WEGENER'S GRANULOMATOSIS

What is Wegener’s granulomatosis?

Its cause is unknown but it's a multi-system disease, which means many parts of the body may be affected. 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.

Around 50 per cent of people with the disease develop arthritis.

Symptoms

As Wegener's affects so many parts of the body, it can cause a host of symptoms.

The first symptom is often a runny nose, caused by inflammation of the nasal passages (rhinitis). However, it's important to remember that this symptom is still most likely to be the sign of a cold or allergy as these are very common, and Wegener's is very rare.

Recurring episodes of sinusitis may occur, as can chronic ear infections. Many people experience general malaise and weakness, unexplained fever and night sweats, weight loss and poor appetite, joint and muscle pains.

The eyes are often affected, resulting in conjunctivitis and swelling. When the kidneys are involved, blood may appear in the urine as a consequence of glomerulonephritis.

If the lungs are affected, there may be coughing (sometimes with blood), wheezing and shortness of breath.

Serious complications can occur, especially if the condition is not treated. The major concern is kidney failure which, if left untreated, results in death.

Chronic inflammation of the eyes (conjunctivitis), perforation of the nasal septum causing a hole to develop in between the nasal passages, and respiratory failure are possible problems if treatment isn't adequate.

The diagnosis is made by the presence of a combination of:
•oral ulcers or nasal discharge
•chest x-ray changes such as nodules and cavities
•sediment in the urine
•blood vessel biopsies

Causes and risk factors

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.

Wegener's granulomatosis can affect anyone of any age, but it's more common in people between the ages of 30 and 50 and is rare in other age groups. Up to 90 per cent of people with Wegener's are Caucasian.

Treatment and recovery

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.

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