What is Takayasu’s Disease?
Takayasu’s disease (arteritis Takayasu, a disease of lack of pulse, brachiocephalic arteritis and other names) is a chronic granulomatous arteritis with a primary lesion of the aorta and its main branches. More often the aortic arch, the nameless, carotid and subclavian arteries are involved in the process, somewhat less often the celiac, mesenteric, renal, ileal, coronary and pulmonary arteries. Inflammatory granulomatous changes initially begin in adventitia and the outer layers of honey; there are histological accumulations of lymphocytes, plasma and reticular cells, to a lesser extent neutrophils and giant cells are present. In the later stages, granulomas are fibrosed, tears and hardening of the media occur and marked intimal proliferation leads to a narrowing of the vessel lumen and facilitating the development of thrombosis. The disease is relatively rare. It is found in various regions of the world, but most often, apparently, in Japan, where its signs are established in 0.03% of all autopsy examinations. Over 80% of all cases are women, and mostly between the ages of 15 and 25 years.
Causes of Takayasu Disease
The previous assumptions about the direct effect of infection and the presence of antibodies to arterial tissue have not been confirmed. Currently, much attention is paid to genetic predisposition. There are indications of a more frequent combination of Takayasu’s disease with HLA-DR4 and the B-cell antigen MB-3. D. Scott et al. Based on their research, it is assumed that cytotoxic T-lymphocytes are directly involved in the occurrence of pathological changes in the arteries in this disease.
Symptoms of Takayasu Disease
In the clinical picture can be divided into two stages.
- In the initial, acute stage, systemic symptoms prevail – low fever, weakness, weight loss, night sweats. In the same period arthralgia and arthritis are possible, which leads to an erroneous diagnosis of RA. Much less common pleurisy, pericarditis, scleritis, skin nodules, which even more simulates RA.
- After a very long period (from several months to several decades, an average of 8 years), the second, chronic, stage of the disease develops, which has clinical symptoms of narrowing or blockage of the affected arteries in accordance with the zones of their spread.
Characterized by the absence of a pulse in at least one of the most frequently involved in the process of the arteries – radial, ulnar, external carotid, subclavian, femoral. The affected arteries on palpation can be painful (especially sleepy), stenotic noises often appear above them. Corresponding limbs are cold, many patients have pain in these limbs during exercise and paresthesias in the distal regions. At least half of the patients have hypertension due to a narrowing of the thoracic or abdominal aorta, or as a result of damage to the renal arteries. It should be borne in mind that the usual method of determining blood pressure on the hands may be untenable because of the sharp narrowing of the corresponding arteries, which makes it necessary to measure pressure on the legs or by means of ophthalmodynamometry. The defeat of the coronary arteries, especially in combination with hypertension, can lead to the development of angina, myocardial infarction, circulatory failure. Stenosing aortitis can cause relative aortic valve insufficiency. Involvement of the carotid arteries in the process causes headaches, dizziness, seizures, fainting, loss of intelligence, visual disturbances (the latter are aggravated when the head is tilted back). A significant narrowing of the mesenteric artery causes abdominal pain, diarrhea, and gastrointestinal bleeding.
With a long course of the disease and a sharp stenosis of the arteries in a number of patients, dystrophic changes of the ischemiases are expressed.
Diagnosis of Takayasu Disease
Laboratory values are non-specific. Characterized by a significant increase in ESR, the increase in the level of acute-phase proteins and immunoglobulins, hypoalbuminemia, moderate normochromic or hypochromic anemia. The number of leukocytes is often unsharply increased.
The diagnosis is made on the basis of the absence or weakening of the pulse, the appearance of noise over the affected arteries, regional symptoms of ischemia. Arteriography can be a direct proof of the involvement of large arteries in the process, with the help of which they detect the greatest changes in the area of the aortic arch and its main branches, less often in the abdominal aorta and its branches. These changes are manifested in conical or uniform narrowing of arteries with a smooth inner contour, sometimes they are combined with the expansion of blood vessels. For dynamic monitoring of the state of the aortic root, it is advisable to use echocardiography.
Treatment of Takayasu Disease
Without adequate treatment, Takayasu’s disease takes a progressive course with occasional exacerbations and worsening of blood circulation in the affected areas. Spontaneous remissions are possible, but are very rare. The main causes of death are cardiac decompensation and cerebral circulation disorders. The main treatment method is corticosteroids. The initial daily dose is 30-60 mg of prednisolone. This treatment in most patients leads to a rather rapid improvement with the reverse development of fever and general symptoms, recovery of the pulse and reduction of symptoms of regional ischemia, normalization of laboratory parameters. With obvious signs of positive dynamics (usually after 2-4 weeks), the daily dose of prednisone is gradually reduced to the minimum maintenance, ensuring a state of stable improvement. The total duration of treatment should be at least a year. Simultaneously with corticosteroids, it is advisable to take long-term administration of immunosuppressants or plaquinil, but there has been no targeted assessment of their role in the general system of therapy. Vascular preparations (such as prodectin or complamine) and anticoagulants, as well as cardiac glycosides and antihypertensive agents, are also used as aids, especially for specific symptoms of local ischemia. In some cases, surgery is necessary with plastic replacement of sharply narrowed areas of the arteries or the creation of an artificial bypass path of blood flow. With the timely start of active therapy, the prognosis of the disease is relatively satisfactory – a 5-year survival rate exceeds 80%.