The term vasculitis is used to describe several autoimmune diseases whose common characteristic is inflamed blood vessels. The symptoms and course of vasculitis can vary widely depending on the severity of the disease.
What is vasculitis?
Vasculitis is an inflammation in the blood vessels caused by reactions of the body’s own immune system. It can be divided into a group of different autoimmune diseases. Thus, there is primary vasculitis, which includes independent inflammatory rheumatic diseases. These include vasculitides of large vessels, vasculitides of medium-sized vessels and vasculitides of small vessels. Furthermore, secondary vasculitis is known, which results from infections, rheumatic diseases, drugs or other autoimmune diseases. The different forms of vasculitis also differ in terms of their frequency. Every year in Germany, up to 20,000 people suffer from inflammation in the large vessels. Women are more affected than men. Secondary vasculitis is diagnosed in more than 10,000 individuals within Germany each year.
Causes
The causes of primary vasculitis are as yet unknown. Possible triggers of secondary vasculitis include viral infections, rheumatoid arthritis, malignant tumors, blood as well as lymphatic diseases, drugs, and the autoimmune disease systemic lupus erythematosus. Among the causes, a disturbed function of the immune system is important. Some forms of vasculitis are caused by antibodies directed against the body. These autoantibodies can, for example, fight against the white blood cells. Immune complexes can also cause vasculitis. In this case, the body’s own antibodies bind drugs or particles of bacteria, creating immune complexes that are deposited in the walls of the vessels. This damages the vessels. For example, hepatitis viruses are found in some variants of this disease. Depending on the severity of the vasculitis, reactions are subsequently triggered such as vessel occlusion or the formation of aneurysms, granulomas and ulcers.
Symptoms, complaints, and signs
Vasculitis initially leads to nonspecific symptoms that subsequently expand unambiguously. Above all, a subjective feeling of illness is noticeable in the early stages. Affected individuals are no longer able to perform in the usual way. Fever and joint complaints are also present. A sudden loss of weight may occur. The inflammation of the blood vessels expands in a few days to include further complaints – depending on which organ is affected. Pain and signs then appear locally. Patients describe especially the attack on the heart, kidney, lungs and nervous system as extremely painful. On the heart, muscle inflammation may develop, accompanied by constant chest pain. Kidneys and the lungs are at risk of loss of function. Those affected by vasculitis of the kidneys have blood in their urine. If the lung tissue is inflamed, one coughs up blood several times a day. The nervous system is affected by paralysis. Also, cramps often set in. The head and the psyche suffer. A stroke is possible. If the skin is affected as the largest organ, reddening of the skin and sometimes open skin areas occur. The reduced supply of nutrients and oxygen proves its effect here. At the muscles the vasculitis provides for rheumatic complaints. On the stomach, if affected, there is constant abdominal pain. Sometimes vasculitis also occurs in the eyes. Then visual disturbances and an affection of the sclera are possible.
Diagnosis and course
Various diagnostic methods are available when vasculitis is suspected. Laboratory tests are performed because white blood cell count, erythrocyte sedimentation rate, and CRP levels increase with vasculitis. Furthermore, other characteristic blood values can also indicate a certain form of vasculitis. These include values such as ANCA, complement values and viruses. An important part of the diagnosis is also a sample of tissue or a biopsy. Depending on the type of vasculitis, X-ray imaging of the vessels may also aid in diagnosis. If medium and large vessels are diseased, characteristic changes can be detected with the aid of X-ray contrast media.How the respective vasculitis develops further depends decisively on its severity. However, with early diagnosis and consistent therapy, the symptoms of most forms of vasculitis can be alleviated.
Complications
Vasculitis can lead to many different symptoms, all of which have a very negative impact on the quality of life of the affected person. Patients suffer from severe fever and equally from sweating at night. This also causes sleep problems, so that most patients suffer from irritability or depression and other psychological upsets. Furthermore, the disease leads to severe weight loss and further also to severe pain in the joints. There is a general weakness, so that the affected persons feel permanently tired and weary. The ability to work under pressure also decreases significantly. Patients are no longer able to perform strenuous physical activities or participate in sports. If vasculitis already occurs in a child or adolescent, the disease leads to a significant delay in development. As a rule, the symptoms are alleviated with the help of medication, although a complete cure of vasculitis cannot be achieved. Further complications do not occur. A healthy diet can also have a positive effect on the course of the disease. Whether vasculitis leads to decreased life expectancy in patients cannot be universally predicted.
When should you see a doctor?
With vasculitis, the affected person usually always requires treatment by a physician. It is not possible for this disease to heal on its own, so the affected person should contact a doctor at the first symptoms or signs of the disease. Only by starting treatment early can further complications and discomfort be avoided. A doctor should be contacted in case of vasculitis if the affected person suffers from sudden weight loss and high fever. The symptoms occur without any particular reason and do not disappear on their own. Furthermore, severe chest pain may also indicate this disease and should also be examined by a medical professional. Most patients with vasculitis also show bloody urine or severe cramps in the muscles, and vision problems may also occur. Vasculitis can be detected by a general practitioner, although the treatment itself is usually carried out by a specialist and depends on the exact nature of the symptoms. It cannot generally be predicted whether there will be a reduced life expectancy for the affected person.
Treatment and therapy
Treatment of vasculitis adjusts to the severity and extent of the disease. Often, although it cannot be cured, it can be treated very well. Strong medications are often used for this purpose if the patient’s organs or life are threatened. In most cases, cortisone must be taken, the dose of which is slowly reduced. In addition to this drug, immunosuppressants are often necessary. These are drugs that are supposed to slow down the immune system that is working too aggressively. In severe forms of vasculitis, it is therefore impossible to avoid taking the immunosuppressive drug cyclophosphamide. Due to the strength of the drugs, they are usually not taken for longer than six months. Subsequently, the patient takes less aggressive drugs to maintain the improved state of health. The many side effects of drug treatment are prevented with the help of urine and blood checks and a sensible diet. If standard therapy fails, which happens in about 10% of all patients, new approaches to therapy are available. These include TNF blockers. Mild courses of vasculitis can be treated with less potent drugs if diagnosed early.
Prevention
Currently, no reliable measures are known to prevent vasculitis. However, if vasculitis already exists, it is important to interpret potential recurrences early. Thus, an unfavorable course can be prevented by a quick therapy. The first warning symptoms of vasculitis include rheumatic complaints, night sweats, reddened eyes, and unexplained weight loss or fever.
Aftercare
Because vasculitis is not curable in most cases but can flare up again and again, extensive aftercare measures are necessary for affected patients, depending on the specific course of the disease and the affected vessels. The core of aftercare treatment is the administration of anti-inflammatory drugs such as cortisone. For some time now, so-called biologicals, i.e. modern immunomodulating drugs, have also been playing an increasingly important role. The aim of this medication in aftercare is to maintain the therapeutic success achieved up to that point and to prevent a possible flare-up of the vasculitis. Depending on the course of therapy, other drugs that suppress the patient’s immune system can also be used under close medical supervision. Depending on the type of vessels affected, follow-up care may also be extended to counteract diseases of the heart, for example. In many cases, compression therapy is also used. Affected patients are then instructed to wear compression stockings. This is intended to improve blood flow to the vessels, which can have a multiple positive effect on vasculitis. On the one hand, compression therapy prevents further deposits from forming on the walls of the vessels. On the other hand, compression also has a beneficial effect on the healing process associated with pre-existing inflammatory lesions.
What you can do yourself
Depending on the extent and localization of vasculitis, various therapeutic measures are available. Medical treatment, which always includes the administration of medication, can be supported by various self-help measures. Small vessel vasculitis is treated with immunosuppressants, cytostatics, antibodies and steroids. It is important to note the side effects and interactions in a medication diary and to inform the doctor about the complaints. In case of severe side effects, the treatment must be discontinued or switched to another preparation. In the case of vasculitis of medium-sized and large vessels, vascular wall prostheses may have to be inserted. Patients are advised to take it easy and rest. Strenuous physical activities should be avoided, although regular aqua-jogging or swimming can support healing. In addition, the diet should be changed. An inflammation-reducing diet with vegetable oils, little meat and a protein-rich diet inhibits the spread of internal inflammation. Specific measures apply to special forms of vasculitis. For example, nicotine must be avoided in the case of endangiitis obliterans. It is also important to be informed about risks such as stroke, heart attack or kidney failure. In the event of a medical emergency, the emergency medical services must be called immediately.