WEGENER'S GRANULOMATOSIS

Wegener's granulomatosis is an inflammatory condition that affects many tissues in the body. It was originally described as a "flu-like" illness in the first patients in whom this condition was diagnosed, because its principal symptoms are similar to those of a viral illness. The dominant organs involved in the inflammatory process are the lungs, kidneys, eyes, and muscles. The disease ranges in severity from mild pain and weight loss to sudden kidney failure, shortness of breath with pneumonia, or severe eye pain (because of inflammation of those tissues).

There is no known substance or agent that gives rise to this condition. It is classified as an autoimmune disease, which means that the body develops immune proteins (called antibodies) that attack various cells and cause damage. Wegener's granulomatosis is a rare disease, although the exact incidence is unknown. Typically, the patient is older than 40 years at the time of presentation, and women are affected more often than men, although it is not clear why.

There is no tendency for the condition to be passed on to children. It does not have a vector for transmission. Often patients have Wegener's granulomatosis diagnosed because of the "constellation" of symptoms present that raises the doctor's concern: some patients initially have mild kidney failure, fatigue, fevers, and a pneumonia on chest x-ray. In others, Wegener's granulomatosis is more dramatic in its presentation, and affected patients require hospitalization for close care. There is an antibody that can be detected in the blood of most affected patients (called the ANCA antibody). The presence of this antibody has been closely linked to the presence of Wegener's granulomatosis.

With modern treatments, patients do extremely well in disease remission.

Patients with Wegener's granulomatosis may have kidney failure, shortness of breath, or fevers and weight loss. The treatment offered today seems to alleviate most of the symptoms. As with most chronic inflammatory diseases, patients can become debilitated over time, especially if they don't respond to or comply with medical therapy. Depression may occur, and patients who don't take care of themselves can become malnourished.

Your physician will be offering you two principal treatments. All patients require steroid medication in the form of prednisone. In addition, studies have shown a better cure rate with potent medicine called cyclophosphamide (Cytoxan) or azathioprine (Imuran). Trimethoprim-sulfamethoxazole (Bactrim, Septra) may reduce the incidence of relapses of Wegener's granulomatosis.

If you have severe Wegener's granulomatosis, your doctor may admit you to a hospital to treat you with intravenous medications and a special form of blood treatment, called plasmapheresis. This is where the antibodies in the blood are removed quickly, and fresh proteins are administered.

All treatments carry risk of side effects.

Steroids may raise blood pressure; raise blood sugar (induce diabetes in some patients); cause stomach ulcers; cause bruising; increase susceptibility to infection; cause flushing, and with long-term therapy, may cause cataracts or worsen osteoporosis. There are more potential side effects your physician can describe for you.

Cytoxan may cause nausea, hair loss, a lowering of blood cell counts (with more chance of infection), and rarely infertility in women.

• Medications: prednisone is best taken with milk to avoid irritating the stomach. It is very important to take the prescribed steroid course because abrupt cessation can be harmful. Do wear a Medic-Alert bracelet, or some alternative, to let others know you are receiving this important treatment.
• Diet: do eat well and take supplementary calories as necessary, to avoid getting debilitated. Your dietitian can help.
• If your blood sugar goes up with prednisone, your doctor may place you on a type of diet used in diabetic patients. Do stick to the diet to avoid serious complications of high blood sugars.
• Exercise: do stay as active as possible to minimize the time spent recuperating. Patients who are bed bound are slower to recover from the illness.
• Your doctor will need to keep a close eye on you and your blood counts during treatment: it's very important to keep the appointments you set. Patients on these drugs need close supervision.

• Don't take over-the-counter medications unless you receive the doctor's advice to do so. Some medications should be avoided until your body is healed.
• Don't skip doses of prednisone. To do so can be harmful to you.
• Don't cheat on any dietary advice you might receive. Patients with high blood glucose levels (mild diabetes) and kidney problems need to restrict their food intake to avoid serious illness. The provider and his team will let you know of any restrictions you should observe.
• Don't overdo work/exercise to exhaustion. That will drain your reserves further.

• Should you experience any symptoms associated with treatment, or if you cannot tolerate or forget to take the treatment, you should call. Also, you should call if you experience new symptoms that you are concerned about, such as cough, shortness of breath, pain (especially ulcer pains), difficulty swallowing, and sore throat. Finally, if you get a fever or chills during your treatment, call if you have any concerns.

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