Wegeners with no major organ involvement?

[2ringers]

I'm 47 year old male from central Pennsylvania. I don't know if I have wegeners or not.

History:
I have asthma, allergies, IGA deficiency and cronic nasal problems. I also had many benign cysts removed from my abdomen.

I would get congestion, sinus infections and the sinus problems would trigger the asthma. They would put me on prednisone and antibiotics and my sinuses would clear up until I stopped the prednisone. I have had sinus surgery 3 times to remove nasal polyps. About 10 years ago I bent over and felt my ear close and I suddenly lost all hearing in the right ear. I went to the ENT and was given prednisone and antibiotics and most of the hearing came back. Over the next few years this kept happening and each time less hearing returned. My ears would not drain because the fluid was so thick. I eventually lost all hearing in my right ear.

I was fitted for hearing aids, but they just made the drainage worse. I went to Hershey to see a specialist in conductive hearing loss. She said I had polyps in my ears and did a tympanomastoidectomy on my right ear. It did not help.

Several years ago I suddenly lost all hearing in my left ear. Now I was completely deaf. Went to ENT, more prednisone and some hearing returned. I was eventually sent to vocational rehab for my hearing loss. They referred me to a otoneurologist. His first thought was you have wegeners. They did blood work and said we can't say you have wegeners due to the lab results and the fact you have no major organ involvement. He belives it is autoimmune related and said he has seen 2 cases like mine. He did surgery and I now have BAHA implants on both sides and he also did a tympanomastoidectomy on the left ear.

He sent me to a rheumatologist and they did more testing and said they couldn't diagnose me with wegeners. They did belive it was autoimmune related and started me on methotrexate because of the fact that every time I stopped the prednisone, my sinuses and ears would flare up. I started the methotrexate the first week of January 2013, and I have not needed the prednisone since. My sinus symptoms are much better and the drainage from the ears has not stopped, but it is much less.

Now for the past couple of months I have been having arthritis symptoms. Pain and weakness in both hands, pain in both knees, both feet and left elbow. I'm not scheduled to go back to the rheumatologist until early March. It is an 8 hour round trip to see her. I could not find a rheumatologist around home that would treat me without a positive diagnosis. I did schedule an appointment to see a local rheumatologist for the arthritis, but I can't get in until early January.

So, I don't know if I have wegeners or not.

Ron

[Pete]

Hi Ron,

the best suggestion I can offer is to see a Vasculitis specialist. Although either would be a bit of a drive, Cleveland Clinic or Johns Hopkins ought to be able to give you a definitive diagnosis.

Good luck and better health.

[annekat]

Welcome, Ron! I hope you don't have Wegener's, but it sort of sounds like it, and it would sure be nice to know, wouldn't it? Getting proper diagnosis and treatment can be a tough one. Being on the east coast, you have better access to Vasculitis specialists than I do in the Pacific Northwest, even though it may be quite a drive to the one you choose. Try this list: VF Medical Consultants (http://www.vasculitisfoundation.org/mcm_resources/medical-consultants/) . And some forum members may know of other good docs in your area who aren't on that list.

I'm glad you found the forum, as it is the best place in the world to be if you think you might have Wegs. There is lots of info available by searching the archives. Keep us posted on what transpires!

[pberggren1]

Sure sounds like it could be Wegs. I would go to Cleveland Clinic, that is probably the closest for you. Many on here to there. That is one of the top 10 places in the US to get Vasculitis care and treatment. Once you are established as a patient there with your first visit most go back there only once or maybe twice a year. Many consults can be done with your local docs with your main doc at CC.

[SpaceflightAddict]

I second what everyone here has said... go see a Vasculitis specialist.

I ended up at John's Hopkins earlier this year, with only sinus issues, and they diagnosed me while I had negative ANCA results and two inconclusive nasal biopsies.

Best of luck to you, and if you have any questions at all feel free to ask.

EDIT: From my understanding, the doctors on the VF consultant list that anne posted earlier will do phone consults with your regular doctor. It could not hurt to ask your doc to give one of them a call.

[Pete]

Hi Ron,

I see Dr Alexandra Villa Forte at Cleveland Clinic. I think she's excellent, and she is very thorough. I usually have a written list of questions and concerns with me at each appointment, and she takes the time needed to address everything.

Another doc at CC who may still be taking new patients is Carol Langford. Some other folks on this forum speak highly of her as well.

[Alysia]

Hi Ron, and welcome.
I wish for you that you don't have wg, but something easier. it sounds like it can be.
I hope that you don't mind if I use your story to ask something... ?
I have a question to the experts around, I hope it is not a silly one :blushing:
here it comes: there are some docs that I met, who do diagnosis also according to the influence of meds. so: if pred is "clearing" symptoms when antibiotic don't, can we consider symptoms as "wg-activation-related" ? and if the antibiotics is the one who do the job, can we consider the symptoms as not wg-related ?

[2ringers]

Yes prednisone will clear the symptoms without antibiotics. For 2011 and 2012 it was prednisone alone. I had tried just antibiotics and it was no help whatsoever.

I will have to check next week if my insurance will cover me going to the Cleveland clinic.

[me2]

: if pred is "clearing" symptoms when antibiotic don't, can we consider symptoms as "wg-activation-related" ? and if the antibiotics is the one who do the job, can we consider the symptoms as not wg-related ?


To the first question: No. If antibiotics are not clearing up the problem that does not mean it is not infection. It may simply be that the specific antibiotic is ineffective against the specific infection. To then treat with pred will mask either an infection or wg. Remember, pred doesn't really treat wg anyway. It treats inflammation which could be caused by many things - like infection. In most cases we luck out and we get results that are easy to interpret. I'm just pointing out that it is not a formula that is always that simple. I'm finding myself dealing with this right now.

Second question: Usually yes, but not always. This disease is wily and there have been rare cases like this one:


Sulphamethoxazole?trimethoprim in the treatment of limited paranasal Wegener's granulomatosis (http://rheumatology.oxfordjournals.org/content/41/5/589.full)

Diagnosis by influence of meds is not wrong but with this disease we can't prematurely assume it always works.

[gilders]

You do have Wegener's symptoms and the meds that have worked for you are ones that would be used and often work for getting Wegener's into remission.
Although an ANCA blood test isn't definitive for active Wegener's, it would be a simple test to help indicate Wegener's. Biopsy is the only completely accurate way to diagnose, but nasal biopsies aren't always conclusive. As your sinus' are the area where Wegener's sees to be "attacking" then a nasal biopsy would still be a good idea.
Wegener's is sometimes very hard to diagnose, but that doesn't mean drs shouldn't at least attempt investigations to rule it out!

[Texastatoogal]

Could you tell me what they
do for a nasal biopsy. What is involved? I might be getting one done on Monday and I am a little nervous.

[gilders]

Could you tell me what they
do for a nasal biopsy. What is involved? I might be getting one done on Monday and I am a little nervous.
There was a recent thread that was started about nasal biopsies,
http://www.wegeners-granulomatosis.com/forum/general-wg-chat/3562-nasal-biopsy.html
Reply #9 gives you a fairly detailed story of my biopsy from a couple of months ago.:rolleyes1:

[me2]

Could you tell me what they
do for a nasal biopsy. What is involved? I might be getting one done on Monday and I am a little nervous.

Results may vary. I got scared reading Gilders account. Most people have little discomfort from the procedure as was the case for me. The procedure is done in the office and is very simple. They spray your nose with numbing agents and then take a little snip of skin from inside. Like a big bug bite size. A little tissue for the nose and you go home. In my case the biopsy was definative for wg and it meant an end to the endless doctor visits and antibiotics and on to proper treatment. Does this mean you are still looking for a diagnosis?

[2ringers]

If I were to have the nasal biopsy done, would the fact that I'm on methotrexate affect the results?

When I had the first tympanomastoidectomy done, they did a test on a sample of the polyp. I don't know what they tested for, but they said nothing was found. This was before any mention of wegeners. I wonder if the test would have shown this if they were checking for it.

[annekat]

I have seen first hand how pred can clear up Wegs symptoms, way before I ever knew I had Wegs, and how it often worked even better than the antibiotics to do so. I remember my ENT sometimes prescribed it and sometimes not, because he thought I'd had too much of it. Little did he know I'd be solidly on it in the future. But I agree it only improves the symptoms and doesn't fight the disease.

Interesting case study, Kirk. I've read on here before about Bactrim (the sulfa med in the case) being useful in maintaining remission but not as a sole treatment. I know I took Bactrim pre-wegs as one of the antibiotics used to fight "sinus infections", and it worked as well as any, in the short term. But it was never the DS version (double strength?) that many of us take 3 times a week. I'm inclined to want to keep taking it even if I quit some of the other meds someday, as long as it continues to work over time.

[Texastatoogal]

I saw a rheumatologist on Friday for the first time. My blood work from my primary was negative for wegener's and he will not start me on any immune suppressants until he is convinced I have it. So I am doing more blood work, a ct scan, and he is sending me to an ENT because he said my nasal area is red and inflamed.

[Dirty Don]

If I were to have the nasal biopsy done, would the fact that I'm on methotrexate affect the results?

MTX affects the AI to my understanding...thus controlling the effects of the WG...it still may present in areas.

[drz]

If you are in central PA I would look up Merkel in Philadelphia. He definitely one of the world known experts. You should be able to a consult with him quickly and a visit to his clinic if needed. My doctors consulted with him and Specks at Mayo.