Takayasu's syndrome

Takayasu syndrome is a granulomatous inflammation of the aorta and its main branches, usually beginning at the age of up to 50 years. The first described the disease D. Savon (1856), but its name was obtained through the work of Japanese ophthalmologists, one of which was M. Takayasu (1908).

Epidemiology

Takayasu is found mainly in the countries of Asia and South America, but numerous cases of the disease are recorded in other geographical regions of the globe, including in Russia. The annual incidence of Takayasu's syndrome ranges from 0.12 to 0.63 cases per 100,000 people. Women are most prone to disease of young and middle age.

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How does Takayasu's syndrome manifest itself?

The course of the Takayasu syndrome has a certain stage. In the early stage of the disease or during its exacerbations, the clinical picture includes nonspecific symptoms caused by a systemic inflammatory process (weight loss, weakness, drowsiness, myalgia and arthralgia, abdominal pain). Often, in the onset of Takayasu's syndrome, unexplained fever, acceleration of ESR and anemia take place for a long time. In this period, up to 10% of patients do not make any complaints at all.

In the developed stage of the Takayasu syndrome, as a result of progressive occlusive arterial lesions, there are signs of ischemia of individual organs and tissues. The absence of systemic inflammatory reactions does not exclude active vascular inflammation, which leads to pain at the site of the projection of the affected vessel or pain when it is palpated. Signs of vascular inflammation occur within the first year of the onset of the disease. Most often (40%) note a feeling of weakness, fatigue and pain in the muscles of the forearm and shoulder, which are predominantly one-sided, their severity increases with physical activity. But lack of a pulse or a decrease in its filling and tension on one of the arms, asymmetry of systolic blood pressure on the brachial arteries is noted in 15-20% of cases.

In 7-15% of patients with Takayasu syndrome, there are pain in the neck, dizziness, transient moments of visual impairment, increased blood pressure, shortness of breath and palpitations. In 70% of cases, one can listen to systolic murmur on the common carotid arteries, and 15% of patients observe their soreness (carotidinia). Such physical data are often found in the auscultation and palpation of the abdominal aorta, especially in the II and III anatomical types of the disease.

And the third phase of Takayasu's syndrome in the vessels is dominated by signs of fibrosis, there are stenoses of vessels and signs of vascular insufficiency. During this period, compared with the early stage of the disease, the symptoms of intermittent claudication of the upper and lower extremities, lesions of the cardiovascular system, brain and lungs are much more frequent (50-70%). Clinical signs of pulmonary involvement are met in less than a quarter of patients. There are pains in the chest, dyspnea, unproductive cough, rarely hemoptysis.

Patients note pain in the muscles of the proximal parts of the hands, often left, reducing the strength of the hand occurs with less physical exertion, at rest or during sleep. Sometimes the pain extends to the left shoulder, the lower jaw, the neck area and the left half of the chest. In 85-90% of patients with Takayasu's syndrome, the pulse on the arteries of the forearm disappears and there are differences in systolic blood pressure measured on the brachial arteries.

With the progression of the inflammatory process in the vessels, both the frequency and the severity of the clinical symptoms caused by ischemic events in individual organs or systems increase. However, despite the occlusion of subclavian or axillary arteries, a critical degree of ischemia of the arms is rarely observed, which is associated with slow progression of stenosis and development of collaterals.

Classification

Several classifications of pathological changes of the vessels in the Takayasu syndrome have been proposed, the ranchers between which are not of a fundamental nature. There are four anatomical types of disease. At the first, there are changes from the side of the arch of the aorta and the arteries that leave it. With this variant, simultaneous lesions of the left subclavian and common carotid arteries are common. The second type of disease leads to an isolated lesion of the thoracic and / or abdominal aorta and their branches. In the third type, the pathological process in the arch of the aorta and / or its branches is combined with changes in the thoracic or abdominal aorta. In the fourth type, the pathological process is localized in the main pulmonary trunk or its branches in combination (or without it) with any of the first three variants.

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How to recognize Takayasu's syndrome?

Normally, normochromic normocyte anemia, moderate platelet and hypergammaglobulinemia is usually found. Especially characterized by an increase in ESR, the extent of which depends on the inflammatory activity of the disease. However, in a third of patients, despite the clinical signs of inflammatory activity, ESR can remain within normal limits. In urinalysis, moderate proteinuria (up to 1 g / day) and microhematuria are noted. An increase in the concentration of CRP is observed in 60-70% of cases. When ophthalmological examination, decrease visual acuity, more often one-sided, and loss of visual fields.

Instrumental research

One of the basic instrumental methods of examining patients with Takayasu's syndrome is angiography. Its results are important for both confirming the diagnosis, and for assessing the dynamics and prevalence of the inflammatory process. According to angiography, changes in the pulmonary artery occur in almost 60% of patients. The stenoses and / or occlusions of lobar and subsegmental branches predominate, especially the blood supply of the upper lobe of the right lung. Pulmonary hypertension, dilatation and aneurysms of the pulmonary trunk, as well as isolated changes clinically reminiscent of pulmonary artery thromboembolism, are rare. The results of angiography do not allow to differentiate the presence or absence of an active inflammatory process in the wall of the pulmonary artery. For these purposes, CT and MRI angiography are preferable. Ultrasound angiodopplerography is of great importance for examination.

The diagnosis of Takayasu's syndrome can be made according to the classification criteria of the American College of Rheumatology and the criteria offered by other authors.

How to treat Takayasu's syndrome?

Treatment of Takayasu syndrome includes the appointment of prednisolone (1 mg / kg body weight per day, not more than 60 mg / day), which allows 60% of patients to achieve remission and reduce the dose of the drug. Patients refractory to prednisolone use methotrexate, cyclophosphamide, azathioprine. Methotrexate (17.5 mg / week) and combination with small doses of prednisolone allows to achieve remission in 81% of patients, faster to reduce the dose of glucocorticosteroids and to maintain a longer remission. To stop the inflammatory process, monthly courses of pulse therapy with methylprednisolone and cyclophosphamide are used. In view of the long-lasting inflammation in the wall of the arteries, its duration should be at least 6-9 months.

To reduce proliferative changes in arteries, the appointment of acetylsalicylic acid (at a dose of 100 mg / day) and statins is indicated. The duration of maintenance therapy with glucocorticosteroids depends on the dynamics of the main clinical signs, acute phase parameters (ESR, CRP) and, as a rule, is not less than 2-5 years.

Surgical treatment of Takayasu's syndrome

Surgical intervention on the aorta and trunk vessels is desirable in the first five years after diagnosis. Indications for its purpose serve the lumen of the lumen of the artery at 70% and more in combination with ischemia. However, even in the presence of these conditions, the operation should be performed in the inactive phase of Takayasu's syndrome.

Forecast

With Takayasu's syndrome, the 15-year survival rate is 80-90%. The most common causes of death are stroke (60%) and myocardial infarction (about 25%), less often - aortic aneurysm rupture (5%). With the defeat of the coronary arteries in the first two years from the time of the onset of symptoms of cardiac pathology, mortality is 56%. Takayasu's syndrome has an unfavorable prognosis in patients with complications such as retinopathy, arterial hypertension, aortic insufficiency, and aortic aneurysm. In patients who have two or more of these signs, the 10-year survival after diagnosis is 58.6%, with most deaths occurring in the first five years of the disease.

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