Wegeners Granulomatosis
What is Wegener's Granulomatosis?
Wegener's granulomatosis (WG) is an uncommon disease of unknown cause. It can occur at any age, including children but is most common in elderly patients. Both men and women are affected equally. It is an auto-immune condition, where the body reacts against itself.
WG is characterised by inflammation of the small blood vessels (vasculitis) including the capillaries, arterioles and venules. Inflammation 'can lead to damage to vital organs, such as the kidneys and lungs. Wegener's granulomatosis is similar to microscopic polyangiitis but differs by the presence of granulomas, collections of inflammatory (white) blood cells near affected vessels, in the upper airways these granulomas may present as ulcers.
Symptoms associated with Wegener's Granulomatosis
The symptoms of WG vary and may include fever, tiredness, and loss of appetite and weight loss.
Most patients first notice symptoms affecting the respiratory tract. These include a runny nose with nasal crusting and sores, nosebleeds and facial pain due to inflammation in the nose and sinuses. Deafness or earache due to inflammation in the middle ear. Cough or shortness of breath and occasionally production of blood stained sputum due to inflammation in the lungs.
Other symptoms can include muscle and joint pains, skin rashes and eye involvement with redness and pain. The kidneys are often involved by inflammation but this does not generally cause symptoms until the kidney function has deteriorated, with a build up of poisonous substances in the bloodstream. Symptoms of ankle swelling, reduced urine production and shortness of breath may then occur.
Making a diagnosis
For the most effective and successful treatment diagnosis should be made early. WG often presents in a similar way to other disorders and can be difficult to diagnose. Your doctor wi 11 make a diagnosis from your complaints and by doing further blood tests.
WG is associated with an antibody called ANCA, which is directed against white blood cells (called neutrophils). Its presence supports a diagnosis of WG but does not itself prove the diagnosis. Other tests will include an assessment of your blood and protein and blood tests for measuring kidney function (urea and creatinine). A kidney biopsy, where a small piece of kidney is taken to look under the microscope, will probably be performed to assess inflammation in the kidneys. Other blood tests will be performed to assess the disease activity and include ESR and CRP. A chest X-ray will be performed to look for lung involvement.
Treatment
Steroids (prednisolone) and Cyclophosphamide are usually used in the early stages of treatment. Steroids are initially given at high dose and reduced as the inflammation improves. (These are not the same as the anabolic steroids that some athletes use). Once the disease becomes quiet (enters remission) the Cyclophosphamide is usually stopped and replaced with azathioprine. Both these drugs are immunosuppressants (suppress the white blood cells causing inflammation). Another newer immunosuppressant, mycophenolate mofetil, is being used more often, particularly if azathioprine does not suit someone. It is also likely that drugs which knock-out the white cells (B cells) that help make ANCA, will become more commonly used.
Does the treatment have side-effects?
Both steroids and the immunosuppressants reduce inflammation by suppressing the activity of the white blood cells. However by doing this, these white blood cells are less able to fight infection. The immunosuppressants can also suppress the production of cells from the bone marrow. Blood tests wi 11 be performed to mon itor closely the white blood count and haemoglobin (to detect anaemia). If you have signs of a fever or infection you must seek medical attention.
Steroids can cause or aggravate diabetes, thinning of the bones, high blood pressure, weight gain and bruising of the skin. The dose of steroids will be reduced as quickly as possible to reduce side effects.
Cyclophosphamide can cause inflammation of the bladder and infertility if used over a long period of time.
Can WG relapse?
Unfortunately about half of all patients will relapse in the months or years following the initial illness, which is why your doctor may continue your medication following disease remission despite no evidence of the disease activity and continue to see you in the clinic on a regular basis.