For those with Sjogrens
 

So, I have not yet been diagnosed with Sjogrens and I was in a bit of denial that I had it because I don't have the traditional dry eyes, dry mouth. But I recently read a book about Sjogrens and it was like my entire life made sense to me. Chronic bronchitis, sinus infections, dry cracked lips, watery eyes, choking on food, anemia, chronically dry skin - MY WHOLE LIFE summed up. And now this small fiber neuropathy taking over.

Here's my problem: I don't have the antibodies and I have RA, so my rheumatologist is dead set on me not having Sjogrens. But I sincerely think I do. How do I fight her for a lip biopsy? Do I just need to get a new rheumatologist? I am so annoyed that I have to sit over here and diagnose myself while I worsen.

And what does treatment for Sjogrens look like as far as neuropathy is concerned. Do DMARDs really do anything for neuropathy? Is the only option IVIG? I just need a dang diagnosis so I can go forward.

Thank you!

I think I'm probably ideally placed to reply to this, having been diagnosed with seronegative RA five years ago and treated with four DMARDs which I had severe reactions to. My RA symptoms never came back but the neurological symptoms have continued and progressed. Finally I've been rediagnosed with primary Sjogrens on the strength of a very positive lip biopsy result three months ago. I had to wait for RA drugs and steroids to be fully out of my system to be taken seriously though. I've also relocated a couple of times, which led to my autoantibody bloods being retested in a new hospital. My ANA had switched from negative to clear positive and this led the new rheumy to ponder what could be causing my clinically diagnosed small fibre neuropathy - hence lip biopsy. You would need to be well clear of all RA meds to make this worthwhile though. And secondary Sjogrens can be just as severe and sometimes more active and more of a problem for you than the primary disease. However lots of rheumies seem not to realise this and refuse to acknowledge that Sjogrens can be the more severe disease or even that RA is secondary to the pre-existing Sjogrens - as I believe was the case with me. I too read the Sjogrens Book and my life seemed to fall into place. I think I've had it forever and my hypothyroidism and RA were both part of my Sjogrens. My new rheumatologist and neurologist both agree with my theory.

The bad news is that, apart from Plaquenil, there are usually no treatments offered for Sjogrens apart from topical ones and symptomatic nerve pain meds. I know that this is a great source of misery for many sufferers and things do seem to be improving in the US - with Sjogrens World Foundation and Dr Mandel's excellent articles and Professor Birnbaum's work at the John Hopkins. But I am not sure this has yet filtered out across the international rheumatology or neurology communities yet.

I am diagnosed with "borderline" sjogrens as the cause of my full body neuropathy. I had the lip biopsy and now 2 years later still have a numb spot in my lower lip. I agree with the bad news of plaquenil being the only other autoimmune treatment offered. For this reason I wouldn't recommend the biopsy. I decided not to try the plaquenil due to the fact that I have no swelling at all, and that seems to be what it is helpful for. I might change my mind at some point, but for now I continue to try for other opinions and options.

I think the decision over having a lip biopsy should be made according to what you personally would gain by having a diagnosis of Sjogrens. In my case I'd have a hundred more for the result it gave - i.e. very high numbers of lymphocytes on each salivary gland. But if you are already diagnosed and treated for RA and you know that you do have RA, then I'd be wary of having a biopsy. The drugs for RA can mask and give false negatives and treatments are similar anyway.

Secondary Sjogrens can be just as severe and debilitating as primary Sjogrens so knowing you have it is the thing. It helps to have your doctors looking out for Lymphoma though as having Sjogrens significantly increases your risk.

Personally I have no more numbness from my lip biopsy than I previously suffered (a fair bit) and barely a scar to show that I had it done 4 months ago. However my rheumy said he couldn't recall the last positive of this kind that he'd seen so I may be quite unusual in this respect.

For me it's very worth knowing that Sjogrens is my significant disease just for peace of mind and long journey and research and powerful instincts and some battles with the Scottish medical profession, all vindicated at last!
As I can't tolerate the RA meds or symptomatic drugs I am in two minds about treatment options although I'd love to at least be offered IViG or Rituximab and would settle for trying Cellcept/ Mycophenolate. I do think Sjogrens is a seriously underestimated disease and I get very cross that other rheumatic and neurological diseases take precedence with big gun medications.

Sounds like you need to find a new Rheumy if they won't order a lip biopsy. At least 40% of those with Sjogren's are what they call 'sero-negative'...meaning the antibodies are negative. Some may have a positive ANA but still have negative SSA & SSB (Sjogren's antibodies) as in MAT52's case. The lip biopsy is the gold standard, as they say for Sjogren's, so it's important to have this done...by someone that is experienced and read by a pathologist using an appropriate grading scale.

Don't worry about primary vs secondary...it really makes no difference and treatment is NO different for primary vs secondary. If you have Sjogren's then it can be mild or severe in either case. It's just a matter of knowing, so you can treat accordingly.

As for treatment, DMARDS do NOTHING for neuropathy!!! DMARDS are for symptoms relating to the Sjogrne's itself (like fatigue, dryness, and joint pain) and to help slow progression.

IVIG is the best treatment for neuropathy...and some immune suppressors, like Cellcept.

Do you have a skin biopsy to confirm the SFN? You'll need this for approval of IVIG in most cases (due to cost and insurance reluctance to approve).

So talk to your rheumy again and directly ask for the lip biopsy to confirm or rule out the Sjogren's. If he/she won't do it, then find someone else. it is really not uncommon have to see more then one or two rheumys before getting properly diagnosed. I saw several and it was almost 12 years before I was Dx...because I am sero-negative. However, my lip biopsy was so positive, it stated that it showed Sjogren's at end stage.

Be you own advocate and persistent about getting the care (and Dx) you need. The longer you wait, the harder it is to reverse nerve damage!!

Hi enbloc,
what treatments have helped you the most? Do you have a daily regimen?

I take Plaquenil (DMARD) daily for the Sjogren's itself and it's related symptoms and to help slow progression.

I also receive high dose IVIG for the neuropathy as a result of the Sjogren's...and it also benefits my primary immune deficiency (CVID).

The neuropathy damage is mostly permanent for me, as I went so long undiagnosed or treated. However, I do get some benefit from the IVIG to help with nerve/SFN related pain and some autonomic symptom relief.

Sjo by Nicox
 

Has anyone had this test done? My ophthalmologist is looking into it for me. I tested negative for the Ro and La antibodies. I have not had the lip biopsy done and thought this might be a good alternative.

Mskari, I have been asking for a lip biopsy but my rheumatologist and a colleague of that rheum all refused to do a lip biopsy where there is nothing else. I have full body Sfn but no positive Ana or dry mouth. I think that sjogrens primarily involves B cell activity (I think from memory so don't quote me). If this is the case there are particular drugs that target B Cell proliferation specifically. I think the key one is rituximab. This one has already been mentioned in a previous post. And of course there is the ivig. I think it would be helpful to know. With no other key 'markers' such as the eyes, and mouth issues and blood to go on it seems to be the norm that rheums will refuse to do a lip biopsy

Thank you immensely for all of your replies here. My rheumy won't orchestrate a lip biopsy - just saw her on Monday. She is convinced I don't have sjs. She said she'd see it in my blood work somewhere, even if I am an antibody negative, and then she retested the whole spectrum of autoimmune and inflammation blood markers.

Interestingly enough, my RA has been quiet, so not surprisingly my CRP and ESR came back normal. She attributes all of this to the methotrexate and I'm sure she's right, but I also stopped eating grains, dairy, and all gluten, too AND I am on LDN.

I saw an eye doctor who told me that my eyes are not dry. I just don't know what to think or what to do. My neuropathy is much better when I don't eat sugar, grains, gluten, and dairy but I can't tell if it's reversing in any way. And then to have these normal inflammation markers now makes me feel like RA is my only autoimmune disease and now that it is under control and I don't have any inflammation - what the hell is causing my neuropathy?