Vasculitis

Introduction

Vasculitis is the inflammation of blood vessels. This can affect all blood vessels in the body. Arteries, veins and the very small capillaries.

The term vasculitis is a generic term and includes various diseases which may have different courses, but all belong to the autoimmune diseases. In an autoimmune disease, the body itself forms antibodies against the body’s own structures and thus destroys them. There are two forms of vasculitis, the primary and the secondary. Primary vasculitis is a disease in its own right, which is due to autoimmunological processes. Secondary vasculitis is triggered by the intake of drugs, by infections or by certain autoimmune diseases.

Causes

Vasculitis can be caused by a variety of factors. However, every vasculitis is a reaction of the immune system. Primary vasculitis is an independent autoimmune disease with an inflammatory rheumatic cause.

However, the exact causes are unclear. Secondary vasculitis is caused by other factors. Underlying autoimmune diseases can lead to vascular inflammation.

These include rheumatoid arthritis, Crohn’s disease and systemic lupus erythematosus. Infections in the body caused by viruses or bacteria can also be the cause of vasculitis. Streptococcus infections, hepatitis, HIV or borreliosis are possible triggers.

Malignant tumors can also cause vasculitis. In addition, the intake of certain drugs can cause inflammation of the blood vessels as a possible side effect. This can be the case with antibiotics, cytostatics and so-called ACE inhibitors, for example.

The body’s own antibodies can form complexes with drugs, viruses or bacteria that are deposited in the walls of the blood vessels. This leads to damage to the walls of the blood vessels and, as a result, to inflammation. As an inflammatory reaction, the walls swell and lead to a narrowing of the blood vessels. This hinders the blood flow.

Symptoms

The inflammation of the blood vessel walls and the resulting narrowing lead to a disturbed blood flow in the body. It is usually arterial vessels that are affected by vasculitis. The organs located behind the affected vessels are then less well supplied with blood.

This means that the oxygen and nutrient supply is also reduced. Depending on which vessels are affected, the symptoms vary. In most cases, however, vasculitis initially manifests itself through general symptoms such as fatigue, fever, weight loss and often night sweats.

Many patients also report joint pain. The further symptomatology depends on which organs are damaged by the vasculitis. Often the skin is affected or at least also affected.

This becomes noticeable by redness and sometimes also by open spots. Involvement of the eye can be shown by visual disturbances and redness. If the heart is undersupplied, this is manifested by chest pain and possibly by inflammation of the heart muscle or pericardium.

Breathing difficulties and haemoptysis are indications of lung involvement, while high blood pressure and blood in the urine indicate kidney damage. If the nervous system is affected, seizures, strokes, headaches or paralysis may occur. There are different forms of vasculitis and depending on which form it is, mainly certain organ systems are affected.

In vasculitis of the skin (cutaneous vasculitis), the blood vessels of the skin are inflamed. In this case, the small blood vessels in particular show deposits in the vessel walls. In most cases, vasculitis of the skin vessels does not occur alone and as an independent disease, but is caused by other influences or diseases.

A frequent cause is the intake of medication that leads to vasculitis. This can be the case, for example, by taking antibiotics. Bacterial infections or vascular inflammation in other areas can also be triggers for cutaneous vasculitis.

The inflammation of the skin vessels is often manifested by a skin rash, which can appear in various ways. Red spots, blisters, lumps or a flat rash are possible conspicuous features. In addition, pain or itching may occur.The diagnosis can be confirmed by taking a tissue sample.

In addition, a blood test provides information on how far the inflammation has progressed in the body. Depending on the underlying cause, skin vasculitis is treated in different ways. If bacterial pathogens are the cause, antibiotic therapy is initiated.

For other causes, therapy with anti-inflammatory drugs such as cortisone is recommended. In pronounced cases, treatment with immunosuppressive drugs is used. Vasculitis often affects the larger and smaller vessels of the legs.

The various types of vasculitis can manifest themselves differently. Purpura Schönlein Henoch often manifests itself by small bleedings (petechiae) on the legs and feet. It mainly affects the small vessels.

It can also lead to joint problems in this area. Some patients also suffer from oedema (water retention) in the feet and legs. Endangiitis obliterans affects the small and medium-sized vessels of the legs.

Young men (under 40) are particularly affected. Heavy smokers are also more frequently affected. The disease manifests itself mainly through pain and a feeling of cold in the legs – especially at rest.

Microscopic panarteritis also manifests itself on the legs. Small nodules and palpable bleeding under the skin are typical. The legs are particularly affected.

In cutaneous leukocytoclastic angiitis, the legs are also most frequently affected. Punctiform redness is observed, which does not disappear when pressure is applied with the finger (purpura). In addition, blisters filled with blood and painful nodules in the skin can occur.

If the vasculitis affects the eye, this is mainly noticeable by a reddening of the affected eye. Visual disturbances can also occur. The eye is also affected within the scope of various basic diseases.

Especially when vasculitis affects smaller vessels in the body, the eye is often affected. Vasculitis of the brain can affect the vessels of the brain and spinal cord. A distinction is also made between primary and secondary vasculitis in the brain.

Symptomatically, vasculitis in the brain can manifest itself through many different symptoms affecting the central nervous system (CNS). Typical for this disease is the combined occurrence of central symptoms and other physical manifestations. These often include headaches, confusion or paralysis that occur on one side of the body.

Since vasculitis, just like a stroke, leads to a reduced supply of certain areas of the brain, the symptoms of the two diseases are similar. The exact diagnosis of brain vasculitis is difficult, as the symptoms are very unspecific. They can also be similar to those of a brain tumor, meningitis or damage to the spinal cord.

To make the diagnosis, cerebrospinal fluid (cerebrospinal fluid diagnostics) is usually taken. Here, increased inflammation values indicate the presence of a vascular inflammation and other diseases of the brain can be excluded. With the help of MRI images of the head, inflammation can be detected.

To confirm the diagnosis, it may be necessary to take samples of the brain or meninges (biopsy) in order to exclude an infection by bacteria or viruses. Vasculitis of the brain is treated in the same way as other vascular inflammations by the administration of anti-inflammatory or, in more severe cases, immunosuppressive drugs. In vasculitis, an inflammatory change in the vessels can damage the surrounding nerves.

This can lead to sensations, tingling and pain. The sensation of touch and temperature can also be altered. Frequently, an existing vasculitic neuropathy improves only moderately under vasculitis therapy.