Vasculitis deutsche rheuma liga bundesverband e. V

The guidebook brochure "Vasculitis – a rheumatic disease with many facets of the German Rheumatism League explains forms of vasculitis, encourages those affected and gives practical advice.


The disease – what is vasculitis?

Vasculitis (plural: vasculitides) is a generic term for different forms of inflammation of blood vessels. Vasculitides often cause pain in joints or muscles, occasionally joint swelling, and are classified as rheumatic diseases. Some vasculitides are caused by other known diseases, e.g., vasculitis.B. Infections or other rheumatic diseases, such as rheumatoid arthritis. In other forms of vasculitis, a triggering cause is not sufficiently known. These used to be called primary vasculitides.

Vasculitides are rare. Estimates show that in Germany ca. 200.000 people suffer from vasculitis. Depending on the specific form of vasculitis and u.a. depending on which blood vessels are affected (z.B. large-vessel vasculitis or small-vessel vasculitis).

Causes – how vasculitis occurs?

The vascular inflammations result from a faulty immune response. The immune system mistakenly regards the body's own substances as foreign and fights them off. In the episode the blood vessel ignites. This can lead to a disturbance of blood flow and also to a leakage of blood and inflammatory cells into the tie. Depending on the blood vessels affected, this can cause damage to a wide variety of organs.

Why this reaction occurs is not known. Scientists have been able to show that part of the pathogenesis of the disease can be traced back to genetic causes. Other factors, such as presumably environmental influences (viruses, toxins u.a.) are not yet sufficiently known.

Symptoms of vasculitis – what are the warning signs??

Signs of vasculitis can be:

– Complaints in the musculoskeletal system (muscle and joint pain, rarely joint swelling) – Eye redness, especially if it is very painful – Visual disturbances – Headaches – Ear, nose and throat problems (bloody rhinitis or hearing loss) – Numbness (for example in the feet) and paralysis – Complaints in the gastrointestinal tract (bloody diarrhea, abdominal pain) – Skin changes (red spots, nodules, ulcers) – Coughing up blood – Bloody urine

The first general symptoms may be:

Many of the symptoms can also have other causes. However, the physician should become attentive if several of the signs occur in different organs.

Diagnosis – how does the doctor determine the disease?

If vasculitis is suspected, the diagnosis begins with a questioning of the affected person (anamnesis) and a physical examination. If the symptoms have already manifested themselves in the past, this can be an important indication. If the initial suspicion hardens, the next steps follow.

Laboratory tests
With the help of laboratory tests, the physician can clarify whether an inflammation exists in the body. The erythrocyte sedimentation rate (ESR) is accelerated. The level of a certain protein (C-reactive protein) is elevated. However, the examinations do not reveal anything about the type of inflammation. If so-called auto-antibodies are found in the blood, this is also an important indication. Antibodies are proteins that the body produces to fight off foreign bodies. Auto-antibodies also attack the patient's own body in the process. Their existence indicates an auto-immune disease.

The type of auto-antibodies provides information about which auto-immune disease is involved. Typical for some vasculitides are ANCA (=anti-neutrophil cytoplasmic antibodies). However, if autoantibodies are absent, this does not rule out vasculitis. A urine analysis can also provide important information – especially about whether the disease has affected the kidneys.

Imaging procedures
With the help of imaging procedures, the doctor can find out where in the body something has changed. This is important for diagnosis, but also for deciding where a tie sample is useful. In addition, imaging techniques help to assess whether a therapy will be effective. With the help of sonography, magnetic resonance imaging, computer tomography or positron emission tomography, the doctor can detect changes in the head and internal organs. An X-ray can reveal whether the disease has affected the lungs.

A tie examination (biopsy) can reveal whether there are inflammatory changes in the blood vessels. For this purpose, the doctor takes a tie sample – for example from the nose, lung, kidney or from a branch of the temporal artery.

Therapy – what are the options?

Vasculitides are still not curable, but they can be treated well today. Therapy depends on which vessels and organs are affected and how far the disease has progressed.

1. Drug treatment
Glucocorticoids (often simplified as "cortisone") almost always play an important role in the treatment of vasculitis. It quickly inhibits inflammation and slows down the immune system in its attack on its own body. However, this can have side effects, including:

– Weight gain – Skin changes – Eye lens opacity – Increased intraocular prere – High blood prere – Diabetes – Tendency to infections

These side effects can be reduced if patients follow certain rules when taking the medication (therapy rules for cortisone).

For some forms of vasculitis, treatment with "cortisone" may be sufficient (usually for giant cell arteritis, IgA vasculitis (formerly Purpura Schonlein Henoch)); for other forms, additional agents that inhibit the immune system are necessary. One example is the drug cyclophoshamide. It is used when the situation is life- or orhgan-threatening. If the disease is less severe, the active substance methotrexate may be sufficient and help to save cortisone. Once the inflammation has subsided, treatment can be switched to better tolerated agents. Modern protein medications, the so-called "rheumatism drugs", are also used. Biologics, are increasingly used in the treatment of vasculitis. Examples include tocilizumab for giant cell arteritis. Rituximab in ANCA-associated vasculitides.

To avoid relapses, patients usually have to take these drugs for years, often with a small dose of cortisone in addition.

2. Non-drug treatment
All vasculitis medications have side effects. But there is no alternative to them. Patients who forgo conventional medical treatment risk their lives. Therefore, in any case, it is important for patients to ask their doctor before starting any complementary healing procedure. Given the rarity of vasculitides, patient education is also particularly important.

Exercise also makes it easier to cope with the disease. Physiotherapy and functional training keep painful joints and muscles mobile. In addition, exercise is important for the inner balance.

Relaxation exercises can also contribute to a balanced basic attitude. They can also reduce pain. Effective relaxation techniques include autogenic training or Jacobson progressive muscle relaxation. The associations of the German Rheumatism League offer various exercise and relaxation courses.

Psychological help
A chronic disease, such as vasculitis, not only puts a strain on the body, but also on the psyche. The constant fatigue, the drop in performance, and the fear of the future is a cause of concern for many patients. In addition, the drugs often have a negative effect on mood. In these cases a psychologist can help. It is often easier to talk about these problems with an outsider than with friends or relatives. In addition, the professional view makes it easier to deal with the problems. And psychologists do not only bring understanding to the patients. They also know how to solve the problems. It is important to clarify before treatment whether the health insurance company will cover the costs of psychological therapy.

3. Operation
In rare cases, larger vessels can be dilated with a stent (tube) if they are occluded as a result of vasculitis. Widening of the arteries (aneurysms) also occurs. Can also be treated surgically. In addition, surgeries to repair secondary damage, z.T.

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