Wegener’s granulomatosis is relatively rare and strikes men and women equally. Wegener’s typically occurs in middle age, but is found in people of all ages. Although it very rarely affects children, Wegener’s can occur in people at 70 or 80 years of age.

The first signs of Wegener’s granulomatosis are often vague and can include fever, loss of appetite, loss of weight and fatigue. Almost always there are symptoms related to the upper respiratory tract which includes: sinus pain, discoloured or bloody fluid from the nose, blocked ears and, occasionally, open sores in the mouth, nose or on the face. A common sign of the disease is an almost constantly running nose or other cold symptoms that do not respond to normal treatment or that become increasingly worse.

The lungs are affected in most people with Wegener’s granulomatosis. Symptoms can include a cough with or without blood, shortness of breath, and chest discomfort.

Kidney problems can present as red, brown or foamy urine, high blood pressure or swelling in the feet and ankles.

Muscle and joint pain or, occasionally, joint swelling affects two-thirds of all people with Wegener’s granulomatosis. Although joint pain can be very uncomfortable, it does not lead to permanent joint damage or deformities.

Nearly half of all people with Wegener’s granulomatosis develop skin lesions. These often appear as small red or purple raised areas or blister-like ulcers or nodules that may or may not be painful.

Up to 60 percent of people with the disease will experience eye problems. These can include pain, redness, altered vision and protrusion of the eyeball.

The cause of Wegener’s granulomatosis is not known. Wegener’s is an auto-immune disease with the body’s own immune system attacking the blood vessels and other tissues. Genetics appear to play a relatively small role, although it is very unusual for Wegener’s to occur twice in the same family. It is thought that infection may contribute as a trigger for Wegener’s, but no specific infection has yet been identified.

With appropriate treatment, the outlook is good for people with Wegener’s granulomatosis, and it is not unusual for people with Wegener’s to see their symptoms disappear completely. However, about half of all people with the disease may require long term use of medications to keep the disease in remission. The disease may flare frequently within two years of stopping medication but can also occur during treatment. For this reason it is very important that people with Wegener’s continue to see their doctors regularly, even if they have felt well for several years.

Medicine

In most cases Wegener’s granulomatosis is treated with medication, consisting of a combination of prednisone and a cytotoxic medicine (like cyclophosphamide, Methotrexate, Azathioprine or rituximab).