Description, Causes and Risk Factors:
Alternative Name: Takayasu arteritis, Takayasu disease, Takayasu syndrome, aortic arch syndrome, nonspecific aortoarteritis, and the pulseless disease.
Takayasu's arteritis is rare, with an approximate incidence worldwide of 2-3 cases per year per million head of population. The annual incidence in the UK is 0.15 per million. Takayasu's arteritis affects women more frequently than men with a 9:1 female to male ratio. It affects young adults up to the age of 40, but is most common in the age range 15-20. It has been reported all over the world, but is most common in Asia, particularly Japan.
Takayasu's arteritis is one of many types of vasculitis, in this inflammation occurs in the walls of large arteries, the aorta and its major branches, which supply blood to the head, arms, legs, and internal organs. Inflammation may cause arterial walls to thicken resulting in narrowing of the inside diameter of the artery.
C1assilication of Takayasu Arteritis May Include:
Type IIa: Involves ascending aorta, aortic arch and its branches.
Type IIb: Involves the ascending aorta, aortic arch, its branches and thoracic descending aorta.
Type III: Involves descending thoracic aorta abdominal aorta and or renal arteries.
Type IV: Affects mainly the abdominal aorta and/or renal arteries.
Type V: Combines features of both types IIb and IV. Moreover involvement of coronary and pulmonary arteries is designated as C+ and P respectively.
Type I: Primarily involves the branches from the aortic arch.
Causes and Risk Factors May Include:
A number of factors like connective tissue disorders, infections, genetic factors and immunological phenomenon have been implicated in the etiology of TA. However, the issue remains far from settled.
Connective tissue disorders like rheumatoid arthritis, SLE, sarcoidosis, ulcerative colitis and Crohn's disease. However, any definite proof of causal association between connective tissue disorders and TA is lacking.
A number of reports of occurrence of the disease in monozygotic twins and in sibs, point towards a possible genetic predisposition in this disease.
The possibility of an immune mediated etiology has been supported by findings of high gamma globulins, circulating immune complexes, presence of aortic antibodies and failure to detect an etiologic agent in walls of vessels affected by the disease. Dense NK cell infiltration has been shown in wall of the aorta and it has been proposed that they cause vascular injury by releasing a cytolytic factor called perforin.
A variety of infectious agents have been implicated the etiology of TA viz. streptococcus and mycobacterium tuberculosis. There are several reports of association between TA.
Symptoms May Include:
Muscle or joint pain.
Arm weakness or pain with use.
Signs May Include:
Difference in blood pressure between the two arms.
High blood pressure (hypertension).
Decreased radial pulses (at the wrist).
Takayasu's arteritis is usually diagnosed by an angiogram, a test that demonstrates how well blood flows in arteries. Various types of angiogram are now available, including conventional angiograms involving injection of dye directly into an artery, as well as less invasive techniques such as computerized tomography angiography (CTA) or magnetic resonance angiography (MRA). An angiographic procedure is usually requested on the basis of symptoms in combination with physical exam findings, such as loss of pulse or blood pressure in an arm, or abnormal sounds (“bruits”) heard over large arteries with a stethoscope. Angiographic studies reveal narrowing of one or more large arteries. It is important to try and distinguish between narrowing due to vasculitis (inflammation of arteries) and narrowing due to atherosclerosis (“hardening” of the arteries). There are other causes of arterial narrowing, including another rare disease called fibromuscular dysplasia that leads to narrowing of major arteries, usually in women.
Blood tests for inflammation including measurements of the erythrocyte sedimentation rate (ESR), C?reactive protein (CRP), are often, but not always, elevated in patients with Takayasu's arteritis, and a large number of other inflammatory diseases produce similar abnormalities in these tests.
Treatment of Takayasu arteritis is difficult, but patients who do have the right treatment can see positive results. Early detection is important. Most patients are treated with steroids and immunosuppressive drugs.
Surgical procedures are sometimes required to increase the flow of blood through an artery and procedures undertaken may include:
Vascular bypass procedures.
Aortic valve replacement.
Suction thrombectomy of the subclavian artery.
Angioplasty with stenting.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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