Takayasu syndrome: symptoms, treatment and disease predictions

Among the diseases that affect the arteries and blood vessels of a person, there are many such that are still a mystery for doctors and scientists.

These include Takayasu's disease( Takayasu's syndrome, brachiocephalic arteritis, pulse-free syndrome, nonspecific aortoarteritis) - its causes and mechanisms of occurrence have not been clarified, which significantly complicates early diagnosis and can lead to early disability of patients.

Contents

  • 1 Description and statistics
  • 2 Reasons and risk factors
  • 3 Classification
  • 4 Symptoms
  • 5 Dangers and complications
  • 6 When should I see a doctor?
  • 7 Diagnosis
  • 8 Treatment
  • 9 Prevention and prognosis

Description and statistics


Takayasu's disease is a chronic autoimmune inflammation, that affects primarily the aorta and its major branches .

The arc of the aorta, the carotid, anemia, and subclavian artery are most often involved in the process, less often - mezentrial, iliac, renal, coronary and pulmonary arteries.

For the first time the disease was described by a physician Mikito Takayasu in 1908, who noted specific vascular manifestations on the retina of the patients. Some of his colleagues noted that such manifestations are often accompanied by a lack of pulse on the wrist arteries.

Pathology is rare enough - about 2-3 people out of a million, and it is mostly found in countries of Asia and South America and is more common in women than in men( 8: 1).The average age of patients with this diagnosis is 15-25 years.

Causes and risk factors

The etiology of Takayasu's disease is not clear, but studies have shown its clear association with infectious-allergic and autoimmune factors.

The most likely genetic predisposition of patients to pathology, which is also confirmed by a number of studies. People with this diagnosis often have lymphocyte antigen MB-3 and HLA-DR4 gene .

Classification of

At the onset of the disease in the walls of the aorta and its large branches, the begins an inflammatory process that progresses with time .

The immune complexes accumulate in the vessels, after which the microradia of the choroid, the sclerosing of the walls, the thrombus formation of increases. In later stages, inflammation is transformed into an atherosclerotic lesion of the vessels involved in the pathological process.

Based on the localization of inflammation, in the clinical course of nonspecific aortoarteritis, there are 4 types of :

  • I - the disease affects arteries that branch off from the aortic arch;
  • II-is characterized by lesions of the abdominal and thoracic aorta;
  • III - the aortic arch is involved in the process, together with the abdominal and thoracic sections;
  • IV is a common aortic lesion that affects the pulmonary artery.
In addition, the disease has two stages: acute and chronic, each of which is characterized by different symptoms. The first stage proceeds quickly enough and in the absence of adequate treatment passes into a chronic one( on average, this occurs after 8-10 years).

Symptoms of

In an early stage, Takayasu's illness shows symptoms similar to other vascular and arterial diseases:

  • slight increase in temperature;
  • chills and night sweats;
  • weight loss, lack of appetite;
  • increased fatigue even after light physical exertion;
  • pain in large joints;
  • sometimes - skin nodules, pleurisy, pericarditis.

A characteristic feature of the pathology by which it can be distinguished from other vasculitides is lack of pulse on at least one artery involved in the inflammatory process of .Affected arteries can be painful on palpation, sometimes stenotic noises are noted at the bottom. If the disease affects the thoracic, abdominal or renal aorta, about half of the patients have hypertension.

In the absence of appropriate treatment, Takayasu's disease passes into a second, chronic stage, which is characterized by the clinical symptoms of narrowing or plugging of damaged arteries.

In the long course of the disease and severe tissue damage, some patients develop dystrophic changes and ischemic ulcers on the limbs, and involvement in the carotid artery process can lead to tooth loss and hair loss, as well as facial skin lesions.

No less dangerous is another vasculitis - Behcet's syndrome. Compare the symptomatology in order not to confuse these diseases.

Symptoms and details of treatment of rheumatoid vasculitis, as well as their differences from other types of disease, are described in this material.

Find out all about the treatment and symptoms of Kawasaki's disease in children - this dangerous pathology is important to notice on time!

Dangers and complications of

Complications of the pathology depend on its location( arteries that are involved in the pathological process):

  • changes in the arterial vessels of can lead to aortic failure, which causes myocarditis, myocardial infarctions and coronary artery disease;
  • in cases of lesions of the abdominal part of the aorta , blood circulation of the lower extremities worsens, the patient may experience pain while walking;
  • if an renal artery is involved in the inflammatory process, it can lead to its thrombosis;
  • pulmonary artery disease may cause shortness of breath and chest pain.
Death from Takayasu's illness comes only in the most neglected cases - usually the cause of death is cerebral circulation disorders and cardiac decompensation.

When should I see a doctor?

Given that nonspecific aortoarteritis is diagnosed by primarily in the age of 35-40 years of , patients should be alerted to severe muscle weakness, joint pain, coordination disorders, headaches and other symptoms that are uncommon for young people.

If they occur, should be sent immediately to the rheumatologist , as such signs may indicate an arterial pathology, including a Takayasu disease.

Diagnosis

Takayasu's disease is diagnosed in patients in those cases, when there are at least 3 symptoms of :

  • weakness or lack of pulse in the arteries of the upper limbs;
  • the age of the patient is less than 40 years;
  • intermittent claudication( lameness, which alternates with periods of normal health);
  • the difference between the arterial pressure of the hands is not less than 10 mm.gt;p.
  • noises in the aorta, which are determined by listening;
  • narrowing or occlusion of arteries, revealed by a detailed examination;
  • aortic valve failure;
  • regular increase in blood pressure;
  • increase in ESR, which has no specific cause.

As additional research, the patient can be assigned:

  • blood test, designed to detect abnormalities in blood quality indicators( usually moderate anemia, prolonged increase in ESR, leukocytosis);
  • Ultrasound of blood vessels, with the help of which the speed of blood flow and the state of coronary vessels are evaluated;
  • angiography - the method of radiographic examination of blood vessels, which allows to reveal the sites of narrowing of the arteries;
  • chest X-ray is performed in those cases if there is a suspicion of involvement of the aortic and pulmonary artery in the pathological process;
  • echocardiography allows you to evaluate the functioning of the heart muscle and identify possible violations;
  • electroencephalography and rheoencephalography - through these studies can diagnose violations of the blood vessels of the brain and the level of its activity.

Treatment of

Taktika treatment of Takayasu disease has three objectives:

  • to stop and, if possible, eliminate the inflammatory process in the arteries;
  • control of ischemic complications;
  • elimination of symptoms of hypertension.

The main method of treatment of the pathology is corticosteroids, mainly a preparation called " Prednisolone ".

If it does not work properly, the patient is assigned one more effective drug " Methotrexate ".It refers to cystatics and has an antitumor effect, as well as the ability to suppress DNA synthesis.

In addition, to reduce the risk of atherosclerosis and cardiovascular disorders, it is recommended to take anticoagulants( drugs that dilute blood) - the simplest of them is acetylsalicylic acid.

Another method for treating Takayasu's disease is the extracorporeal hemocorrection , which is also called gravity blood surgery. It is the processing of blood elements, which is carried out outside the patient's body to isolate the particles that cause the development of the disease.

With pronounced violations of the functions of the brain and limbs, the patients are shown surgical treatment of , which is designed to restore impaired blood supply to tissues and organs.

In cases of vascular complications, often raises the question of angiographic intervention of ( by bypassing the narrowing site, endarterectomy, percutaneous angioplasty), and stenosis of the coronary arteries accompanied by stenosis of the renal arteries and myocardial ischemia is performed by vascular plastic surgery.

Prophylaxis and prognosis


Primary preventive measures for Takayasu have not yet been developed by ( primarily due to the unclear etiology of the disease).Secondary prevention consists in appropriate care for patients, prevention of exacerbations and sanation of foci of infection.

With adequate treatment, the prognosis of nonspecific aortoarteritis for patients is quite favorable - a 5-year survival rate in this case exceeds 80% of .

The situation worsens in the presence of complications( arterial hypertension, retinopathy, aortic insufficiency, aneurysm) - only 58% of these patients live more than 5 years.

Takayasu's disease belongs to rare pathologies, but is insured against it, unfortunately, it is impossible to .To avoid the development of possible complications accompanying nonspecific aortoarteritis, it is very important to take responsibility for your health condition - especially for young people who often neglect anxiety symptoms of arterial disorders.

For patients with this diagnosis, , it is important to remember that this pathology is incurable, but adequate therapy allows patients to lead the normal life of without inconveniences and painful sensations.

More about the syndrome of Takayasu, see: