Question: I have been diagosned with Wegener's. Could you please recommend treatment for this medical condition?
Dr. Hibberd's Answer:
Wegener's granulomatosis is an uncommon disease of the immune system. It can be a challenge to recognize, and is diagnosed by tissue examination in conjunction with X-ray and blood and urine analysis with an ANCA (anti-neutrophil cytoplasmic antibody ... an abnormal protein belonging to our immune tissue and called an abnormal immunoglobulin or autoantibody) titre that seems to correlate with disease activity.
Wegener's is an autoimmune disorder where the immune system attacks its own body tissues. An inflammation of blood vessels (called a vasculitis) starts a cascade of tissue destruction and the formation of clusters of nonfunctional tissue called granulomas. It frequently involves lung, sinuses, eyes, ears, nose, kidneys, skin, and other organs. Its treatment depends upon its stage and severity.
Often, cancer drugs, such as cyclophoshamide and methotrexate; immune suppressants, such as Imuran and prednisone; and modulators of immune function, such as Cellcept, are used under close supervision by an oncologist or immunologist. Generally speaking, induction of remission is initiated by chemotherapy under the supervision of a rheumatologist/hematologist/oncologist or an immunologist.
Unfortunately, treatment is not curative, but this disease can be induced into remission that eliminates all signs of disease.
If the disease and its flare-ups can be treated early enough, remission can be maintained by medications and close supervision for periods of 20 or more years in some patients.
Not all patients require drugs during their remission periods, but it is essential to treat flare-ups aggressively. Not bad for a disease that had a first-year 82 percent mortality 50 years ago, and where most patients could expect to live five months.
After the disease is in remission, many patients live normal lives. Unfortunately, once induced into remission, over 50 percent of its victims suffer periodic flare-ups. Treat these flare-ups aggressively and the outlook is good.
The main complications of treatment concern the toxicity of the chemotherapy, so close monitoring by your doctors is strongly advised.
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