POTS, antibodies and immunosuppressant drugs.

[Castorp]

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 Rituximab or Azathriopine (Imuran). 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.

@Gingergrrl @kangaSue @Learner1

 

[Judee]

Also perhaps @crypt0cu1t although I haven't been keeping up with where everyone is in treatment currently.

 

[Gingergrrl]

I fell ill in March 2020 with what seemed to be a virus and I have still not recovered.

Do you think it was COVID or were you not able to get tested back in March 2020? Are you in the US? If so, you can do a COVID antibody test (although if you have been vaccinated, I am not sure if the test would be able to distinguish if the antibodies were from the virus vs. the vaccine)?

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.

I also had a diagnosis of Autoimmune POTS (in addition to many other autoimmune antibodies & diagnoses).

(In addition, I also have positive ANA results, although the presence of another autoimmune condition have been ruled out so far).

What was your ANA (titer & pattern)? Also, which other autoimmune panels have you done?

I have attempted pulse steroid therapy

What does this mean?

IVIG and plasmapheresis

I did high dose IVIG for 2 years but never did plasmapheresis b/c all of my doctors felt that it was not safe for me at that time.

Having reached this point, my doctors are considering to use immunosuppressant drugs such as Rituximab or Azathriopine (Imuran)

Azathriopine (Imuran) is very dangerous and wipes out your entire immune system. I've never tried it but IMO would personally avoid it if there is an alternative. I did two years of Rituximab which is a much safer option b/c it is specifically targeted to wipe out only B cells and nothing else. In my case, Ritux was the right choice b/c I had 11 autoantibodies and my illness was progressive and getting worse until I started the IVIG.

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.

That makes sense but I don't know if you have other B-cell mediated autoantibodies or all the other factors of your situation.

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?).

I tested positive for 7 Cell Trend autoantibodies (and we ran the tests twice to confirm) and I had very severe POTS so the autoantibodies were pathogenic (in my case).

Is there anyone who has used this type of treatment from the results of an antibody screen?

When you say "antibody screen" do you mean only Cell Trend or also other autoantibody panels? In my case, the Cell Trend tests were further confirmation of autoimmunity but the deciding factor (for me) were several Mayo Clinic Panels combined w/my entire clinical situation.

I tested positive for the "N-type Calcium Channel Autoantibody" that correlates w/LEMS and I had progressive muscle weakness that affected my ability to breathe and was weakening my lungs & diaphragm. The IVIG & Rituximab put my muscle & breathing weakness into complete remission and it has never returned (even after stopping Rituximab).

Edited to add: I saw that you tagged me and let me know if this is helpful or if you have other questions?

 

[Haru]

This is my same exact issue. I’m trying to dm you but it doesn’t seem you have that feature

 

[Castorp]

@Gingergrrl, many thanks for your reply. I am very happy of hearing that you are in remission.

In view of the date (March 2020) and the sudden onset, long COVID may be a possibility. I live in Spain and I had no access to a COVID-PCR test during the first days of my illness. However, over the last months and prior to the vaccine, I have made numerous antibody tests, with negative results. Accordingly, there is no evidence that I had COVID.

My ANA titer is 1:320 (I do not know the pattern). My doctors have checked numerous antibodies associated with many autoimmune conditions, but all the results were negative. For this reason, they have ruled out (at least for the moment) the possibility that I have another autoimmune illness (in addition to autoimmune POTS).

After the diagnosis of autoimmune POTS, I attempted pulse steroid therapy, which consists of intravenous high doses of steroids. As far as I am aware, this is a standard treatment in many autoimmune conditions. However, in my case it did not work.

Next, I 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, I wonder if only two cycles may have not been enough to see a response.

Finally, I attempted five sessions of plasmapheresis, but without any positive result. As far as I am aware, plasmapheresis removes the antibodies from the blood. Accordingly, I think that if the alpha and beta adrenergic antibodies were pathogenic in my case, this treatment should have an effect (at least partial) on my symptoms. However, I did not experience any positive change. This is why I am reluctant to employ Rituximab or other immunosuppressant drugs, as suggested by my doctors. In any case, at the same time, I need desperately a treatment that allows me to improve my current situation.

Thank you again for your help.

 

[Castorp]

This is my same exact issue. I’m trying to dm you but it doesn’t seem you have that feature

@Haru, I am new on this site, and I do not know how to send you a private message.

I have seen in a previous post that you have very similar symptoms to mine. Did you find something useful to reduce the intensity of this unbearable malaise? I have attempted numerous medications and supplements, but nothing seems to work.

 

[Rebeccare]

@Haru, I am new on this site, and I do not know how to send you a private message.

@Castorp @Haru I'll be in touch with both of you!

 

[Gingergrrl]

In view of the date (March 2020) and the sudden onset, long COVID may be a possibility. I live in Spain and I had no access to a COVID-PCR test during the first days of my illness. However, over the last months and prior to the vaccine, I have made numerous antibody tests, with negative results. Accordingly, there is no evidence that I had COVID.

Do you know what type of COVID antibody test you did? It might have a different name in Spain vs. the US.

My ANA titer is 1:320 (I do not know the pattern). My doctors have checked numerous antibodies associated with many autoimmune conditions, but all the results were negative. For this reason, they have ruled out (at least for the moment) the possibility that I have another autoimmune illness (in addition to autoimmune POTS).

That is a pretty high ANA titer. Mine is pretty consistently 1:160 (speckled pattern). There was a period right after my treatment that my ANA titer was negative at 1:40 but now it is positive again at 1:160 (speckled pattern).

Were you able to do any of the Mayo Panels (or similar tests in Spain) for less common autoantibodies? It sounds like you have good open-minded doctors (since they have recognized Autoimmune POTS) so I was wondering if they could order some of the Mayo panels to send to the US (similar to how you sent the blood sample to Germany for Cell Trend).

After the diagnosis of autoimmune POTS, I attempted pulse steroid therapy, which consists of intravenous high doses of steroids. As far as I am aware, this is a standard treatment in many autoimmune conditions. However, in my case it did not work.

That is interesting and I am not familiar with pulse steroid therapy. I take a low dose of oral hydrocortisone (Cortef) daily but for a different reason.

Next, I 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, I wonder if only two cycles may have not been enough to see a response.

It is definitely possible that two cycles of IVIG were not enough to see results (or it is possible that you were not a responder to IVIG).

Finally, I attempted five sessions of plasmapheresis, but without any positive result. As far as I am aware, plasmapheresis removes the antibodies from the blood. Accordingly, I think that if the alpha and beta adrenergic antibodies were pathogenic in my case, this treatment should have an effect (at least partial) on my symptoms.

Like you, it is my understanding that you would see very quick results with plasmapheresis and know if you are a responder. The results would be temporary and it is not a long-term solution but they could be very useful diagnostically for autoimmunity. I never did plasmapheresis so I can't comment from direct experience.

However, I did not experience any positive change. This is why I am reluctant to employ Rituximab or other immunosuppressant drugs, as suggested by my doctors. In any case, at the same time, I need desperately a treatment that allows me to improve my current situation.

So you did not see any changes whatsoever in your POTS symptoms from any of the treatments that you tried? Did you ever get any benefit from oral meds for POTS (beta blockers, Florinef, Midodrine, Mestinon, Ivabradine, etc)? Not that you would try ALL of those, but they are often things that a doctor will initially try w/POTS so I was curious if you tried any of them, and if they brought any symptom relief, (even though they would not address the underlying cause in Autoimmune POTS).

Thank you again for your help.

I am happy to help if I can and I truly hope that my experience will be useful to others in the future. Do you have any muscle weakness (of your limbs or your core muscles like your lungs & diaphragm)?

 

[Castorp]

@Gingergrrl, thanks again for your interest. I find your comments and suggestions very helpful.

Do you know what type of COVID antibody test you did? It might have a different name in Spain vs. the US.

The test was an Elisa test, a serological test that detects antibodies to the SARS-CoV-2 virus. This is the most reliable COVID test available in Spain. I have repeated this test several times over the last months, and in all cases the results have been negative. Accordingly, there is no evidence that COVID was the trigger of my illness. Despite this fact, some of my doctors do not rule out definitively that I had had a mild form of COVID.

That is a pretty high ANA titer. Mine is pretty consistently 1:160 (speckled pattern). There was a period right after my treatment that my ANA titer was negative at 1:40 but now it is positive again at 1:160 (speckled pattern).

I have revised my blood work, and my ANAs are also speckled pattern.

Were you able to do any of the Mayo Panels (or similar tests in Spain) for less common autoantibodies? It sounds like you have good open-minded doctors (since they have recognized Autoimmune POTS) so I was wondering if they could order some of the Mayo panels to send to the US (similar to how you sent the blood sample to Germany for Cell Trend).

To date, my doctors have checked numerous antibodies associated with many autoimmune conditions, as well as onconeuronal antibodies related to paraneoplastic neurological syndromes. Nevertheless, I do not know the Mayo Panels. I am going to research on this issue in order to discuss it with my doctors in the next appointment. Thank you very much for your suggestion.

That is interesting and I am not familiar with pulse steroid therapy. I take a low dose of oral hydrocortisone (Cortef) daily but for a different reason.

This is a common treatment in many autoimmune conditions, but it is not usual in autoimmune POTS. In fact, I only could find this previous case in the literature:

https://www.autonomicneuroscience.com/article/S1566-0702(07)00411-0/fulltext

It is definitely possible that two cycles of IVIG were not enough to see results (or it is possible that you were not a responder to IVIG).

I aim to discuss with my doctors the possibility of repeating this treatment before resorting to Rituximab or other immunosuppressant drugs.

So you did not see any changes whatsoever in your POTS symptoms from any of the treatments that you tried? Did you ever get any benefit from oral meds for POTS (beta blockers, Florinef, Midodrine, Mestinon, Ivabradine, etc)? Not that you would try ALL of those, but they are often things that a doctor will initially try w/POTS so I was curious if you tried any of them, and if they brought any symptom relief, (even though they would not address the underlying cause in Autoimmune POTS).

My doctors have also attempted to treat the POTS symptoms with different medications (beta-blockers such as propanolol or bisoprolol, ivabradine, antidepressants or 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, which is my worst and most disabling symptom. 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).

I am happy to help if I can and I truly hope that my experience will be useful to others in the future. Do you have any muscle weakness (of your limbs or your core muscles like your lungs & diaphragm)?

I do not have muscle weakness. Nevertheless, I usually have chest pain. It is very difficult to describe, but I feel it like a spasm in the area of the diaphragm and the lungs. This is one of the strangest symptoms I have. All the tests (CT-scans, EKGs, echocardiograms,..) done to find out the origin of this pain are normal, and my doctors have been unable to provide a reasonable explanation for it.

 

[Gingergrrl]

@Gingergrrl, thanks again for your interest. I find your comments and suggestions very helpful.

Thanks!

The test was an Elisa test, a serological test that detects antibodies to the SARS-CoV-2 virus. This is the most reliable COVID test available in Spain. I have repeated this test several times over the last months, and in all cases the results have been negative. Accordingly, there is no evidence that COVID was the trigger of my illness. Despite this fact, some of my doctors do not rule out definitively that I had had a mild form of COVID.

I would assume from these antibody tests that you did not have COVID (although I agree that there is no way to rule it out 100%). Have you tested for other viruses to try to determine what virus you might've had in March 2020 if it was not COVID?

I have revised my blood work, and my ANAs are also speckled pattern.

So you and I are similar in that regard (positive ANA w/speckled pattern).

To date, my doctors have checked numerous antibodies associated with many autoimmune conditions, as well as onconeuronal antibodies related to paraneoplastic neurological syndromes.

This is what I had wondered and did not realize that you'd been tested for paraneoplastic autoantibodies. These were the panels (done by Mayo) that were the game-changer in my case. A neurologist ordered them and expected all of the results to be negative but I tested positive for two paraneoplastic autoantibodies (and we repeated the test again at Mayo and also w/another lab to confirm it was correct).

Nevertheless, I do not know the Mayo Panels. I am going to research on this issue in order to discuss it with my doctors in the next appointment. Thank you very much for your suggestion.

I don't know that the Mayo Panels would necessarily be better than the ones that you've done in Spain. But in the US, Mayo is one of the top labs to test for obscure autoantibodies. I would NOT recommend Mayo for treatment, and I have never been there in person, but their lab is excellent.

I aim to discuss with my doctors the possibility of repeating this treatment before resorting to Rituximab or other immunosuppressant drugs.

So you only did two cycles of IVIG? It is hard to know if that would be enough to determine if you would be a responder (for autoimmunity). Did your doctor think that it was enough or were you forced to stop due to insurance or other non-medical factors?

My doctors have also attempted to treat the POTS symptoms with different medications...

I assumed that you had tried many POTS meds before the IVIG.

I do not have muscle weakness. Nevertheless, I usually have chest pain. It is very difficult to describe, but I feel it like a spasm in the area of the diaphragm and the lungs. This is one of the strangest symptoms I have. All the tests (CT-scans, EKGs, echocardiograms,..) done to find out the origin of this pain are normal, and my doctors have been unable to provide a reasonable explanation for it.

In addition to POTS, I also had chest spasms & chest pain (plus profound muscle weakness that was weakening my breathing and it all seemed to go together). I did a treadmill test w/EKG in 2014 that showed an abnormality and my (former) cardiologist suspected that I was not getting enough blood perfusion to the heart when I was standing. He thought it was "microvascular angina" (although in retrospect, I do not think this was the case).

I did two tilt table tests that showed severe POTS (but we already knew that I had POTS so it didn't really change anything). The tests that were the most abnormal for me were PFT's (pulmonary function tests). I failed every single one and they showed pulmonary restriction (due to the muscle weakness). But it was still unclear why. Although after a few months on the IVIG, I was finally able to pass a PFT test for the first time!

Once I found the LEMS autoantibodies, I did muscle testing that showed that my left phrenic nerve to my diaphragm was only working at 57%. A that point, I was able to get approved for IVIG in mid 2016 after a huge insurance battle. The IVIG almost immediately put my allergic reactions & anaphylaxis into remission and then slowly started to improve my muscle weakness.

After I had done the IVIG for a full year and was a clear responder, we were able to get approval for Rituximab. I overlapped the IVIG & Ritux for a year and then stopped the IVIG but continued the Ritux for another year for maintenance infusions. My muscle strength & breathing returned to pre-illness level and stayed in remission.

I hope I did not write too much! I want to wish you the best in figuring out your situation and it sounds like you are on the right track and have good doctors.

 

[Castorp]

@Gingergrrl, thanks once again for your reply.

I would assume from these antibody tests that you did not have COVID (although I agree that there is no way to rule it out 100%). Have you tested for other viruses to try to determine what virus you might've had in March 2020 if it was not COVID?

During the first months of my illness, I was tested for various virus and bacterial infections (Epstein-Barr, cytomegalovirus, parvovirus, Lyme,..), but all the results were negative.

I don't know that the Mayo Panels would necessarily be better than the ones that you've done in Spain. But in the US, Mayo is one of the top labs to test for obscure autoantibodies. I would NOT recommend Mayo for treatment, and I have never been there in person, but their lab is excellent.

Thanks for attracting my attention on the Mayo Panels. I think this may open a potential line for further research, particularly taking into account that my doctors consider that autoimmunity is likely to play a key role in my illness.

So you only did two cycles of IVIG? It is hard to know if that would be enough to determine if you would be a responder (for autoimmunity). Did your doctor think that it was enough or were you forced to stop due to insurance or other non-medical factors?

The duration and dose of my IVIG treatment was decided by a doctor with a long experience in treating "traditional" autoimmune conditions (e.g. Lupus, Sjogren, ...), but unfamiliar with autoimmune POTS. He told me that in these traditional autoimmune conditions two cycles are usually enough to see a response (at least partial). Nevertheless, I have read about other POTS patients who have received IVIG during several months, like in your case. Did you notice a positive effect from the beginning of your IVIG treatment or you need more time to see a response? What dose did you use?
As far as I am aware, IVIG is the best treatment option available nowadays for autoimmune POTS, although unfortunately not all patients improve with this therapy. However, considering the potentially serious side effects associated with Rituximab and other immunosuprressant drugs and the null effect of plasmapheresis in my case, I aim to propose my doctors the possibility of repeating the IVIG treatment.

In addition to POTS, I also had chest spasms & chest pain (plus profound muscle weakness that was weakening my breathing and it all seemed to go together). I did a treadmill test w/EKG in 2014 that showed an abnormality and my (former) cardiologist suspected that I was not getting enough blood perfusion to the heart when I was standing. He thought it was "microvascular angina" (although in retrospect, I do not think this was the case).

I did two tilt table tests that showed severe POTS (but we already knew that I had POTS so it didn't really change anything). The tests that were the most abnormal for me were PFT's (pulmonary function tests). I failed every single one and they showed pulmonary restriction (due to the muscle weakness). But it was still unclear why. Although after a few months on the IVIG, I was finally able to pass a PFT test for the first time!

Once I found the LEMS autoantibodies, I did muscle testing that showed that my left phrenic nerve to my diaphragm was only working at 57%. A that point, I was able to get approved for IVIG in mid 2016 after a huge insurance battle. The IVIG almost immediately put my allergic reactions & anaphylaxis into remission and then slowly started to improve my muscle weakness.

After I had done the IVIG for a full year and was a clear responder, we were able to get approval for Rituximab. I overlapped the IVIG & Ritux for a year and then stopped the IVIG but continued the Ritux for another year for maintenance infusions. My muscle strength & breathing returned to pre-illness level and stayed in remission.

I hope I did not write too much! I want to wish you the best in figuring out your situation and it sounds like you are on the right track and have good doctors.

Thanks for sharing your experience. I am going to ask my doctors, but I think that some of these tests may be useful in my case. I am very happy that you could discover the origin of your symptoms and stay in remission. Your story provides me hope that it is possible to find a solution, although sometimes it seems an extremely difficult task.

 

[crypt0cu1t]

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 Rituximab or Azathriopine (Imuran). 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.

@Gingergrrl @kangaSue @Learner1

How much ivig did you do? Was it 2g/kg and for how long?

 

[Castorp]

Hi @crypt0cu1t !
I received two cycles of IVIG during two months (2 g/kg/cycle over 4 days each month). In view of the null effect on the symptoms, my doctors decided to stop the treatment. However, I wonder if only two cycles may have not been enough to see a response.

 

[Gingergrrl]

During the first months of my illness, I was tested for various virus and bacterial infections (Epstein-Barr, cytomegalovirus, parvovirus, Lyme,..), but all the results were negative.

That is interesting that all of those were negative in your case. I had severe Mono from EBV in 2012 which re-activated about 10-months later in 2013. At that point I was testing positive for EBV on about 5 different tests which showed an active infection. In 2014 I tried anti-virals (famvir and then valcyte) but neither of them helped and my condition continued to worsen.

Thanks for attracting my attention on the Mayo Panels. I think this may open a potential line for further research, particularly taking into account that my doctors consider that autoimmunity is likely to play a key role in my illness.

I think that the Mayo Panels could be helpful and I don't see any downside (besides the difficulty and cost of shipping your blood sample overseas).

The duration and dose of my IVIG treatment was decided by a doctor with a long experience in treating "traditional" autoimmune conditions (e.g. Lupus, Sjogren, ...), but unfamiliar with autoimmune POTS. He told me that in these traditional autoimmune conditions two cycles are usually enough to see a response (at least partial).

I had not heard before that two cycles are enough to see if there is a response to IVIG in autoimmunity. But he may be correct and I just had not heard it.

Nevertheless, I have read about other POTS patients who have received IVIG during several months, like in your case. Did you notice a positive effect from the beginning of your IVIG treatment or you need more time to see a response? What dose did you use?

My POTS did not improve from the IVIG and it was not until I was getting Rituximab for about nine months that it went into remission (and has not returned). I had multiple different medical issues and the LEMS (muscle weakness) immediately started to improve from IVIG, especially the weakness in my arms. My MCAS and allergic reactions also went into remission very quickly from the IVIG (which I was not expecting and was a huge surprise)! But the shortness of breath, chest pain, and POTS (all of which forced me to use wheelchair), did not go into remission until the Rituximab.

 

[Learner1]

@Castorp My apologies - I see that you tagged me, but for some reason, I didn't see it and am just catching up now.

You haven't mentioned if your immune function was tested and what tests were run. That might provide some insight.

During the first months of my illness, I was tested for various virus and bacterial infections (Epstein-Barr, cytomegalovirus, parvovirus, Lyme,..), but all the results were negative.

What tests were run, specifically, and what were the results, with ranges? I ask because, many times, viral tests are interpreted as negative when they are, in fact, positive. And sometimes only antibodies are measured, not PCR. (My EBV was missed by several doctors because no one ran a PCR test - treating it was helpful.) Having a serious infection can also lead to other infections reactivating, which can happen at any time, so even if you were negative a year ago, things could have changed in the last few months, so It would be wise to be very thorough here.

With multiple autoimmune antibodies, it may be you have some infection triggering them. Doing Rituximab without identifying and treating an infection could be dangerous.

Thanks for attracting my attention on the Mayo Panels. I think this may open a potential line for further research, particularly taking into account that my doctors consider that autoimmunity is likely to play a key role in my illness.

I've attached a list of antibody tests collected from people in this forum.

However, depending on when you last did IVIG and plasmapharesis, testing antibodies may not be accurate. Carful discussion with your doctors on what to expect would be wise.

The duration and dose of my IVIG treatment was decided by a doctor with a long experience in treating "traditional" autoimmune conditions (e.g. Lupus, Sjogren, ...), but unfamiliar with autoimmune POTS. He told me that in these traditional autoimmune conditions two cycles are usually enough to see a response (at least partial). Nevertheless, I have read about other POTS patients who have received IVIG during several months, like in your case. Did you notice a positive effect from the beginning of your IVIG treatment or you need more time to see a response? What dose did you use?

I am immunodeficient and have autoimmunity. In the US, insurance must approve IVIG, and the doctor must make a case. My doctor decided that it was easier to get approval for the immunodeficiency, which is typically given at .4g/kg per month, But he increased the frequency to every three weeks and got the equivalent of 75 g / kg per month approved. I have been on it for 4 years. It alone did not help my autoimmunity, but it did help with fighting the infections that I had. It wasn't until I did Rituximab after I had been on IVIG for 18 months and he had thoroughly tested me to make sure I didn't have any active infections that I got relief from my autoimmune symptoms.

As far as I am aware, IVIG is the best treatment option available nowadays for autoimmune POTS, although unfortunately not all patients improve with this therapy. However, considering the potentially serious side effects associated with Rituximab and other immunosuprressant drugs and the null effect of plasmapheresis in my case, I aim to propose my doctors the possibility of repeating the IVIG treatment

I have a hyper version of POTS, with high blood pressure. My symptoms have been helped with, first, propranolol, but then, when that stopped working, metoprolol, as well as acetylcholinergic substances, like pyridostigmine, neostigmine, and Huperzine. A. Huperzine A has worked the best over time. These gave me relief before I did the Rituximab. Like @Gingergrrl I had mast cell activation, but the Rituximab has allowed me to get off all of the meds I was on for it, except for when I'm exposed to an allergen.

Currently information you have provided, it might be wise to thoroughly examine your immune function and thoroughly investigate whether you might have any smoldering infections lurking, before contemplating rituximab. The fact that you did not respond to plasmaphrasesis makes it a little iffy whether the bother of doing the rituximab would be worth the risks at this point. You do not want to do Rituximab If you have an infection - there have been cases like Olaf Bodden and Whitney Dafoe, where it seems that they got much worse with Rituximab because they still may have had infections and wiping out their antibodies at that point was disastrous.

The other things that you haven't mentioned would be looking into other causes for fatigue. Many times, there are multiple causes of fatigue. Some things to look at:

1. Adrenal function - aldosterone, ACTH, and a 24 hour saliva or urine cortisol test
2. Thyroid function - not just TSH, but Free T3, Free T4, and reverse T3.
3. Sex hormones, and maybe HGH
4. Nutrient status - methylmalonic acid (not serum B12), folate, vitamin C, glutathione, iron, zinc, amino acids. Also test nitrotyrosine for peroxynitrites.
5. Mitochondrial nutrients - see attached. Infections can cause mitochondrial fragmentation, affecting energy production

Best wishes

 

[Castorp]

@Learner1, thank you very much for your detailed reply. I find your comments and suggestions extremely helpful and I aim to discuss them with my doctors in the next appointment. I only have a question. Please, could you provide me further information about what specific tests should be done to examine the immune function?

Thanks again.

 

[Learner1]

@Learner1, thank you very much for your detailed reply. I find your comments and suggestions extremely helpful and I aim to discuss them with my doctors in the next appointment. I only have a question. Please, could you provide me further information about what specific tests should be done to examine the immune function?

Thanks again.

You're welcome. There are many - I've listed the more common ones there are more esoteric ones - starting at the top, and working your way down the list:

1. Immunoglobulins with subclasses
2. CD57 profile
3. NK cell, number AND function
4. Lymphocyte Subset Panel - B cells
5. T cell panel
6. Cytokines (CytoDX and others)

 

[Lara]

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 Rituximab or Azathriopine (Imuran). 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.

@Gingergrrl @kangaSue @Learner1

hi!
did u do the rituximab treatment?
did it help u?

 

[Deebeewaldo]

Hi @Castorp ,

I have just seen your post and I am intrigued as I sound similar in some ways.

I initially fell ill in March 2018, but got another viral infection in late December 2019, some doctors do wonder if it was a mild case of Covid 19, but there was obviously no testing then. I was not able to access a COVID antibody test until 12 months later in January 2020 and this was negative, but I have since read a study showing that something like 30% of people infected with Covid-19 do not subsequently produce detectable antibodies, so who knows if it was that or not.

I am also in Europe - and just recently made contact with a doctor in Spain who is familiar with the Celltrend tests, and the concept of autoimmune POTs (none of the doctors where I live - Ireland - seem to be familiar with this, or interested in learning, sadly).

I was wondering if you would mind sharing with me who your Spanish doctor is? To see if it is the same doctor I am working with? I am due to visit this doctor in Spain in November hopefully. I have not yet been trialed on IVIG, and hoping this Spanish doctor might consider it for me.

Because I also have other overlapping autoimmune issues - although not clear what exactly, positive speckled ANA, titer varies from 1:80 to 1:1280!, multiple symptoms, vasculitic lesions, erythromelalgia, and a bout of autoimmune hearing loss in April 2021 which was responsive to high dose steroids. In Ireland, doctors have given me plaquenil and ldn, both of which have done nothing unfortunately.

In Ireland, the doctors are now considering Rituximab since the bout of autoimmune hearing loss I had in April, but I would prefer to try IVIG first, given we are in a pandemic, plus my husband and I would like to try have children as soon as possible if my health stabilises.. This is also partially because when I was previously given steroids (not for the hearing loss but before that), I was given a high dose for a short period, and tapered very quickly - this caused an outbreak of shingles. So it seems it is quite easy for infections to react in my body while on immunosuppressants, so I would much prefer to try IVIG first compared to Rituximab. In Ireland IVIG is not used for cases like mine, hence why I have started to look in other countries for doctors who are familiar with this.

Of course I understand if you prefer not to share the doctor's name, and hope you have success with treatment.

Thanks for any help, Dee

 

[Nord Wolf]

My doctors have also attempted to treat the POTS symptoms with different medications (beta-blockers such as propanolol or bisoprolol, ivabradine, antidepressants or LDN). The beta-blockers and ivabradine help to reduce my heart rate and the intensity of tachycardia when standing.

I was recently placed on Ivabradine for PoTS with strong tachycardia and elevated BP upon standing. Do you recall about how long after starting Ivabradine you started noticing decreased tachycardia?