Takayasu syndrome

Takayasu's syndrome is a granulomatous inflammation of the aorta and its main branches, usually beginning before the age of 50. The disease was first described by D. Savon (1856), but it received its name thanks to the work of Japanese ophthalmologists, one of whom was M. Takayasu (1908).

Epidemiology

Takayasu syndrome is found primarily in Asian and South American countries, but numerous cases of the disease have been registered in other geographic regions of the globe, including Russia. The annual incidence of Takayasu syndrome ranges from 0.12 to 0.63 cases per 100,000 people. Young and middle-aged women are most susceptible to the disease.

[ 1 ], [ 2 ], [ 3 ], [ 4 ], [ 5 ], [ 6 ], [ 7 ], [ 8 ]

How does Takayasu syndrome manifest itself?

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

In the advanced stage of Takayasu's syndrome, as a result of progressive occlusive arterial damage, signs of ischemia of individual organs and tissues occur. The absence of systemic inflammatory reactions does not exclude active vascular inflammation, leading to pain in the projection site of the affected vessel or tenderness during its palpation. Signs of vascular inflammation occur already during the first year from the onset of the disease. Most often (40%), a feeling of weakness, fatigue and pain in the muscles of the forearm and shoulder are noted, which are predominantly one-sided in nature, their severity increases with physical exertion. However, the absence 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 are noted in 15-20% of cases.

In 7-15% of patients with Takayasu syndrome, neck pain, dizziness, transient moments of visual impairment, increased blood pressure, dyspnea, and palpitations occur. In 70% of cases, systolic murmur can be heard in the common carotid arteries, and in 15% of patients, their pain (carotidynia) is observed. Similar physical data are often detected during auscultation and palpation of the abdominal aorta, especially in anatomical types II and III of the disease.

In the third phase of Takayasu's syndrome, signs of fibrosis predominate in the vessels, vascular stenosis and signs of vascular insufficiency are noted. During this period, compared with the early stage of the disease, symptoms of intermittent claudication of the upper and lower extremities, damage to the cardiovascular system, brain and lungs are encountered significantly more often (50-70%). Clinical signs of lung damage are found in less than a quarter of patients. Chest pain, shortness of breath, unproductive cough, and rarely hemoptysis are noted.

Patients report pain in the muscles of the proximal parts of the arms, more often the left, a decrease in hand strength occurs with less physical exertion, at rest or during sleep. Sometimes the pain spreads to the left shoulder, lower jaw, neck area and left half of the chest. In 85-90% of patients with Takayasu syndrome, the pulse in the arteries of the forearm disappears and differences in systolic blood pressure measured in the brachial arteries occur.

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

Classification

Several classifications of pathological changes in blood vessels in Takayasu syndrome have been proposed, the differences between which are not fundamental. There are four anatomical types of the disease. The first type involves changes in the aortic arch and the arteries branching off from it. This variant often involves simultaneous damage to the left subclavian and common carotid arteries. The second type of the disease results in isolated damage to the thoracic and/or abdominal aorta and their branches. The third type involves a pathological process in the aortic arch and/or its branches combined with changes in the thoracic or abdominal aorta. The fourth type involves a pathological process localized in the main pulmonary trunk or its branch in combination (or without it) with any of the first three variants.

[ 9 ], [ 10 ], [ 11 ], [ 12 ], [ 13 ], [ 14 ]

How to recognize Takayasu syndrome?

Usually normochromic normocytic anemia, moderate thrombocytoma and hypergammaglobulinemia are detected. Especially characteristic is the increase in ESR, the degree of which depends on the inflammatory activity of the disease. However, in a third of patients, despite the clinical signs of inflammatory activity, ESR may remain within the normal range. Moderate proteinuria (up to 1 g / day) and microhematuria are noted in urine tests. An increase in the concentration of CRP is observed in 60-70% of cases. During ophthalmological examination, a decrease in visual acuity, often unilateral, and loss of visual fields are noted.

Instrumental research

One of the main instrumental methods of examination of patients with Takayasu syndrome is angiography. Its results are important both for 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. Stenosis and/or occlusion of its lobar and subsegmental branches predominate, especially those supplying the upper lobe of the right lung. Pulmonary hypertension, dilation and aneurysms of the pulmonary trunk, as well as isolated changes clinically resembling pulmonary embolism, are rare. The results of angiography do not allow differentiating the presence or absence of an active inflammatory process in the wall of the pulmonary artery. For these purposes, it is preferable to use CT and MRI angiography. Ultrasound angio-Dopplerography is of greater importance for the examination.

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

How to treat Takayasu syndrome?

Treatment of Takayasu's syndrome includes prednisolone (1 mg/kg body weight per day, no more than 60 mg/day), which allows 60% of patients to achieve remission and reduce the drug dose. In patients refractory to prednisolone, methotrexate, cyclophosphamide, and azathioprine are used. Methotrexate (17.5 mg/week) in combination with small doses of prednisolone allows 81% of patients to achieve remission, reduce the dose of glucocorticosteroids more quickly, and maintain longer remission. Monthly courses of pulse therapy with methylprednisolone and cyclophosphamide are used to relieve the inflammatory process. Due to long-term inflammation in the arterial wall, its duration should be at least 6-9 months.

To reduce proliferative changes in the arteries, acetylsalicylic acid (at a dose of 100 mg/day) and statins are indicated. The duration of maintenance therapy with glucocorticosteroids depends on the dynamics of the main clinical signs, acute phase indicators (ESR, CRP) and, as a rule, is at least 2-5 years.

Surgical treatment of Takayasu syndrome

Surgical intervention on the aorta and main vessels is desirable to be performed in the first five years after diagnosis. Indications for its appointment are narrowing of the artery lumen by 70% or more in combination with ischemia. However, even in the presence of these conditions, the operation should be performed in the inactive stage of Takayasu syndrome.

Forecast

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

[ 15 ]