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?

Were you ever tested for celiac with blood work or scope? That can take a long time to heal the nerves. Also RA is an autoimmune process that has a name. Many don't. Whatever is attacking your joints can also be going after nerves. Do you also have dysautonomia?
Also, would your doctor let you try the plaquenil for the RA? I have a friend who only has an official diagnosis of RA and her doctor offered it. As far as I know that would the only other thing that would be offered. Were your vitamins and minerals checked? b-12, b1, b6, D, E, folate, iron, ferritin, copper, ceruloplasm, zinc?

Hi health girl. Yes, I was tested for celiac. Negative. I did have positive anti-gliadin tests showing the gluten antibody.

And you're right about autoimmune processes without names. Even my rheumy told me she sees neuropathy mediated by autoimmunity that doesn't fit into any existing autoimmune disease. She said it could just be a separate mechanism where my immune system is going after my nerve tissue. I don't know about gluten, to be honest.

I have been anemic all my life (iron deficient) and still have issues getting my iron up, although supplements DO help, it's just slow. B6 was lowish, but normal. B12 was lowish, but normal. B1 was fine. D was extremely low, but improving. copper and zinc were fine. Folate was lowish, but normal. I am supplementing all of these things.

When I was 19 (I'm 31 now) my longest toe on each foot went numb and have been numb since. I never thought much of it until all of my toes went numb last year and progressed to this full body neuropathy I am dealing with now. I think the incident when I was 19 was the beginning and I have had an autoimmune mechanism happening for a lot longer than I initially thought (RA didn't start until I was 27) - something definitely proceeded the RA. but with these normal inflammatory markers and no other symptoms, I am lost.

Have you seen Dr. Oaklander's lecture about SFN?
She talks about SFN that starts in young age, the high prevalence of autoimmunity and the effectiveness of immunological treatments.

I must say thanks for posting this video here stillhoping. What an amazing woman Dr Oaklander is. I've read some really excellent published material by her as well but never seen her talking on video before.

I'm interested in this thread because I do have confirmed Sjogrens,which presented as RA for a few years. But also because I think my SFN is milder than what many describe, but is very longstanding and widespread/ diffuse and has plateaued so that most of my sensory issues relate to past damage of small nerve fibres, which I believe is permanent i.e cannot be regenerated now.

However, having spent most of the past two years off immunesuppressants and steroids, my full on RA symptoms haven't returned. And the burning and tortuous SFN pain have mostly disappeared so I think damage has been done now and my confirmed Sjogrens has been responsible for what has been and is now busy attacking my tendons everywhere instead.

For this reason plus a marked increase in my more classic Sjogrens symptoms, I'm starting Cellcept today, another disease modifying antirheumatic drug and also an immunesuppressant. As Imuran hospitalised me twice last year with pancreatitis, this is no small undertaking but I feel the rheumatologist would not have recommended it had it not been important to dampen my overactive immune system and tackle system wise inflammation levels once more.

To me your rheumatologist seems quite wise to say that your peripheral neuropathy is most likely to do with a pre-existing inflammatory process, but I think that if you want to find out about Sjogrens you should be allowed to have a lip biopsy. However in my case a lot of things only showed positive when I came off my gluten free diet and stopped taking Methotrexate and steroids. If your RA symptoms have gone, your bloods are normal but your need to know what is causing your numbness is still great - then perhaps you need to stop the Methotrexate and give your body a chance to be free of the toxicity (I mean by this that it is a cytotoxic drug) and see where this goes?

This is what I did about two and a half years ago but I only had a diagnosis of seronegatuve RA that was non erosive and was tolerating, even a low dose of injectable Methotrexate, very badly (GI issues). Now I at last know what my real problem was all along, i.e. primary Sjogrens, a lot of things have fallen into place for me. And neuropathy has been more of a worry to me than the joint and tendon pain - especially now I now that this is kind of RA is non erosive in my case.

So I think many doctors including my neurologist, do a great disservice to us who suffer from what they class as ideopathic neuropathy, by not confirming or relentlessly tracking down the cause (or giving us support and free reign to). All info on SFN says that if you can find the source then you should treat it ASAP i.e. if prediabetes then treat the diabetes etc.

Looking back my late dad had neuropathy from a young age and also gout and hypertension, but he only developed late onset diabetes when he was in his late 50s and he had his first full scale heart attack. However he used to walk with a cane from my first memory of him and often suffered from disequilibrium and fell easily. My mum always maintained this was because he was mildly deaf and over anxious about his health. But he had hyperacusis rather than hearing loss and I think this was probably part of his neuropathy. Looking back I can see a lot of my own symptoms in him and for me it has manifested as Hashimoto's and Sjogrens so I'm more autoimmune than he was. Nevertheless I'm now walking with a cane and can relate to him much more strongly with hindsight.

So I'd advise you to do whatever you need to do to get clarity on what is behind your own neuropathy -whether that leads you to lip biopsy, endocrinology and neurology and even brain MRI and lumbar puncture as I had. For me it's been worth every horrible investigation to know what is going on for myself. And once you've found out you can push for a more targeted treatment? But of course if the RA comes back you may need steroid shots and Methotrexate again so it is a definitely a risk you need to weigh up for yourself.

And as you know Coeliacs is an autoimmune disease too as is Pernicious Anaemia - so untreated these might well have done some long term damage to your peripheral nervous system? Perhaps your rheum is right to say you need look no further? Mat