Vasculitis

Vasculitis definition

Vasculitis refers to the inflammation of blood vessels that is triggered by an immune reaction. In other words, your body's own immune system attacks the blood vessels.

This can result in circulation problems and consequently organ damage or failure. Vasculitis is generally a very rare disease, and different types can occur at any age.

Cause of vasculitis

Vasculitis is classified into primary and secondary according to its origin.

A primary form indicates that the disease originates in the blood vessel. In vasculitis, this is usually due to the immune system. Like many autoimmune diseases, the exact cause of vasculitis is not known. In medical terminology, this seemingly spontaneous development is called “idiopathic”. However, certain triggers are associated with vasculitides, such as nicotine consumption.

Secondary vasculitides result from another disease (such as hepatitis B, C, tumors) or they occur as a side effect of medications, toxins, and drugs.

In addition, vasculitis is classified according to the size (large, medium, small) and type (arteries, veins, capillaries) of the affected vessels. Lastly, it is also classified by the presence of ANCA antibodies.

ANCA antibodies are produced by the body against its own leukocytes. Their presence is associated with many forms of vasculitis and with other autoimmune diseases. While it is not known how the antibodies are formed, it is suspected that both environmental and genetic factors can contribute to the development of the disease.

Vasculitis symptoms

The symptoms of vasculitis can vary greatly depending on the type of vasculitis and the region in which it occurs. As vasculitis is a rare disease, the symptoms are often mistakenly thought to be caused by other diseases.

One symptom that is easily recognizable is cutaneous vasculitis, characterized by pin-point redness that may merge.

Symptoms such as coughing up blood, narrowing of the airways, prolonged ear and sinus infections, nerve disorders, and chronic kidney diseases are difficult to attribute. While more common causes are suspected of these symptoms, vasculitis must also be considered. Testing for antibodies can provide more insight.

Vasculitis therapy

Treatment of autoimmune diseases involves immunosuppression (medication that lowers the immune system) to stop or delay the progression of the disease. This involves the use of corticosteroids, which are very effective in fighting the signs of inflammation but have significant side effects.

The dose of corticosteroids can be reduced by adding methotrexate. Methotrexate suppresses the immune system by damaging the cells (a cytotoxic effect). Sometimes vascular constrictions can also be surgically removed, e.g. with a stent.

Vasculitis risks

If the arteries are inflamed (arteritis), the inflammation can weaken the vessel wall which may promote the formation of an aneurysm (bulging of the vessel wall). In this case, there is an increased risk of bleeding in that location.

The swelling caused by the inflammation can also restrict blood flow. There may be either reduced blood supply (ischemia) to the affected organ or in the event of vessel occlusion, this can ultimately lead to an infarction, where the affected tissue dies due to lack of oxygen.