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**Jenny** · 08-16-2008, 06:34 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 .

Thanks, Jenny

**RCOSSIO** · 08-19-2008, 01:28 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, 08:03 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, 12:53 PM

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, 01:04 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, 03:42 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, 01:03 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, 07:53 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, 07:55 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?