autonomic dysfunction tied to small fiber???

[trc1962]

Posted so often here about my journey to find health again and how hard it has been to get answers. I began a flare in June that began like my flares always do - with sore feet and neuropathy that moves up my legs and arms. This time though it kept moving despite the prednisone, reached my lower back and stopped my bowel from working and my stomach from emptying. Now it has moved up my back into my neck and around my head. My neck feels shaky and it requires tiny doses of clonazepam to stop it through out the day. I have to take large doses of laxatives and then use a suppository twice a day to get rid of any waste. I had a small fiber biopsy in July which they said was inconclusive and therefore negative. The neuro refused an EMG and told me there was no neuropathy. I have dealt with my rheumy and he has tried to get me neurological help and treat my symptoms. Yesterday I finally saw a neuro and he listened as I told him everything and looked at all my scans and tests. There is no small fiber neuropathy he said because it was negative however, he said you probably have dysautonia and it is a problem and probably why your bowel isn't working. I am confused, my eye lids twitch and droop and my mouth gets tired chewing and that to me is neuropathy. In 2003 I responded very well to aziathiaprine and got my life back but in 2011 I had to stop the drug when it caused jaundice and liver issues at higher doses. Since then they have tried me on mycopholenate but usually just no drugs unless I start a flare and then it is good old prednisone and it isn't working this time. My sjogrens diagnosis is presumed, with a positive eye test and a lip biopsy that checked every box but one - now they want another one 11 years later. This new neuro said "I doubt you have sjogren's as it is rare and you don't have the markers and maybe it isn't sjogrens but how to get to someone who can diagnose this and help me? I am so tired of just surviving and taking Saturdays to be home so I can drink milk of magnesia and have a weekend "clean out." I love my job as a health and fitness teacher and I am scared I won't be able to do it much longer without help.I never knew the medical community was so inept as I have pushed very hard for answers. So sorry this is long but I am so tired of It all.

[irish]

I am going to throw a wrench in the works and suggest that you get a second opinion. You have some signigicant issues and with no positive Sjogrens diagnosis it is a quandryl

There are so many other autoimmune diseases that can cause these symptoms and also some off the wall neurological issues that are not so common. If it was me I would go to either another neurologist for assessment and possibly an immunologist. When you call to make the appointment ask for a doctor who deals with autoimmune issues and issues that fall through the cracks. There are some docs out there who like a challenge and that is the kind of doctor that you want. Good luck and let us know how your come out. Irish

[trc1962]

Thank you Irish. I am going to follow through with this second lip biopsy to see if it can guide if this is sjogrens or something else. I am also reaching out to the local neuro here to ask him to please look outside the box away from sjogren's and see if he has any other ideas. The neuro doing the EMG has promised a referral to U of W to an autonomic specialst he interned with if there aren't other answers. so we have some direction. I am going also to see a local naturopath who deals with mast cells issues as I have dealt with sensitivity to histamine for quite a few years. When all is done I shall report back. Thanks.

[MAT51]

Posted so often here about my journey to find health again and how hard it has been to get answers. I began a flare in June that began like my flares always do - with sore feet and neuropathy that moves up my legs and arms. This time though it kept moving despite the prednisone, reached my lower back and stopped my bowel from working and my stomach from emptying. Now it has moved up my back into my neck and around my head. My neck feels shaky and it requires tiny doses of clonazepam to stop it through out the day. I have to take large doses of laxatives and then use a suppository twice a day to get rid of any waste. I had a small fiber biopsy in July which they said was inconclusive and therefore negative. The neuro refused an EMG and told me there was no neuropathy. I have dealt with my rheumy and he has tried to get me neurological help and treat my symptoms. Yesterday I finally saw a neuro and he listened as I told him everything and looked at all my scans and tests. There is no small fiber neuropathy he said because it was negative however, he said you probably have dysautonia and it is a problem and probably why your bowel isn't working. I am confused, my eye lids twitch and droop and my mouth gets tired chewing and that to me is neuropathy. In 2003 I responded very well to aziathiaprine and got my life back but in 2011 I had to stop the drug when it caused jaundice and liver issues at higher doses. Since then they have tried me on mycopholenate but usually just no drugs unless I start a flare and then it is good old prednisone and it isn't working this time. My sjogrens diagnosis is presumed, with a positive eye test and a lip biopsy that checked every box but one - now they want another one 11 years later. This new neuro said "I doubt you have sjogren's as it is rare and you don't have the markers and maybe it isn't sjogrens but how to get to someone who can diagnose this and help me? I am so tired of just surviving and taking Saturdays to be home so I can drink milk of magnesia and have a weekend "clean out." I love my job as a health and fitness teacher and I am scared I won't be able to do it much longer without help.I never knew the medical community was so inept as I have pushed very hard for answers. So sorry this is long but I am so tired of It all.

I can relate to everything you are going through. If you read my recent neuro update post you will see that my neurologist takes a different view of the punch biopsies but my lip biopsy did show up very decisively for SS. One thing I really want to tell you is that, if you repeat the lip biopsy you do need to be fully clear of steroids and other medications such as Mycophenolate I've read. My immunology only showed clear positive when I was entirely clear of steroids and other anti-rheumatic medications. Mind you having a solid diagnosis has helped my self confidence and ensured I'm not dismissed but I'm still not being allowed to try Mycophenolate, Rituximab or IViG. Can't win!

https://fhs.mcmaster.ca/otolaryngology/documents/February2010MinorsalivaryglandbxsjorgrenBoscoLuiPGY5.pdf

[trc1962]

I had no idea that I have to be drug free for the lip biopsy to show, thank you for that information.
I am so very sorry that you are treading water like me and everything just moves at a snails pace at best. I am very happy you have a diagnosis and now we can hope for treatment that will improve things. I have lost a lot of faith in the medical community as a whole through out my ordeal and it is very discouraging. I don't care what I have, just figure it out and decide what treatment might work! I still feel very sure this is a form of neuropathy even if it didn't show. Thinking positive thoughts for you and hope for better treatment going forward.

[MAT51]

It has taken me six years to get the right diagnosis. In that time I've tried four DMARDs including Methotrexate, Plaquenil and Azathioprine and have had severe allergic reactions two three of these. Now they tell me that the other options, apart from Mycophenolate, are the big guns and without organ involvement I can't try these.

My skin biopsy was negative last year but the neurologist says this doesn't matter - I still have a clinical diagnosis of SFN from her and the previous neuro. But I'm sure the reason I would not qualify for a treatment such as IViG is that I need a skin biopsy result to confirm. So I kept asking and she got quite avooyed but surely it's my choice and I don't trust her clinical judgement alone is going to get me what I need. It's awful that we want bits punched out of us in this way but it is the reality. She did say that it might not show nerve damage because mine is so diffuse rather than glove stocking distribution, that it might be hard to locate the right part of me to biopsy effectively. If your symptoms tarry with a very diffuse small fibre neuropathy it might take more time to show up by biopsy.

[Nymph]

I have dysautonomia (but not the gastroparesis, thank God) and have had a skin biopsy that showed that my small fiber nerves are marvelously healthy. For me I think that my dysautonomia preceeded my Sjogren's so has a different cause. Sorry you are going through all this.

BTW, there is a blog called lethargicsmiles by a young woman with Sjogren's, SFN, and dysautonomia that is quite interesting and may be helpful to you in terms of tips on obtaining diagnosis and care. She is a very tenacious person.

[MAT51]

Thanks so much for tip about strong SS person blog. I think of myself as SS worrier as opposed to RA Warrior sometimes. I will check out this lethargicsmiles. Out of interest how can you be so sure that the dysautonomia preceded the Sjogrens? I have read that neuro symptoms of SS can start many years before the sicca symptoms appear.

I had many symptoms of both SS sicca and disequillibrium when I was in my early 30s. Now, inevitably, I wonder.

[trc1962]

I know I have small fiber neuropathy because I can feel it and it is very diffuse over much of my body including may neck, head and face. The small fiber sample was taken from a place where I have little symptoms plus I do believe that small fiber takes awhile to show up. I don't think I have CNS as the last neuro said is the only option and all that's left (they all look at the negative skin patch biopsy as the governing law). I don't have CNS symptoms and my scans are very clear with nothing but an old UBO I have had forever. The neuro wants a lumbar puncture but I have a chiari with herniation and to me it is too risky - wouldn't want to add new problems to what I already have. Why can't they just say you have small fiber symptoms and since we have ruled everything else out lets treat it. My rheumy mentioned IVIG but wants a new lip biopsy first and he said being on prednisone won't effect the outcome - who to believe? This is a long frustrating issue and I am hoping for treatment besides prednisone soon. Wishing you all a good Saturday.

[MAT51]

Your choice of course but I'd come off the Prednisilone for a good few months before having the lip biopsy. I can see why you don't want the lumbar puncture though. There are only so many hoops you can reasonably jump through. I've jumped through all of them and all have shown up sjogrens and an immune mediated SFN as widespread as it can possibly be. The lumbar puncture took 7 attempts and I travelled home in a wheelchair. I also have ganglionopathy/ dysautonomia. But no one has even mentioned IViG as a possibility. So you've more options than me at least

[Nymph]

I believe that my dysautonomia may be caused by a genetic collagen disorder called Ehlers-Danlos hypermobility syndrome. I have hypermobile joints, although not to an extreme. And from childhood I have had what I now know id blood pooling in my feet, and exargerrated autonomic responses to small changes in blood sugar (the shakes). This got worse with Sjogren's but seems to be worsened with immune activity of any kind. It is possibly one of the most common causes of POTS. That is why I think that. However, my immunologist thinks it is the other way way round, and he's a pretty bright cookie, so my opinion is certainly not conclusive.