Wegener's Disease and Vasculitis
What is vasculitis?
Vasculitis is a group of auto-immune diseases where the body’s immune system attacks it’s own blood vessels instead of defending them against infection. This causes an inflammation of the blood vessels. Because blood vessels run to every part of the body all organs can be affected, although the most common forms of vasculitis affect the skin, kidneys, lungs and joints. Typical signs of inflammation are redness of the skin, swelling or feeling of warmth or pain of the affected area.
There are different types of blood vessels in the body and each different type of vasculitis will affect each type of blood vessel in a different way. The main blood vessels that carry blood from the heart to the major organs are called arteries. Inside the organs, these then branch out into smaller arteries called arterioles and then branch out again into even smaller blood vessels called capillaries. The blood carries oxygen and nutrients to the organ and, once those supplies are used, travels back to the heart to be pumped around the body once again.
Different forms of vasculitis affect different sized blood vessels. Takayasu’s arteritis and giant cell or temporal arteritis, for example, may target the largest blood vessels, the aorta and the biggest arteries. Polyarteritis nodosa and Kawasaki’s disease may target the medium sized blood vessels as they transition into the organs. The majority of other types of vasculitis target the small blood vessels and this includes Wegener’s Granulomatosis.
The vascular diseases which target the small blood vessels are often divided into the diseases where anti-neutrophil cytoplasm antibodies (ANCA) are found in the blood and diseases where ANCA is not found. Wegener’s Disease is an ANCA associated vascular disease.
Although the specific symptoms of each type of vasculitis differ depending upon the disease, there are symptoms which are common across most types of vasculitis. These general symptoms include fatigue, weakness, loss of appetite, weight loss and fever.
Many of the vascular diseases which target the small blood vessels can cause kidney damage. This can manifest as loss of appetite, fatigue, and swollen legs and breathlessness due to fluid retention. In the worst cases toxins can accumulate in the body leading to nausea, confusion and epileptic fits.
A simple ‘dipstick’ urine test for blood and protein will confirm whether there is inflammation of the kidneys. If the kidneys are inflamed and go untreated it can lead to permanent damage and the need for dialysis or even transplant.
A firm diagnosis of vascular disease generally follows a biopsy of the affected organ such as the kidneys, skin, lungs or brain.
There are generally two stages to dealing with any type of vascular disease. The first is the initial treatment which aims to control and limit the active phase of the disease. Once the disease is under control (inactive) the second stage is maintaining remission and trying to prevent a relapse. These relapses are usually referred to as flares by vasculitis sufferers.
The drugs used in the first stage are usually of two types; low level chemotherapy drugs such as cyclophosmamide are usually employed alongside a co-corticosteroid such as prednisone. In the second stage low dose co-corticosteroids tend to be administered. The purpose of the drugs is to suppress the immune system and stop the body from attacking itself.
The causes of vasculitis are unknown but they are thought to have a genetic component with an environmental component triggering the disease. Vasculitis is not contagious.
The majority of vascular diseases cannot be cured, they can only be controlled.
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