Small Fiber Neuropathy And Pots - Chicken Or Egg....?

[Goschi]

Hi folks,

I am among the 50% POTSies who have small fiber neuropathy along with my dysautonomia. So far, so "good". I just read through the numeous studies I collected about this issue and realised that there is already no doubt about a clear connection between both conditions.

But reading the studies again, I started to wonder that I never found particular any remarks about whether the small fiber neuropathy is the (possible) cause of POTS - or maybe just the other way round, POTS may actually cause SFN...? Does anybody know about studies or other medical Infos where docs say more than that these two conditions are "connected"....?

Thanks in advance for any help!

[Nymph]

I don't know, either, but would be interested to find out as I too have both.

[gjensen]

I would say POTS is the result of peripheral denervation (SFN) in cases where it is present. Not the other way around. I would say to chase the chickens and eggs would be to discover (if it could be) what caused the SFN.

[Sunshinegirl]

I don't have any real answer for this but I did get a sort of answer from my neurologist when he took nerve samples from my legs. I asked him what this would have to do with POTS. He said that the same small nerves that he was collecting were present in the rest of the body such as the heart. If there was evidence of nerve damage in one part of the body, it would likely point to small nerve damage in other areas of the body. If the nerves in the heart are not firing as good as they should, it would account for the elevated heart rate. This may be why many people with POTS show this pathology.

For this reason, he said I had do to a list of things to protect my heart from working harder than necessary while also not becoming deconditioned. The main things were to wear compression stockings not just to increase blood pressure but to assist the legs (the second heart) in brining back up the blood, To sleep at a 45 degree angle upward and to do specific exercises that focus on leg strengthening that work in a pumping like motion such as treading water or the recumbent bike. I also have to have a ton of salt and water to combat low blood volume so my heart does not have to work as hard. When I put these things to use, I saw near immediate benefit from a few of them.

[Goschi]

Thanks to everybody's feedback!!

I was also tempted to believe that SFN means you could have general autonomic neuropathy which eventually causes POTS. That sounds quite logical.

However, it doesn't fit at all with my personal timeline. My first POTS symptomes date back to 2011 (maybe even 2010).
In 2013 my POTS finally reached it's full flare, yet still undiagnosed. At the same time, I had my first skin biopsy for SFN - negative! A couple of weeks later I eventually got my POTS diagnosis.
6 months later I had my second skin biopsy - "borderline" SFN! Shortly after that, my POTS started to slightly improve. Another 6 months later, my POTS was definitely improving more and more. At the same time, I got my third skin biopsy - now definite SFN!
Today my POTS is still improving and I am on good way to recovery I would say - however, I do have clear SFN...
And the POTS recovery can not be due to meds, ("masking" the neuropathy) as I am tapering down all of them (which even helps me more!)

So, in my particular case, POTS definitely preceded the SFN - and today I have mostly recovered from POTS, but seem to have full blown SFN - I actually experience the various symptoms of neuropathy in my legs (pain, tingling, numbness, etc.).

Being very confused - any ideas/experiences from anybody...?

[Sunshinegirl]

I too relate to you experience but... Let me think out loud for a moment...because..

Its not to say that one causes the other. It just happen to be present in at least 50% of the population. Relation does not always mean causation. Some things, like your SNF issue can be progressive. Pots can wax and wane for some and be debilitating for others.

If we think about the nervous system and what we know about dysautonomia, how it got its name, we are talking about the part that goes haywire... in this case is when the autonomic system runs amuck. This is not due to the small nerve fibers alone. What causes demyelination or neuropathy may only be related.

To me, this condition has been like doing a 1,000 piece puzzle with all the pieces turned upside down. One of the most telling things for me about these conditions are when you read the medical history under peoples profile and then tabulate the similarities. (I still don't know how to create mine). Look at how many had Lyme. Look at how many have MTHFR, MCAD (mast cell), GI issues....the list goes on but they are all very similar.

Lyme for example: We know that the most in the medical profession don't know how to properly treat this nor will health insurance companies pay for the full treatment....that is why people only get 8 weeks of antibiotics. Speak to many with Lyme and you will find that most don't ever really feel "right" again...or not for a very long time. Putting these pieces together with my own experience of how many neurologists first ask me when I had Lyme.... I did not have Lyme. This does not mean that I don't have dysautonomia. I have MTHFR, MCAD, POTS, TBI, SNF damage.

These are just my observations and I think I just cracked all my eggs

[Nymph]

In my case, I do not know whether one caused the other. Sjogren's could have caused both. Or hypermobility could have predisposed me to POTS and Sjogren's, which then caused the SFN. Or... or...

It does seem like SFN is commonly caused by Sjogren's. I will be interested if I ever get treatment for that, if it also helps my POTS or slows the progression. I'm not sure how I would know if it slowed progression of anything, though, as my symptoms of everything fluctuate ALL the time!

Must be time for a nap.

[Goschi]

@sunshinegirl: I think, you are totally right - who actually knows whether one condition causes the other at all? Docs seem to be not at all sure about that! And you are again right about the "1000 piece puzzle" - I

too have MTHFR (proofed!) and probably MCAD in addition to POTS and SFN. Alle those conditions MAY be connected together, (probably they are, I guess) but what actually causes them ist mostly

unknown - especially (!) to the medical community... So we have to go on researching on our own....

@Nymph: You also describe the re dilemma quite well... Btw, you seem to be one of the rarer cases here on this forum who has Sjogren´s (?). Sjogren´s is often mentioned in medical literature as a possible

underlying cause for dysautonomia, but in different dysautonomia forums one only meets Sjogren´s patients very rarely. Do so many of us get undiagnosed? Anyway, treatment for Sjogren´s should be

available, what kind of problems are you experiencing getting treated...?

[Nymph]

It's possible. Sjogren's is under-diagnosed. I think that there are more people on the Sjogren's forum who report dysautonomia. However, this is also underdiagnosed and awareness is low so many with Sjogren's probably don't know that they have it. They just think that they feel like crap because of Sjogren's. That's what I thought. You don't come across dysautonomia often in the Sjogren's literature. SFN is mentioned much more frequently.

[Goschi]

Hm, interesting aspect - Sjogren´s patients may often feel so sick because of actually having dysautonomia from it!? Never thought about that.... May I ask you, which tests you had to confirm your Sjogren´s? (I do have "probable Sjogren´s", but docs stopped diagnosing half way...) Do you know an Explanation why Sjogren´s may directly cause SFN?

[Nymph]

I also do not have a firm diagnosis, it's more of a working diagnosis. However, since I am seropositive for RA antibodies (anti CCP), and that does show up in about 5% of Sjs patients, and I do not yet display signs/symptoms of RA... well. It can be frustrating because little is known about what it means! SSa and SSb are the classic blood results ofr Sjs. However, there are new antibody tests such as the SJO/Immco test that are sometimes used.

The lip biopsy is the "gold standard" but I have not had it yet. I may get it soon if I go to Johns Hopkins. I think that they require it.

Sjs causes a number of neuropathies. I believe that trigeminal is the most common, followed by SFN-sensory. But it can cause other kinds, including central, motor, etc. Why? That gets into the immune process and I then get lost! I am not sure if it is a direct autoimmune attack (antibodies) or indirect (cytokines, etc) due to inflammation. There is so much unknown about Sjs. Thankfully, the research pace seems to be picking up.

Sjogren's makes you feel sick and tired anyways. But if you have dysautonomia, too, that's going to add on another layer that may be hard to distinguish, especially if you aren't informed! I actually think that my Sjs is pretty well under control (arthritis, malaise, glands, etc) except for the neuro aspects. I now think that many of my daily struggles are now due to POTS, which may or may not be caused by Sjogren's. To test that I would really have to get on stronger drugs to control the progress of neuropathies and see if it helped. My POTS is fairly mild, though. But I don't want it to get worse. Also, I've started dropping things - possibly due to neuropathy - and I don't want that to progress! Basically I am seeking a way to halt this right here because I really want to at least maintain the quality of life I have now and not become disabled. Either arthritis or neuropathy could sideline me if they progress but right now the neuropathy is pulling ahead.

Are you being treated for your probable Sjogren's?

[Sunshinegirl]

So if I only had blood tests for Sjogrens was that not good enough? What is this lip test?

[Nymph]

Correct. A significant number of people are sero-negative, and especially with neuro complications. The lip biopsy is just that - they take a slice from the inside of your lip, put it under a microscope or something, and look for infiltrates - B-cells that are damaging tissue in there. It has to be done right and read by a pathologist with experience with these. A lot of people never get the biopsy because it hurts. Also, it might not become positive until later in the disease. Personally I would get the Immco test, which is another blood test that measures other stuff.

If you think you have Sjogren's I would recommend contacting a local support group through the Sjogren's Foundation and asking for a doctor recommendation. That is how I found my doctor who is up on the literature and is willing to treat a non-textbook case.

Basically if you have arthritis, dry eyes, mouth, or nose and throat, swollen parotid glands, and/or SFN along with POTS it might be worth looking into, more so if you have several of these. Check out the Sjogren's Foundation for more info.

[Nymph]

A few helpful articles:

http://www.ncbi.nlm.nih.gov/pubmed/22976617

http://www.the-rheumatologist.org/details/article/6904931/Systemic_Sjogrens_More_Than_a_Sicca_Disease.html

http://brain.oxfordjournals.org/content/128/11/2518.short

[Goschi]

Thanks, Nymph, for the links! Didn´t know any of them!

I should really pursue my Sjogren´s diagnosis, I guess... Up to now, I do have a pathological test for dry eyes, a pathological test for the parotis (too little saliva produced) but lab tests all came back normal, as well as no sign for arthritis - however, I have SFN and POTS, so altogether it should be worth undergoing the lip biopsy. Problem is I didn´t yet find a doc here who is willing to do it. They all don´t take the dysautonomia as a possible part of the whole picture into consideration... :-(

The next question would be about treatment options - usually only the sicca syndrome is treated. That´s not what really bothers me, to be honest, I didn´t even notice that I have dry eyes and not enough saliva.

I would "simply" be interested in treating the dysautonomia caused by Sjogren´s, but that seems to be another challenge...

[Goschi]

And thanks once more Nymph for telling me about the Immco-Test! I have never heard of it before, but I guess, I should be able to organise this one here on my own at labs - without too many disputes with unwilling doctors... ;-)

[Nymph]

You are welcome! I hope that you find some answers and a way forward.

I was told by my rheumy that to treat the neuropathy they use Rituxan or Imuran. Both of those have some potentially serious side effects. A few people get igiv. I think it is rare to get that approved. Thankfully there are more drugs in the pipeline for Sjs. I am not sure which ones will prove helpful for neuropathy. Take a look at this for the drugs being tested: http://www.sjogrens.org/files/sq/WinterSQ2015.pdf

Best

[Nymph]

WHoa - just looking at this again. This looks quite safe and has shown promise in MS:

BCG Vaccine

This vaccine already has a 95-year safety profile
due to its use worldwide for TB and at very high dose for bladder cancer. BCG induces TNF, a cytokine that promotes Treg generation and death of autoreactive T cells. Ongoing Phase III trials are taking place in Mul- tiple Sclerosis (MS) in Italy, Phase II type 1 diabetes in Boston, Massachusetts and Phase II prevention trials
in Denmark, Australia, and Turkey. Phase I trials in Sjögren’s are in the planning stages at the NIH. In multi- ple sclerosis, the use of BCG shows outstanding efficacy and safety in new onset disease and with seven years of follow-up is showing better outcomes than currently licensed drugs on the market.

(CF Sjs Foundation link above)

I will definitely be keeping an eye on this.

[TCP]

I know that 8 years ago the dysautonomic issues and neuropathy came on dramatically at the same time.

[TCP]

In my case I would say that back in 1984 after Glandular Fever I had what was later believed to be ME/CFS, which I now think was maybe Autonomic Dysfunction. Then 8 years ago, POTS symptoms flared and neuropathy. I do think I have the neuropathy so mild before that which resulted in no pain, but nervous system changes/sensations. This also applies to people with diabetic neuropathy in the early stages no actually nerve sensations are present but damage is already being done.

Once the neuropathy was fully switched on 8 years ago it's spread all over my body and it intense burning. Luckily it calms down at night when I lie down. Last year I was diagnosed with EDS at 54! All of these symptoms tie-in and make a more complete picture, but there are still many unanswered questions.

I'm actually going for some nerve testing soon. About time.