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Jenny
08-16-2008, 05:34 PM
Hi I'm from Canada. I was diagnosed in April' 08. I'm taking cyclophosphamide and prednisone. I'm still not doing that great. It started in my right ear (October' 07), then my lung (March 08), gums, throat, skin and sinuses really quickly and had to be hospitalized almost died from the lung complication. My ear and sinuses are still pretty bothersome :( I also have facial paralysis on my right side, doctors don't know if it is the wegener's from the inflammation from my ear or Bell's Palsy. Anyone else have this? Also my heart beats really fast mostly around 130bpm without doing any physical activity and high blood pressure. My doctor doesn't seem too worried about it but I have read that WG can affect your heart too and want to know the symptoms of it before it is too late :confused:.

Thanks, Jenny :)

RCOSSIO
08-19-2008, 12:28 AM
I am sorry to hear that Jenny! :(

Right now I am having nerve pain on the right side of my face as well, some numbness on my chin and my teeth are very sensitive. Went in for a CT scan Friday will update and let all of you know, thanks!

andrew
08-19-2008, 07:03 AM
Hi Jenny...
I assume your heart wasn't beating that fast prior to diagnosis/getting sick?? Why isn;t your doc worried about it? I guess uit could be a side-effect of one of the drugs but it would be good to check. The high BP is a concern though. Have you been given any meds for that? What does the doc say about it?

I have certainly heard of a few people with WG having Bells Palsy but I guess that doesn't really mean that the paralysis is related.

Welcome to the site!

Jenny
08-19-2008, 11:53 AM
Hi Thanks Andrew for your reply. :) It was beating fast before the diagnosis and in the hospital. So my lung specialist did an echocardiogram and an ultrasound on all my organs while I was in the hospital. He says everything besides my lung seemed fine. Thank goodness it didn't go to my kidneys. So I'm going to ask my rheumatologist about it when I see her on the 26th. I had shortness of breath and chronic bronchitis for about six years leading up to all of this (which most likely was the start of the WG in the lungs) I had a grapefruit sized mass on my left lung in March, so now I'm not taking any more chances and being passive about my health. Were you diagnosed early or did it take them a while to figure out what was wrong with you?

Jenny
08-19-2008, 12:04 PM
Hi Richard! My teeth started to hurt and became really sensitive. They became really inflammed and bleed (like half a cup of blood) when I brushed them. I couldn't eat anything that hard like crackers or toast or I would just bleed. Now that I am on treatment the gums went back to normal but receeded way below the gum line and I have no gums in between my bottom teeth. My teeth are still really sensitive especially cold. WG can attack your gums and teeth. I got x rays today at the dentists and I have some bone destruction. So they told me to brush my teeth after every meal and I have to go for scalings every three months. How are your ears? I got the paralysis on my right side a little after the WG in my right ear (ottitis media or whatever they want to call it) I'm permantly deaf in that ear now. The neurologist thinks because my ear is so inflammed it is pressing on the seventh facial nerve and that might be the cause. Do you have sense of taste on that side of your tongue? I have lost the sense of taste. What meds are you taking? I found this article online about facial palsy in WG. I'm going to ask my rheumatologist about Remicade since my ear and paralysis are still not getting better. How long have you had WG?

Recovery from Multiple Cranial Nerve Palsy of Wegener's Granulomatosis with Infliximab

To the Editor:

Wegener's granulomatosis (WG)-related intracranial involvement is classified into 3 forms: contiguous invasion of granuloma from extracranial sites, remote intracranial granuloma, and central nervous system vasculitis1,2. We describe a case of WG in which the extension of granuloma from extracranial sites of the skull base resulted in refractory multiple cranial nerve palsy that was dramatically resolved by infliximab therapy.

A 42-year-old man had developed bilateral hearing loss and facial nerve palsy since November 2004. Pulmonary nodules were observed and partial resection of the left upper lobe was performed, with a pathological finding of necrotizing vasculitis with granulomatous formation. Otitis media, facial nerve palsy, hearing loss, and swallowing disturbance become apparent, and he was referred to our hospital in June 2005. On admission, neurological examinations revealed peripheral cranial palsies of bilateral trigeminal, facial, vagal, and hypoglossal nerves. Other cranial nerves, pyramidal tract, and extrapyramidal tract were not involved. In audiometry, the right hearing level was scaled out and the left was 111.3 dB. The C-reactive protein (CRP) level had increased to 7.95 mg/dl, but proteinase 3 antineutrophil cytoplasmic autoantibodies [PR3-ANCA (PR3-ANCA kit; Euro–Diagnostica AB, Malmö, Sweden)] were negative. T1-weighted magnetic resonance imaging (MRI) of the brain with gadolinium-diethylenetriamine (Gd-DPTA) infusion showed a remarkably enhanced lesion from the skull base. According to the international classification criteria3, he was diagnosed as having WG complicated with intracranial granuloma formation, extending from the skull base.

He was treated with oral prednisolone (PSL; 60 mg/day) and cyclophosphamide (CYC; 100 mg/day) for 2 months. CRP improved slightly from 7.95 mg/dl to 4.87 mg/dl; however, neither cranial nerve palsy nor abnormal MRI lesion changed with treatment (Figure 1A). Moreover, manic-depressive psychosis, probably due to corticosteroid therapy, had appeared, leading to reduction of PSL dosage from 60 to 30 mg/day at 2 months. Considering the therapeutic time course, this patient appeared to have WG that was difficult to cure by standard therapy. A protocol for therapy with infliximab was approved by the Institutional Review Board of Nagasaki University, and scheduled according to the same sequential regime used in rheumatoid arthritis in Japan (3 mg/kg), with continuation of CYC and PSL.


[click, then close, image]
Figure 1. Axial and coronal T1-weighted MR images with gadolinium enhancement before infliximab injection (A) and after infliximab injection at Weeks 14 (B) and 80 (C). Abnormal MRI features were unresolved by conventional therapy (A). However, the area was reduced by infliximab therapy (B and C).


After the second infusion at 6 weeks, CRP diminished to 0.08 mg/dl and the Birmingham Vasculitis Activity Score (BVAS)4 fell from 22 to 4. Swallowing disturbance as well as trigeminal and facial nerve palsy gradually improved. In nerve conduction velocity studies, the amplitude of the facial nerves also improved: improvement was found in the right orbicularis oculi muscle (from 0 to 200 µV), left orbicularis oculi muscle (from 500 to 920 µV), left nasalis muscle (from 270 to 1610 µV), and left orbicularis oris muscle (from 104 to 540 µV) at 14 weeks after the third infusion. The right auditory disorder did not resolve, but the left hearing level improved from 111.3 dB to 45.0 dB. Pulmonary nodules also disappeared. MRI detection of the enhancement area at proximate infliximab infusion (Figure 1A) was significantly reduced compared with 14 weeks (Figure 1B) and 80 weeks (Figure 1C). Remission (BVAS 0) was achieved at 14 weeks. Infliximab infusion was continued every 8 weeks up to 78 weeks (11 infusions), when PSL was tapered from 30 to 5 mg/day. CYC dosage reached 10 g, then was switched to azathioprine. BVAS remained 0 at 78 weeks, and then infliximab was discontinued. Remission still remained at 102 weeks.

This is a case of ANCA-negative, biopsy-proven WG with severe intracranial granulomatous involvement with multiple cranial nerve palsies. Reinhold-Keller, et al reported that ANCA-negative WG shows more serious CNS involvement than PR3-ANCA-positive WG5, which is consistent with our case.

Efficacy of TNF blockage agents in WG is reviewed in several reports6. On the basis of high rates of adverse events, etanercept use is not recommended for WG; however, Mukhtyar and Luqmani have described an excellent effect of infliximab, demonstrating that 43 of 53 patients (81%) given infliximab experienced remission. In addition, 37 of these 53 patients (69%) had been classified as refractory cases with standard treatment6. Infliximab, in most cases, is administered with concurrent treatment6. Since the therapeutic duration of CYC and PSL is short, 2 months in this case, a delayed efficacy of CYC and PSL could become apparent after infliximab. However, high BVAS, abnormal MRI lesion, and high serum CRP concentration were still present, before infliximab, regardless of the administration of 100 mg/day CYC and PSL (at least 30 mg/day) for 2 months, and these indicators rapidly improved in 6 weeks after infliximab. We suggest these therapeutic outcomes indicate the efficacy of infliximab.

Our case is the first infliximab-induced recovery of intracranial involvement of WG. We hope this experience encourages the consideration of infliximab treatment for refractory and life-threatening WG.

KEITA FUJIKAWA, MD; ATSUSHI KAWAKAMI, MD; KATSUMI EGUCHI, MD, Unit of Translational Medicine, Department of Immunology and Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, 1-7-1 Sakamoto, Nagasaki 852-8501, Japan. Address reprint requests to Dr. Fujikawa.

DAVEY8105
08-22-2008, 02:42 PM
Hello Jenny! I was just hospitalized over the weekend because my lungs had become so inflamed despite 7 months of Cytoxan infusions and Prednisone every day. They are now trying Rituxan infusions for me and huge amounts of Prednisone, and I do feel better. It is so frightening to be in a situation where your life feels threatened and it feels like it is all spiraling out of control. However, since you have been diagnosed, it is best to really listen to your body and if anything seems out of whack, get it checked out immediately. I learned the hard way to become really pro-active about my health. I hope all goes well with you! Hang in there!

RCOSSIO
08-27-2008, 12:03 AM
Jenny,

Just had a scan done it showed granulomatosis and otitis media. In addition they did see a lesion on the mandible and I am set for an appt Sept 5th with a Head/Neck surgeon.

The doc didn't seem concerned but it sure is painful. Back on the Lyrica for the nerve pain on the right side of my face, sinus and jaw.

I'll keep you informed.

Jenny
08-28-2008, 06:53 AM
Good luck with the head and neck surgeon. Sorry to hear that it is in your ear and jaw :( It is very painful. Are you on cytoxan? Do you have any side effects from the Lyrica? I'm thinking of asking my doctor if I can try it. If you get surgery done to your ear it doesn't hurt afterwards it's nothing compared to the ear pain from the Wegener's.;)

Jenny
08-28-2008, 06:55 AM
Hi Davey thanks for your reply. How are your lungs? That is scary that you were on cytoxan and prednisone and that still happened. Are you tolerating the Rituxan ok?

RCOSSIO
08-28-2008, 09:59 AM
Am on Cytoxan 150mg, just bumped up the pred. Pain has gotten better with Lyrica. Hopefully your doc can recommend it to you.

DAVEY8105
08-29-2008, 01:15 PM
Hi! I just had my second infusion today and the Dr. said it will take several weeks to know if it will work. I don't feel as sick right now as I did with the Cytoxan, just swollen hands and feet mostly. The eight infusions of Cytoxan i had didn't do much to stop the disease so, of course, now I'm scared the Rituxan may not work either. Just trying to stay positive and rely on my Dr.'s.

debbie
09-01-2008, 12:38 PM
Hi Jenny, I am from Canada aswell, Edmonton, Alberta. I was diganosed in 2007 and have been the Cytoxan infusions and Prednisone ever since, WG has mostly effected my upper airway and sinuses. I am finding now that my ears are starting to bother me, but have been ignoring it hoping it will just go away. I don't have any ear pain, but there is alway like a pressure build up. Did you have constant pain right from the start in your ears?

Jenny
09-03-2008, 09:26 AM
Hi Debbie. I had fullness feeling in my ear and then partial hearing loss (about two weeks and then deafness in that ear) then lots of fluid, excrutiating pain and blood. That all lasted for about 7 months and my ear and behind my ear still hurt but no fluid or blood. The deafness is permanent with me the WG destroyed the nerves in there too so the hearing will never come back :( Maybe ask your doctor if you can get a ct scan of that ear to see if there is a lot of inflammation in there and then they can up your prednisone. I don't want you to go through what I did. It has eaten away some of the mastoid bone behind my ear.

debbie
09-06-2008, 12:07 PM
Thanks Jenny, I go back to the doctor in a couple of week and will have them checked out.

jola57
09-08-2008, 05:39 PM
Hi Jenny, I'm from Mission, BC. I've had WG since Nov 2006, been on cyclo from dec 2007 to June 08 and now back in the dumps - lung lesions, blood in urine, tinitis (permanent ringing in my left ear), lowered hearing, sinuses, both feet parasthesis (can't feel them) - have I left out anything?, oh yes cramps in legs and hand and my eyes hurt. I keep lowering and upping pred depending on how I feel.
Doc's don't do much - I guess so little is known that each specialist is waiting for another to have me go back on cyclo. I'm not complaining I seem to be lucky and I feel good despite all. I just wish to keep it at the level it is at and not have any permanent damage. My ANCA is back above 200 and CRP and ESR well above normal.
Debbie don't ignore the ears, I believed my family doc that it was just an ear infection at the beginning and took to long to go on pred. Now I don't think I will always hear the ringing in my left ear.:(.
Hugs,
Jolanta

RCOSSIO
09-09-2008, 06:49 AM
Just got back results from CT scan no concern other than another scan of the jaw three months from now. Doc didn't think anything other than a benign bone lesion due to Fosomax. Stopped taking the Fosomax and the pain got better after my 2 weeks of antibiotics.

Will follow up with an ear specialist for the inflammation but the pain went away again after the antibiotics.

Will see how it looks 3 months from now!

Jenny
09-12-2008, 09:03 AM
Good Luck to you all. It doesn't sound too easy to go on to Rituxan in Canada. My rheumatologist said it is really hard to get a hold of. Cytoxan is doing a bit of good but I'm still bad. Do any of you have joint involvment? Richard I can feel bumps on my jaw and temple area but my doctor didn't really say much about it. :confused:

Jenny
09-12-2008, 09:04 AM
I was trying to remember your online name in my reply about your jaw. Sorry...my brain isn't like it used to be

andrew
09-12-2008, 01:03 PM
Good Luck to you all. It doesn't sound too easy to go on to Rituxan in Canada. My rheumatologist said it is really hard to get a hold of. Cytoxan is doing a bit of good but I'm still bad. Do any of you have joint involvment? Roscoe I can feel bumps on my jaw and temple area but my doctor didn't really say much about it. :confused:

What about getting Rituxan from an oversease online pharmacy (a real one :))?

I'm don't know about laws in Canada that govern importing drugs but here in Oz, as long as it's not a large amount and/or I have a prescription I can produce then it's all good.

RCOSSIO
09-13-2008, 09:18 PM
Jenny,

Yes joint pains are constant but not bad. My Rheumy up my pred by 10mg but now insist on motion exercise and unless my sed rates go up, otherwise go back above the normal of 31 then he will adjust.

The jaw pain went away with the Lyrica as it was affecting what they call the avleolar nerve. Will let you know after 3 months after the CT scan how my jaw looks again.

Jenny
09-15-2008, 08:12 AM
On thursday I was put back on prednisone 10mg for the joint problems. Now that I'm feeling a bit better I'm going to start doing some exercise. Yesterday I started to cough up mucous/fluid and there was some blood too :( See the lung doc on the 24th. Good luck to all of you too and keep me updated on how you are doing. Thanks for the support :)

andrew
09-15-2008, 08:10 PM
Exercise! Nice! Just don't overdo it :) I should really do some exercise myself. I'm very...um...'stocky' :D

let us know how you go with the lung doc.

Sangye
12-02-2008, 12:44 PM
I had a racing heart for quite a while. It started before I got diagnosed and kept on for many months once I began pred and cytoxan. I finally figured out it was due to the severe anemia I had. My bp went high for awhile, too, as a result of the pred. Pred can also cause a racing heart, as it revs up the adrenals. Both symptoms have gone away, but that racing heart really made me nuts for awhile!

Rodger
12-04-2008, 12:17 PM
I also had teeth and gum problems in the beginning before I was diagnosed: numbness in the roof of my mouth, and before things got better my teeth loosen and shifted. Thank god the folks never went whole-hog on the braces as a kid. My gums receded so now I see a periodontist regularly as well as my regular dentist. Neither of them were particularly familiar with WG and still think the repeated high doses and variety of antibiotics may have contributed. That part has stabilized. My dental hygiene has begun as much as priority (quasi-obsession) as hand washing.

I've also started the ear symptoms recently. Constant fluid from the ears too. When does the fun stop? ;->

crackers
12-05-2008, 10:23 AM
Hi Jenny
I live in the uk my husband became ill two yrs ago and reading your story brings back so many scary memories.John ended up with pnuemonia and sepsis also high bp and fast heart rate.I cant say if meds or wg cause fast heart rate etc but yes the same happened to John and even though he is on meds for it now his bp is still high
I wish you a lot more good days
Sue xx

Natalie Makeef
01-05-2009, 10:39 PM
Hey there from New Zealand. I was dignosed in Nov 06 and I had similiar symptoms. I am now taking Methotrexate after taking Cyclophosphamide. I have been off predisone for about 5 months now. I am still not that great. It started with a prolonged flu, bleeding nose inflamed tissues in my eyes so I my eyes were all read around the pupils. The disease spread to my throat felt like I had a big lump and my hearing was going in both ears. Then I cough up blood from my lungs thats when I went to hospital. I had an xray done and the doctor noticed that my lungs didn't look right and suggested I might have WG. Also my heart beats really fast too even if I don't do any physical activity. I do think it is the medication. I recently caught a virsus and I have been coughing my life away so am taking a ventalin to relieve the tightness in my chest. I am 36 and have been married for 4 years and cannot have children because of the medication and the WG has effected my ovaries.
I recommend that you rest, have less stress in your life and pace yourself. I wish you all the very best, Natalie