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Proposed criteria for diagnosis of MCAS (Read 303 times)
NZNancy
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Proposed criteria for diagnosis of MCAS
07/15/11 at 18:03:34
 
The article is:
Mast cell activation syndrome: Proposed diagnostic criteria; Cem Akin MD PhD, Peter Valent MD, Dean D Metcalfe MD; Journal of Allergy and Clinical Immunology 2010 (my copy was pre-publication so I don't have issue and page number)

There are many diseases that involve mast cell activation. "Primary" mast cell activation is seen in mastocytosis and "monoclonal mast cell activation syndrome"; these involve mast cells that are abnormal and self-activated.

Secondary activation of mast cells is the result of various stimuli acting on (apparently) normal mast cells. For instance, allergic disorders, mast cell activation due to chronic inflammatory disorders or tumors, urticaria triggered by heat or cold or exercise, chronic autoimmune urticaria. some infections activate mast cells.

Idiopathic disorders, where there may be activation of mast cells, or the symptoms may be caused by activation of other immune system cells instead, such as basophils or macrophages or others. These could include idiopathic anaphylaxis, idiopathic angioedema, or idiopathic urticaria.

OK, so those secondary causes of mast cell activation need to be ruled out along the way to making a diagnosis of "Mast cell activation syndrome". They are mast cell activating disorders, though.

The proposed criteria for the diagnosis of MCAS, then, follow:

1. Episodic symptoms consistent with mast cell mediator release and affecting 2 or more organ systems, for example
a. Skin: urticaria, angioedema, flushig
b. Gastrointestinal: nausea, vomiting, diarrhea, abdominal cramping
c. Cardiovascular: hypotensive fainting or near fainting, tachycardia (rapid heart rate)
d. Respiratory: Wheezine
e. Naso-ocular: redness and swelling of the conjunctiva, itching, nasal stuffiness

2. A decrease in the freuency or severity or resolution of symptoms with antimediator therapy (H1 and H2 antihistamines, antileukotriene medications (Singulair, Zyflo), or mast cell stabilizers (cromolyn, ketotifen)

3. Evidence of an increase in a urinary or blood serum marker of mast cell activation: increase of tryptase level above baseline on 2 occasions or only once if the baseline level of tryptase is 15ng/ml or higher. This is the preferred method of indicating mast cell activation. Less specific markers are 24-hour urine with elevated level of histamine metabolite or prostaglandin metabolite, PGF2.

The point is to establish a definite diagnosis for MCAS, separating it from the other mast cell activating disorders.

I'm interested in knowing how many people in this group can pursue this diagnosis.

Nancy


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peter
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Re: Proposed criteria for diagnosis of MCAS
Reply #1 - 07/16/11 at 12:27:56
 
Good topic nancy this could help A lot of people find there trigers

And for people with abnormal SELF ACTIVATED mast cells to know

That the Simptoms can be self activated. As looking for trigers is very

STRESFUL and couse self activation .

I hope this topic can be done with out any infighting

peter



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NZNancy
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Re: Proposed criteria for diagnosis of MCAS
Reply #2 - 07/16/11 at 21:14:54
 
Thank you, Peter. I agree - infighting gets nobody anywhere. The whole idea in this group is for each person to come as close as possible to their true diagnosis, so they keep their attention in the area where there next helpful information is going to come from.

My personal feeling is that people who have the symptoms but do not have mastocytosis are far more sick than people who actually have mastocytosis (and no other diseases that cause ongoing illness). Over time, I am aware that people with mastocytosis move along a relatively predictable path and can plan their lives more easily [i]as a rule[i].

I've followed people without mastocytosis for nearly 15 years and repeatedly brought their plight to the attention of the most active researchers in the mast cell area. These things take time, and once TMS was able to fund some research things began to move.

It is very interesting to me to see how this description of MCAS will pan out, and how it can benefit patients, which is the whole idea after all.

Nancy
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Lisa
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Re: Proposed criteria for diagnosis of MCAS
Reply #3 - 07/17/11 at 08:32:21
 
I'm one of those you helped, Nancy!  I honestly don't think I would have gained Dr. Castell's support if you hadn't spoken with her about my case before hand!

Yet I can't help you much with this situation here.  My case appears to be an MMAS autoimmune/autoinflammatory case.  We've not found the neoplasm itself, but we've found other pathological damage which SM is known to cause.  We know the neoplasm is there and we've also found it with my son who is also an autoimmune/autoinflammatory MMAS case.  Due to the difficulty in testing the bone marrow, due to the fact that only a few labs in the world can do it, we've got to wait for our tryptase to rise to confirm it.  

So, sorry, I can't help you with this poll you're doing.  I hope others will join in however!


Hugs!

Lisa
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Donīt forget, there is so much more to life than being sick!
 
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NZNancy
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Re: Proposed criteria for diagnosis of MCAS
Reply #4 - 07/17/11 at 12:26:00
 
Lisa, I know you have had a real struggle along the way!

One thing, MMAS (monoclonal mast cell activation syndrome) can be diagnosed only with a bone marrow biopsy and the specialist staining that is done in the mastocytosis centers. It is not autoimmune or autoinflammatory, but of course there is no "one person, one disease" rule, so anyone could have MMAS plus any other disease on earth!

A tryptase rise in itself will not confirm MMAS. One must have two of the four "minor criteria" for systemic mastocytosis, and the other three (besides elevated trypase) require the special bone marrow staining.

I'm interested in what kind of damage you have managed to find? Being in Brazil has really created a problem for you with diagnosis. On the other hand, think of all the doctors you are educating!

I think a number of people with a family history of autoimmune diseases will be found to be making an autoimmune molecule that turns on  mast cells. This is, in my mind, the most exciting area for research, because if we can find how the immune system is turning on mast cells we will be learning something really valuable about mast cells.
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Susan
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Re: Proposed criteria for diagnosis of MCAS
Reply #5 - 07/17/11 at 13:50:14
 
Quote:
OK, so those secondary causes of mast cell activation need to be ruled out along the way to making a diagnosis of "Mast cell activation syndrome". They are mast cell activating disorders, though.


Nancy, are you saying that if you have some cause of the mast cell activation, then it isn't mast cell activation syndrome?

I have been in the process of being diagnosed, and wondering what it all means for me. My allergist ran blood testing for allergies (IgE), found nothing, my total IgE was in the 90s, tryptase was 8.5 or so, and he said I have neither mastocytosis nor allergies. He has no interest in finding out what I DO have.

However, I am still reacting to many things, and do MUCH better since starting higher dose antihstamines, both H1 and H2. My doctor started me on ketotifen, and I pick up a script for Singular tomorrow. My GI doctor is checking me for the GI type symptoms, and believes I have eosinophilic esophagitis. My endoscopy on Thursday showed the type of ring like structures in my throat they expect to see, but we have to wait for the biopsy to be sure.

Anyway, if I have mast cell activation due to chronic infections, or EE, Hashimotos thyroiditis, or unidentified food allergies, does that mean it is NOT MCAS? Even when I fit all of the criteria? Are you saying that if they can find some activator of the syndrome, it is not a syndrome? I just haven't found anything else that ties together all of the reactions that I have.
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Susan

Chronic Fatigue Syndrome/ Chronic neurological infections
Diagnosed with Mastocytosis August 2011
 
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NZNancy
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Re: Proposed criteria for diagnosis of MCAS
Reply #6 - 07/17/11 at 15:27:46
 
Thanks for asking, Susan, because it can be difficult.

If you have one of the already-established diagnoses that cause or are associated with mast cell activation, such as allergy, irritible bowel syndrome, thyroid cancer, autoimmune urticaria, allergy, idiopathic anaphylaxis, idiopathic angioedema, or idiopathic urticaria, then THAT disease is your diagnosis.

However, if you do not have any of those diseases,
and you can establish that your symptoms are connected to release of mast cell mediators,
and that you are better with antihistamines/singulair/ketotifen etc,
then your diagnosis is MCAS.

If that you still can't fit a diagnosis of MCAS, you are in the default group of mast cell activation disorder, which means that you still don't have a "group" to go with that will help researchers sort out what exactly is going on inside you, but it appears that mast cell mediators are the source of your symptoms.

This effort to establish a group called MCAS is in order to have a "like" group, so research studies can look more closely at people in that group in order to gain further understanding.

Just as with, ISM (indolent systemic mastocytosis) where there is a huge amount more knowledge than even 5 years ago, because researchers were looking at a group that all fit the criteria for this diagnosis. If the patients that researchers are studying are too dissimilar, then the research is confused and random and takes longer to arrive at an understanding of some mechanism that will lead to better treatment.

It isn't somehow better or worse to be in one category or the other. The important thing is to be in the category where you belong, because only then will information start fitting together better for you and the chance of getting real help increases as we inform the researchers of our observations in each category. It's a search for truth here, not status, because status has no place here.

Susan, have you been able to get any testing, like a 24-hour urine collection for metabolites of prostaglandins or histamine? Or a tryptase level within 4 hours of the start of a severe episode of symptoms? That would be extremely helpful.

Be sure you get from your allergist a copy of all reports from testing. These will be extremely worthwhile along the way, and the easiest time to get them is now.

Nancy
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Susan
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Re: Proposed criteria for diagnosis of MCAS
Reply #7 - 07/17/11 at 16:23:25
 
Nancy, thank you for explaining.

I see what you are trying to do. I did have some testing, but not all. The allergist tested me for 24 urine histamine (normal) and 5HIAA (also normal). The tryptase was only a baseline test, not during a reaction. We tried to get it during a reaction, but the lab screwed up the test three times.

I do have the test reports. I learned long ago that it is essential to keep them, or things get lost when going from doctor to doctor.

My GI specialist is supposed to have the biopsies tested for mast cell issues, as well as the eosinophilic esophagitis. Since he was doing it already, I figure it would be useful.


I would very much like to get a clear diagnosis as to what is going on, but it is not easy when most of the doctors don't know much, something I am sure you hear often.
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Susan

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NZNancy
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Re: Proposed criteria for diagnosis of MCAS
Reply #8 - 07/17/11 at 17:15:49
 
Ah, yes, Susan, I went back and looked at your initial message and you mention a tryptase result of 4. Your hematologist is undoubtedly watching your white blood cell count and your platelet count, which is important. If you feel you are better on your present meds, that's an indication that the chemicals released by mast cells (and some other cell types) are causing symptoms. Tryptase is the only chemical we know of right now that is made in any quantity by mast cells, which is why it would be important for you to keep on trying to get a symptomatic sample of blood drawn.

The other thing is to just wait for the reports on those biopsies.

Meanwhile, I hope your precious son is doing well and is a normal little boy with dirty hands!

Nancy
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Susan
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Re: Proposed criteria for diagnosis of MCAS
Reply #9 - 07/18/11 at 06:45:51
 
Since my cute little boy is 31, I think you must have read about someone else! ...

Here is my story: http://mastcelldisorders.wallack.us/yabb/YaBB.pl?num=1303781804
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Susan

Chronic Fatigue Syndrome/ Chronic neurological infections
Diagnosed with Mastocytosis August 2011
 
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peter
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Re: Proposed criteria for diagnosis of MCAS
Reply #10 - 07/18/11 at 12:01:36
 
Is it that hard to see mast cells in the BMB

Arfter my second bmb my doc could see the mast cells  in his offes without spelicalist stuff

untill the people know where they FIT thay carnt FIX IT

or pervent mast cell disease and demage from immune disorders

peter  bone marrow mastocytosis

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Re: Proposed criteria for diagnosis of MCAS
Reply #11 - 07/18/11 at 20:20:37
 
Oh Susan, I am so sorry for getting a little boy into your household! You have plenty of problems already. I'll blame brain fog......

Nancy
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Deb
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Re: Proposed criteria for diagnosis of MCAS
Reply #12 - 10/12/11 at 16:50:38
 
NZNancy wrote on 07/17/11 at 15:27:46:
If you have one of the already-established diagnoses that cause or are associated with mast cell activation, such as allergy, irritible bowel syndrome, thyroid cancer, autoimmune urticaria, allergy, idiopathic anaphylaxis, idiopathic angioedema, or idiopathic urticaria, then THAT disease is your diagnosis.


HI! I just wanted to clarify this just a little. In general I agree with what was said here. But as far as 'diagnosis' some things in this list don't count. Allergies are tested and able to be proven. So yes that is a diagnosis. Cancer and autoimmune disorders are also diagnoses. But irritable bowel syndrome is a symptom. It can have many causes. Idiopathic (means unknown cause) anaphylaxis is also a description of a symptom. Anything that says idiopathic is not a diagnosis. It just means you have a symptom and they don't know why. So maybe mast cells are the cause or maybe not.
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juliegee
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Re: Proposed criteria for diagnosis of MCAS
Reply #13 - 12/24/11 at 15:15:30
 
Great thread. I'd love for Nancy to expound some more. I have to say that I agree with Deb. Irritable bowel syndrome, idiopathic anaphylaxis, (and even interstitial cystitis and GERD) are all DXes that POINT to MCAD. They comprise the "unique constellation of symptoms" that Dr. Afrin talks about.

Traditional allergies, a pheo, or carcinoid syndrome- THOSE could explain suspected MCAD symptoms and be the patient's ultimate DX.

I am one of those who has never caught a laboratory marker, BUT would have daily anaphylactoid attacks w/o treatment. I suffer from all of the DXes described in the first paragraph (and lots more!) These all point to an MCAD DX according to Dr. Castells.

Am I missing something?

Thanks-

Julie

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« Last Edit: 12/24/11 at 16:40:48 by juliegee »  
 
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Re: Proposed criteria for diagnosis of MCAS
Reply #14 - 12/24/11 at 17:21:54
 
The article listed at the top of this thread is older than the "consensus proposal" currently in pre-publication:

http://www.ncbi.nlm.nih.gov/pubmed/22041891

If you look closely you'll also notice that the 2010 article was written by Akin, Valent, and Metcalfe... the new one includes 16 other doctors from a variety of countries.  IMO, the new article carries more weight.  I also happen to think that it's much more clear and inclusive Smiley

Heather
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