ANAC and tissue sample confirmation

[LisaMarie]

how many of you have heard that you have to have one or the other positive to have WG?

[pberggren1]

I know that some on the Forum here have had both negative so it depends on symptoms and other blood work and other diagnostic procedures and of course the docs experience.

[chrisTIn@]

I think you mean ANCA?
My ANCA was positive but tissue-samples were negative.

Still the fact that I have WG can not be doubted,
according to my WG-specialist.

[drz]

how many of you have heard that you have to have one or the other positive to have WG?

I have never heard anything about this one way or other. I was told my ANCA scores were highest Mayo lab had seen and very suggestive of Wegener's disease so I guess it is not viewed as 100% indicative marker. My diagnosis was confirmed by a kidney biopsy the next day.

[LisaMarie]

Thanks for your alms input.....Not being able to see Dr specks has really made this a rough week...hopefully I will get to see him end of this week...seeing docs who do not specialize in WG really sucks :glare: wish me luck with getting into ENT and having biopsies done.the doc who did the bronchoscopy is concerned since they also found some abnormal tissue in both bronchial tubes and down in both sides...more on the right then the left..I hope thr bad dog has not swakened and moved there. though i sm not he ever went to sleep

[Geoff]

Wishing you well LisaMarie, I with DRZ in so far as my ANCA (PR3) scores were sky high but Wegs was only confirmed absolute after a kidney biopsy. Throw that nasty Wegs dog a bone and tell him to go sit in his kennel.

[Sangye]

Where's that Wegs dog lullaby when you need it? :wink1:

[Daggar]

Holly had the elevated Proteinase 3 Antibody (PR3 - ANCA?).... they did a kidney biopsy to confirm WG.

Did anyone experience elevated Anti-MPO levels?

[LisaMarie]

My ANAC is negative so they are grabbing biopsies where ever they see inflammation or funky stuff..they took 2 biopsies of my stenosis and 5 from my bronches....waiting for ENT ...Hopefully will be seen and biopsied by them in my nose and sinuses...want to ask them the ringining in my left ear and hearing loss in my right...praying i see Dr Specks tomorrow....if not on Monday...i have never had biopsy done for whatever reason and they deleted the pics of my stenosis prior to surgery will keep u all posted....i know some of use have both negatives in ANAC and tissue...i was just wandering how many....this is such an hard disease to diagnosis...and with all of us being different it is more apparent to me how important a specialist is...the docs here who do not specialize in WG are definately not the ones to see :unsure:

[cathy2330]

Hi LisaMarie, I haven't been on the website for awhile but I am always browsing and reading just haven't posted anything for some time. When reading your story here it is very similar to mine so I felt compelled to write today. I am Anca Negative and have had biopsies of my nasals and trachea and bronchi all negative. I have sinus issues , inflammation narrowing and have tracheal stenosis and bronchi stenosis. It has been such a difficult road as I really do not fit into a catergory. My rheumis best guess is Wegs or as she says some sort of vasculitis. She has called the mayo clinic (I am in BC canada) and discussed my case and they agree. My ESR has never been over the normal range, my CRP is within normal and at diagnosis approx. 1 year ago no kidney involvement. Around 8 yrs ago, had one bronchial narrowing in the left bronchi and was just monitored with a ct scan every year which showed no changes. Last Jan. I had been struggling with my breathing for over 4 months and went for CT scan which showed nothing new. Doc did a bronchosocpy and from there everything downhill. I spent 6 weeks in the hospital with all sorts of docs trying to sort me out. Seems I had gotten new stenosis in both of my bronchis , now having 2 areas in left and 1 large area in right and tracheal stenosis right near the vocal cords, believe they consider subglottic stenosis. Doc tried putting in stents area was too narrow, failed. During the past year I have had approx. 13 balloon dialations, Have done 6 months of cytoxan, currently on pred. 10 mg and during the last 3 months my GFR and Creatine all out of normal range. Also had a ultrasound on my kidneys as I have been having pain on my right side for the past three months. Ultrasound showed a mass on the upper right kidney. My last round of cytoxan (IV) was in Dec. and have only been on pred. /bactrim since. Seeing kidney specialist on Mond. as I need to know if this is vasculitis related or something totally different.

Had a balloon dialation in Oct. but was noticing by Dec short of breath getting worse, had another bronch and balloon dialation in mid Jan. and Thoracic doc decided that we will need to do at least every 3 months, scheduled for mid Apr. but will do sooner if starting to get into difficulty. Seems it is the trachea that is closing up the quickest right now. Rheumy does not want anymore cytoxan, it did not seem to help much and am considering rituxan or cellcept or nothing. It is a very frustrating thing as there is not a lot of proof if rituxan will help existing stenosis's ( I have been researching as best as I can) and doc who did bronch. does not see any ACTIVE inflammation. I am getting another CT scan on sinus in Mar as approx. 1 year ago had nasal surgery to clean out all passages. Problem for me is deciding if I am having active disease then would really feel rituxan is best option, but do not want to put any more toxins in my system if it will not do anything. It seems I will have to be constantly dialated but also concern as more dialations can cause more scarring and quicker which is not good. Also, as I have had so many dialations after each one now more complication, oxygen sat. dropping, needing adrenal and having to stay in hospital for 1 - 2 nights as narrowings are swelling and breathing very bad until swelling starts to reside.

Anyways, I always do read on here but my situation is so wierd but I as wondering if you knew anymore about stenosis's (as you seem to have same issues) and whether the meds will help. I know I do not (as doc say CANNOT afford anymore narrowings for him to keep dialating) but I don't know as through my ESR/CRP etc it does not seem to be active and right now I just need to control the existing. Also need to get confirmation on kidneys, do you know if biopsy is the only way to seem if inflamed????? Thanks for any advice and if you have any questions that might help you I would be happy to answer if I can.
Good Luck with everything. Cathy

[LisaMarie]

Cathy
I feel your pain.....I know I have WG...I am just wired weird...i fit all the symptoms except positive ANAC and we will see about the tissue samples....My CRP has not been normal once since Diagnosis....I have lost hearing in my right hear and have problems with my left ..my right mandible remains swollen and sore...in the broncho they only took tissue in the "angry areasand also from my stenosis....i took rituxan and imuran and ended up in the hospital for 14 days with unknown funky lung stuff and resp distress..I almost died..so I can offer no help on meds...I just had to stop cellecept because my liver enzymes tripled ...as they did on imuran...i got severe mouth lesions with nausea and emesis with mtx....so here I am at Mayo praying for a better plan....will keep you posted...my kidneys have been ok so far....I am back up to 40 mg of pred....i just can t seem to go below it without major issues....also my left extremeties swell more than my right...but the right side of my face swells more then the left...just call me unique i guess...will keep you posted...hope you have a good day:biggrin1:

[JanW]

I have stenosis as well (only subglottic, very limited tracheal involvement) and am wondering if surgery was ever an option for you. My doctor actually cut into my scarred tissue to make the opening wider and then balloon dilated it. So far, I am coming up on a year with no complications and no need for further surgeries or dilations. It really did change my life.

To my knowledge, no medication plays much of a role in affecting stenosis; I take mtx alone to control my disease, which so far is in remission.

[cathy2330]

When he balloon dialates the area does open wider the problem is that because the stenosis is so calcified hardened that it narrows back very quickly. I have been doing some research and there has been some documented that rituxan has helped with stenosis but of course that is very limited. The real problem also is the bronchi narrowings because if they close off a tracheotomy will not help so I am dealing with both issues of the subglottic and the bronchis. I am also just pondering if the medication will help to prevent any new areas. It is a very difficult decision to make hard to know what is the right thing.

[Daggar]

Now that I've had a brief lesson in c-ANCA vs p-ANCA and the PR3 enzyme vs the anti-MPO enzyme I can understand what most of you are talking about!!! Yikes, you need to be a med student to get all this.

I asked the doc about Proteinase 3 – he explained to me that it is the enzyme/antigen that causes the immune system to produce c-ANCA... Holly was c-ANCA positive at the time of diagnosis but now negative as the levels of the Proteinase 3 enzyme/antigen has fallen into the “normal” range.

Not sure I got this one right -- the anti-MPO is the enzyme/antigen that causes the immune system to produce p-ANCA... found more often in other forms of autoimmune disorders/vasculiitis – ie Microscopic Polyangiitis but isn’t ruled out in WG.

There is also an x-ANCA but that's found in non-vasculitis disorders.

Has anyone been p-ANCA positive at the time of diagnosis??

[Bren]

I have Wegner's & Limited subglotic stenoises.. I have ANCA P that is Positive and my ANCA C is not so my Rhym don't think I have a true case of Wegners but have many symptoms.. crusting in the nose -Major, draniage down the back of my throat,, ear problems like swishing, heart pounding. High BP, I have 2 dialations in 2 years my ENT Dr don't like to do surgery's due to scaring, I really don't feel that he understands.. I was as small as a straw before the surgery's I just had my appendix out they did not rupture but I didn't have much time to spare.. I have had an apt to see Dr Speck on Monday the 28th for a month now I m still going down.. I m the one that my Rhymi Dr is going to talk to Dr Speck and come up with a game plan for me.. they have taken me of All Meds as they want Dr speck to see more of Wegner's.. my blood work was really off white bloods count was way high, they used a 5 air way tube for my surgery.. can't wait till Monday comes to get an experts answer from Dr Speck and Dr Kaslebauer .. my Dr had me on Meth then I had a problem breathing and they put me on cytogen.. Dr speck he would not put me on that he would of put me on ritoxon? -(not sure on spelling) I hope my healing is going to go fast they did a cut instead of laperscopic due to it would be less time with a tube down my throat and less for swelling.. love to hear from anyone else

[LisaMarie]

Bren
I just saw Dr Specks today and he was great...will see him again in May when I am down to atleast 10mg so wish me luck....it is definately better to have your doc talk to Dr Specks prior...I will be repeating things in Mayo .....but all in all it was worth the visit..and I highly recommend it.:biggrin1:...

[Bren]

LisaMarie, sounds good the two Dr's have talked and that is why I have not been on Meds the Stomach-Appendix issue scarred me as I thought the Wegners affected my Kidneys... I can't wait to get a game plan and to start feeling better and know the meds going in me is the ones that will work as Dr Speck knows his stuff and not put things in me like I have been having and not know if it works. my understanding Subglotic area really has its mind of its own, Med don't really help control .. anything can make it flare up to the subglotic area??
So glade things went so well for you, and the trip was worth your time.. keep in touch

[Daggar]

Sorry to hear you're having a rough time of it Bren -- good that you are going to see a WG specialist.

Just asked Holly's doctor today and he wouldn't rule out WG with a positive pANCA result. He's treated a few that were positive pANCA but much less often.

Hope Dr. Speck gets you on the right track to recovery!

[Sangye]

LisaMarie, what is Dr Speck's treatment plan for you?

Daggar, there's an x-ANCA? Wow, what'll they think of next. It does sound cooler than C-ANCA though. Like x-Files disease or something. :wink1:

[pberggren1]

I'll take the x-ANCA as well!:flapper:

[Daggar]

x-ANCA can make you feel pretty "crappy" as well..... it's associated with inflammatory bowel disorder!! seriously .

[pberggren1]

I don't want any ANCA then.

[LisaMarie]

LisaMarie, what is Dr Speck's treatment plan for you?

Daggar, there's an x-ANCA? Wow, what'll they think of next. It does sound cooler than C-ANCA though. Like x-Files disease or something. :wink1:

Well the short of it is Dr Specks wants me down to 10mg of prednisone then have more tissue samples taken.....so i go back in May to see him and ENT....still waiting on some labs to come back...my CRP is up to 21.....the doc who did the bronxh felt it was WG but the samples only showed inflammation under the microscope...so being on 40mg of pred is not helping in the plan process...he is checking me for B cells and immunology .he felt the rituxan should have put me in remission....so i may or may not have WG Limited...he did not understand why no one ever biopsied me....he agreed to keep me and i will use my WG doc down here as a go between....i am a mystery...mostly points to WG....i just am not responding towards the meds like i should, so i have them scratching their head ..so i am waiting on more results..something is brewing in my brim bronchs....so once it cultures out I will be on meds for that ..it may be aspergilis ...so wish me luck...the oddball duck that I am

[pberggren1]

I sure hope it is not Aspergillus Fumigatus Lisa Marie.

[LisaMarie]

Me too Phil...me too...the med is so expensive and super hard to get rid of :crying:

[pberggren1]

Ya, I took 3 weeks worth at 200mg per day and it cost me 220. If it was not covered it would have cost me 2600. Vfend is a nasty drug with not so nice side effects. I sure hope you do not have Invasive Asperillosis or Chronic Pulmonary Aspergillosis. These are very hard to treat. Are you giving regular sputum samples to be tested for Acid Fast Basilis and bacterial and fungal cultures?

[Sangye]

It's a sad day when we're hoping that it's "just" Wegs, isn't it? :glare:

I hope whatever it is, they can treat it easily and get you strong again Lisa Marie. So glad Mayo is going to be overseeing your care.

[elephant]

LIsa Marie so glad you got to see Dr Specks, are you still on oxygen?

[LisaMarie]

for now at night only....and per id here when i am out and about buying groceries pushing a cart...i tend to loose air due to de conditioning...so he hopes the mask and o2may help ward off infections...hoping i can tolerate getting down to 10mg and getting back up to Mayo flaring where they can get some good tissue samples...need to have a sleep study done and try cpap at night since the pft show the wonderful pred has cause some airway obstruction...so i will do what Dr Specks wants and pray he gets his answers to give me a plan....:biggrin1:

[LisaMarie]

Ya, I took 3 weeks worth at 200mg per day and it cost me 220. If it was not covered it would have cost me 2600. Vfend is a nasty drug with not so nice side effects. I sure hope you do not have Invasive Asperillosis or Chronic Pulmonary Aspergillosis. These are very hard to treat. Are you giving regular sputum samples to be tested for Acid Fast Basilis and bacterial and fungal cultures? Phil when i was released from the hospital i had to buy 5 tabs for $ 230.00,,,so who knows how much it will cost...

[RCOSSIO]

Phil...is that what happened to you????

[pberggren1]

Ya, I had to take Vfend for 3 weeks just to be on the safe side because I had one sputum sample come back positive with Aspergillus Fumigatus. 5 pills for 230 is insane.

[LisaMarie]

I was on the Iv form in Sept/Oct for my 14 day stay at the hospital and had switched to oral just prior to Discharge to home and had to take 5 more tabs and it was $230...so trust me I am hoping I do not have that again...I had positive antibodies for it during that hospitalization...so I am waitng on final results from my bronch last week at Mayo to see if I need anything or if it is just my funcky body being wierd again...you known none of use are the same or present the same.....Bren good luck today...You'll like Mayo and Dr Specks!!!!!!!

[RCOSSIO]

even the name sounds UGLY