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!