MCAS: Mast Cell Activation Syndrome (or Disorder)
 

Hi all,

This thread is an offshoot from one below where some posters here recently discussed how antihistamines seemed to help their PN symptoms. I also participate in a dysautonomia forum, where many members have MCAS. I never paid much attention. This is a newly-recognised disease and I had not heard of it.

Yet I thought of it in reference to a member here who wrote of his PN, hives, and GI problems....all typical if MCAS. I then did some more research (sorry I can't upload articles..I'm writing on my reader..Wikipedia is actually a good starting point). Mast cells are produced in our bone marrow and are deployed to fight infection. They can become inappropriately activated, and release histamines and other substances that can be neurotoxic.

Many people with MCAS have POTS or other cardiac dysautonomic symptoms, PN, GI problems, and allergic symptoms, including anaphylaxis. Light bulb moment: my dysautonomia and eventual PN were set off by an extreme reaction to antidepressant medication. I always assumed the meds were somehow neurotoxic (I had neurological symptoms for over a year), but no one could make sense of the fact they ultimately caused both anaphylaxis as well as a hypertensive crisis that nearly killed me. I didn't get it either....until now ...possibly.

Here it is over a year later....and I am basically bedbound with severe dysautonomia...my worst symptom is very labile blood pressure. This week I started a beta blocker (Propanalol) which really helped...but set off allergies. This is another sign of MCAS...very weird reactions to meds.

I just wish to put this out here. Testing is difficult...there are about five docs (primarily hematologists) who do this for a living in the U.S. I live in NYC...no one here! One, Anne Maitland, is no longer taking patients. I then wrote Lawrence Afrin in Minnesota and Cem Akin in Boston. Both had assistants write me back. Both have year-long waits, but each agreed to work with a physician here for testing. Nice guys. My PCP is game.

Treatment involves a variety of histamine blockers and a low-histamine diet (scary...all sorts of things verboten). But I'd do it in a heartbeat if warranted. I do want to be tested first...it's really restrictive...but easy to look up on line.

I'm wondering if anyone here has been diagnosed with MCAS.

I can tell, from the antihistamine thread, that some people here might wish to consider testing. It just would be great if I had an underlying disorder causing SFN with treatment that is not life-threatening!

Sylvie

If you don't mind, are you able to disclose the anti depressant medicine?

Hope you get some answers soon.

No problem. I was taking a high dose of Effexor (an SNRI) which became ineffective. Instead of weaning me off first, my doctor added a high dose of Paxil (SSRI). I was also taking Trazadone for sleep. I had Parkinsonism for about 14 months when I had the crisis. I thought it was a variant of serotonin syndrome. Then abrupt withdrawal. This was Thanksgiving 2014.

I was a mess, but recovering.....had to go on sick leave. Dysautonomia hit in March. PN began in May. Positive SFN 11/15. Positive POTS and neurocardiogenic syncope last month (I couldn't figure out why I've been bedbound...that's the reason!) As you can see, testing taking forever.

I'm still a mess.. I'm a professor, and was really grieving about what looks like a necessary early retirement. I'm moving into the "acceptance" mode of my grief stages!

Thanks for your good wishes!

Sylvie,

What is involved in the testing? Have you considered just trying the antihistamines to see if they work? Many conditions are actually diagnosed by trying treatments for them...ie., like inflammatory neuropathies are sometimes determined by seeing if a course of steroids work? It's not like there is a specific test for inflammatory neuropathy...and the short course of steroids can show immediately whether inflammation is a factor int he neuropathy.

Speaking of bedridden, what have you tried for your dysautonomia? I don't want to detract from the point on your thread (which I think is perfect based upon some members comments and symptoms). I have a long history of dysautonomia (19 years) and might be able to offer you some information to help you get out of bed.

I am one of the many here with strange allergies, but that is also common with any autoimmune disease, due to the direct tie between the immune system and allergies (IgG, IgA, Igm...& most importantly, IgE (for allergies). So I have an interest in where this topic goes and all the good input that will likely get posted.

There is a free-access PDF which discusses diagnostic criteria and possible treatments for MCAS here; http://www.jacionline.org/article/S0...11)00675-0/pdf.

Hi En Bloc,

Thank you so much for your interest....your posts are always so intelligent, informed and interesting......based on your history, I was wondering if you had been tested for MCAS.

Testing:...tryptase levels (sometimes normal with disorder); histamine, prostaglandins, and leukotrienes in an iced, 24-hour urine sample; sometimes a bone marrow sample.

Antihistamines:
I'm hesitant to try antihistamines because they tend to act antithetically and make me hyper ....yes Benadryl keeps me awake (although they do help my allergy symptoms). I would just like to know if I have MCAS before taking anything. I have so much trouble sleeping anyway (I'm sure you can relate).

Bed:
I am extremely uncomfortable standing and even sitting with my head unsupported. I do have a body worker/yoga teacher coming 3x/wk. She's great. I also push myself outside as much as possible. I never feel "normal." (I was very high-energy and athletic before all this). I thought it was simple fatigue....neuros concurred.

So I had my first tilt table test two weeks ago. My BP dropped from 149/90 to 80/60 before I was vertical. I began passing out and they stopped the test. I had no idea my BP is so labile.....this is why it is so hard to stand/walk. I also have POTS. I can't believe my neurologists were not on to this. (I found my own cardiologist...he thinks the cardio stuff is "severe," and found other heart issues as well.) I had a nuclear stress test last week and I'm waiting for results...I'll be going in Tuesday.

I would be very open to your suggestions and would love to confer....how do we PM on this site?

By the way, I had three panels of testing for autoimmune disease and nothing!

Again, thanks so much!

Sylvie

Thanks Kiwi!

I posted a number of things on this thread after Silvie mentioning MCAS. I did lots of research, basically reading all the literature I could find, including the short piece Kiwi provides here.

As I said on there, lots of useful links here (this woman is both an MD and has Celiac+MCAS): http://www.thepatientceliac.com/2014...drome-madness/

Including to this long piece bu Dr. Afrin, which is worth reading:

https://www.novapublishers.com/catal...ducts_id=42603


Sylvie,

Where did you call or write to? I would like to get my GP involved and see if Dr. Afrin is willing to work with her. I have no problem seeing the plausibility of this in my case too.

Thanks for posting this.

I have classic neurological based Sjogren's with ganglionopathy. The allergies I have are weird, but not wide spread and I don't get any hives or such. Just weird like being allergic to EVERY antibiotic in all the drug classes. I have also tested positive for allergies then tested negative for the same allergies. But these types of things are quite common with autoimmune disease due to the improper and erratic actions of the immune system. I don't really have any unexplained allergies or symptoms...everything (now) seems to fit in a neat little box. But of course it wasn't always like that and it took many years to understand the 'what & whys' of my condition.

That being said, I'm very interested in the MCAS and plan to discuss it with my rheumy/neuro at Hopkins this spring...after I've had a chance to educate myself a little, and that starts with some of your links and the discussion you (and DavidHC) began in the last few days. So I'm not ruling this out as a possibility or additional aspect of my condition.

As for the NMH (neurally mediated hypotension--from your tilt table) and the POTS. Although these two conditions can come by them self, they are most commonly paired together. Actually, the NMH 'causes' the POTS in many cases from the low BP causing the heart rate to NATURALLY increase during these positional changes. When the brain detects the drop in BP, it automatically increases the heart rate in order to get blood to the brain. It's called a compensatory reaction. So these two conditions go hand in hand.

The complete autonomic testing takes a while but it sounds as if you have a good idea that your autonomic system is failing to perform as it should. Of course, this will leave you unable to stand for any length of time...even sitting up can be difficult. There are many other aspects of dysautonomia...that effect other systems.

I will sit down in the next couple days and write out some of the things I have done to help and medications commonly used to treat dysautonomia. I will also discuss some of the other testing you may need and what to expect from it. I'll PM this info later in the weekend. There is a link in the top right corner of each page that says 'private messages' (just underneath your 'welcome ...user name'). When I PM you, this will become bold and show an unread message...just follow the links to read it. You can reply directly from my message or PM any time by right-clicking on my user name from any place it appears on the forum or thread. The drop down on this right-click action will give an option to PM.

Like I said, I'd rather do it this way than try to discuss two separate topics within the same thread. I don't want to detract from the MCAS topic in any way.

This is interesting. Note the case that begins on page 149:

https://www.google.ca/url?sa=t&rct=j...PsZzUA&cad=rja

Hi Sylvie,
There is an exercise training program by Dr Leviene from Dallas that might be helpful for POTS.
http://www.sciencedirect.com/science...66070214002021
You can read about it in this blog -
http://potsgrrl.blogspot.co.il/2011/...-protocol.html
There is a lot of information about tests and treatments for POTS in that blog.

According to what you wrote here and on previous posts, if your blood pressure is falling when standing, I am not sure the diagnosis is POTS, as far as I know it seems NMH/OH.

Just a quick note about POTS and NMH/OH: POTS can present by itself. But patients that have NMH/OH almost always have POTS as a secondary Dx...because of the very thing I discussed earlier in this thread. When the BP drops upon standing, the body naturally and automatically increases heart rate (to tachycardic levels) to compensate for the low BP to get blood to the brain quickly. So, they end up with POTS secondary to the NMH/OH...because both conditions are a result of postural/orthostatic changes. I hope this makes sense.

Hi David,


Here are some contacts for you: please look up full addresses, for I don't have them ..sorry.

The two I contacted:

Lawrence Afrin
University of Minnesota -Hematology
Afrinl@umm.edu
His assistant is Georgio Walberg
612-625-9604

I wrote Afrin and Walberg wrote me back--stated Afrin read my letter...nice.

Cem Akin
Brigham and Women's Hospital, Boston
617-278-0300
(I called and assistant returned call)

Other docs:

Maria C. Castells
Brigham and Womens
Colleague of Akin

Theoharis Theoharides
Also writes with the two above
716-636-6898

And by the way, you know the Afrin article you posted up is on Mastocytosis, a far more serious disorder than MCAS?...I just skimmed it, but will check out page 149. You know you inspired my post...I really hope MCAS is the answer for you. I also had to smile at your sense of ageism (although I get it...how can a big, healthy man complain about obscure, subjective symptoms? ) Yet it ain't easy being a 60-year-old woman who flings around medical jargon. (I'm still working on my patient persona.) I'm so lucky I found a couple of smart, open doctors. My cardiologist is a peach. Yet, I'm still hunting for the elusive smart and empathetic neurologist!

En Bloc,

Speaking of smart....I again have to thank you. Is your neuro/rheumy Julius Birmbaum at Hopkins' Sjogrens center? My rheumatologist suggested I go there, and I know another patient of his from another forum (perhaps it's you....forum aliases are confounding!). My sister is in Baltimore, so it could be an easy stay for me. I'm eager to see if he'll test you ...perhaps I should see him?
.
...I look forward to your message (no rush!).

Sylvie

I have both....as noted by En Boc, they are interrelated. I've heard about the Leviene protocol, but have not read anything except patient experiences....thank you for these references!

I have POTS for many years and I have tried betablockers and fludrocortisone with high salt and liquid intake. A healthy nutrition helped me more than these treatents.
My doctor gave me the detailes of the Leviene protocol, if you want I can send you.

Florinef (Fludrocortisone) is to help increase BP by retaining fluids...as is the high salt diet. So I guess you had (at one point, at least) some drops in BP to warrant trying these. Are you not having any problems now with BP?

Were you ever Dx with NMH/OH?

I would love a copy of the protocol...thank you!

My baseline BP is very high...so Florinef and salt are out. DX is neurocardiogenic syncope and POTS....you understand the relationship!

And yes, my BP is all over the place...even with three meds. Lots of tachycardia too.
As noted, this is why I feel so sick. I also think I'm now deconditioned...My muscles are a shadow of what they were just 18 months ago. But I am stronger in yoga, and my balance is good....

I'll know a lot more on Tuesday when I meet with the cardiologist.

Thanks!

YES, my doctor is Julius Birnbaum. I have seen him for the last 8+ years (before he was even at the Sjogren's Clinic)? He is, by far, the best in the nation for neurological manifestations of Sjogren's (or other autoimmune disease). He is the only one in the entire country to be both a rheumy and neuro. If you can get in to see him, then by all means, go for it.

Have you been on the Sjogren's World forum? I have spoken of Dr. Birnbaum many times on that forum...and here. So you may know me from there...my user name there is my real name, Anita.

I sent you a private message

OK. I had written this reply in follow-up to stillhoping's comment about the trying the Florinef and salt...yet I thought she said she didn't have BP problems, only POTS. I was a bit confused for why she would be Rx'd this med if she didn't have BP problems, as that it why it is used.

But, now I know more about your condition. I now realize my PM to you probably made no sense, given your high BP. So, I will rethink this with the more info I have and get back to you.

Thank you so much!
S

I don't have NMH/OH, just POTS.
I tried this treatment twice. The first time was when my POTS was just diagnosed, I had normal BP and the Florinef, salt and water raised my BP so I had to add propranolol. I am very sensitive to medication so it took a few months to find the exact combination, and I continued for about 2 years.

I tried to return to this treatment recently, my BP now is lower (100-80/60-70), probably because I hardly eat salt or processed food. So this time I didn't need the propranolol. I couldn't tolerate the salt because of GI and iron issues, so I stopped it.

That's what I thought you said (that you DIDN'T have NMH). That's why I was confused because Florinef is used the treat NMH and to increase BP. Not sure why they would have Rx that for you if you didn't have NMH or low BP. The medicine is designed to retain fluids to raise BP...that is all it does, so it made no sense why they would Rx this for you.

This is also a treatment to POTS, two doctors recommended it to me even without NMH or low BP, and I know others with POTS without NMH who got it.

The goal of this and other treatments for POTS is to increase the amount of blood reaching the head when standing. Either by increasing the blood volume or the vasoconstriction.

Have you used Florinef and salt ?

I have used Florinef, Midodrine (vaso-constrictor) and salt for 19 years.

The sole purpose of Florinef, is as you said, to increase blood flow to the head. it does this by helping the body retain fluids and increase BP. The reason your BP increased when you took it is because that's exactly what the medication is supposed to do.

That goal you mentioned is exactly what those with NMH also need. Because that is what is needed when BP drops too.

Hi Sylvie,

Thank you for this information. I may have found a local specialist who knows Afrin too, so that's good. I'll definitely update this thread when/if I proceed. In all honesty, I want to pursue this path because I want to know the etiology of it all, it's who I am, not because I think it will help cure or heal me. My guess at this point is that I had some sort of infection, whether bacterial, fungal, etc. via the gut that became systematic eventually and has caused autoimmunity. I have no problem believing all sorts of things may have gone wrong in my body and to say that one intermediary mechanism of action was my mast cells going off the rails, so to speak. I want to know and if I can figure it out, I will. Diet is the key here and it is what has made the immense difference for me. I will continue to heal my gut and go from there. But I wish to look into this among other things, and though I may have inspired you to post about MCAS, you inspired or motivated me to look into this. It makes sense. Thanks again.

Also, the article I posted is actually Mastocytosis, well per se, it's about systematic mast cell diseases, and deals quite extensively with MCAS too. One thing that may have to be done for anyone who has issues indicating MCAS, especially after preliminary blood tests and depending on what they indicate, is testing for Mastocytosis. Regardless, if you read on, the meeting concerns primarily MCAS.

I would say it's as bad as it gets for women, given sexism, and older women due to sexism and ageism, for sure. But I can't tell you the number of times physicians will not look into things or take me seriously despite my complaints and when I've already been diagnoses with SFN and other problems with unknown etiology, because I'm young and I "look good", whatever the hell that means. But I can hold my own, and no doubt it's definitely easier for a young male, who doesn't have to deal with sexism and ageism.

Thanks again for your kind words and the information. And good luck on finding competent and empathetic physicians. They exist here and there... :)

Dear David,

Thank you so much for your letter....Lovely and supportive in every way.

I'm sure your great energy and drive will lead to a diagnosis and effective treatment for you. Meanwhile, I and everyone here will continue to learn much from your voyage!
Now I'll read the article you posted for me.

Again, thanks so much.

S

David,

I think this really may have an impact for you based upon some of your specific symptoms and reactions.

Autoimmune disorders are typically triggered by something and infections are at the top of the list. They don't have to be in the gut (mine was upper respiratory infection, same as many others I've talked to with Sjogren's), but it really doesn't make a difference 'where' it took place. It's the action in the immune system triggered by the infection that matters.

Thank you, Sylvie. :) You're too kind. I appreciate the kind words and encouragement. I hope you're right, and I'll do what I can on my end. In the meantime, I've found a real expert in the field and will be referred to him in the next few weeks. It may be months before I see him, but he is an expert in allergy and immunology, with MD and PhD, and diagnoses MCAS too. Let's see what happens. I'll also write Dr. Afrin in the meantime.

Let me know what you think, once you've read it. The case I referred you too - and I recommend it for anyone interested - is one where one of the symptoms is PN, and one other, extremely bizarre symptom. It's fascinating, if nothing else.

Be well!

En Bloc,

Thanks for the encouragement, which is what it effectively is. I agree, obviously. How can it not be? It's already clear that it has a major role in all this, to what extent and precisely how, I can't say, no one can at this point. But that 'infection' is precisely how autoimmunity so often, perhaps if not always, begins is quite telling and worth remembering.

If nothing else, I'll solve or work to heal/improve my gut problems, and that alone is worth it. No one can truly be healthy without a healthy gut, and that's worth working toward.

Thanks again :)

Here's an informative lecture that discusses EDS, POTS and MCAS:

https://www.youtube.com/watch?v=WXAdRiLMG8k

Though I posted this separately, I thought I'd post it in this thread too, given its relevance:

https://chriskresser.com/quercetin-heal-leaky-gut/

I wanted to update this. I saw a specialist who deals with MCAS too, and he ran a couple of blood tests, not the complex 24 hour urine tests, and offered me Cetirizine and Ranitidine to see whether it would improve my condition. I saw him a while ago but have been too busy to update. I still haven't filled the Rx, and don't think I will. I may see him again. He didn't see particularly pushy at all about taking the meds and said he'd be fine if I didn't, but I'm not sure what else he can do if I don't take the meds, since one of the 3 key methods of seeing whether one has MCAS is empiric, which required medication and observation. Testing for markers is imperfect and often fails, and in Canada many of the key markers are not tested as they are in the USA.

I have a followup appointment in May or June, can't recall, but I may push it back or call and see whether he wants to see me if I haven't taken the meds. I took Zantac for years, so Ranitidine, and I think that was a huge mistake. I don't intend on starting it again.

Me too! Can I?

The Cetirizine is just over the counter, Zyrtec...a second generation antihistamine. Is this the treatment protocol for MCAS?? I guess it would 'possibly' be beneficial to see the results of your labs...did he send them to you?

Right. They both are OCT. The treatment protocol is to use meds that suppress the mediators released, histamine being one of them. It can get more involved, complex and expensive, but this was a start, a basic empiric start. I'll see him soon, so perhaps I'll see. But the labs he ran were quite useless serum levels of one or two mediators. He doesn't seem to be taking it particularly seriously, though to be fair he doesn't have the resources of the testing done by the Mayo clinic. I'll have to see him to see the results. I guess I'm also not too keen on taking the meds, so I'm not taking this seriously either.

Very interesting thread and links- thanks. My new neuro was useless and I have an appointment to see my new rheumatologist in a month's time. For myself I have been thinking about neuro Sjogren's, Ehler's Danlos Syndrome (EDS) and Mast Cell Disorders (Mastocytosis) and also wondering about possibly having Amalgam allergy that is causing burning mouth syndrome. I don't think Mast Cell Disorders or PoTS are commonly tested for in Scotland where I live. But I am hoping I'm wrong!

I was getting some relief from Piriton (not sure of possible alternative brand names in US?) but increasingly the relief is wearing off. I'm hesitant about researching PoTS too much until I know whether it is properly tested for or understood by the NHS in Scotland. I just feel I may end up getting more frustrated by doctors not knowing about stuff I've researched than if I let them do the detective work and tell me - if this makes sense?

I'm glad you found it helpful. If you suspect mastocytosis, it may be worth seeing a hematologist, as I hope to soon. My WBC has also been on a downward trend for a while now, so another reason to see one for me.

And it goes without saying that I feel your frustration. The saddest part of it all is perhaps not that they are ignorant where they shouldn't be, but that they are so resistant to learning new things and opening themselves up to well-evidenced alternatives.

Good luck!

I know it has been a bit of time since last post on this thread but I wanted to let you know my daughter has been dx with MCAS. We have been searching for answers for her for the last 6-7 years and have seen all kinds of specialists which came up with nothing. One ER doctor did say she had POTS but that was it. We sought out a geneticists Dr. Maulik Shah who ordered a battery of blood and urine test. He recently dx MCAS and has begun treatment. The latest was a very low dosage of Doxepin. Although this is an anti depressant, it has a anti histamine effect at a lower dose. She only takes 20 mg a day. We have seen great reduction in pain and daily symptoms with this. She most recently started on the quercetin bromelain. To early to tell how it will work.

One thing I will say is that after years of searching MCAS was somethng that was never mentioned before we met Dr. Shah. Once he saw this through her testing, (high tryptase levels and histamine levels) it all made perfect sense. All her symptoms finally fit. I would encourage you to push forward with it. Not alot of doctors are familiar with it but there are some out there.