The autoimmune disease Takayasu’s arteritis begins very nonspecifically with a strong feeling of illness and symptoms that are also present in a conventional viral flu and may mislead the treating physician into an incorrect diagnosis. Subsequently, the disease progresses to a chronic course in which the symptoms caused by the circulatory disturbances come to the fore.
What is Takayasu’s arteritis?
Takayasu arteritis, also known as Takayasu disease, is a very rare autoimmune disorder that affects mostly East Asian women and was named after Mikito Takayasu, who first described the condition in 2008. Takayasu’s arteritis results in granulomatous, and thus vaso-occlusive, inflammation of the outgoing branches of the aorta, and in a few cases, the pulmonary artery. Rarely, bulges, so-called aneurysms, occur in the affected vessels. This form of the disease is generally called vasculitis, a group of diseases that have in common an inflammation of the blood vessels. As a result, the disease then often damages the associated organs or limbs, as there is a reduction in their supply. The vasculitic diseases are among the rheumatic diseases.
Causes
The causes of Takayasu’s arteritis are still largely unclear today. Autoimmunologic processes are suspected. Environmental factors, hepatitis viruses, and bacterial infections with Staphylococcus aureus, among others, are under discussion. A genetic predisposition to the disease has also not been ruled out. However, the ultimate cause of vascular occlusion in Takayasu’s arteritis has now been clarified. After an accumulation of inflammatory cells initially causes vasoconstriction on the vessel walls, the accumulation of cells creates areas that are exposed to high pressure. The vessel walls calcify, scar tissue develops, and subsequently the vessels become stiff and narrow.
Symptoms, complaints, and signs
A distinction should be made between symptoms in the incipient and progressive stages of Takayasu’s arteritis. In the beginning, there are usually general complaints that cannot be clearly associated with inflammation of the blood vessels. Some nonphysicians misinterpret these as [[flu]]. They lead to a weakening of the body. Patients complain of febrile conditions. Night sweats are not uncommon. Without a change in lifestyle, affected individuals lose weight. Pain is present near the sites of inflammation. The joints and muscles feel attacked. If the disease progresses, new vascular occlusions develop. Patients then often complain of pain in the arms. Even lifting light objects can become a strain. In this context, dizziness is not uncommon. The natural blood circulation is disturbed. If the carotid artery is affected, the neck feels sore. Pain there is often accompanied by spontaneous visual disturbances. There is a risk of stroke. Sometimes sufferers complain of breathing difficulties and chest pain in connection with Takayasu’s arteritis. These very symptoms should not be disregarded. They turn out to be fatal if they are not treated professionally. Early cure of the typical signs brings the greatest chances of improvement.
Diagnosis and progression
Usually, Takayasu arteritis begins with a severe feeling of illness. Patients perform very fatigued and weak, suffer from loss of appetite and weight loss, and report joint and muscle pain. There may be fever and night sweats. After the initial illness, chronic symptoms such as visual disturbances, dizziness and fainting, high blood pressure and headaches develop as a result of the circulatory disturbances and the reduced supply to the affected organs. The risk of stroke and heart attack is also increased. Blood pressure in both arms is often different. An ultrasound examination can visualize the vasoconstrictions and inflammatory processes. A computer or magnetic resonance imaging scan can provide a more accurate picture of the vascular system and also visualize any bulges in the vessels.Since there are no disease-specific laboratory values for Takayasu’s arteritis, the examination of the blood is not the main focus in the diagnosis. Only elevated inflammatory parameters, such as C-reactive protein (CRP) and fibrogen, accelerated blood sedimentation, and white blood cell elevation can be detected with laboratory findings.
Complications
Individuals suffering from Takayasu arteritis develop severe joint and muscle pain relatively quickly. Severe complications can develop if the circulatory problems are not treated. Damage to the heart valve on the aorta can then develop, and the risk of heart attack increases. Organ damage and strokes also occur repeatedly in the course of the disease. Many sufferers also experience recurrent dizziness and fainting spells, which can lead to falls and subsequent serious injuries. If the patient has a previous illness, even the typical fever episodes can lead to serious complications. Circulatory failure is relatively rare, but can be fatal for the patient if accompanied by the appropriate symptoms. Drug treatment usually proceeds without major complications. However, immunosuppressants and corticosteroids can cause side effects, interactions and allergic reactions. Intervention via balloon catheter, laser or stent can cause vascular damage and other adverse events. If a stent is placed in the aorta, this can lead to cardiac arrhythmias. Rarely, acute occlusion of the stent and myocardial infarction may occur. Furthermore, hypersensitivity reactions to the contrast agent may occur.
When should you go to the doctor?
People suffering from a general feeling of illness or malaise should talk to their doctor about what they are observing. The condition is often mistaken for symptoms of the flu, so individuals should carefully examine and describe what symptoms they are noticing. Patients often perceive differences in their current state of health compared to influenza illness at an early stage. They should also report these to their doctor accordingly. Fever, night sweats or spontaneous disturbances of the usual vision should be examined and treated. Pain in the chest or upper body area is considered unusual and a sign of a present illness. Impaired respiratory activity, internal irritability, and general weakness must be presented to a physician. A decrease in usual performance and an increase in the need for sleep are evidence of a present health disorder. Since a severe course of the disease can lead to the premature death of the affected person, appropriate action should be taken at the first warning signs of the body. A doctor must be consulted as soon as shortness of breath or a racing heart occur. Anxiety, the feeling of too little oxygen in the organism as well as a pale appearance should be examined and treated. In case of a loss of internal strength as well as the impossibility to move without help, a visit to the doctor is necessary.
Treatment and therapy
The standard therapy for Takayasu’s arteritis is prolonged cortisone treatment. Initially, high doses of corticosteroids are used to control the inflammation of the blood vessels. Then, the dose is slowly reduced as much as possible and continued for at least 6 months to two years before cortisone discontinuation, under strict medical supervision with regular monitoring of blood levels, can be considered. If corticosteroids do not bring the desired success, treatment with immunosuppressants is indicated on a trial basis, although the efficacy of such therapy has not yet been clinically confirmed. In cases of impending vascular occlusion, surgery with lasers or balloon catheters is often performed in an attempt to reopen the vessel. A stent may also be used to stabilize a vessel. Untreated Takayasu arteritis leads to death in most cases. However, if therapy is started early, the prognosis for those affected is very good. The survival rate after 10 years is more than 90 percent. Serious complications occur in about 25 percent of sufferers.
Prevention
To prevent Takayasu’s arteritis, due to the fact that the cause of the disease is largely unexplained today, only general recommendations for a healthy lifestyle can be given, such as an adequate amount of exercise, a healthy, whole-food diet, abstaining from tobacco products, and drinking alcohol in moderation.
Follow-up
In most cases, the person affected by Takayasu’s arteritis has few and also limited options for aftercare. In the first place, the affected person should see a doctor at an early stage so that complications or other complaints do not arise in the further course. The earlier a doctor is consulted, the better the further course of the disease usually is. Most of those affected are dependent on a quick surgical intervention. There are no particular complications, although those affected should rest and take care of their bodies after the operation. They should refrain from exertion or stressful activities in order not to put unnecessary strain on the body. In the event of side effects or uncertainties, a doctor should always be consulted first, and he or she should also be consulted if there are any questions. In many cases, those affected by Takayasu’s arteritis are also dependent on the help and care of their own family. This mainly alleviates or prevents depression and other psychological upsets. It cannot be universally predicted whether Takayasu’s arteritis will result in a reduced life expectancy for the affected person. In this regard, the earlier the disease is detected, the better the subsequent course usually is.
What you can do yourself
Takayasu’s arteritis is treated with medication and surgery. The most important self-help measure is to take prescribed glucocorticoids and TNF blockers carefully. In addition, unusual symptoms must be reported to the physician. A twinge in the limbs, dizziness or pain suggest an aneurysm at risk of rupture, which must be treated surgically. After surgery, it is important to follow the doctor’s instructions. A change in diet can further reduce the risk of an aneurysm. Patients should eat a healthy diet and avoid fatty or high-sugar foods. It is best to consult a nutritionist, who can provide further tips on diet and set up a nutrition plan. The doctor in charge can also put the patient in touch with a physiotherapist, who can give tips on physiotherapeutic measures. If the above measures are followed, the prognosis is good. According to one study, two-thirds of patients can lead a relatively symptom-free life, but they must continue to take the medication prescribed by their doctor to rule out serious complications. In the event of an aneurysm, initial emergency medical care is required. The patient must then be treated in the hospital. The goal is to close the hemorrhage to avoid serious health complications, including death of the patient.
