Takayasu’s disease
Takayasu’s disease (nonspecific aortoarteritis) is an idiopathic inflammatory lesion of the aorta, its departments and large arterial branches. The etiology of Takayasu’s disease is unclear, presumably there is an autoimmune nature of development. The clinical course of nonspecific aortoarteritis depends on the level of damage to the aorta (arch, thoracic or abdominal aorta) or the pulmonary artery with its branches. Characteristic signs are asymmetry or absence of pulse on the brachial arteries, difference in blood pressure on different limbs, aortic murmurs, angiographic changes – narrowing or occlusion of the aorta and its branches. Treatment of Takayasu’s disease is carried out with corticosteroids, cerebrovascular complications are not uncommon.
General information
The causes of Takayasu’s illness have not been reliably determined. Her debut is associated with exposure to infectious and allergic agents and autoimmune aggression. Deposited in the vascular walls, immune complexes cause granulomatous inflammation and narrowing of the inner diameter of the vessel, which contributes to thrombosis. The outcome of nonspecific aortoarteritis is sclerotic changes in the proximal segments of medium- and small-caliber arteries.
The prevalence of Takayasu disease is higher in South America and Asia than in Europe. Nonspecific aortoarteritis affects more often young people aged 10-30 years and women under 40 years.
Classification of Takayasu disease
According to the topographic feature, several variants of the course of Takayasu’s disease are distinguished. The first variant is characterized by an isolated lesion of the aortic arch and its branches – the left common carotid and subclavian arteries. In the second anatomical type of nonspecific aortoarteritis, a lesion of the thoracic or abdominal aorta with its branches develops. For the third variant, a combination of changes in the aortic arch, as well as its thoracic and abdominal sections is typical. The last anatomical type of Takayasu’s disease is characterized by involvement of the pulmonary artery with its branches and possible combination with any of the above options.
Symptoms of Takayasu’s disease
For Takayasu’s disease, the clinic of upper limb ischemia is typical – weakness and pain in the hands, numbness, low tolerance to physical exertion, lack of pulsation on one or both subclavian, brachial, radial arteries, cold hands. There is a clear difference in blood pressure on the affected and healthy upper extremities, as well as higher blood pressure values on the lower extremities. With nonspecific aortoarteritis, there may be pain in the left shoulder, neck, and left side of the chest. When palpating the altered arteries, soreness is felt, when auscultation is heard over them, characteristic noises are heard.
Inflammatory lesion of the vertebral and carotid arteries in Takayasu’s disease causes neurological symptoms – dizziness, distraction of attention and memory, decreased performance, unsteadiness of gait, fainting. Objective focal symptoms indicate ischemia of the corresponding parts of the central nervous system. The lesion of the visual analyzer in nonspecific aortoarteritis is expressed in the deterioration of vision, the appearance of diplopia, sometimes sudden blindness in one eye due to acute occlusion of the CAC (central retinal artery) and atrophy of the optic nerve.
Pathological expansion and compaction of the ascending aorta in patients with Takayasu’s disease often leads to aortic insufficiency, disorders of coronary circulation, ischemia and myocardial infarction. In 50% of patients with cardiac manifestations of nonspecific aortoarteritis, myocarditis, arterial hypertension, and chronic circulatory insufficiency are noted.
Changes in the abdominal aorta cause a progressive decrease in blood circulation in the lower extremities, pain in the legs while walking. When the arteries of the kidneys are affected, proteinuria, hematuria is detected, thrombosis develops less often. Involvement of the pulmonary artery manifests itself as chest pain, shortness of breath, and the development of pulmonary hypertension. In Takayasu’s disease, there is an articular syndrome – arthralgia, migrating arthritis with a predominant interest in the joints of the hands.
Diagnosis of Takayasu’s disease
The diversity of the clinical picture in Takayasu’s disease leads the patient to consult a rheumatologist, neurologist, vascular surgeon. The diagnosis of nonspecific aortoarteritis is considered obvious in the presence of 3 or more of the following criteria: the onset of the disease at a young age (up to 40 years); the development of the syndrome of “intermittent claudication”; weakening of peripheral pulsation; difference > 10 mm Hg when measuring blood pressure on different brachial arteries; pathological noises in the projection of the abdominal aorta and subclavian arteries; characteristic angiographic changes.
A clinical blood test for nonspecific aortoarteritis reveals moderate anemia, ESR growth, leukocytosis. In the biochemical analysis of blood, a decrease in the level of γ-globulins, albumins, haptoglobin, seromucoid, cholesterol, lipoproteins is determined. In the course of immunological studies, the presence of HLA–antigens, an increase in the level of immunoglobulins is established.
With vascular ultrasound, selective angiography, and aortography, partial or complete occlusion of the vascular branches of the aorta of various lengths and localization is revealed. Rheoencephalography data in Takayasu’s disease indicate a decrease in blood supply to the brain; with EEG of the brain, a decrease in functional brain activity is determined. When conducting a vascular biopsy in the early stage of nonspecific aortoarteritis, signs of granulomatous inflammation are found in the resulting material.
Treatment of Takayasu’s disease
In the active period of nonspecific aortoarteritis, inflammation suppression, immunosuppression and correction of hemodynamic disorders are carried out. Patients with Takayasu’s disease are prescribed methotrexate, prednisone, azathioprine, cyclophosphamide, antihypertensive drugs. To prevent thrombosis, antiplatelet agents (acetylsalicylic acid, dipyridamole) and anticoagulants of indirect action are indicated.
The inclusion of extracorporeal hemocorrection methods in the complex therapy of Takayasu disease makes it possible to purify the blood from immune complexes, thrombosis factors, restore blood flow and improve the functioning of ischemic organs. In case of nonspecific aortoarteritis, cryoaferesis, plasmapheresis, lymphocytapheresis, cascade plasma filtration, extracorporeal immunotherapy are performed.
With vascular complications, the issue of angiosurgical intervention (endarterectomy, bypass bypass of the occlusion site, percutaneous angioplasty) is often resolved. Indications for vascular plasty in nonspecific aortoarteritis are the development of coronary artery stenosis with myocardial ischemia, renal artery stenosis with arterial hypertension, intermittent claudication syndrome; critical stenosis of three or more brain vessels; aortic valve insufficiency; thoracic/abdominal aortic aneurysms > 5 cm in diameter.
Prognosis of Takayasu disease
Nonspecific aortoarteritis has a long-term course. Timely diagnosis of Takayasu’s disease and immunosuppressive therapy can save the patient from the need for angiosurgical intervention. Prognostically, the rapid progression of Takayasu’s disease is less favorable. Severe complications (stroke, heart attack, retinopathy, aortic aneurysm dissection) are associated with the risk of disability and death of the patient.
Methods of prevention of nonspecific aortoarteritis are not known.
