Wegener's Granulomatosis, Wegener's Disease FAQ

When all blood and urine tests are normal and you have no overt symptoms, and your physician deems you "in remission". (There are some patients who can only stay in a chemically reduced remission by use of low dose methotrexate or low dose prednisone).

If your symptoms are decreasing and blood, urine, and radiographs are moving toward normal, the meds are working. It is difficult sometimes to tell if symptoms are from WG or from medications side-effects. For that reason, you need to know the usual side-effects (though they differ from patient to patient).

The basics for monitoring disease activity are the Erythrocyte Sedimentation Rate (ESR) and C Reactive Protein (CRP). However, these are measures of inflammation and can be due to viral, bacterial, or parasite infection. The best way to judge disease activity is by the changes in symptoms and clinical exam results.

The cANCA and anti-PR-3 levels may indicate disease activity level, but not always. There's a small percentage of WG patients who at times have normal levels even when the disease is active. Physicians accustomed to
treating WG usually judge disease activity from symptoms, not so much from cANCA or anti-PR-3.

The creatinine level should be monitored during treatment to make sure the kidneys aren't adversely affected.

For more on medical tests, see http://www.wegenersgranulomatosis.net. Go to Section 36 then click on the link to the Medical Tests page.

If you have trace blood and trace protein in your urine, those can be due to any number of conditions other than WG, but in any WG patient that indicates that close monitoring is needed. Any WG patient should
be concerned when blood and protein show in their urine. Traces can be so minimal that they're not visible to the naked eye. Should blood or protein in urine persist or worsen, your physician should be contacted.

WG patients should have their urine monitored periodically during treatment at frequent intervals as your physician deems prudent. Even after remission, the urine should be checked with urine dipsticks for blood and protein bi-weekly. X-rays of lungs can be adequate, however, there are cases where nodules show up on a CT scan that don't show on x-ray.

The prednisone can rapidly reduce inflammation, but the basic disease process of an immune system dysfunction isn't fundamentally affected. It can be normal to feel great at times, but unusually so.

It's a possibility that you are responding the euphoria induced by prednisone that may cause you to be hyperactive.

Generally speaking, in the past, cytoxan would be continued perhaps from 6-12 months after remission.

However, generally the practice these days is to substitute a less harsh immunosuppressive for cytoxan after the major symptoms have been reduced to a level the physician judges safe to make the substitution.

The substitute may be methotrexate, Cellcept (Mycophenolate Mofetil), Sandimmune (Cyclosporin, Neoral, SangCya), Imuran (Azathioprine), Arava (Leflunomide), Etoposide (VP-16, VePesid), or Leukeran (Chlorambucil). The latter two are used more in Europe than in the U.S.

It's probably wise to get copies of all your lab and radiograph reports so that you can track virtually anything. Day to day, the one's to watch are ESR, CRP, and creatinine.

Every WG patient is unique, in symptoms, in responses to medications, and in disease activity. The medications do work, and one can expect the patient to get the disease into remission. Day to day, the medication side effects can be difficult. Sometimes dosages or medications used can be adjusted to work around some undesirable situation.

You need to understand this is a condition that is incurable but almost always controllable. You will have to be followed very carefully during treatment by your physician, and then periodically after in remission for the rest of your life, stretching to perhaps semi-annually. Yet your urine should continue to be checked for blood and protein bi-weekly, using urine dip sticks.

There are no comprehensive books dealing with WG. Other than the WGA web page and patient packet, there are some web pages and on-line medical resources. Take a look at some of the sections of http://pws.chartermi.net/~blader/WG-URLS-V3.html.

You might also download the booklet from the St. James Hospital in Dublin, Ireland. It's in .pdf format so you require Adobe Acrobat to view it. It's at http://www.stjames.ie/ClinicalInformation/. Scroll down to the "Wegener's Granulomatosis Handbook". Click on the link there. Please note that different medications are sometimes used in Europe.

Nothing I know of but I can write you something if you want :-)

This is a little difficult to answer because it can be different for everyone. I'll answer with my own experiences.

This is a form of chemotherapy so expect to feel a little under the weather for a couple of days. My procedure takes about two hours or so and I take the whole day off work. I also usually have it on a Friday so I have the weekend to recover. By Monday I'm starting to feel a bit better but I don't go back to the gym until Thursday.

If you're anything like me you'll also notice an immediate weight gain (I gain about 4 kilos in a day). This is due to the fluid that is administered with the Cyclophosphamide. I lose it within a few days.

Even though it's chemoptherapy, it's nowhere near as big a treatment as cancer patients get.

Yes. Keep a good eye on yourself if you have caught a cold. You don't want it developing into an infection (much like I have at the moment *cough*). If you have lung or sinus involvement then be vigilant. The head-cold I had last week has moved to my chest and (because I had lung involvement) I went directly to my doctor who has given me antibiotics *coughs again*.

My recommendation would be to discuss with your doctor the possibility of getting a flu vaccination.