autoinflammation and dsyautonomia

[Pistol]

Yesterday I had an appointment with my autonomic specialist. I informed him that, since onset of POTS 11 years ago, I have also been suffering from generalized joint pains and flare very badly when exposed to allergens, such as pollen or insect bites. I was seen by rheumo twice, each time with tons of labs to check AI, always negative except for ESR, WBC and RBC. I have been taking Prednisone twice, which makes my POTS worse. 

He explained to me that they now know that Dys is an auto-inflammatory disease, meaning it affects the innate immune system and the tests for autoimmunity will not show anything, since the adaptive immune system is an entire different entity. Here is a good article explaining the difference:

Difference Between Innate and Adaptive Immunity | Compare the Difference Between Similar Terms

The autoinflammatory part of POTS appears to have to do with cytokenes, mastcells and cholinergic reactions, and the labs commonly done for AI are not affected. He said that they had found compelling evidence of this and were ready to prove it by a major study but along cam COVID and shut it all down. Once they can do research again this will all evolve into a great breakthrough in finding a cure for POTS. Isn't that great?

For now I will be taking Plaquenil, which should not only help with the constant inflammation but he claims it will help my POTS, especially the fatigue. A friend of mine on this forum takes it ( she is very similar to me in symptoms ) and she found good relief from it, especially with fatigue. Once it starts working I should be able to exercise more which in turn should improve many of the symptoms of POTS. 

 I will not be able to start it until January ( an eyexam is needed before starting ) and I was told it takes about 4-6 weeks before any improvement is seen,  but I will come back to this post and update how things go. I am just hopeful about the good news that better treatment is on the horizon!!! He said with this development they not only will be able to use the drugs they already have but - more importantly - can develop new drugs to help this disease. Hurray!!!!!

[cmep37]

This all sounds really interesting @Pistol.  New areas of research and new drugs (or even old drugs being used in new ways) offer a little hope for all of us!  Please keep us updated on how you get on with the Plaquenil - is that also used for lupus?

[Ashc]

Keep us posted on the Plaquenil. Thanks for sharing this.

[JimL]

I have joint issues and double vision. The POTS stuff as quieted down mostly though. Reading above, it sounds a lot like myself. Been through 2 primary docs, a cardiologist, electrophysiologist, neurologist, ophthalmologist, neuro-ophthalmologist, GI doc and a GI surgeon. Most either don't know, think I am nuts or some don't care. My daughter sent me an article yesterday about a woman that got COVID and it seems she has POTS symptoms and arthritis. I don't think I was exposed to COVID, it didn't exist then, but I think I got some sort of bug. The only blood test they've done that comes back abnormal is sed rate and it's not that high. Here's the link to that article: https://www.thehour.com/news/article/A-Canadian-woman-has-been-sick-with-covid-19-15787562.php

Maybe Canadian doctors are better. I've had no luck in AZ. 

[MTRJ75]

Same story. I've been to two rheumys, the second at Rutgers University, who told me that in no uncertain terms that all my bloodwork is fine and I don't need a rheumy and I should be happy about this. He admitted I was suffering from something, but there was nothing any rheumy could do about it. 

Every other doctor I go to says I need a rheumy. Or that my AI disease may be the cause of all this, but that's out of their purview. Plaquenil is actually used for Sjogren's, but since I've never tested positive, it hasn't been offered to me. I've also read enough to be concerned about the eye toxicity, that I don't even know if I'd take it if offered. I hope it helps you though. 

EDIT: I'd like to add something else here. I've used White Peony extract in the past, as it's been advocate for in AI conditions in alternative circles. It did help with pain, but I stopped when I started CBD oil because of cost. Ideally, I'd be able to use both still. 

[Guest KiminOrlando]

I fully believe this is a fact. I have been on Plaquenil for 15 years and the more treatment I get for my autoimmune disease, the better my dysautonomia gets. I have proven to have Rheumatoid-arthritis with blood work, but the Rheumatologist does not call it that. He calls it Undifferentiated Connective Tissue Disease because he thinks it is something else that doesn't have a name yet nor a specific blood test. It also has bizarre lung involvement. My blood work has also been positive for lupus, but now it has disappeared. They think this is somehow relevant, but aren't sure how. I truly hope this is further researched. Also, if a Rheumatologist offers further medication to treat an autoimmune disease, it may be worth considering to see if it makes your dysautonomia better.

[MTRJ75]

So it just so happened that I had my quarterly eye doctor visit this afternoon and I asked him about Plaquenil. He said that in the past with larger doses, toxicity to the eyes was more common and potentially progressive even after the medication was stopped. HOWEVER, in recent years, the dosage is much less and rarely ever seen anymore. He estimates treating over 1K Plaquenil patients and only twice has he ever seen an issue after many years of use and in both cases they were able to catch it quickly enough to where the patients recovered. 

Since I see him every 3 months, I'd feel more comfortable asking about this. The question now is: which doctor? 

[Guest KiminOrlando]

My eye doctor told me the original warning on Plaquenil was the old formulation. The new formulation, which is used almost exclusively now, has almost no statistically noticeable difference in retina detachment as people who are not on this drug. He said it probably shouldn't have this warning any more but it is almost impossible to get one removed since technically it is the same drug. Reformulations aren't considered new drugs. The recent hysteria over this drug may have been from conflating chloroquine and hydroxychloroquine. 

For reference, my dose is 200mg of hydroxychloroquine twice a day... for 15 years. My understanding is that this is standard dosing for an adult. Your doctor may choose something different for you.

If you have concerns, feel free to send me a msg and I'm happy to discuss my experience or your concerns. This med really got a bad reputation recently. 

[DizzyGirls]

Wow!  This is so interesting and fits right in with my oldest.  Ever since she had to stop taking Motrin, her pain has been off the charts.  Had an ultrasound show some mild distention and swelling of her left kidney (so far no Nutcracker syndrome).  I think her issues are inflammatory.  She's been on Plaquenil off and on for the past few years, but only at 200 mg a day.  Wonder if that's a high enough dose to do anything?  Just a thought....  My mom has EDS, Lupus, Dermatomyositis, and Sjogren's, so AI runs in the family and my daughter is a carbon copy of her.  Would love to hear updates on this @Pistol!

[p8d]

I am the person @Pistol mentioned in her post. I developed post viral dysautonomia and the first specialist I sought out was a rheumatologist because my mom has 4 autoimmune diseases and her side of the family is rife with it. Of course every test was negative at that time but 18 months later on my third ANA I got positive results for both lupus and RA. I have the diagnosis of undifferentiated connective tissue disease and was put on hydroxychloroquine at that time. My first caution is that this medication is EXTREMELY hard on the digestive tract. I started with a quarter tablet once a day and after months got to 200mg twice a day but still had diarrhea from it. My gastroenterologist gave me the ok to take a tiny dose of Imodium daily to counteract this and gain the benefit of the drug. It took 12+ months, definitely not 4-6 weeks, for me to see any benefit from it and another 2+ years to achieve significant relief from the malaise (flu like feeling), joint pain and swelling and fatigue. It did absolutely nothing for my blood pressure or heart rate so please don’t think it’s an all around panacea. I did eventually, after 18 months, feel tolerable enough to start physical therapy in person in a water treadmill and did PT until the pandemic stopped the in person visits.

I have a neurologist and cardiologist who are ahead of the curve on this being autoimmune and I started subcutaneous IG (SCIG) 2 years ago and that has lead to more improvement in malaise, joint pain and swelling and fatigue. My joints are improved enough that the rheumatologist decreased the Plaquenil to 300mg/day last year which is what is recommended for maintenance for my weight. @DizzyGirlsthe dose is extremely important in my pain levels. When hydroxychloroquine became all the rage earlier this year and became difficult to get my dose was decreased to 200mg/day and the pain/swelling returned. My hypertension recently decreased and I cut my BP meds in half but whether this is the result of an increase in the dose of SCIG a couple of months beforehand or to stopping gabapentin I don’t know. Both those things happened at about the time the BP dropped. I do have SFN so it’s *possible* my nerves are growing back now that they aren’t being attacked by my immune system. I still have extremely labile blood pressure with orthostatic hypotension and supine hypertension and *anything* that causes “stress”, writing this or difficult emails, phone calls, a pleasant Zoom call with family all send my BP way up but so far not to crisis territory recently. Having said all this I still get fatigued easily just not as badly as before and I haven’t left the house except for lab work and a flu shot since March so it’s hard to really tell the impact of the increased dose yet. I can say I don’t feel like death every minute of every day anymore and I think Plaquenil, SCIG and exercise all have helped. As has mindset. I think every little thing adds up and I try everything I am offered.

 I did send my blood to Celltrend in Germany and tested positive for the alpha a-1 autoantibody. I am very encouraged by what @Pistol’s specialist said about this being autoimmune and am hopeful that they will identify which pieces of the innate immune system might be targeted with new or better drugs. Hopefully we are finally getting close to a real treatment for this! 
Thank you so very much for posting this @Pistol It offers real hope!

[Hippopotsamus]

@Pistol thanks for sharing that hopeful info! I think all of the research they are doing with regard to immunity and “autoantibodies” in particular is going to be key to solving the POTS puzzle.I got the double whammy of having POTS and “Long-Covid” so I have been following the support group research threads and this is going to be the laser focus of new research- autoantibodies, the mast-cell histamine connection... because so many are developing dysautonomia and ME post-Covid they can’t ignore it any longer. Way too many young people going to need vast healthcare resources they were not planning for unless they figure it out. 

[Pistol]

@Hippopotsamus - you know, I was just thinking .. in 20 years medicine may have changed to the point that dysautonomia is a common diagnosis, and treatments may be available by then ( certainly hope so ). In literature they mention that dysautonomia has been around forever, but the docs never knew what it was and were baffled by the symptoms. I read that in the recent past it was always brushed off as anxiety, hypochondria or hysteria. And before that they had different names for it but no treatment, they used to put the people ( mostly women ) on bedrest and there they would stay indefinitely. How sad! I wonder if that is what happens to all the females in classic novels that had a mysterious illness and did not come out of the room or had to move to the sea for a better climate. 

Well - makes me almost relieved to have it in this day and age ...

[dizzytizzy]

Reigniting this thread to say that I have a slew of AI issues -- hashi, oral lichen planus, lichen planopilaris, dysautonomia, etc. Electrophysiologist mentioned that same study to me at my last appt in November. He was bummed that COVID shut it down. He said that in many cases, they now believe that POTS-like syndromes are related to post-viral infections that impact/alter immune function or some other thing that triggers a change in immune function. He feels strongly that there is an AI component. 

My dermatologist just prescribed Plaquenil last week for me -- 200/mg twice daily. She's hoping it helps with all my lichen stuff going on. Ran it by EP and he's cautiously optimistic that it may help dysautonomia symptoms as well. I'm only on day 3, but I've noticed that I do not feel so heart poundy all the time now. Usually it is quite prominent when I am sitting down. I have had stomach cramping here and there with it, but not nausea, vomiting, diarrhea yet....and I hopefully don't. 

Off to read the article @Pistol shared!

[Guest KiminOrlando]

@dizzytizzy it may take 3 months or more before you know if the Plaquenil works for you. I hope it does. Please keep us posted. 

[Pistol]

@dizzytizzy - I have been on the Plaquenil for about 3 months and I am seeing less joint pains, I know that because I simply just realized while reading your post that I have not been hurting. Even the stiff fingers every morning are less severe! And I have absolutely ZERO side effects from it! But that's just me, I know of Others who are not that lucky. I hope it helps you!

[p8d]

@Pistol that’s wonderful! I am happy for you. I have always thought that dysautonomia is an autoimmune disease, it is wonderful to see so much research starting to show it.

After several months on plaquenil for post POTS diagnosed AI disease it was the first drug to help with fatigue/joint pain/malaise. I did have significant diarrhea for a couple of years but stuck with it. SCIG helps even more and I recently started LDN for the neuro inflammation and pain, joint and migraines, that the other two don’t help. I still get flares of quite bad joint pain and fatigue which is why we started LDN . So far my pain is less but it’s too soon to really tell as I am ramping up the dose. LDN is causing insomnia but I get that if you look at me wrong anyway and it’s supposed to help with sleep after the adjustment period. 
 

Good luck with it @dizzytizzy

[JimL]

Yeah, I kind of suspect that POTS is probably AI reaction in a lot of folks. I had a poorly treated H Pylori infection. I even sent a link to a paper on one of the government websites about it to my doc. Ignored. I get bouts of dry skin, losing hair and constipation with just feeling off and lousy with worsening esotropia and my standing HR can get a little high, but not like it was. I also get fullness feeling in my left ear.  I can see the esotropia in picts now. I've lost faith in doctors and I will leave it at that. Unless you get one that's curious and has an open mind, it's going to be mixed results at best. I think the problem where I live is that we have a lot of retirees and they are just used to processing patients like an assembly line and go home. I just hate to go through the process again of trying to find a good doc and then wonder if I will be labelled a nut again. 

[dizzytizzy]

On 3/13/2021 at 11:20 PM, KiminOrlando said:

@dizzytizzy it may take 3 months or more before you know if the Plaquenil works for you. I hope it does. Please keep us posted. 

@KiminOrlando Dang it! I didn't get the notifications that people had responded to this. Sorry for the delay.

Anywho, that's what the dermatologist advised me too. So we'll see how it goes. The heart thumpiness has definitely subsided **knock on wood** But then I started having sun sensitivity about a week into taking the meds and she had me come back in to the office to check it out. I was mid-sentence and she interrupted me and was like, "What the heck is going on with your neck and chest?!" Apparently, I had hives (that's how my skin tends to look which I thought was normal) and she kind of freaked out. Then she grabbed her little bamboo stick thing and scratched it across my back. She said, "Holy dermatographia!" Kind of funny 😁

She said it's really interesting that it seems to be helping my heart symptoms and mentioned the emerging research about Plaquenil being used to treat dysautonomia. Then was said that she thinks it's worth doing a screening for lupus, due to all of the weird rashes I get, the lichen planus, the dysautonomia, the small fiber neuropathy. So I'm waiting for an ANA screen to come back. I had a positive one in 2015 (ANA positive, but none of the antibodies for anything were positive), did the rounds with a rheumy who eventually gave up and said he thought something was going on but it wasn't confirmed with labs so there was nothing more he could do for me. 

I'm not holding a lot of faith in the ANA to come back positive this time. And I'm really reluctant to do the rheumy circuit again. At least I'm on the Plaquenil now for the lichen planus crap, so hopefully it will help all the other things going on too. 

[dizzytizzy]

@JimLTotally agree on the doc situation. So hard to find one who is willing to stick it out. I think a lot of people, like me, have just learned to live as best they can with their symptoms. Or at least until the next big health crisis hits, and then the cycle of trying to figure it out starts again. 

The dermatologist I'm seeing now has a very "can do" attitude and seems intrigued and perplexed by my symptoms. I think she sees it as a challenge, and I'm like, "Good luck. I'm an open book. But just know that many have gone before you!" 

 

[Guest KiminOrlando]

@dizzytizzy If your ANA is positive, let us know. Mine was positive but negative for anything specific and I was denied treatment. This ended up being disastrous for me. Some doctors don't believe in sero-negative autoimmune diseases. Some don't believe in a diagnosis of Undifferentiated Connective Tissue Disease but that is what I have and getting meds for the autoimmune disease helped my dysautonomia. I also had the lupus rash. 

I hope you find some answers. 

[Pistol]

@dizzytizzy @KiminOrlando I have had joint pain since onset of POTS 12 years ago. Blood testing always only showed an elevated ESR. My joints get red, swollen, hot and very painful, that is ALL joints. I saw a rheumatologist twice, he ordered tons of outlandish sounding labs and even xrays, but everything always checked out normal and he recommended steroids, which i cannot take due to HPOTS When I told him I cannot take that and asked for Plaquenil instead he said that is only for people with RA. Eventually my autonomic specialist put me on Plaquenil, saying it might help my POTS as well. I ahve been on it for 3 months and it seems to have cut back on my joint pains, although I am having some sort of flare right now. 

I do not know why the rheumatologist community is so bent on lab results. Obviously if a patient has visibly inflamed joints there is something going on. But apparently they are not yet familiar with auto-inflammation. Which surprises me b/c it really is not a new concept. 

[p8d]

@dizzytizzy The first rheumatologist I saw said everything was fine with my labs (there was some weird results that he didn’t interpret as abnormal) but did offer me steroids. I refused him but my then PCP offered a medrol dose pack that really helped. 24 months later when an ANA came up positive with autoantibodies my PCP recommended a different rheumatologist who is wonderful and treats based on symptoms not just labs. Can you ask this wonderful dermatologist for a referral? She might know a sensible one.  The dermatographia sounds like MCAS. Maybe she can recommend an immunologist? I have found that Drs affiliated with academic medical centers are sometimes more curious about we weird ones. Just a thought. Please let us know how you do. Just FYI it took me almost a year to see improvement from plaquenil but I titrated up extremely slowly so don’t expect quick results.

[JimL]

4 hours ago, Pistol said:

@dizzytizzy @KiminOrlando I have had joint pain since onset of POTS 12 years ago. Blood testing always only showed an elevated ESR. My joints get red, swollen, hot and very painful, that is ALL joints. I saw a rheumatologist twice, he ordered tons of outlandish sounding labs and even xrays, but everything always checked out normal and he recommended steroids, which i cannot take due to HPOTS When I told him I cannot take that and asked for Plaquenil instead he said that is only for people with RA. Eventually my autonomic specialist put me on Plaquenil, saying it might help my POTS as well. I ahve been on it for 3 months and it seems to have cut back on my joint pains, although I am having some sort of flare right now. 

I do not know why the rheumatologist community is so bent on lab results. Obviously if a patient has visibly inflamed joints there is something going on. But apparently they are not yet familiar with auto-inflammation. Which surprises me b/c it really is not a new concept. 

That's the only thing that comes up with me, somewhat elevated ESR and it's discounted. 

[dizzytizzy]

@KiminOrlandoANA screen came back normal/negative this morning. I go back and see derm in a month, but I don't think she will pursue further testing unless something weird symptomatically crops up again. 

[dizzytizzy]

@p8dI'll be sure to ask about next steps/specialists. Since my ANA screen came back negative, I'm not exactly sure where she might go next. At this point, since she is willing to prescribe plaquenil, I'm somewhat content to give her some time to see how it goes. She switched me from daily zyrtec to allegra, as she's had better luck with that in managing hives. I suspect I have something MCAS-like going on and that she is thinking along those lines too, but I'm going to have that specific convo when I go back for my follow-up. 

Many of my health issues started after a bout of suspected mono in my early 20s, and seem to be the result of a triggered or overactive immune response, so I'm hopeful that plaquenil may be just what is needed to calm it down. It was over a decade later that the dysautonomia diagnosis came and it seems like every few years now I add another diagnosis! I was on the full 400mg of plaquenil, but we decided that might have been too much too soon, so I'm taking 200mg/day right now and will hopefully increase in a few weeks. Some of my heart thumpiness has come back. Maybe it will decrease when I'm on the higher dose and after several months. 

I also have some pituitary stuff going on and was diagnosed with adult growth hormone deficiency a few years ago. Mixed bag of oh so fun health stuff like most of us here.