Diseases whose pathogenesis involves an abnormal function of the immune system are usually quite dangerous and can lead to a wide variety of health problems. One such condition is Takayasu's disease, also known as Takayasu's arteritis, aortic arch syndrome or pulseless disease.

Takayasu disease is not a common entity - according to available statistics, it is diagnosed in less than three per million people from the general population each year. The majority of sufferers are women, typically the first symptoms of this entity appear in patients aged between 10 and 40 years. For reasons that are as yet unclear, most cases of Takayasu's disease are diagnosed in patients of Asian origin.

Causes of Takayasu's disease

In the course of Takayasu 's disease, arterial vasculitis occurs. Most often, the pathology occurs in the aorta and its main branches, but the abdominal aorta and its descending vessels may also be affected. Although rare, there is a possibility that inflammation associated with this disease will occur in any other arterial vessels. Typically, a feature of Takayasu's disease is the appearance of narrowing within the involved arterial vessels. Other vascular pathologies that are encountered with this entity are sometimes vascular aneurysms, as well as a tendency for thromboses to appear within the narrowed arteries.

The fact that inflammation in large arterial vessels occurs in people with Takayasu's disease and that an overreactivity of T-lymphocytes, among others, is associated with this has already been detected. However, scientists have not yet succeeded in finding the specific cause of Takayasu's disease. However, inherited genes are suspected of playing a role in the onset of the condition, as it has been noted that certain variants of the HLA system predispose patients to develop pulseless disease.

Symptoms of Takayasu's disease

The problem with Takayasu's disease is not only that it is a severe entity, but also that the complaints that appear in patients are very non-specific and can be treated as symptoms of completely different conditions. The first disorders that appear in the course of pulseless disease are flu-like symptoms. Patients may experience, among other things, subfebrile states, weakness or muscle and joint pain. These disorders usually resolve fairly quickly - even completely on their own.

More severe symptoms of Takayasu's disease occur with the development of inflammation within the arterial vessels. Different patients suffering from this entity may have different complaints - depending on the exact arteries in which the inflammation is taking place. In general, pulseless disease may present with a variety of symptoms related to arterial stenosis and obstruction - such problems may be:

• weakness or even absence of a pulse in the upper limb, associated with pallor and weakness of the limb,
• visual disturbances,
• episodes of transient central nervous system ischaemia,
• dizziness,
• fainting,
• chest pain,
• dyspnoea.

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The above complaints occur when pathological inflammation affects the aortic arch and its main branches. Other problems may arise when the disease affects other arteries - for example, if the renal arteries are narrowed, hypertension may occur, while involvement of the abdominal aorta and its branches (such as the visceral trunk) may be accompanied by abdominal pain, gastrointestinal bleeding or diarrhoea.

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How is Takayasu's disease diagnosed?

The diagnosis of Takayasu disease is not easy. This is due, for example, to the fact that it is necessary to exclude other, more common potential causes of the presenting symptoms, such as vascular lesions associated with atherosclerosis or upper thoracic outlet syndrome, before a diagnosis of this entity can be made.

In order to diagnose Takayasu's disease, patients undergo a variety of tests, including laboratory tests (in which inflammatory markers are determined), as well as various imaging studies - vasoconstrictions can be detected during examinations such as CT scans and MRI. Angiography is also a valuable test used in the diagnosis of Takayasu's disease.

Criteria have been developed for the diagnosis of Takayasu disease. There are six features of this entity - at least three of these must be present in the patient to make a diagnosis of pulseless disease and are:

• patient's age at onset less than 40 years,
• detection of arterial stenosis,
• the presence of chroma in any of the limbs,
• the detection of a weak or absent pulse on the brachial artery,
• in the measurement of blood pressure in both upper limbs the detection of a difference in the results obtained exceeding 10 mmHg,
• the detection of an audible murmur over the subclavian artery or over the abdominal aorta.

photo: shutterstock

Treatment of Takayasu's disease

The main preparations that are used to treat Takayasu's disease are glucocorticosteroids. Treatment with these preparations for this entity usually lasts for many months. If, despite treatment with glucocorticosteroids, the patients' condition does not improve satisfactorily, they may be given other drugs that affect the function of the immune system, such as cyclophosphamide or methotrexate.

Sometimes patients with Takayasu's disease require surgical treatment. This may be necessary, for example, in the case of critical arterial stenosis leading to severe ischaemia of various organs or when the patient develops a large aneurysm.