Nonspecific aortoarteritis (Takayasu's disease)

Nonspecific aortoarteritis (aortic arch syndrome, Takayasu's disease, lack of pulse) - destructive-productive segmental aortitis and subaortic panarritis of the arteries rich in elastic fibers with possible damage to their coronary and pulmonary branches.

ICD Code 10

M31.4 Syndrome of the aortic arch (Takayasu).

Epidemiology of Takayasu's disease

Nonspecific aortoarteritis begins more often between the ages of 10 and 20, mostly female patients. In the overwhelming number of observations, the first symptoms of the disease appear at the age of 8-12 years, but the onset of the disease may also occur at preschool age.

The disease is most common in South-East Asia and South America, but cases of Takayasu's disease are registered in various regions. The annual incidence varies from 0.12 to 0.63 cases per 100 000 population. Most often ill teenage girls and young women (under 40 years). Cases of NAA in children and elderly people are noted.

[1], [2], [3]

Causes of Takayasu disease

The etiological agent of this disease is unknown. The relationship of the disease with streptococcal infection is revealed, the role of mycobacteria of tuberculosis is discussed.

At present, it is believed that the imbalance of cellular immunity is of particular importance in the development of autoimmune disorders. In the blood of patients there is a violation of the ratio of lymphocytes; CD4 + T-lymphocyte count increases and CD8 + T-lymphocyte count decreases, Increased number of circulating immune complexes, elastin peptides content and increased elastase activity, cathepsin G, increased expression of MHC I and II antigens.

Pathomorphological changes are most pronounced at the sites of the arteries from the aorta. In the middle shell, foci of necrosis are observed, surrounded by cellular infiltrates consisting of lymphoid cells, plasmocytes, macrophages and giant multinucleated cells.

What causes Takayasu's disease?

[4], [5]

Symptoms of Takayasu's disease

For early stages of the disease, fever, chills, sweating at night, weakness, myalgia, arthralgia, anorexia are characteristic. Against this background, the signs of a common vascular disease (coronary, cerebral, peripheral), especially with the involvement of the upper limbs (lack of pulse), should be alarming.

The unfolded stage of Takayasu's disease is manifested by the defeat of arteries that depart from the arch of the aorta: subclavian, carotid and vertebral. On the side of the lesion, the fatigue of the arm develops under load, its cooling, numbness and paresthesia, the gradual development of atrophy of the muscles of the shoulder and neck, the weakening or disappearance of the arterial pulse, a decrease in blood pressure, systolic murmur on the common carotid arteries. Characteristic are also pain in the neck, along the vessels and painfulness of their palpation due to the progressive processes of inflammation of the vascular wall, transient ischemic attacks, transient visual impairment.

Symptoms that are caused by the defeat of arteries that depart from the abdominal part of the aorta are significantly less frequent: the development of a vasorenal AH of malignant course, attacks of the "abdominal toad", caused by the lesion of the mesenteric arteries, the emergence of intestinal dyspeptic syndromes and malabsorption.

In NAA, coronary artery disease (coronary artery disease) occurs in 3/4 of the patients; its feature consists in the defeat of the mouth of the coronary vessels in 90% of cases, with distal departments being affected less often. The beginning of the disease with isolated stenosis of the coronary artery with the clinic of acute coronary syndrome or myocardial infarction (MI), often without characteristic ECG changes, is described. Coronary infection may also be manifested by the development of ischemic DCM with a diffuse decrease in heart contractility due to myocardial hibernation. Often described as a lesion of the ascending aorta - a densification in combination with dilatation and the formation of aneurysms. Patients with NAA often develop aortic regurgitation due to dilatation of the root of the aorta and / or aortitis. AH occurs in 35-50% of cases and can be caused by the involvement of the renal arteries or the development of glomerulonephritis, less often - the formation of coarctation of the aorta or ischemia of the vasomotor center against the background of carotid artery vasculitis. CHF in arteritis Takayasu occurs due to AH, coronary artery and aortic regurgitation. Cases of thrombosis of the heart cavities as well as myocardial damage with the development of myocarditis, confirmed by endomiocardial biopsy with the detection of necrosis of cardiomyocytes, mononuclear infiltration and associated with the active phase of the disease are described.

Symptoms of Takayasu's disease

Classification of Takayasu's disease

In accordance with the nature of the strain, stenotic, deforming or combined (a combination of aneurysms and stenosis) variants of nonspecific aortoarteritis are isolated. On the localization of lesions, 4 types of nonspecific aortoarteritis are distinguished.

Types of nonspecific aortoarteritis in the localization of lesions

Types	Localization
I	Aortic arch and arteries from it
I	Descending, abdominal parts of the aorta, celiac, renal, femoral and other arteries
III	Mixed variant {widespread lesion of vessels of an area of an arch and other departments of an aorta)
IV	The defeat of pulmonary arteries, combined with any of the three types

[6], [7]

Diagnosis of Takayasu's disease

Laboratory changes: normochromic normocytic anemia, small increase in the number of platelets, hyper-y-globulinemia, increased ESR, fibrinogen concentrations, a2-globulins, circulating immune complexes, rheumatoid factor. The increase in the concentration of CRP is more closely related to the activity of the disease than the ESR. 20-35% of patients have AFL (IgG, IgM), associated with the presence of vascular occlusion, arterial hypertension, and valve damage. In the analysis of urine, moderate proteinuria, microhematuria is revealed.

Of the instrumental methods used ophthalmoscopy, note the angiopathy of the vessels of the fundus, assess visual acuity (as a rule, it decreases).

The main method of instrumental diagnosis in Takayasu's disease is arteriography. With its help, confirm the diagnosis, as well as assess the state of the vessels in the dynamics. It is necessary to study the entire aorta: changes are manifested by narrowing of long sections or occlusion of arteries, the aortic region and the mouths of its visceral branches. Doppler ultrasonic angioscanning and MRI are also used. Their advantage is in the ability to diagnose the disease at an early stage. Histologically, Takayasu's disease is manifested by pan arteritis with localization of the infiltrate mainly in the adventitia and outer layers of copper, but if the results of angiography and clinical symptoms are typical, then a biopsy of the vessel is not required.

The classification criteria for Takayasu's disease (Arend W. Et al., 1990)

• Age of patients (debut of disease <40 years).
• Syndrome of intermittent claudication of the limbs - weakness and discomfort, soreness in the lower extremities when walking.
• Attenuation of the pulse on the brachial artery is the weakening or absence of pulsation on one or both humeral arteries.
• The difference in blood pressure in the brachial arteries is greater than 10 mm Hg.
• When auscultation - a noise over the subclavian artery from one or both sides or over the abdominal aorta.
• Angiographic signs - a narrowing of the aorta lumen and its large branches up to occlusion or dilatation, not associated with atherosclerotic lesion or pathology of development.

The presence of three or more of any criteria serves as the basis for a reliable diagnosis of "Takayasu disease."

Differential diagnostics is performed with other systemic inflammatory diseases, including AFS, infections (infective endocarditis, syphilis, etc.), malignant neoplasms (including lymphoproliferative diseases), atherosclerosis of large vessels.

Diagnosis of Takayasu's disease

[8], [9]

Treatment of Takayasu's disease

Treatment is aimed at suppressing inflammation in the vascular wall, immunopathological reactions, preventing complications, compensating for the symptoms of vascular insufficiency.

Patients with arteritis Takayasu are sensitive to glucocorticoids. The recommended treatment regimen is prednisolone at a dose of 40-60 mg / day for 1 month, followed by a gradual decrease, maintaining a dose of 5-III mg / day for at least 2-3 years. With insufficient effectiveness of monotherapy, improvement can occur with a combination of low doses of glucocorticoids and cytostatics. Preference is given to methotrexate (7.5-15 mg / week). Cyclophosphamide (cyclophosphamide) is used in severe cases with high activity of the inflammatory process. In the treatment-resistant cases, pulse-therapy with cyclophosphamide is performed once a month for 7 to 12 months.

Some patients need either a percutaneous transluminal intervention for the purpose of revascularization, or surgical prosthetics of sharply stenosed areas of the vessels or the installation of a prosthetic aortic valve. Indications for surgical treatment - stenosis of the artery more than 70% with signs of ischemia. With stenosis of the coronary arteries, aortocoronary prosthesis is performed.

Obligatory antihypertensive drugs (with arterial hypertension), anticoagulants (for the prevention of thrombosis), according to indications - statins, antiosteoporetic drugs, antiaggregants,

In cases of non-stopable surgically vasorenal hypertension, beta-blockers, AG1F inhibitors are possible, but they are contraindicated in bilateral stenosis of the renal arteries.

How is Takayasu's disease treated?

Prevention of Takayasu's disease

Primary prevention is not developed. Secondary prevention consists in preventing exacerbations, sanation of foci of infections.

Prognosis of Takayasu disease

Conducted adequate treatment contributes to achieving a 5-10-15-year survival rate in 80-90% of patients.

Of the complications, the most common cause of death is strokes - 50%, myocardial infarction - 25%, rupture of the aortic aneurysm - 5%. K. Ishikawa identifies 4 main groups of complications, retinopathy, arterial hypertension, aortic insufficiency and aneurysms (mainly an aortic aneurysm). The prognosis in patients with these complications is much worse. Thus, 5-year survival in patients with at least two of these syndromes is 58%

Background

In 1908, the Japanese ophthalmologist M. Takayasu reported unusual changes in the retinal vessels during the examination of a young woman. In the same year K. Ohnishi and K. Kagoshimu noted similar changes in the fundus in their patients, which was combined with the absence of pulsation of the radial artery. The term "Takayasu's disease" was introduced only in 1952.

[10], [11], [12], [13]