Maybe WG, have questions

[mcpucho]

Hi friends,

Best version of complicated long story made short...

Have autoimmune disease for a few years without firm diagnosis. Just started with a new rheumatologist - Dr Robert Speira in NYC - and our working diagnosis is Sjogren's. We discussed WG (aka GPA) as I have had ANCA and P3 AB on serology in past 2 yrs but results are inconsistent. My Sjogren's AB is also inconsistent, and not unheard of to have WG and Sjogren's, so case still open.

Main issues are small fiber neuropathy, muscle weakness (maybe myositis), horrible fatigue, general stomach issues (GERD, etc), recurring sinusitis, and weather related migraines (cannot say they are related to autoimmune, just a general pain in the ass).

I started Rituxan (RTX) summer 2013 with GREAT results. I did 375mg x 3 weeks. In 2-3 months I went from a housebound, painful and miserable existence, to being able to exercise regularly, travel, socialize, work part time. Almost a normal life!

I was told the RTX is more or less effective 6-9 months and then repeated. It lasted 6 months for me. I slowly but surely returned to baseline level of misery over next 3 months - fatigue, neuropathy, muscle weakness, the miserable works. I was retreated with RTX at 10 months, and at a higher dosage.

On May 15 and May 30 2014 I received 1000mg infusions. Today I am 1 month post treatment and my neuropathy is much improved, muscle weakness almost gone, and stomach issues resolved. At this point after my first RTX treatment in 2013 my energy was much improved and I was going to the gym. But today my energy is still crap, and that's because of inflammatory sinusitis problems which I think they may be WG. That's why I'm posting.

Early May 2014 I had a cold or allergy which lasted 1-2 days. Didn't feel sick and thinking allergy I bought some Allegra and it literally dried right up. Over next few days got bloody crusting and nostril swelling. Got RX for Nasonex from ENT and swelling cleared up.

It's important to state here that in general my sinus inflammation has generally worsened over last 4 years as my autoimmune issues have worsened. ENT has no idea what it is cause of inflammation except that it is likely "immunological". I have seen 2 other ENT's and they were more clueless so I have stuck with my guy.

So, thought allergy, took Allegra and Nasonex, thinking I'm all good. Notice some sinus pressure on face and headaches in AM over next couple of weeks. Bothersome, but not terrible. I was looking forward to my RTX treatments and didn't pay sinus issues much attention.

About a week after second RTX infusion my sinuses suddenly berserk. Extreme fatigue, nasal congestion galore, neck stiffness, terrible nasal swelling to the point of nostrils being painful to the touch, 24 hr sinus pressure and pain, headaches. Total sinusitis fiesta.

Back to ENT and he says "turbinate and adenoid hypertrophy". No infection. RX prednisone 60-40-20 over 9 days. Improvement of swelling in nostrils, but really just getting worse. Now I have dizziness and ear pain, super duper dry eyes and mouth. I return to ENT and ask for allergy tests and CT scan. He offers more prednisone but I don't want to get on that horse so RX of inhaled steroid (budesonide) and antibiotic because (sure, why not?).

ENT calls me yesterday. I'm expecting some polyps or other type of obstruction and positive allergy test. Tells me no positive allergy and CT scan of sinuses is actually better than prior scan. Says maybe I need more immunosuppressive drugs. I remind him I just had RTX a few weeks ago. Says maybe I should see the neurologist. I say, no it's definitely sinuses. As my sinuses are worsening my neurological complaints are simultaneously improving because of the RTX.

Really he doesn't know what to say. We're both perplexed. I say let me try the RX budesonide and I'll make a followup appointment. If no response maybe we'll do the "turbinate reduction" procedure, see you in 2 weeks.

That's not much of a plan, and it doesn't get the source of the problem. Why I am having worsening sinus issues? Great, back on the dreaded merry-go-round of investigation again. So, what are we missing? I think WG, and here I am with questions.

Should I have a nasal biopsy?

When I started with new rheumy we had discussed nasal biopsy but as I was just about to be treated with RTX (RX from immunologist) we saw no reason to push nasal biopsy.

As I have exacerbating sinus issues that ENT cannot diagnose, and my autoimmune disease has no exact diagnosis, I was wondering if I should have a nasal biopsy done to rule WG in or out? I know that, like all tests, nothing is 100% and any diagnosis has to be big picture, but nonetheless.

Also what is involved with biopsy, etc?

For those with WG and sinusitis issues, what problems have you incurred and what has helped?

I had a good amount of bacterial sinus infections, but none of the inflammatory issues I have today, until about 4 years ago when my autoimmune issues started to be kick in and become apparent (had to stop working, etc). Since then I started to get extreme swelling and inflammation after bacterial or viral infections that warranted steroids. Sometimes a few courses.

Started getting to get crusty blood in nostrils seemingly all the time, and worsening sinus pressure in general. Also notice worsening dry eyes and photo sensitivity with sinusitis. A few times I have had adenoid hypertrophy with inflammation. This is first time I have had turbinate hypertrophy as well.

I found RTX, and am very grateful for that, but I thought that was the beginning of the end of my journey, so to speak. Seems otherwise in light of this inflammatory sinusitis that ENT cannot put a finger on. I can only come to the conclusion of WG, or maybe CSS, and at the far end of speculation, allergic fungal sinusitis, but that is a reach. Nasal biopsy could be helpful in determining one of the above.

Reuhmy and ENT are at same hospital and have good relationship. I see both in about 10 days and I would like to best inform myself before my respective appointments so I can have them carry out a plan for me.

I appreciate any insight and help you can give me from your own experiences.

Thanks for reading and best of health to you and yours,
Nathaniel

Go USMNT!

[BookNut]

I do not have Wegeners, but have debilitating sinusitis that often ends up causing extreme shortness of breath and wheezing. this has been going on for almost 4 years. After an allergist, 3 pulmonologist and two ENTs, my PCP suggested taking clarithromycin three times a week. I have not been on it long enough to make a judgement yet...but it is something to look into. It is evidently useful in reducing the number of exacerbations in COPD and also chronic sinusitis. I am also meeting with my allergist this week to talk about a possible IgG subclass 2 deficiency which can cause sinus problems. Have you had your IG levels measured? There is IgE, IgG, IgM and some otherr, and then there are subclasses as well. Perhaps the meds to surpress your immune system might have caused a deficiency? Just speculating.

You will soon get answers from the folks who are experts on wegeners...but I thought I would put in my two cents in case it might help.

[windchime]

Hi Nathanial and welcome to the forum. You have an interesting story and certainly a puzzler. Are you doing any nasal rinses? You don't mention them and many on here find them extremely helpful if not a necessary part of their routines. I use them them for my chronic post nasal drip and it helps a lot. I'm sure someone else will chime in soon with other advice on the rinses.

[Alysia]

welcome to the forum Nathaniel.
rtx should be given every 6 months, and it takes some time for some of us, untill you can feel its influence. so maybe you can still be optimistic about it.
as for nose biopsy, I did it twice. both were false negative. (I have wg for sure). if you will get it positive for wg, then you have it. but negative biopsy does not mean that you don't have wg. don't let any doc be misleading by that. (like it was in my case).
can you describe more, please, sorry for the questions: what is going on with your nose ? is it bleeding ? do you have crusties getting out of it ? are your eyes red ? did you went to eye doc (you should) ? do you have bleeding gums ? do you have pains in your joints ?
I have also colon involvement of wg so it is possible to have stomach problems, although it is more rare.
I hope you will get more anaswers soon, and more important, start to feel better. please update us.

[LisaT]

I'm not an expert, but it sure sounds like WG/GPA. Why put yourself through the pain and inconvenience of a biopsy if your docs are willing and able to treat you for WG/GPA without it? A few others here have had a difficult time with the biopsy. My diagnosis is still not 100 percent because it's based on symptoms, and I'm ANCA-negative. None of my docs in Canada have suggested a nasal biopsy even though they feel my nose shows obvious signs of the disease. They did suggest doing a biopsy of my lung, but the nodule of concern disappeared between CT scans (thank G-d). I think you said your ANCA is positive and that's pretty specific to the disease, from what I understand. As long as you're getting the right treatment, and they're not withholding drugs until confirming a diagnosis, that's what really matters, in my opinion. It would be nice to know for sure but if they treat it and the treatment works, that confirms it... I wish you luck and hope you start feeling better pronto.

[drz]

Dr Spiera is recognized as one of the world experts on Wegs and should be able to answer all your questions. You are in good hands.

VF Medical Consultants (http://www.vasculitisfoundation.org/mcm_resources/medical-consultants/)

[Dirty Don]

Welcome Nathaniel...I had/have 'hamburgered' sinuses. ENT was of little help tho she did a lot of other clean up in there. The only thing that helped my sinuses calm down and start to feel better was pred. It's not a nice horse to ride, but if there is no other way out of town...you'd better hop on that horse. Seriously...the sinuses will calm down but the inflammation of whichever disease you have needs to be controlled first...pred does that. Best to you.

[mcpucho]

Hi all. Thanks for replies. Will try to answer questions summarily.

- I have an IgG defeciency and was diagnosed with Common Variable Immune Deficiency (aka hypogammaglobulinemia) in 2006. I have had sinus issues dating back to 2002, with a surgery in 2003. I have received IVIG monthly since 2006, and twice a month for the last 18 months as my autoimmune disease went into full throttle. Immunologist ordered 2 days consecutive every two weeks around RTX treatment as to not get over suppressed. Hoping to go back to once a month treatments soon because it's a pain in the ass treatment.

- Yes, I do saline nasal irrigation regularly, and have also used AYR saline spray multiple times a day for the last year. Doesn't really help unfortunately.

- Have had nasal crusties develop and worsen over last 2-3 years. Worsened during recent gap in RTX treatment but stopped when my turbinates and adenoids hypertrophied (swelled) a few weeks back. Right now no crusties just sinus pain/pressure, headaches, etc.

- I am aware that a negative nasal biopsy would not rule out WG, but it could if it could provide a de facto cause for my sinus issues that would be very helpful. My ANCA and PR3 has been very inconsistent. Also, if I need more immunosuppressive meds I need a compelling reason or my immunologist won't sign off. Moreover, I've had autoimmune disease for a few years and it would be nice to have a firm diagnosis!

- My eyes have been dry off/on for a few years, but only started to get really gritty and red in last 2-3 weeks, with "veins" protruding from corner of eyes and towards pupils. I don't know if "uveitis" is the right term? Last eye DR I saw was 2 years ago and everything was normal, but things have progressed since then. Probably a good idea to get checked out, thanks.

What do folks use for eye issues? I haven't found any OTC solutions to be helpful.

- Joint pain is super mild and almost non-existent. I'd put it at the far end of my complaints. Neuropathy, fatigue and sinus issues are main complaints.

- RTX treatment has been very good for me, and I know it takes a few months to get full results so no despair at all. Dr Spiera stated we should repeat at 6 month intervals in the future. Still, not sure why sinus issues continue to worsen, along with eyes, even after RTX treatment. My neuropathy and other symptoms are improving simultaneously.

- I just started with Dr Spiera and glad to hear he is considered a "world expert" in WG. I was referred to him by an oncologist who is treating my mom for cancer (she is doing great btw). Interestingly I thought I may have a vasculitis type autoimmune disease such as WG but I couldn't get any Dr - all of them excellent Drs - do any type of biopsy over the course of 3 years. Dr Spiera was first to truly entertain the idea and mentioned a nasal biopsy, which I didn't even know was a diagnostic option. Alas, he was the first Dr to put the sinus issues into the bigger picture and the first to mention WG as a potential diagnosis.

Wondering... does anyone take steroid sparing meds like MTX or Azathioprine with RTX? Are they helpful for WG? Like everyone, I hate prednisone. Just did a 9 day course and took me a week to recover from moodiness and subsequent energy crash. The thought of doing high dose pred and taper again is a nightmare, as I'm sure you all know to well.


Glad I stopped here and decided to post. I really appreciate all of your help and will keep you posted as to what develops.

Nathaniel

[mcpucho]

Welcome Nathaniel...I had/have 'hamburgered' sinuses.

Hi Don. What kind of issues specifically got you "hamburgered"? (nice description btw).


The only thing that helped my sinuses calm down and start to feel better was pred. It's not a nice horse to ride, but if there is no other way out of town...you'd better hop on that horse. Seriously...the sinuses will calm down but the inflammation of whichever disease you have needs to be controlled first...pred does that. Best to you.

I know, I know, but hate the pred. Have you ever used nebulized steroids like budesonide? I have had success with that in past but only in combo with nebulized antibiotics for infections. This is first time I am using it solely for inflammation. Dr RX'd only because I said I didn't want more pred. I have a 4 week RX.

I am using the "Atomizer" from ASL Pharamacy. They don't charge for the device, only co-pay for meds - ASL Pharmacy: Topical sinus therapy for patients suffering from chronic rhinosinusitis (http://www.aslrx.com/products.html)

Unfortunately as disease has progressed, sinus issues are always worse than previously and bigger doses of pred are needed. Going to give this atomizer a run for the money for at least 2 weeks before more pred. I have 3 pred burst refills available at pharmacy taunting me. I live less than a block away, fighting the urge to give in.

[pberggren1]

This thread is broken. Hopefully this will fix it.

[chris.wg]

Hi Nathaniel. Nasal biopsy is a tricky call. My doctors didn't even consider it because of damage already caused to sinus cavity plus the fact that it has a high rate of false negatives. If you are on rtx though I don't know if they will find another area of inflammation to test. My diagnosis involved a needle biopsy of my lung with infected tissue. As for the prednisone horse you may already be on it and not realize it. I was not aware my rtx infusion had methylprednisolone, which while technically different is very similar and almost the same thing, as part of the infusion. Getting on a high dose is always no fun. But many have gone with a low dose prednisone for managing symptoms after they are under control. Not ideal but if your doctor suggests it I would give it serious consideration. Only other advise I really have for you is keep looking and pushing for a diagnosis. It sounds like they are treating you for WG but kind of touch and go. If it is Wg it will allow your doctor to be a bit more aggressive knowing this. And if it ends up being something else your treatment options may vary from alittle to ALOT and go a long way to making you feel better.

[pberggren1]

Fix please.

[godgirl]

Hi, Nathaniel. Welcome to the forum.

I have a lot of eye issues with my stuff. For a while, when my dry eye syndrome was first diagnosed (a couple of months ago), the eye doc put me on prednisone drops. They helped, but then I had to find something for after it, since it wasn't going to go away. I use Systane Balance, but I also use the Systane Overnight Therapy gel. I put it in right before I turn out the lights. Personally, I've found this helps a LOT. It's a thick gel and since I'm sleeping, it stays put. But I use Systane Balance drops throughout the day. Hopefully, you'll be able to figure out everything that's going on, and get better soon!

[mcpucho]

I use Systane Balance, but I also use the Systane Overnight Therapy gel. I put it in right before I turn out the lights. Personally, I've found this helps a LOT. It's a thick gel and since I'm sleeping, it stays put. But I use Systane Balance drops throughout the day.

I happened to buy Systane Balance today. Works much better than the Systane Ultra. I'll look for the Overnight formula, as I also wake up very dry.

[godgirl]

I agree with you. The Systane Ultra didn't work as well. The Balance was much better.

Also, if there's any pain with your eyes, get to the eye doc. Scleritis, uveitis, etc can be part of a flare. And without treatment (prednisone-ugh), you can damage your sight.

[annekat]

Welcome, Nathaniel. Just wanted to say my nasal biopsy was no big deal and it provided a positive diagnosis. I already had a saddle nose at that point, so there was already some damage, plus the nose made the dx pretty obvious. But the process was not overly unpleasant, there was a numbing agent given, and it was done right in the ENT's regular examining chair, on the spot, as soon as I went in and showed him my saddle nose. There was some bleeding during the process, but in my case I think he was mainly taking scrapings from the hole in the bony part of my septum, which I guess could be less invasive than cutting into inflamed tissue. In any case, if a positive biopsy was able to be obtained, things would be more clear and you could proceed from there. RTX sounds great, but I agree that some high dose pred would help a lot. I don't know how much was in your RTX dose, but you might need to continue it; people I know who use RTX haven't stopped the pred. I know some of us hate it more than others and am truly sorry if you have a big problem with it. I wonder if it is possible you could get used to it. In any case, I too have heard great things about Dr. Speira, and it would be interesting to know whether he thinks you should get a biopsy and what he thinks about the pred. I apologize if I'm missing some of what you said here without going back to the first page.

[chris.wg]

Well Annekat right now I am slotted to be pred free again once I step down. Remember I am switching from Cytoxan pred free to a rtx treatment had to get on the pred again to manage symptoms for the switch but who knows. I am hoping to stay off of it ... but my advice to Nathaniel is as much advice to myself in case when all is said and done the doc says you may need to stay on it. Sort of hoping for the best preparing for the worst. But if you have faith in your doctor and trust your doctor then you should never dismiss what he says lightly. If you don't then find a new doctor.

[annekat]

Chris, I sure hope you are able to get pred free! Some are able to and some aren't, and true about finding a doc you trust so you can follow his suggestions with no worries. I don't have the best doc but he seems to able to work with me on the pred, trusting my judgement if I need to go slower than he suggests. So far I haven't been able to get below 7.5mg without flaring. I'm on 15mg. MTX and wonder if upping that just a bit might help me need less pred. It certainly is a balancing act. I do think I need a doc who is more experienced with WG. There is no one quite as well thought of as Nathaniel's Dr. Speira in my area, but I hear there are some competent vasculitis docs within 70 miles or so of my home.

And I do appreciate clarification of your situation with having to start pred again after starting RTX. I, too was on Cytoxan, but then switched to MTX and have never been off pred that whole time. RTX could be in my future, but I won't rush into it unless I become quite a bit sicker than I am now.

[chris.wg]

My old doctor, <old because he suggested I go else where not because I didn't trust him, as he put it he was not ashamed of anything he has done in his treatment but he isn't sure how much further he can take me> Wanted to get me off the prednisone as soon as possible. It took a few months to step down from 60 and as I was stepping down he kind of left the judgements up to me he gave me guide lines but said if you start to feel pains or flares and need to step it back up do so. Just keep me informed. I only had to do this once my initial step down he went from 60 to 50 and that caused some trouble so we stepped it back to 55 and did 5 mg step downs from there out. So when I seen my new doctor he was pleased to hear I wasn't on prednisone but also knew what I was about to face in the switch and talked with me about it. We are both hopeful and optimistic I wont need it again but .... as I said time can only tell.

[lag713]

Hi Chris and welcome! I'm sorry I'm a bit late to join the discussion. I think you're in good hands with Dr. Spiera. I've heard good things.

I only have sinus involvement but I did not get a nasal biopsy because my case was caught early and likelihood of a false negative is very high. I do sinus rinses and use nasonex but I haven't found much to help really. Massages are my favorite alternative medicine.

I hope you get a definitive answer soon. Wishing you the best! :)