Wegener's Disease, also known as Wegener's Granulomatosis or Granulomatosis with Polyangiitis, is a rare disease in which the blood vessels become swollen and can leak leading to poor blood flow. It is a form of vasculitis. The inflammation generally attacks the sinuses, nose, ears, lungs, kidneys but can also affect the skin, brain, heart, bowel and nerves. The symptoms include a constant fever, sinusitis and night sweats. Wegener's can lead to tissue damage and organ failure and if left untreated, it can be fatal.
According to the National Health Service website around 500 people each year are diagnosed with Wegener's in the UK alone. It is estimated to affect 3 out of every 100,000 people globally. According to my rusty maths that means over 200,000 people are suffering with Wegener's right now. Men and woman are equally at risk, but the disease rarely attacks children. The most common age to be diagnosed with Wegener's is in your forties or fifties.
What exactly is Wegener's disease?
Wegener's Disease is a form of vasculitis - a group of diseases characterised by inflammation of the blood vessels. Vasculitis is a disorder that destroys blood vessels by inflammation and arteries and veins can be affected. In Wegener's the inflammation is usually restricted to the small and medium sized blood vessels.
Organ damage can occur as a result of this granulomatous inflammation. A granuloma is an imprecise term for a group of inflammatory cells. They form when the immune system attempts to wall off substances, such as bacteria and fungi, that they recognise as foreign but cannot entirely eliminate. Granulomatous means characterised by granulomas. Many other diseases involve this type of inflammation including Tuberculosis, Leprosy, Sarcoidosis and Crohn's disease.
In 1936 Freidrich Wegener recognised the disease as a unique clinical syndrome and this is where the term Wegener's Granulomatosis comes from. Following the discovery of Wegener's Nazi past the name of the disease has been officially changed to a more descriptive name: Granulomatosis with Polyangiitis (GPA). Polyangiitis simply means the inflammation of multiple blood vessels.
The exact cause of Wegener's is unknown, but what seems to happen is that the body's immune system, the system of biological processes designed to fight off disease, malfunctions. The immune system sends out antibodies, protein molecules which have the ability to merge with bacteria and viruses, and thereby neutralise them. In Wegener's, these abnormal ANCAs (Anti-Neutrophil Cytoplasmic Auto-antibodies) attack the cytoplasm, the inside of certain white blood cells called neutrophils. This in turn causes the white blood cells to attack the walls of small blood vessels in different cells, tissues and organs of the body. This attack by the altered white blood cells upon the vessel walls causes swelling and the symptoms of Wegener's.
The particular type of inflammation is very distinctive when tissues are examined under a microscope, as the white blood cells arrange themselves in a certain way called a granuloma.
Image provided by Pulmonary Pathology under Creative Commons Licence.