Castorp

@Sarah Tee, many thanks once again. I had reached a point where I didn't know what else to try. That's why I appreciate your suggestions so much.

@Sarah Tee thanks again. I have a (supine-standing) catecholamines test done to rule out hyperadrenergic POTS. 

I did not know that a CCB can be used to increase blood flow to the brain, but I think it may be worth a try in my case (I also had a slightly high BP).

@Sarah Tee Thank you very much for your suggestions, I find them very interesting.My neurologist has never mentioned them to me, and I suspect it must be difficult to obtain them in Spain, but I would like to discuss it with them on my next visit. Do you know any literature on this?

Thank you for posting about this study, @MTRJ75. I find it really interesting. I hope that these findings can shed light on the role played by autoimmunity in SFN and dysautonomia.

9 hours ago, CallieAndToby said:

What kind of antibody testing are y'all getting? Even after the positive small fiber neuropathy biopsy results, nobody has done any further digging or antibody testing and IVIG helped me in the past with dysautonomia but I only got approved for 6 months for autoimmune encephalitis. It's too expensive as well and the insurance companies don't want to pay for it, a close family member has an FDA approved condition of primary immunodeficiency and she can't get it either but has constant infections. I personally can't tolerate any immunosuppressive medications (steroids, rituximab). 

I live in Spain and here the employment of IVIG for dysautonomia mainly depends on the criterium of your doctor. In my case my doctor considered that the presence of positive adrenergic receptor antibodies was enough to justify the use of IVIG. Nevertheless, in view of the null response and probably for economic reasons, I only  received two cycles of IVIG.

39 minutes ago, RecipeForDisaster said:

I just saw my neurologist yesterday and he confirmed he is interested in IVIG - BUT I a have to have the right findings on my biopsy, even though I have an insanely high (and increasing) IGG abs FGFR3.

Curiously I also have a high level of FGFR3 antibodies, although this finding is not particularly relevant for my doctors. However, as far as I am aware, FGFR3 antibodies are positively associated with small fiber neuropathy. I hope that the findings of your biopsy allow you to attempt IVIG, which is an  useful treatment for many people.

Thank you so much for your replies.

@supertired, in my opinion IVIG is clearly worth trying if there are signs of autoinmunity (e.g. positive antibodies,...).  In fact, IVIG  has been used increasingly with significant efficacy in the treatment of autoimmune forms of dysautonomia and small fiber neuropathy. As an example,

https://www.karger.com/Article/Fulltext/498858

Unfortunately, there is not one-size-fits-all solution in these diseases, and not all patients improve with IVIG. Nevertheless, theoretically failure to respond to IVIG does not preclude a  positive response to other immune-modulatory therapies such as plasmapheresis, rituximab ororal immunosupressant drugs (e.g. Cellcept). However, taking into account the potentially serious side effects of these other treatments, I think that it is a good idea to start with IVIG.

I have used acetyl-l-carnitine and coenzime q10 because their ability to enhance cellular energy production. Nevertheless, as with all the treatments I have attempted during these two years, these supplements did not have any effect on my crushing fatigue.

In view that currently I have no treatment,  I am thinking about attempting mestinon again.@RecipeForDisaster@supertired, what dose of this medication do you take? How long did you have to wait before see the effect its effect?

Thanks again for your help.

During the last two years I have been experiencing various debilitating symptoms, including tachycardia when standing, problems of temperature regulation (chills and hot flashes, although the temperature is usually below 98.6), unrefreshing sleep and chest pain. However, the worst symptom I have is a non-stop crushing fatigue-malaise 24/7. I have been suffering this unrelenting fatigue-malaise every time since the beginning of my illness, even while resting and my heart rate and blood pressure are okay. This horrible feeling is really incapacitating and never goes away.

After ruling out other conditions, my doctors thought that I may have CFS/ME. However, this diagnosis was dismissed because I have no post-exertional malaise (PEM), which appears to be the cardinal symptom in this condition. Finally,   my doctors diagnosed me with POTS from the results of a Tilt test. My POTS seems to be apparently autoimmune, as I  am positive for various adrenergic receptor antibodies. Since then, I have attempted numerous treatments:

-Treatments to address the (apparently) autoimmune cause of POTS: Steroids, IVIG, plasmapheresis and CellCept.

-Meds to treat the symptoms: beta-blockers (bisoprolol, propanolol), ivabradine, mestinon, LDN, antidepressants (mirtazapine, duloxetine), amantadine, gabapentin and anti-inflamatory drugs.

-Supplements: vitamin B12, vitamin D, magnesium, acetyl-L-carnitine, coenzime Q10, inosine, tyrosine and fatty acids.

Unfortunately, none of this treatments have worked for me. Indeed, they have had absolutely no effect on my symptoms, which means I continue to suffer every time. I am really desperate and my doctors seem not to know what to do. Are there other treatments that I could try to improve my situation? I would be very grateful if you could share your experiences.

Thank you so much for your help.

Many thanks @Sushi for your reply. I have been googling and I see that Strattera is a pure norepinephrine reuptake inhibitor, without any opioid or serotonin effects. I aim to ask my doctors about this med in my next appointment.

I have a diagnosis of autoimmune POTS and my blood tests persistently show low levels of norepinephrine. However, my doctors do not attach any importance to this finding. I wonder if these low levels of norepinephrine are usual in  POTS patients. How can I increase norepinephrine?

Many thanks for your help.

As far as I am aware, IVIG is a potentially useful treatment for autoimmune mediated dysautonomia and/or SFN. In fact, the results by Schoefieid and Oaklander show that around 75%-80% of their patients experience an improvement with IVIG. Nevertheless, this treatment did not work for me. At least, I did not have any serious side effect, only a mild headache after the infusions.

Hi @RecipeForDisaster,

To the best of my knowledge FGFR3 autoantibodies seem to be linked to autoimmune mediated dysautonomia and SFN. In my opinion, this and a positive ANA should be enough to justify the employment of IVIG (and eventually plasmapheresis).

If you are interested, you may have a look at the following papers and discuss them with your doctors:

https://pubmed.ncbi.nlm.nih.gov/33792960/

https://pubmed.ncbi.nlm.nih.gov/30889595/

https://pubmed.ncbi.nlm.nih.gov/32171889/

I fell ill in March 2020 with what seemed to be a virus and I have still not recovered. My main symptoms include an unrelenting and profound malaise 24/7 every single day during the last 21 months, a feeling of fever (although my temperature is usually below 98.6°F) and tachycardia when standing. I am positive for alpha1, beta1 and beta2 adrenergic antibodies (CellTrend test), which has been used by my doctors to justify the diagnosis of autoimmune POTS. Additionally, I also have elevated FGFR3 antibodies, which in combination with the results of a corneal confocal microscopy and a sudoscan test seems to suggest the presence of autoimmune SFN. Moreover, I have positive ANA results, although the presence of another autoimmune condition have been ruled out so far.

Following the recommendations in the literature, during the last year I have attempted pulse steroid therapy, IVIG and plasmapheresis, but unfortunately I have not experienced any change in my symptoms. This means that I spend most of the day in bed, suffering constantly, without any improvement for the last 21 months.

At this point, my doctors have decided to start with CellCept (mycophenolate), an immunosuppressant drug used to prevent rejection in organ transplantation and off-label in some autoimmune conditions. I am a bit concerned about the potential risks of this treatment and I wonder if any other of you with autoimmune POTS/dysautonomia or SFN have gone this route. If so what was your experience like? Did your condition improve? Did you experience serious side effects from CellCept?


Thank you in advance for your help.
 

1 hour ago, Pistol said:

@Castorp - I have HPOTS and NCS and since onset of these I also have been experiencing generalized joint pains, trigger finger, swelling and IC, esophagitis and other inflammatory conditions. Over the years all autoimmune markers were always negative except for ESR, which essentially just shows that inflammation is present. My autonomic specialist is currently part of the research that has found a connection between autoinflammation ( not autoimmune ) and dysautonomia. He started me on Plaquenil ( Hydroxychloroquine ) 9 months ago, and he was right: not only did my joint pains and the swelling improve but so did the POTS symptoms! I have been able to greatly cut down on BP meds and my Beta blocker decreased from 25 mg to 6.25 mg! Other BP meds, like guanfacine, were cut all together. My fatigue has improved ( although i still have to watch what I do ). 

In my case the inflammatory response DID contribute to the POTS symptoms, and getting it under control my quality of life has improved, and I no longer live in constant pain. 

@Pistol, thanks for your reply. I find the link between dysautonomia and autoinflammation particularly interesting and I am really curious about Plaquenil. To the best of my knowledge, this is not a standard POTS-dysautonomia treatment. Nevertheless,  I have heard  about a  few POTS patients who have used Plaquenil with positive results, but I did not know why it works in some cases. If I have understood correctly, you started on Plaquenil in order to reduce the inflammation shown by your high ESR. All my inflammation markers (including the ESR) have been repeatedly negative, so I assume that there is no evidence of autoinflmmation in my case. Nevertheless, I do not know if this reasoning is correct and Plaquenil may also be useful for me. For this reason, I would be very grateful if you could provide me further information about this, in order to discuss this potential treatment with my doctors.

11 hours ago, p8d said:

How long did you try the IVIG? It took me several months (9+) and a couple of dose changes to see benefits from SCIG (I never did IVIG). I have a positive ANA with markers for lupus and RA plus a positive alpha 1 from Celltrend. I started Plaquenil a couple of years before the Ig and both helped a bit. It’s taken me several years and many, many meds in addition to 4 years of physical therapy (pre Covid) and extreme pacing to not suffer the unrelenting fatigue and malaise. I still flare badly with all the symptoms you listed if I overdo anything even a little bit but the immunotherapy has helped me more than anything else.

@p8d, thank you very much for your reply. I have received two cycles of IVIG during two months  (2 g/kg/cycle over 4 days). Taking into account the null effect on my symptoms, my doctors decided to stop the treatment. However, in view of your case, I wonder if only two cycles may have not been enough to see a response.

You also mention that you were prescribed Plaquenil, which helped you a bit. Did you use this medication to treat POTS-dysautonomia? I have heard  about a  few POTS patients who have also improved with Plaquenil, but I do not know why it may work in some cases.  In fact, to the best of my knowledge, this medication is not a standard treatment for POTS. I would be very grateful if you could provide me further information about this potential treatment in order to discuss it with my doctors.

I fell ill in March 2020 with what seemed to be a virus and I have still not recovered. My symptoms include an unrelenting profound malaise 24/7 every single day during the last 18 months, a feeling of fever (although my temperature is usually below 37°C (98.6°F)) and tachycardia when standing.  I am positive for alpha1, beta1 and beta2 adrenergic antibodies (CellTrend test), which has been used by my doctors to justify the diagnosis of autoimmune POTS. (In addition, I also have  positive ANA results, although the presence of another autoimmune condition have been ruled out so far).

Following the recommendations in the literature, during the last months I have attempted pulse steroid therapy, IVIG and plasmapheresis, but unfortunately I have not experienced any change in my symptoms. This means that  I  spend most of the day in bed, suffering constantly, without any improvement for the last 18 months.  

Having reached this point, my doctors are considering to use immunosuppressant drugs such as Azathriopine (Imuran) or Rituximab. Nevertheless, taking into account the null results of my previous treatments (especially IVIG and plasmapheresis), I have serious doubts that this strategy may be useful in my case. In particular, I wonder to what extent the presence of adrenergic antibodies is a sensible guide to treatment with immunosuppressants in this context (i.e. are these antibodies pathogenic?). Is there anyone who has used this type of treatment from the results of an antibody screen?

Thank you in advance for your help.

@cmep37 and @Pistol, thank you for your comments and suggestions. I will discuss them with my doctor in the next appointment.

58 minutes ago, cmep37 said:

I understand your doctors have attempted to treat the underlying condition that is causing POTS but have they prescribed anything for the POTS symptoms themselves?  I am like you - I have secondary POTS caused in my case by hEDS.   There is nothing they can do for my faulty collagen that means my veins are super stretchy but my doctors still tried to lower my HR by prescribing fludrocortisone, Midodrine, beta-blockers (low dose propranolol) and ivabradine.  None of them worked in my case so I am just managing with increased fluids, salt, compression tights and as much exercise as I can manage.  If you haven't tried some of these measures it would be worth seeing a EP cardiologist to get his input - I can't help wondering if your malaise would lessen if your dysautonomia symptoms improved.

Thank you again for your interest @cmep37. My doctors have also attempted to treat the POTS symptoms with different medications (beta-blockers such as propanolol or bisoprolol, ivabradine, antidepressants, LDN). The beta-blockers and ivabradine  help to reduce my heart rate and the intensity of tachycardia when standing. However, they have no effect on the unrelenting malaise. I have not used so far midodrine or fludocortisone because my blood pressure is not particularly low (in fact, when standing I tend to have  dyastolic hypertension).

@MikeO thank you for your suggestion. I have had this generalized malaise every single day since the beginning of my illness, 18 months ago. During this period, I have taken different medications, without any effect on the malaise.  For this reason,  I do not think that this is related to a possible side effect.

It is difficult to describe this generalized malaise. It is not simply not feeling well. I try to describe it to my doctors as feeling like I have a severe flu every single day during the last 18 months (although without respiratory symptoms). There is also a feeling of fever, yet normal values appear on the thermometer. It is really incapacitating and never goes away.  I have attempted numerous treatments, but nobody seems to know what to do to reduce this terrible malaise.

@cmep37 thank you very much for your reply. ME/CFS was considered as a potential diagnosis by my doctors, but I do not fulfil the criteria. In particular, I do not have PEM, which seems to be the main symptom of ME/CFS. After testing positive for various adrenergic antibodies, I was finally diagnosed with autoimmune POTS. My doctors have attempted different treatments (IVIG, plasmapheresis), but nothing has worked for me and I continue to suffer daily this horrible malaise.

Hello! This is my first post. I have been ill for 1.5 year  and finally diagnosed with POTS-dysautonomia.
Neverthelss, I feel like something is still being missed. I have tachycardia when standing and problems of temperature regulation (chills and hot flashes, although the temperature is usually below 37ºC). However, the worst symptom I have is a non-stop profound malaise 24/7. I have experienced this unrelenting malaise since the beginning of my illness in March 2020, even while resting and my heart rate and blood pressure are okay. The feeling is similar to the malaise caused by the flu and  is often unbearable.
I am really desperate and I wonder if this daily malaise is actually a dysautonomia symptom. Does anyone else experience a similar feeling?

Thank you for your help.