Suspicion of MS after Wegener's Granulomatosis

[hrnn]

Hi all!

I'm writing on behalf of my wife who's been diagnosed with WG a bit over a year ago. She started with joint pain and swelling in her lower limbs, and a possibly related ear infection although not for sure.

She's been doing quite well on prednisone lowering the dose and Imuran. She got diagnosed very early so the doctor decided for a milder approach.

A few weeks ago a few things happened at once. She lowered her prednisone from 5mg to 2,5mg, she was under heavy psychological stress due to some family problems. She started feeling pain behind her left eye, quite intense. After a few days on-off she lost sensation on her palate for a few hours and the next day she lost vision on her eye.

After a prednisone shock she got alright again, but now the doctor is suspecting it could be something different to WG, that is Multiple Sclerosis.

I'm by no means an expert but from what I could gather her case is not very MS-like (she didn't have any of the other symptoms described for MS), and even I fould on the forum a very similar case involving the eye (but not the sensitivity loss). My bet is that, if anything other than WG, could be a different myelin degenerating disease (I don't have links handy right now, but I read about a few that would fit a bit better). Also, though not even conclusive for WG, she tested C-Anca positive.

What are the community's thoughts about this?
Thanks in advance!

[Debbie C]

Hi and welcome to the forum ( unfortunately ). Sorry to hear about the problems your wife is going through. I am no expert and really can not say for sure but it could be the result of weaning of the pred to fast.How long has she been at 5 mg ? I also am taking 5 mg. and cannot not even go 1/4 less without having severe headaches and other effects. As so many others on here will tell you.. you have to wean off VERY slowly and some ( like me ) ma be stuck at this point.Maybe if she trys going back up to 10 for a couple of days she may start to feel better. But by all means talk to a pro. in wg before doing anything. Best of luck and tell us more about your wife when you can.

[me2]

Hi hrnn,
I don't know if I have any light to shed on this. From what I understand so far I don't know why it would be considered something other than WG if your wife has a WG diagnosis. Also, I don't know much about MS and the treatments for that.
(I could talk a long time about what I don't know)

Eye problems like you describe definatly fall within the realm of WG and must be taken very serious.

What kind of doc does your wife have? Where do you live? Has she had this doc about a year? How was a WG diagnosis decided?
It would be rather amazing to me if she was diagnosed for WG with just joint pain and swelling. Most of us have to develop a lot more symptoms to finally get a diagnosis. My own case started with sinus problems and nose bleeds. For which I was given lots of antibiotics that didn't work. Then a sinus biopsy was conclusive for WG.

Welcome to the bestest Forum on the interwebs!!

[mrtmeo]

Can you contact the Vasculitis Foundation for a Dr knowledgable in vascuitis?
Contact Us (http://www.vasculitisfoundation.org/about/contact/)

[Thinker]

I had eye involvement right before my diagnosis of WG. I developed redness, pain and blurred vision in my left eye and was diagnosed with anterior uveitis and treated by an ophthalmologist with steroid drops in addition to prednisone and ctx after my diagnosis. I also had migratory arthritis for several months prior and ear infection symptoms for several weeks prior. If she hasn't seen an ophthalmologist it may be a good idea to see one. They can check her eye for any signs of inflammation that her other doctor may not notice and help rule out or confirm her diagnosis.

[hrnn]

Hi! thanks everybody for writing! I'll try to address every question:

after reading the forum I also suspected it could be related to reducing the prednisone too fast (there's even a post with a similar issue related to the optical nerve). but the doctor said no.

Luckily they also considered the issue to be very serious and they admitted her to the hospital just to make all sorts of studies on her and get rid of the symtom (blurred vision).

Her doctor is a rheumatologist and has a few other pacients with WG. She had a different doctor at first and they suspected she could have rheumatoid arthritis, and the hospital derived her to the actual one after realizing it was not. back then they checked her on every single possibility and as far as I can remember she also had something with the kidneys which ended confirming the diagnosis.

right now the plan is to change to rituximab after ruling out MS, for which we have an appointment with an MS specialist on the 3rd.

we are in Vienna, Austria. on one side it's very good because the public health system is taking care of everything and even on the same hospitals she can get consults with specialists on different areas. the problem is that there's not much alternative to get a second opinion.

I'll keep you all posted. Thanks for the feedback!

[annekat]

I just want to say welcome to the forum. It sounds like you have a good handle on the situation and dealing with what you have been told, and will be waiting for more input from various docs. I would disagree with the one doc, however, who said that lowering the dose of pred from 5mg. to 2.5 could not be causing the problem. But true, that might not be all of it. I wish you and your wife the best, and hope you will keep us updated!

[mrtmeo]

I agree with thinker that an opthamologist can give cyclosporin or prednisolone drops for the eyes if there is inflammation.
This is what helped my mom's right eye.

During and right after Rtx treatments, her eye bothered her every day for a total of 3 months and then, the pain was gone and has not returned.
The cyclosphorin and prednisolone drops really helped during that time.
She had PUK (Peripheral Ulcerative Keratitis) which may have been due to the high Rheumatoid Factor which is now pretty low from the rtx.

[hrnn]

Hi! thanks again for the answers!

when she got admitted to the hospital they checked her eyes thoroughly. even in the following days she got them checked many times so they ruled out a problem with the eyes themselves.

what the doctor told her this morning is that there's actually an infection on the nerve and she could have had that for a while already. also that in general her case is coming out of a wegeners and he suspects that what she has could be some other type of vasculitis. we will have to wait to know.

thanks again for all the answers. she's actually feeling fine but on a heavy dose of prednisone. it's just the uncertainty that's a bit unsettling.

I'll keep you posted!

[KathyB]

You seem to be getting up-to-speed on the different symptoms and diseases! Great Job! I've always understood a WG diagnosis is only confirmed through biospy -- kidney, lung, etc. I agree with those posting info for you. Prednisone is tricky stuff. Eyes may definitely be affected by WG. Most weggies report Rituxan is an awesome drug. One thing I might add -- we all have individual cases, no two are exactly the same even though we have the same disease. You'll gather a lot of info on this site but some may not apply to your wife's case. Most importantly, you have "food for thought" and can follow-up, research and ask questions specific to her case. Best wishes and welcome!

[hrnn]

Hi all! thanks again for all the feedback!

so, the update: it's definitely not MS. her brain and LP came back spotless and there should be at least two different signs on that to consider MS as a possible diagnosis. it's also not stress related as the decreased function on her eye was too extreme.

the official story right now is that it;s not WG but Microscopic Polyangitis, as she never presented any granulomas (which is roughly the main difference between these two vasculitises).

she'll start rituximab infusions on friday, with the next one two weeks after that.

I read very good things about Rtx but I'm uncertain what to expect from her overall condition right after the infusion (will she be feeling weak, a la chemotherapy or is it not as extreme?). I did a bit of research on the subject and I know already it;s not a chemical but a treatment biological, still there are a few things unclear about it.

thanks again to you all!
and I hope I'm still welcome given that I'm just a proxy of someone with something other than WG ;) (just kidding!)

[annekat]

Yes, you are still welcome, hrnn! We don't like to let people go once we get to know them. And there are so many similarities between the two conditions and how they are treated. I'm glad to hear it is not MS! I don't know which is worse, but that doesn't sound like something anyone wants to deal with. Keep us posted.

[mrtmeo]

Hi hrn,
My mom has MPA and was treated with one pulse dose for a week of ctx and 4 weekly infusions of rtx.
She tolerated the rtx very well, but she is in her 4th month from first treatment and not in remission, yet.
However, many respond very quickly.
Please come back and tell us how things are going.

[kmac14]

In my experience, the ear 'infection' was one of the first things that I presented with. It was wegeners. Lots of fluid build up behind the drum. I wear two hearing aids and have no problem with hearing but deaf without aids. The ear thing for me was very typical wegeners. I never had eye involvement but that too is typical of wegeners.

[annekat]

In my experience, the ear 'infection' was one of the first things that I presented with. It was wegeners. Lots of fluid build up behind the drum. I wear two hearing aids and have no problem with hearing but deaf without aids. The ear thing for me was very typical wegeners. I never had eye involvement but that too is typical of wegeners. Absolutely right about the ear infection.... we have heard that time and time again on here. A big, antibiotic-resistant ear infection is what I had, out of the blue, at age 58, having never had an ear infection in my entire life. Followed by a couple of years of recurring sinus infections, which also were not at all typical for me. These things should be a big warning to doctors to check for Wegeners. They don't spend enough time with patients to find out their background, whether they have ever had ear or sinus infections before, and so on. Mine were pegged on allergies, which seemed to make sense at the time, although the ear infection was not at my usual allergic time of year. And I also wear two hearing aids and can't really get along without them.

[mrtmeo]

Absolutely right about the ear infection.... we have heard that time and time again on here. A big, antibiotic-resistant ear infection is what I had, out of the blue, at age 58, having never had an ear infection in my entire life. Followed by a couple of years of recurring sinus infections, which also were not at all typical for me. These things should be a big warning to doctors to check for Wegeners. They don't spend enough time with patients to find out their background, whether they have ever had ear or sinus infections before, and so on. Mine were pegged on allergies, which seemed to make sense at the time, although the ear infection was not at my usual allergic time of year. And I also wear two hearing aids and can't really get along without them.

Anne,
Is the hearing dysfunction due to actual tissue damage or is there fluid in the ear?

[annekat]

Anne,
Is the hearing dysfunction due to actual tissue damage or is there fluid in the ear? I think it can be either or both. If it is just fluid build up, and that can be drained somehow, then the person can regain hearing, especially if the inflammation in the eustachian tubes can be lessened, so that the fluid can drain again like it is supposed to. Ear tubes installed in the drums can help drainage, too. But this fluid can become thick and glue-like, to where it can't easily be drained and maybe could be removed surgically, I'm not sure, or else one would have to wait for it to disappear on its own, or not. In my case, I'm not sure whether my fluid turned to glue or what, but the uneven pressure has caused my drums to cave in to where they don't function as well, for one thing, and there has now been almost complete erosion of one ear drum and the little bones behind it, which I guess would qualify as tissue damage from the WG. In what is now the better ear, the infection caused some nerve damage early on, which results in permanent neurosensory hearing loss. Hearing in both ears did improve for awhile after the infection, as presumably the conditions causing the conductive hearing loss were alleviated some, plus the infection had caused holes in both eardrums, which eventually healed, but while still open, allowed drainage of fluid. I was already a candidate for hearing aids at that time but could get by without them until things got worse again, perhaps a resurgence in WG activity causing more e-tube inflammation, fluid build up, formation of glue-ear, renewed infections, and some tissue damage. Somewhere along the way an outer ear infection developed in one ear, which wasn't too bad but dragged on and on, and I think may have triggered disease activity in that ear, causing the severe erosion of the ear drum and the little ossicles, or bones, that are next to the eardrum and relay sound waves to the inner ear. Sheesh, it is all so complicated and confusing. Anyone can correct me if I've erred in any of this explanation. All I know at this point is that what used to be the bad ear is now the good ear, though it still has some nerve damage which I think is irreversible, caused by the infection which was caused by WG though I didn't know it at the time. And what used to be the good ear is now the bad ear, which has no nerve damage, but has a destroyed ear drum and ossicles, though as the ENT says, "some sound can still get in there". With the hearing aids, I do pretty well most of the time.

[mrtmeo]

Anne,
Do you think prednisolone drops in the ears would help lower the inflammation?
Topical steroids seem to help when there is active inflammation in the bronchials, so maybe it would work for the ears.
I wonder if that would work to prevent saddle nose?

[annekat]

Anne,
Do you think prednisolone drops in the ears would help lower the inflammation?
Topical steroids seem to help when there is active inflammation in the bronchials, so maybe it would work for the ears.
I wonder if that would work to prevent saddle nose? I don't know. Putting drops in the ears, the med will stay on the outer side of the eardrum, unless there is a hole in the eardrum. Right now, one eardrum is gone, and I'm using antibiotic drops there to stave off infections, not every day, but 3 days a week with a break in between. There isn't really an inflammation issue there right now, and the hearing loss is more from permanent damage or alteration of the structures, I think. Inflammation in the eustachian tubes can also cause permanent tissue damage so that they never function properly again. As for saddle nose, I think that is more related to inflammation and granulomas in the sinuses and nasal cavity than anything to do with the ears. Before I ever knew I had WG, I got a steroid shot in my sinus area, which helped a lot with what I then thought was allergies. There are also the topical steroid inhalers for the nose; I don't know that they would be concentrated enough to help prevent saddle nose. I already had saddle nose at the time of dx, so no one would have been thinking along those lines, and I had been using the oral steroid inhalers to help with breathing. My WG treating doc doesn't like me to take extra steroids in any form, beyond the pred I'm taking, but I could probably get my ENT to give me a steroid shot if he thought it would help something. At this point, there is not much going on except the residual effects of earlier damage.

[mrtmeo]

Anne,
How long does it usually take to develop saddle nose?

[annekat]

Anne,
How long does it usually take to develop saddle nose? It may be different for everyone. I was undiagnosed and untreated for 2.5 years and it must have started to happen during that time, but I never noticed anything until around the time of my diagnosis. It seemed to happen all of a sudden, with no warning signs such as pain or visual clues that I noticed. Others have seen their nose change shape little by little and/or feel a lot of pain in the area before it happens. I think once someone is properly diagnosed and treated, and they see progress being made in their bloodwork and symptoms, if saddle nose hasn't happened it is then less likely too, although it still can. That is all I can think of to say about it right now. I know there was a thread on it not too long ago where you might get more answers, or on other threads in the archives.

[kmac14]

I didn't get the saddle nose and that has been a very long time. They diagnosed by taking a huge chunk out of my nose (horrible and very painful memory) and I do have a damaged septum inside my nose. My ENT said to me that if it ever did occur then there is corrective surgery.

[Carol]

After bing sick for about an month and being on Imuran, Pred and Bactrim I lost feeling in both my arms within about 1/2 an hour and had to then start on cyclophoamate? infusions. I also lost sight on one eye and had to have the Pred upped. regards
Carol Australia

[hrnn]

Hi! a quick update:

the rituximab treatment was posponed until last week due a runny nose and that the doctor didn't want to take any chances. bad news is she had a severe allergic reaction to the rtx so it had to be stopped after 30 minutes and it's definitely off the table.

so this week we'll meet with the doctor to discuss the alternatives. so far it seems that his medication of choice is cyclophosphamide, which we are not exactly happy with due to its toxicity and the possibility of leaving her infertile.

if so far the aza and rtx didn't work, the classical options are what? mtx and ctx alone?

I've been googling and there are suggestive results on cellcept and also infliximab, but they are not yet mainstream choices.

would someone want to input on this?

thanks all!

[mrtmeo]

Cytoxan is the drug of choice when there is organ or life threatening symptoms.
If she had a problem with imuran, it could be due to a TPMT deficiency that she could be tested for or maybe taking a much lower dose to see if she reacts.
Mofetil and Imuran both caused my mom nausea, vomiting and diarrhea, so I am doing Mycophenolate Sodium which enteric coated and has a better tolerability.
There are studies showing Mofetil effective for induction of remission.
There are many immune suppressants out there, but not much data on their efficacy for vasculitis.
tacrolimus and cyclosporine are 2 I remember reading about.

Cytoxan is the big guns and sometimes necessary to put the disease in remission.
My mom only got one week dose of it, so don't know how well she would tolerate it on a regular basis but her nephrologist doesn't think she could handle it.

[annekat]

hrnn, I'm sorry the RTX didn't work. You did say your wife had a mild case to begin with, but it seems like now alarming things are happening that might make it more severe. You mentioned the stress she was having before this new stuff started, and I think that is SO typical. I don't know how many docs acknowledge the stress factor; I'm not even sure how much mine does. But I think it is very real and should not be dismissed. So whatever is going on, if she can't take RTX and they decide she should take CTX, I wouldn't worry and go ahead and do it, as far as the toxicity factor goes, since you can do a lot to avoid that being a problem, the main thing being to take it in the AM, drink lots of water, and urinate often! Another would be to be on the correct dose for her body weight and go for it with the idea of making it as limited a number of months as possible. Some say no more than three months for CTX but many of us have been on it for much longer. As soon as significant improvement is seen, it would be ideal to switch to a milder drug such as MTX or Imuran. But all the drugs have their issues. As for the fertility issue, I can understand your deep concern over that. I would do some more investigating as to how many women have been able to go ahead and have babies after being on CTX for some time. I think it is possible and that there has been discussion of it here on the forum, so you can search the archives. Good luck with it all, and we look forward to hearing how it goes!

[hrnn]

Hi! thanks for the replies!

I spend the day doing research yesterday and I found a review article comparing the present alternatives to medication for ANCA+ vasculitis available (I posted it in the drugs/medication section of the forum). I did also some research on some particular medications such as cellcept, IVIg and even an experimental one (which I can recall the name at the moment) which struck me as the most suitable choices for the present condition of my wife. I'll look more papers on the subject so I'm prepared to meet the doctor on thursday.

By no means I'm considering myself an expert, but from what I gathered so far, although she is having now her eye involved, I don't think she qualifies as a severe case (I should read some more on the disease activity scales as well), so I'm inclined to consider the CTX an overkill (pun intended) and that less aggressive alternatives are just as appropriate.

Still lots of reading to do.

Thanks again everyone!

[drz]

so this week we'll meet with the doctor to discuss the alternatives. so far it seems that his medication of choice is cyclophosphamide, which we are not exactly happy with due to its toxicity and the possibility of leaving her infertile.

would someone want to input on this?

thanks all!

Some people have harvested eggs before under going chemotherapy just in case they are needed later.

[hrnn]

Hi!

yes, harvesting eggs is a posibility but very expensive to say the least. we are considering it, of course, if it comes to no other alternative than taking this medication.

an update on the case:

the situation with her eye got worse. she got an inflammation in the area again which is pushing the eyeball out giving her double vision. they haven't decided yet if what appears to be pushing is a granuloma or not, so maybe we are back on GPA.

after insisting a lot for alternatives the doctor is going to give her a mix of infliximab and metroxetate starting anytime this week, and she got her prednisone up to try to get the eye back to normal.

hopefully this will finally work. I'm somehow possitive about it as I read good things about mtx in general as the prospect of mixing it with infliximab as well.

thanks again for the feedback, everybody!