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It is classified as a connective tissue disease. It occurs under several names in the medical literature – Takayasu syndrome, aortic arch syndrome, or pulseless disease.
The essence of the disease is chronic inflammation of the arteries of the aortic arch and its branches, as well as of the proximal coronary vessels, renal and pulmonary arteries. The abdominal aorta and its branches are less frequently involved. The disease usually affects young women between the ages of 15 and 45. The cause of the disease is unknown, but there is increasing talk of a genetic predisposition due to changes in HLA.
The first symptoms of the disease affect the entire body. Malaise, fever, night sweats, joint pain, loss of appetite and weight loss appear. Further symptoms depend on the degree of impaired circulation in the vessels affected by the disease. No pulse is felt in the subclavian and peripheral arteries. The pressure measured on the upper limbs is significantly different.
Choroba Takayasu – typy
There are four basic types of Takayasu syndrome.
Type I — there are symptoms of cerebral ischemia resulting from changes in the aortic arch,
Type II – have high blood pressure caused by changes in the aorta or arteries in the kidneys
Type III – there are changes above and below the diaphragm,
Type IV — there are changes in the aortic wall or its branches.
Takayasu’s disease – what tests should I do?
Laboratory tests show accelerated depletion of red blood cells, leukocytosis, anemia, sometimes the presence of IgM rheumatoid factor. Angiography reveals narrowing or even occlusion of individual arteries. Currently, when examining patients, Doppler ultrasound is most often performed, which shows serious changes in the vessels.
How is aortic arch syndrome treated?
According to the guidelines of rheumatic societies, the diagnosis of Takayasu syndrome can occur when the patient develops three of the six characteristic symptoms. These are:
– onset of the disease below 40 years of age,
– upper limb claudication,
Weak or no pulse in the brachial artery
– systolic pressure difference between the upper limbs,
– murmur over the abdominal aorta or subclavian artery,
– angiographic changes, e.g. aortic obstruction or narrowing.
It is important to quickly diagnose the disease and initiate treatment to prevent serious complications (stroke in young women). Treatment includes pharmacotherapy (in most cases) and surgery. Glucocorticosteroids (GKS) are used in patients. If GCS therapy is unsuccessful, the patient takes methotrexate. Invasive treatment is carried out when vasoconstriction disturbs the supply of oxygen and nutrients to internal organs. The disease requires constant monitoring and treatment. 95 percent people survive 15 years from diagnosis if treatment is properly administered.
It is worth knowing that the disease is rare in Europe. The Italian population has the greatest number of cases of the disease. Takayasu’s disease type III is the most common.