Takayasu's arteritis

Content

Introduction

Takayasu's arteritis (TA) is a rare inflammatory disease of the great arteries, of unknown origin. It mainly affects the aorta (the main artery that carries blood from the heart to the rest of the body) and its main branches: the coronary arteries (which allow blood to reach the heart) and pulmonary arteries (which send blood to the lungs) .

Inflammation of the arterial wall in 90% of people causes a variable degree of narrowing of the internal cavity of the artery (stenosis), up to the closure and, consequently, to the reduction or blocking of blood flow. This phenomenon results in the loss of the ability to hear the heartbeat in the peripheral arteries such as, for example, those of the wrist. In 25% of patients there is a weakening and dilation of the wall of an artery (aneurysm). In others, dilation of the aorta can lead to defects in the aortic valve (located in the heart) which, if severe, may require replacement.

TA is a rare disease that appears between 5 and 40 years of age. Although both sexes can be affected, 80-90% of sufferers are women. It occurs predominantly in the oriental race and is more common in people from the Far East, Japan and the Asian sub-continent.
It is not a hereditary genetic disease but the increase of its diffusion (prevalence) in some parts of the world indicates a genetic component in the susceptibility to get sick.

Symptoms

The initial symptoms (symptoms) of TA are usually poorly specific and may include:

  • general feeling of being unwell
  • profound tiredness
  • fever
  • night sweats
  • weight loss
  • muscular pain (myalgia)
  • joint pains (arthralgia)
  • skin rashes
  • dizziness / slight lightheadedness
  • shortness of breath
  • cramps or pain in the arms, legs or chest

Carotidodynia, pain in the carotid arteries in the front of the neck, is present in about 25 percent of sufferers.

Causes

The cause of Takayasu's arteritis remains unknown, as well as why an individual develops the disease at any given time. Environmental factors associated with a genetic predisposition are likely to intervene. A common but unproven hypothesis is that TA is triggered by an "infection. The rarity of the disease makes it difficult to identify the cause.

Diagnosis

The factors that may suggest the presence of Takayasu's arteritis are:

  • onset of the disease at an age of 40 years or less
  • severe muscle pain in a limb caused by cramps due to narrowing of blood vessels (limb claudication)
  • reduced pulsation of the artery of the arm (brachial artery)
  • difference in maximum blood pressure value (greater than 10 millimeters of mercury) between the two arms
  • murmur of the aortic or subclavian arteries
  • angiographic anomaly

In practice, the medical examination shows reduced or absent pulsations in the arms and, albeit less frequently, in the legs. Using a stethoscope, a loud can be detected hissing noise (murmur) on the neck, chest or kidney indicating a restriction in the functioning of the arteries. High blood pressure is another common disorder (symptom). To aid in the diagnosis and identify inflammation, narrowing or dilation of the arteries and extension of the disease, the following are used:

  • positron emission tomography (PET)
  • angiography with magnetic resonance imaging (MRI)
  • Doppler echo high resolution
  • computed axial tomography (CT scan)

Diagnostic imaging is useful for ascertaining and monitoring TA over time. Its use allows you to discover the disease before arterial damage becomes severe.

There is no blood test that can be used to definitively ascertain (diagnose) the disease, but tests such as, for example, the erythrocyte sedimentation rate (ESR) and the C-reactive protein (CRP) can help measure its activity and the response to treatment.

Positron Emission Tomography (PET): involves the administration of a small amount of radioactive fluorodeoxyglucose (radiopharmaceutical). After being injected intravenously, the radiopharmaceutical is distributed in the body allowing to obtain images which, interpreted by specialist doctors, allow to investigate the functioning of organs and systems.
PET is useful for detecting inflammation of the arterial wall and is often used in combination with CT (CT-PET). PET can identify disease early and can help distinguish active disease (which needs further treatment) from scars resulting from previous "inflammation.

Magnetic resonance angiography (RMA): Nuclear magnetic resonance (MRI) is commonly used to assess the extent of TA and, sometimes, a contrast agent may be used to improve images of the arteries. time (monitor) the progress of the disease and the response to treatment It also allows to obtain a good visualization of the blood flow in the carotids and arteries with the possibility of verifying the possible presence of flow restrictions or aneurysms.

High resolution echo-doppler: it is a non-invasive method that allows you to view the main blood vessels and study the flow of blood within them. It uses an ultrasound probe which, passed over the carotid arteries of the neck, detects the arterial wall and its degree of narrowing or dilation.

Computed Axial Tomography (CT): is a diagnostic imaging method that uses ionizing radiation (X-rays) and makes it possible to reproduce sections or layers (tomography) of the patient's body and perform three-dimensional processing.

Therapy

The treatment aims to improve the ailments (symptoms) and prevent further damage and / or scarring of the blood vessels. It is based on the use of so-called corticosteroid drugs, usually prednisolone, to which an immunosuppressive drug can be added in case of non-response.
Commonly used medicines include:

  • methotrexate (or methotrexate)
  • azathioprine
  • mycophenolate

They increase the efficacy of steroid therapy and help reduce its dose.
In people who have been ill for a long time, or in very severe form, cyclophosphamide may be recommended.

In recent years, biological therapies have been used successfully in unresponsive (refractory) disease, including tumor necrosis factor inhibitors (TNF) such as etanercept and infliximab.

Among the additional treatments often prescribed for AT:

  • medicines to lower blood pressure (antihypertensives)
  • statin drugs to reduce the amount of cholesterol and the risk of heart disease. Even if cholesterol levels were normal, statins could be indicated in the case of TA due to the beneficial effect they have on arterial function
  • low dose aspirin to reduce the risk of developing blood clots

Surgery

Surgery is indicated only for the most severe cases of AT and the decision involves a team of specialist doctors including the rheumatologist, the vascular surgeon and the radiologist. For best results, the surgery must be performed when the disease is in remission.
The interventions that can be performed include:

  • revascularization, technique that involves bypassing the occluded artery with a bridge graft to overcome the obstruction. In a long-term study, survival rates, 20 years after transplantation, are greater than 70%. However, the results are variables and further confirmatory studies are needed
  • percutaneous transluminal angioplasty (PTA), a method that allows you to totally or partially dilate the occluded arteries by inserting balloon catheters. Thanks to an extremely small caliber metal guide, the catheters are made to proceed inside the coronary arteries until they reach the narrowing that occludes the vessel. Once the narrowing point is reached, the balloon is inflated, returning an adequate diameter to the blood vessel.
  • stent, metal mesh which, in some cases, is inserted into the artery to keep it open

Complications

Takayasu's pregnancy and arteritis

Most women with Takayasu's arteritis have delivered one or more healthy babies.
However, both the disease and the drugs used to treat it can be risky for the mother and baby. It is therefore very important for the woman to plan a pregnancy and develop, together with the specialist, a global plan to limit the possible complications: for example, to maintain normal blood pressure which, in people with TA can be difficult (in pregnancy the values ​​can increase to dangerous levels).

AT itself does not affect fertility in men or women, however, some of the drugs used to treat it can reduce it, while others can increase the risk of fetal defects. It is therefore essential for both women and men. , consult your specialist to discuss all aspects of a pregnancy.

The long-term evolution of AT is good. About 20 percent of people will have only one inflammatory episode (monophasic self-limiting disease) but, typically, the disease will follow an ups and downs characterized by periods in which the disorders will be present (relapse) and others in which they will be absent (remission).

Available data on long-term outcomes are limited and vary depending on the severity of the disease. In most people, the disease appears to stop after 2-5 years and therapy can be gradually reduced.

Below, the data from surveys carried out around the world:

  • in 25% of people, AT does not affect daily activities
  • 25% of individuals are able to carry out normal daily activities when the disease is in remission
  • 50% of sufferers are unable to perform, or maintain, the full range of daily activities
  • up to 25% of people are unable to work due to AT

With treatment initiated in the initial (early) stages of the disease and following its course over time in a specialized center, AT can be treated effectively.

Mortality rates are low with survival in the United States and the United Kingdom of up to 98 per cent at 10 years.
Mortality directly associated with TA is usually the consequence of a heart attack, heart failure, ruptured aneurysm, stroke or "kidney failure.

Bibliography

Vasculitis UK. Takayasu arteritis (English)

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