Wegener's granulomatosis is an uncommon disease characterized by inflammation of the blood vessels, or vasculitis (pronounced vas-cue-lite-us) and a type of damaging inflammation of the tissues referred to as granulomatous. Vasculitis can also damage important organs of the body by limiting blood flow to those organs and destroying normal tissue.
 
Although the disease can involve any organ system, it mainly affects the respiratory tract (including the sinuses, nose, windpipe, lungs) and kidneys.

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. Almost always these include symptoms related to the upper respiratory tract. Symptoms can include sinus pain, discoloured or bloody fluid from the nose, blocked ears and, occasionally, open sores in the nostrils. 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.
 
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. 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 cause of 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 experience a return of their symptoms. This occurs most 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, both while they are taking medication and after their medication has been stopped.

Medicine

In most cases Wegener’s granulomatosis is treated with medication, consisting of a combination of prednisone and a cytotoxic medicine (like cyclophosphamide).
 
Prednisone
Prednisone is similar to cortisol, a hormone naturally produced by the human body. As symptoms improve, the prednisone dose is gradually decreased until it can be discontinued completely, usually within six to 12 months.
 
When prednisone is taken the body stops making its own natural cortisol, and as the prednisone dose is gradually reduced, the body resumes making cortisol. It is extremely important that prednisone never be stopped suddenly because the body requires prednisone (or cortisol) for normal functioning and may not be able to immediately make the cortisol it needs.
 
Prednisone can affect the body's ability to fight infection. People taking this medicine should immediately report any symptoms of infection, and especially any fever, to their doctors. Prednisone can cause weight gain, cataracts, brittle bones, diabetes, and changes in mood and personality.
 
Cyclophosphamide
Cyclophosphamide (Procytox) is the most commonly used cytotoxic drug used to treat Wegener's granulomatosis. The drug is taken by mouth once daily in the morning followed by drinking a large amount of liquid.  Cyclophosphamide is generally given until the disease goes into remission. Patients are then often switched to another medicine such as methotrexate or azathioprine .
 
Cyclophosphamide is a powerful drug that works by suppressing the immune system. People taking the drug must be monitored carefully and have frequent blood tests. Cyclophosphamide can cause an increased risk of infection, lowering of blood counts, sterility, bleeding from the bladder (hemorrhagic cystitis), bladder cancer, and other serious side effects.
 
Methotrexate
Methotrexate has been used in combination with prednisone to bring about remission for people with active but not severe cases of Wegener's granulomatosis. It is also used to maintain remission after a person has initially received cyclophosphamide. Methotrexate is usually given for one to two years, after which time if people stay in remission, it is decreased and stopped.
 
Methotrexate is given once a week usually by mouth, but occasionally as an injection under the skin or in the muscle. People taking methotrexate require regular blood work to monitor their response and to watch for side effects.
 
The side effects of methotrexate include infection, lowering of the blood counts, nausea, soreness and ulceration of the mouth lining, irritation of the lungs (pneumonitis), and inflammation and scarring of the liver. People taking methotrexate cannot drink alcohol, and the drug is not given to people with poor kidney function or to people with underlying liver disease such as hepatitis.
 
Azathioprine
Azathioprine (also called Imuran) is used primarily to maintain remission in people who have initially been treated and gone into remission with cyclophosphamide. It is taken once a day by mouth. Similar to methotrexate, it is usually given for one to two years after which time the dosage is lowered until it is stopped.
 
The side effects of azathioprine include infection and lowering of blood counts. Unlike methotrexate, azathioprine is safe for use by people with poor kidney function or liver disease.

A Word about Medication Safety
The need to effectively monitor new drugs once they have been approved and introduced into the market has been a key advocacy issue for The Arthritis Society for several years. This advocacy helps to ensure that unfavorable side effects are reported, documented, and addressed. For regular updates on medications available in Canada, visit www.arthritis.ca/tips/medications.

All medications have potential side effects whether they are taken by themselves or in combination with other herbal, over-the-counter and prescription medications. It is therefore important for patients to discuss the benefits and potential side effects of all their medications with their doctor.

Health Canada’s Marketed Health Products Directorate (MHPD) has recently developed a new website, named MedEffect. MedEffect’s goal is to provide centralized access to new safety information about health products in an easy to find, easy to remember location. It also aims to make it as simple and efficient as possible for health professionals and consumers to complete and submit adverse reaction reports. Finally, it helps to build awareness about the importance of submitting adverse reaction reports to identify and communicate potential risks associated with certain drugs or health products. To find out more, visit: www.healthcanada.gc.ca/medeffect or call toll-free 1-866-234-2345.

Along with the physical symptoms of Wegener’s granulomatosis, many people experience feelings of helplessness and depression. Learning daily living strategies to manage your disease gives you a greater feeling of control and a more positive outlook. To get the best results, people affected by Wegener’s granulomatosis need to form close ties with their doctors and therapists, and become full partners in their treatment. From our perspective, it's all part of 'living well with your disease.' There are several resources you can use to find out how best to manage your own condition. Here are a few:

• The Arthritis Self-Management Program (ASMP) is a unique self-help program offered by The Arthritis Society to help you better control and manage your arthritis.
• The Open Forum within this website is an opportunity to discuss and share information with other visitors - people who, through their own experiences, may be able to offer some useful insights.

Of course, there are many other valuable resources for people with arthritis. If you're unclear about where to look for help, be sure to call The Arthritis Society at 1-800-321-1433.