Mast Cell Disorders Forum

Mast Cell Disorders Forum
http://mastcelldisorders.wallack.us/yabb/YaBB.pl General Mast Cell Disorders Discussion >> General Mast Cell Disorder Discussion >> autoimmune? http://mastcelldisorders.wallack.us/yabb/YaBB.pl?num=1301101975 Message started by cjoy on 03/25/11 at 14:12:55

Title: autoimmune?
Post by cjoy on 03/25/11 at 14:12:55

New to all of this and my son has idiopathic anaphylaxis. We have a lot of autoimmune diseases on both sides of the family. Are mast cell disorders autoimmune?

Title: Re: autoimmune?
Post by Starflower on 03/26/11 at 00:16:58

Hi Joy,

This question is right up my alley, but I don't have time to answer it right this minute... I'll try to get back to you later today :D

Heather

Title: Re: autoimmune?
Post by Lisa on 03/26/11 at 02:09:00

Big question Joy, but I'll try to make this short. LOL!

Technically no. At least not what is called Systemic Mastocytosis.

So that I don't repeat a LOT of explanation Joy I need you to go read my reply to Adele where I explained today the difference between the proliferative versus activation disease. http://mastcelldisorders.wallack.us/yabb/YaBB.pl?num=1300616439/14#14

This explanation give to you an understanding as to what is clonal versus non-clonal and this is where the major issue lies.

About 20 years ago, before the clonal form was identified the disease was given one name, Mastocytosis. Before then they knew that there was Urticaria Pigmentosa and some other skin lessions that showed all of this but it took research to bring them all under one disease for those patients who don't show any skin lessions created problems for until they began to really look closely they didn't realize that this was all the same disorder. Yet, as research in to "masto" went on, they began to concentrate solely upon the clonal form, since this was the major physical proof that they really could study. So, in this process as they began to study the patients they would take those who had the proof of the clonal problem with their mast cells and put them into the pile and call them systemic mastocytosis. They didn't know then that the cutaneous patients would evolve into the systemic patients - or that the skin disease would go systemic. This they've found out within the last 10 years or so.

The problem with this is that as they began to define what comprised that of SM, they began to exclude anybody who didn't fall into this strict definition and a whole bunch of patients fell outside of this group. Yet, this was the problem, you had a bunch of patients who looked like, acted like and were treated like the SM patients and responded to that trreatment sucessfully, but who in truth were not because they didn't fulfill the strict criteria. WHY? And this is what forced the researchers begin to realize that they had made a mistake in considering that anybody outside of that criteria were not with some kind of form of the disease.

So, with this they recently decided to open up the diagnosis of the disorder so that it appears to now be Mast Cell Disorders, with those who fulfill the criteria for SM on one side of the spectrum. This put everybody else into the MCAD diagnosis, and we are waiting for the precise criteria for this diagnosis to be published.

It is within this group of patients who are showing that there is an autoimmune element here.

For example, Heather and I and several others have positive autoimmune markers. WHY? We don't fulfill any other disease criteria with those markers, so what do they mean? It doesn't seem to be another disease for our disease clearly show's it's masto by either having some kind of positive mediator release or that of having some kind of positive pathological changes, thus reinforcing that it's masto. Where exactly the autoimmune markers come into this, nobody yet knows, Joy for there's not one researcher looking into this yet.

My case seems to be that of the MMAS patient which would mean that I have one foot in both camps, that of the clonal SM patient and also that of the MCAD non-clonal patient. Cases like mine may be the "missing link"cases which prove that there is actual autoimmune form of masto, but for now, it seems that there is a separation in that most cases seem like Heathers, truely non-clonal in nature and the autoimmune patient would be in that MCAD category. But again, until they begin to purposefully study the autoimmune patients and gain more understanding as to what these patients comprise as to their profile and their cause, they suspect it to be so, and they are even saying that it exists, but there is no profile which has been drawn and they don't understand the whys and why nots about it.

We autoimmune masto patients remain a mystery.

I hope this helps!

Lisa

Title: Re: autoimmune?
Post by Starflower on 03/26/11 at 06:04:47

Thanks, Lisa! 8-)

Two things I want to add...

- There actually is ONE recognized autoimmune mast cell disorder... chronic autoimmune urticaria (CAU). To get this diagnosis you need two things: 1) chronic urticaria (a history of hives), plus 2) A positive test for antibodies to either the high-affinity IgE receptors or to IgE (this is a bit confusing, but these are IgG auto-antibodies against IgE, not the kind of IgE antibodies you find in classic allergies). There are two problems with this, however. 1) Some people... like patients with asthma, lupus, and scleroderma... can also have these antibodies (so far, nobody knows why), 2) Nobody has studied the effects of these antibodies on anything but the skin and lungs... although in my case these antibodies seem to be responsible for anaphylaxis (since I've ruled out everything else you can imagine), there's no research to back this up. Like Lisa said, there is much research to be done.

- Autoimmune disorders... whether related to your mast cell disorder or not... will make ANY mast cell disorder worse due to the constant activation of complement... even if your complement levels are within normal ranges. The explanation is long and complicated, but trust me when I say that 99% of doctors do not know this. I've verified it with the researchers and by studying immunology textbooks (I'm a certified geek), but still... I wouldn't even bother mentioning it to a regular clinical doctor.

Anyway... I'm off to the races again! This is a very busy weekend for me. I'll try to check in.

Heather