Suspicion of MS after Wegener's Granulomatosis

[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?