The treatment of Wegener's Disease typically begins with an attempt to bring the inflammation under control in order to prevent organ damage. In moderate or severe cases this will usually be through corticosteroids (steroids) such as Prednisone or prednisolone plus cyclophosphamide, a cancer drug which aims to weaken your immune system. This aggressive intervention can literally be life-saving. The cyclophosphamide will usually be given intravenously every two to four weeks, but sometimes comes in the form of a tablet, while the steroids are prescribed alongside this and are taken orally. The dose of steroids is typically reduced after a period of six months in order to reduce the long term side effects.
If the Wegener's begins to respond to treatment then you may be given milder drugs which suppress your immune system such as methotrexate, mycophenolate mofetil or azathioprine. The aim of these drugs is to keep you in remission for as long as possible. They do not have the potential long term side effects of steroids.
Prednisolone and methotrexate may be given alone if the Wegener's is relatively mild, where there is no major organ damage - kidney involvement for instance.
In very severe Wegener's large doses of methylprednisolone or plasmapheresis (plasma exchange) may also be given.
Antibiotic medication, such as Co-Trimoxazole (Septrin), may be administered to reduce the risk of chest and sinus infections.
Use of these drugs cause up to 90% of all Wegener's sufferers to go into remission. However, the GPA re-occurs in over 60% of sufferers, so medication is generally required for a considerable length of time.
Once the medication takes effect surgery may be required to deal with tightening of the airways (fibrosis), nose deformity, obstruction of the tear ducts, ear problems or even a kidney transplant.
Newer, experimental drugs are being developed all of the time. One such drug which has been successful for a number of Wegener's Disease sufferers is Rituximab.
Drugs and Side effects
Steroids (Prednisolone, Methylprednisolone, Cortisone, Prednisone, Dexamethasone)
Steroids are hormones which occur naturally in the body and are essential for everyday health. Prednisone is a glucocorticoid steroid which is chemically different from the anabolic steroids which some athletes use to boost performance. Steroids are produced naturally by the adrenal glands, and artificial steroids are very good at reducing inflammation and are therefore used to treat vasculitis and other inflammatory diseases. They are often introduced at very high doses to get the disease under control and then reduced sharply to avoid the side effects which are unfortunately very common with steroids. Steroids are normally taken orally.
The possible side effects of steroids are:
- Increased appetite which can lead to weight gain
- Indigestion and stomach ulcers
- Fluid retention
- Disturbed sleep
- Thinning skin which is easily bruised
- Increased hair growth
- Muscle weakness
- High blood pressure
- Weakening of your bones (osteoporosis)
- Stretch marks and a 'moonface' (Cushing's syndrome)
- Injuries and wounds can take longer to heal
- Children can experience reduced growth
- Increased risk of infection, particularly in relation to chickenpox and measles
- Altered moods, such as feeling anxious, depressed, confused or manic
- Rapid mood swings, you may be up one minute and down the next
- Increased aggression and irritability
Taking artificial steroids can disrupt the body's own production of steroids so it is essentially to not suddenly stop taking steroids. The dose must be reduced over a period of time. Doctors will monitor you closely for signs of diabetes. You may be given drugs to take alongside the steroids to mitigate their effects. These may include bisphosphonates, calcium or vitamin D to prevent osteoporosis, and Ranitidine, Lansoprazole or Omeprazole to protect your stomach from side effects.
Cyclophosphamide is usually thought of as an anti-cancer drug, given as part of chemotherapy, but it is also widely used to treat severe cases of vasculitis. In the past cyclophosphamide was used for up to a year until the disease went into remission and then it's use was reduced and eventually stopped. More recently it isn't used at all in some Wegener's sufferers or only given to those with the most serious manifestation of the disease. It is then phased out as quickly as possible in favour of other, less toxic drugs.
Side effects may include:
- Bladder irritation (cystitis)
- Bone Marrow suppression leading to a low white cell count and infection
- Nausea and vomiting
- Hair loss
Long term use of cyclophosphamide can cause infertility in both men and women. It has also been linked to an increase in cancer of the bladder.
Methotrexate and Azathioprine (Imuran)
Both Azathioprine and Methotrexate are widely used immunosuppressants where major side effects are unusual. Like cyclophosphamide there is a risk of bone marrow suppression leading to a low white blood cell count and anaemia. There can also be some lung damage and liver toxicity. More minor side effects are stomach upsets, indigestion, mouth ulcers, diarrhoea, fatigue, headaches, chills, increased risk of infection and altered moods.
Methotrexate is usually taken once per week by mouth, but it can also be administered by injection. Those with underlying kidney or liver problems will generally not be given Methotrexate. You should not drink alcohol while taking this drug. You may be asked to take folic acid when taking Methotrexate.
Azathioprine can be taken by those with liver and kidney issues.
Rituximab has been used very successfully instead of cyclophosphamide in some sufferers more recently, though it is more often used in treating Rheumatoid Arthritis, Lupus and cancers such as lymphoma. Ritixumab selectively reduces a particular type of white blood cell (B-cells) that drive the inflammatory process. The main side effect is that the immune system can be seriously hindered and infections are therefore a greater risk. Rituximab is such a new medicine being used to treat Wegener's that the long term side effects are unknown and are currently being researched.
Image courtesy of e-Magine Art through Creative Commons.