Wegener’s is a rare disease that results in inflammation of blood vessels (vasculitis).
Its cause is unknown but it can affect many parts of the body. The nose, throat, lungs and ears usually become swollen and inflamed. The kidneys can be involved, too, while for some people it affects the eyes and skin.
Serious complications can occur, especially if the condition is not treated.
The precise cause is unknown, but it’s thought to be an autoimmune disease, where the body’s immune system turns on itself.
As with many autoimmune diseases, the condition probably develops when someone who is genetically predisposed to it comes into contact with a specific trigger, such as a bacterial or viral infection.
Unfortunately, because of the lack of understanding about the disease it isn’t possible to prevent it from occurring.
Without treatment, Wegener’s can kill within months. With treatment, people can recover, with the disease going into remission or at least kept in check This is achieved with the use of immunosuppressant drugs, such as cyclophosphamide, and corticosteroids to control inflammation. Plasma exchange may be used in severe disease.
However, even with treatment some people still develop kidney failure, which is why it’s important that a person with Wegener’s granulomatosis sees a specialist regularly.
Treatment needs to continue for a long time – the exact period depends on the individual patient. Some people have relapses, but in most cases treatment can bring the condition under control again.