questions about Sjogrens
 

I was at my opthamologist the other day for a FU as he has been treating me for chronic dry eyes. I'm now on Restasis and this regimen is keeping the chronic blepharitis at bay, finally. He recommended that I be worked up for SS due to this and my complaints of dry mouth, which my peridontist found a change in my readings this year, indicating that this is becoming more of a problem. I have had bloodwork done for SS and that has come back normal, but the Opth. Dr. said that 30-40% of people with SS don't show up positive on the blood tests and still have it. This could also explain some of my other nerve problems aside from PN. Does anyone know which doctor I should bounce all of this off on as the Opth. Dr. indicated that he feels a lip biopsy should be done. I have my GP, neuro, or a rheumatologist to choose from, or perhaps I should consult all three, I don't know. If anyone has any thoughts about this, I'm all ears and would appreciate any guidance/info you can share.

The rheumy would be your best bet for a proper Sjogren's work up. Your ophthalmologist is correct that many with Sjogren's are sero-negative (closer to the 40% then 30%). The lip biopsy is the definitive test. Your ophthalmologist likely did a schirmer's test as well. I have negative blood work for Sjogren's, but a very biopsy lip biopsy result. The biopsy looks to see if lymphocytes have infiltrated the salivary glands. There is specific criteria as to the infiltration for a Sjogren's diagnosis. There must be at least one focus (an aggregate of 50 or more lymphocytes, histiocytes, and plasma cells) within a 4 mm sq area for a positive result. There are several doctors that can perform the biopsy (dermatologist, ENT, and oral surgeon are the most common). It's just as important to have someone with experience do the biopsy as it is the pathologist that reads it. It is not a common procedure so ask about experience. I will be honest, many times the biopsy leaves a small numb area in the lower lip and sometimes a small knot as well. They can be permanent. I have both, but they are not bothersome.

Sjogren's can explain the dry eyes, mouth, PN and many other things as well. I don't know if your ophthalmologist told you, but using a small amount of baby shampoo on a washcloth and washing around your eyes can help control the blepharitis.

If you have any questions, ask away. I'll do my best to answer. If you are on the east coast, Johns Hopkins has a good Sjogren's Center.

Thank you for responding. I've done the baby shampoo eyelash scrub, the steroid drops and gels, etc...and until he switched me to lodamax for a month along with the restasis, and then just restasis, I couldn't get away from the chronic never ending blepharitis. I still have some eye problems and pain, but at least the blepharitis is staying away now. This has been going on for a couple of years now. I hate that Restasis is the latest and greatest and most expensive and it only works as long as I use it, but it is working right now in that respect so I'm staying with it. I'll have more questions as I learn more so I'll take you up on your offer. Right now I have to get records together for the next rheumy appointment and see if he acts on it. I'd love to go to Hopkins but I'm sure my insurance would prohibit it. Do they send all the biopsies there for testing like they did when I had the punch biopsy for PN?

Yes, they can send the slides for examination/reading upon request, I'm sure. You might be surprised that your insurance could cover a visit to Hopkins. It's not like it use to be where we had to stay within a certain radius. The Sjogren's Center does take some time & leg work out to get in...like most elite places.

Thank you, thats good to know. I have an appointment in a week and half with my rheumatologist and I've already got some of the records I need. I'll just have to get to two other doctors offices to get the remaining ones ahead of time and I should be good to go. I'll post his thoughts after my visit with him.

Thank you for responding. So far all bloodwork for RA has been negative. I have been having joint pain recently though and I will tell him about it. I experienced what I think may have been a migraine which included vomiting and I'm still having some pain from it. I'm not prone to migraines and I'm heading to the doctors in a little bit here. I seem to be falling apart lately.

I saw the doctor this morning and he feels there is sufficient basis for ordering the lip biopsy. He said that if it is positive, it would explain some things medically, in my case which seems to so far have a bunch of doctors stumped. Anyway, they are scheduling everything and will call with appointments and such so I just need to wait to hear from them.

Good. Glad to hear they will do the biopsy and maybe get you some answers. Please keep us posted.

Lip biopsy results
 

The ENT's office called that did the biopsy. They said that the results show chronic inflammation and they are deferring to my rheumetologist for further direction. I don't see him for a week or so. Does anyone know what this means?

I chased the Sjogren's bus for 15 years. I have a highly +ANA-nucleolar, and the +lip biopsy, every sign and symptom including dRTA. Many rheumatologists will not even consider it as an option without a +SSA or SSB. I have been nailed with IVIG and IV steroids etc, and none of it helped with my worst symptoms which are all autonomic, including the dry eyes. My thought is, I am going to try to get scleral lenses for that, because I am freaking going blind. I have the diagnosis (negative SSA-B), but it has been meaningless for me. I won't discourage you from searching for an answer, but, it may not be Sjogren's and if it is, treating it doesn't always work, that is, if it is or isn't really Sjogren's. Rheumatology is one of the MOST disorganized specialites. I use the word disorganized because I don't want to call it what it really is.

I stopped my IVIG a few months ago, since it seemed to be making me more sick than not, after 3 years of it. It does wonders for some folks. I am not one of them I guess. But, I would not fault any one for trying.

I am curious tho, why there is such a profound increase in the number of severe dysautonomics and the LACK of solid research and lack of centers for treating this. Autonomic neuropathy is very serious and it seems that there is just no solid organization in terms of diagnosis and treatment, or should I say managment.

I am sitting in bed with :paperbag: over my head right now, so, take this post with a grain of salt. I don't mean to discourage you. Definitely get tested for autoimmune disease, but it is a whole 'nuther can of worms.

You're not discouraging me. I'm really fed up at having so many problems that no one seems to have answers for. I'd really like to know where this is going and I'm still clueless. I have a lot of autonomic problems too, but I'm not sure of what is causing them or where they are going either. Anyway, this is just another confirmation of something I already know and that is I suffer from some kind of chronic inflammation. If nothing else, it gives the docs another piece of objective evidence.

This is not enough information. The results should list a specific number of foci present in a 4 mm sq area (focus=aggregate of 50 or more lymphocytes, histiocytes and plasma cells). You should get a copy of the results.

"Chronic inflammation" is certainly a clue that something is going on, but it could be caused by many things...its the 'infiltration of lymphocytes' that makes it a Sjogren's diagnosis. The ENT's office may not have read the entire result to you (so typical for them to summarize on their own), so get a copy.

Glad you're seeing a rheumy.

Me too, I'm sure I can get a copy from them when I see them next week. In todays world, medical assistants call you with results and they don't have the training to explain results, only parrot what they are told.

Sarcoidosis causes the same kind of results on lip biopsy that you get as 'consistent with Sjogren's'. It's my thought, that if you can get serum correlation, you are on a better track to diagnosis. Many of the treatment for autoimmune disease are worse than the disease.

My doc recently told me I was too sick to have Sjogren's, and that IVIG would have helped by now.....which, was, my impression as well....sigh.

I strongly encourage you to keep pursuing diagnosis, because without a solid diagnosis, you can't treat. Your train may stop and you may get off at a few wrong stops, but, whatever you do, get back on the train!

I have exhuasted most options, it's possibly Sarc....(not all the pieces fit) or some hereditary or 'familial' dysautonomia, which has yet to be isolated on the genome, and unfortunately it doesn't seem like any one is researching it.:(

I just had blood drawn to check for HSAN III, which is THE Familial Dysautonomia, but, I am not confident that my heritage or ancestry is typical of this disease. Again....sigh.:Hum: I am the closest symptom-wise to Familial Dysautonomia....but I have an elevated ANA. I miss the mark on all the other HSANs, so they didn't test for those. I don't like the idea of hereditary, however, it looks like they are treating HSAN III with oral Kinetin, pretty weird, it's a plant hormone!

I have gotten off at a lot of stops. The geneticist is pondering whether or not to have my genome run....that said, he says he has to find some one interested in studying it. Unfortunately, they could have had 4 generations but my mom died 2 years ago.

I'm going to work on eating some appropriate supplements, get the hole in my ticker checked out to see if it is making matters worse, and hope for the best, which would be things not getting a whole lot worse too fast.

I saw the rheumy yesterday and my results aren't stated in the form you indicated. The results state that there is interstitial mild chronic inflammation includes a few plasma cells and while the inflammatory changes are not specific, they could be seen in the setting of Sjogrens if the clinical circumstances are appropriate and it further states that several lobules of involvement are noted. They want to prescribe plaquenil but only after my opthalmologist okays it. So I guess I have it.

There are different grading systems. The one I was referencing is the Greenspan scale which is used at Hopkins (where I had mine done) and many others (but obviously not all places). As long as it looks for infiltrates & inflammation and the findings correlate to your symptoms, which it did.

Yes, you should have a baseline eye exam (to include color blindness and field of vision checks) by an ophthalmologist before starting Plaquenil...and every six months after. The damage to the eyes from Plaquenil is extremely rare, but none the less important to be checked for regularly.

Plaquenil is not a quick fix. It can (and usually) takes up to 3-6 months before you notice any improvement.

So glad you're getting some answers. You may find other unusual symptoms have an answer now. There are many problems associated with Sjogren's. They may address your PN differently now with this diagnosis.

I am curious about that. I asked alot of questions about other problems I have and their relation to this and I didn't get any firm answers. I'll be seeing my neurologist in a couple of weeks and I'm curious to see what he says about it as well as my other doctors. I was seeing my opthalmologist every 4 months prior to this last time when he was finally comfortable seeing me in 6 months since using Restasis is keeping the blepharitis at bay. I also have pigmentary dispersion syndrome in both eyes which they have been following for a long time so they are being extra cautious over my eyes and thats fine with me. I have enough to deal with already.

I have a vitreous detachment. It happened a year ago, and my vision never returned to normal. It's slightly better than when this happened.

Frankly, I am wondering why they are not prescribing more Boston Scleral Lenses....my thought is to look into those. Insurance will be an issue and the cost is also an obstacle.

I couldn't use Plaquenil....I tried, but I itched to high heaven. Sigh.:Red eyes:

Ouch on the vitreous detachment. I have vitreous degeneration. I wonder if its the same thing. I have a long history of intolerance to meds and I worry that I may not tolerate plaquenil also. My list of medical allergies is growing and I now wonder if thats related to this in some way as well. I'm not familiar with the Boston scleral lenses, what are they for?

Speak to the doctor about the Plaquenil (after approved from the ophthalmologist) and starting it slowly. The typical dose is 400mg (total for the day). Some start with 50-100mg BID and work their way up. I too have numerous drug reactions, but am able to tolerate the Plaquenil.

I will do that. I always prefer to start new meds slowly so I'm pretty sure they won't object to it. Thanks for the excellent advice.