessel) or aneurysm formation (parts of the vessel weaken and form bulges). Collateral circulation can also occur in which the blood finds an alternate path around the blocked artery or vein via neighbouring smaller vessels.
  • TA begins with acute inflammation that can last from a few weeks to a few months. The inflammation may then settle but reoccur after a short period of time. As the disease persists, the inflammation becomes persistent (chronic) and the health of the organs supplied with blood from the affected vessels worsens if not treated.

Causes

  • Unknown but speculated to be of autoimmune origin. This means that the immune system of the body turns on itself and begins to destroy normal blood vessels by mistaking them for harmful foreign substances. There is also evidence of environmental triggers and certain genetic factors that increase the likelihood of being affected by the disease.

Demographics

  • TA is seen most frequently in Asian populations, more commonly occurring in females, and affecting all paediatric age ranges from infants to adolescents.

Diagnosis

  • According to the EULAR/PRINTO/PRES framework, a patient must present angiographic abnormalities with an additional classification feature. Note that clinical features, inflammatory markers and specific imaging patterns are also important to consider when making diagnoses.
  • Click here for more detailed information on classifying and diagnosing TA.

Treatment

  • Often times, a combination of corticosteroids and immunosupressants are used in treating patients with Takayasu Arteritis. However, surgery may be used in severe cases involving occlusion and stenosis leading to ischemia (inadequate blood supply provided to the dependent organ).
    • Click here for more detailed information on suggested treatment protocols.

Sources: Khanna NN, Rao S. Takayasu arteritis – Brief review. Journal of Indian College of Cardiology, 2016. 6(1): p. 147-52.

Maksimowicz-McKinnon K, Clark TM, Hoffman GS. Limitations of Therapy and a Guarded Prognosis in an American Cohort of Takayasu Arteritis Patients. Arthritis Rheum, 2007. 56(3): p. 1000-9.