Last chance before we start Rituxan treatment

[TheMitchells]

Ok everyone, I need your help. I posted only once before because me and my wife questioned her WG diagnosis. If you look at my past post, you will see the full story, but for those that haven't, here is a quick summary where we are. She has had inflammation in both ears, with infection in both causing holes. She originally took the ANCA and P-ANCA blood test while her first ear was inflamed and it came back negative. After the steroids she took healed the first, when she went off, the second got infected. Was tested for the ANCA and P-ANCA again, then it came back positive for high levels. Her sedimentation rate was high as well on this one too. She has had severe hearing lose and her right ear, and mild on her original ear since it all began. Sinus CT came in negative also. Both the ENT and Rheu have said she has WG, but all of her other test were negative; urine and liver. We still doubt WG so we asked for a biopsy. The ENT today said her ears look fine today. No swelling anymore, but most likely because of the 3 weeks of steroids; 1st week 80 mg, then 2 weeks of 60mg, which she is still on to this day. ENT saw some signs in her nasal passage today and scheduled a biopsy on Monday. She had a x-ray on her chest a month ago and the Rheu thought he saw something, so they ordered a CT on her lungs. The CT results came in today and they saw at least one lesion on her lungs. He is the best of the best in Houston and very hard to get to see, but because of the Grace of God, we are getting squeezed into his schedule tomorrow morning. Now I have made a list of questions to ask him, but want to know what you all think. My wife and I are starting to come around to the WG diagnostic, but still would like to be 100% sure before they start the Rixtuxin infusion. Like I said before, both Dr. said it is, our insurance accepted the claim after only 24 hours that it is WG. Are we crazy for still wanting the biopsy to be 100% sure even though the ENT said it still might not be inflamed enough to confirm? I know after the hours of research that time is of the most importance right now, and my wife and I both want her to be cut down on the steroids ASAP. I just want to make sure that we don't make a mistake by putting this toxic infusion into her bloodstream without asking the final "right" questions tomorrow before we make up our minds. Remember, we already have the biopsy scheduled on Monday. Should we wait? What do you all think? Her Rhem appointment is in 8 hours. Thank you as always.

[vdub]

Usually, you won't know for certain that you wegs, since they haven't found any specific physical markers for the diseae. They DX you based on symptoms and throwing out what it can't be. A biopsy sometimes helps, but is not conclusive on its own.

Rixtuxin is currently popular and well understood. I wouldn't be afraid of getting it.

[cjhnsn29]

No! You are not crazy, Sorry it took so long to answer. I am new to the support group. I have been going thru this for years. My sed rate was in the 100's. This diease is aggravating and hard to diagnose. I have been to mega Dr's before the Dr's all came to the same conclusion. My ENT has been practicing since 1962 and only had 5 cases. My ANCA also was normal and the sed was out of the roof. If you or your wife would like to discuss the differents of WG. My husband has been great about it.

[MikeG-2012]

In your wife's case, Where would they biopsy? Nasal, sinus and ear biopsies are often inconclusive. I had three sinus biopsies, and all were negative. Trust me, you don't want her to have a lung biopsy. I did that and had definitive diagnosis, but had a chest tube for six and a half very long days. Looking back, it is nice to have a definitive diagnosis, but I wish they had just started treating me immediately. I still have issues with the area where the chest tube was, and that was in April of 2012. A good sneeze with my body in just the right position, my whole body tingles for a few seconds and my ribs hurt like the dickens for 4-5 seconds.

Rituxan is very well tolerated in most who have it, and the side effects are FAR LESS than Cytoxin, a chemo-therapy drug. I've had six in the past year and have had no side effects from any of them. The only thing that I did suffer was a few days of crashing because of the steroids they pump in along with the RTX.

Relax, both of you take a deep breath and it will be OK!!

Have a great day!!!

[Pete]

I think some of us on here were diagnosed by a process of elimination - including me. Like you, I wanted to be 100% sure I had wegs before starting treatment. The pulmy who diagnosed me said that an open lung biopsy (meaning at least another week in hospital) would probably confirm wegs. I elected to proceed with treatment (150 mg/day of cytoxan, 60 mg/day of pred, and bactrim). Doc said if I got better with treatment, it would confirm diagnosis. I got better.

I had two rituxin infusions last fall to arrest kidney involvement. Other than some mild fatigue the day following treatment, I had no problems with it.

[TheMitchells]

We're going to see a Pulmonologist next week to get a fungal etiology from the cavity found on the lung and possibly a biopsy, but after hearing your story and from what the Rheum said today I think we might pass on the biopsy. We went ahead a scheduled the first rituxan infusion for 9/4/14. Thank you for sharing your story.

[Alias]

From what I have experienced and read, physicians differ on whether biopsy is necessary for definitive diagnosis, and when the biopsy is negative it still does not necessarily rule out WG. From what you are saying, the doctors seem to think the clinical picture is convincing enough in your case. A biopsy is an invasive procedure that can cause pain, possible complications, and cost. If the doc is not pushing for it, I certainly would not insist on it.

[BarbW]

You have enough of the criteria for a dx. The positive c or p anca test let's them know your wife has a serious immuno disease. The Rituxan will provide relief.

[MaxD]

My symptoms were very similar to your wife's. I had prolonged sinus problems, recurrent ear infections treated with mild doses of steroids to get relief, as soon as these were stopped I developed Bell's palsy and then roving joint pains. Lung CT showed nodule consistent with GPA (previously known as WG). Rheumatologist did not need biopsy for diagnosis, as ANCA was positive and SED rates off the charts. Got a second opinion from a top NYC expert who agreed with the diagnosis. I started on 75mg prednisone, and tapering down since Rituxan treatments, which ended early May. Now down to 10mg prednisone and doing well overall. Had absolutely no noticeable side effects from the Rituxan. There is a journal publication coming out this fall from a study that shows that Rituxan is more effective for treatment of sinus/ENT related GPA issues.

Your wife seems to be getting very good care - I personally would not insist on the biopsy. Nasal biopsies have unacceptably high false rates and lung biopsies are a major deal and riskier than the Rituxan treatment. Don't be scared off by the warnings about Rituxan - it has been extensively used for the last 10-15 years and is standard treatment for B-cell lymphomas. It is NOT a chemo drug, so not toxic like cyclophosphamide. Its effects are noticeable after 2-3 months. I will likely continue with one infusion of Rituxan once every 6 months for maintenance therapy.

It is great that you are reading up, asking questions and being skeptical. Good luck to your wife and to you too!

[loisann11]

I had double pneumonia and they thought it was caused by WG and talked bout open lung biopsy. My lungs were in too bad a shape for that but I got better on the Prednisone so nothing done. I had lots of other symptoms too, I had "more again in 3 months and went back to hospital. My kidney Doc agreed that it probably was WG but would not start anything until positive. Taking the drugs if you don't have WG can cause all the bad side effects later on and everything I read said don't do it until 100% sure. I had kidney biopsy that verified it. I the had plasma exchange, 4 doses of Rituxin and of course lots of prednisone. I am doing fine now. Kidney biopsy was really easy, like getting a shot in the back, little one. Taking these drugs seem to have more long term issues such as causing cancer than they do early on. Rituxin caused me no problems either going in or afterward. I did not feel my kidneys were involved but just some signs in the tests I guess. Just me but I would want to know for sure before starting any nasty drugs. Good luck.

[Dirty Don]

Just to temper the lung invasion thing, I had one. I was knocked out, surgery done by a fine but crazy surgeon, woke up a bit sore, mostly from someone throwing my arm over my head during the operation (I was told that later), then recovery. Pathology was accurate and back within 24 hours, docs knew then for sure. WG or surgery? Hmmmm...such choices we get to make nowadays...

[Ruotsi1]

I had an open lung biopsy, sure it wasn't the most exciting thing I've done, but it wasn't horrible. I wanted a definitive diagnosis and the cardiologist who did my bx said he knew by looking at the tissue that it was Weg's. 2nd opinion from University of Washington confirmed diagnosis. I was in the hospital for 5-6 days, sore after surgery, but thankful I knew for sure what I was dealing with.

[annekat]

For what it is worth, my nasal biopsy was really easy and yielded a positive dx of WG, although my ANCA was very low. I was lucky, though, and if it had been negative, that would have been a problem, seeing as how I was dealing with docs with little WG experience and they might have concluded I didn't have WG. However, I already knew enough from reading this forum that I could have been proactive and continued to pursue a dx. I had lung involvement, so could have had a lung biopsy. But I also had a saddle nose, which made it pretty obvious what I probably had, and hopefully someone would have gone on that and my lung CT scan together, along with my SED rate over 100, and let me skip the lung biopsy. It sounds good that you have the RTX scheduled, although I have no experience with it, having been treated with CTX. I hope it goes as smoothly as it has for anyone here who has had no problems with it.