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Monday, February 5, 2018

MCAS, mast cell activtion syndrome, nuts and bolts

Mast cell activation syndrome – MCAS – a stand-alone theory of everything.  The disorder is not accepted by mainstream medicine.  Hematologist deal with a set of serious disorders which may involve tumors but that’s is not what we are going to discuss--not to say mast cell activation syndrome is not a serious disorder. It can be deadly serious.  MCAS is a novel way of looking at disease and is used to explain many diseases, symptoms and syndromes. 
What is a mast cell? Mast cells are important actors within the immune system. Stained, under the microscope, they are plump purple cells. They are like other cells found in the blood (eosinophils, basophils) But these cells are located in tissues, various organs, outside the blood stream and around blood vessels. Mast cells have a role in allergies and killing certain parasitic worms. Not the topic of the day. We are interested in inappropriate action of the cells.  
The granules inside the cells contain various substances which cause inflammation. These granules contain things like:  enzymes, histamine, leukotrienes and prostaglandins. 
Activation:  These caustic immune cells release many inflammatory substances causing severe swelling, immune responses and local tissue injury.  Instead of killing parasitic worms or attacking an allergen mast cells are attacking us and damaging our tissues. Mast cells are omnipresent and symptoms vary depending on which tissues are attacked.   For example, if mast cells activate in the intestinal tract symptoms may include bloating, diarrhea, GERD and other dysfunction. Mast cell activation in muscles and joints causes joint pain and swelling. Mast cells activating in the brain may cause brain fog, headaches and neuropsychiatric symptoms.  Diffuse mast cell activation can cause a “multisystem” presentation: fatigue, brain fog, joint pain, muscle pain, bowel and bladder dysfunction, neurological dysfunction, change in mood, confusion and many others. 
Patients may experience one mysterious problem after the next.  Symptoms may come and go over many years. Doctors scratch their heads or diagnose a psychosomatic disorder. Doctors will not think: MCAS.  MCAS is a new paradigm on the edge of medical practice and medical science. Doctors don’t know about it – except for the few. 
MCAS can explain a lot. 
Mast cells don’t typically act on their own. They are triggered by something. Triggers vary widely from one person to the next. 
Certain clinical scenarios make us think MCAS. Patients with hypermobility joint syndrome and POTS invariably also have MCAS. Lyme spirochetes may be a trigger as well as other infections. 
We look for clues: Recurring hives, unexplained itching, dermatographia and facial flushing. Patients may report sensitivity to scents and smells and to variation in temperature – hot or cold. A patient I saw today is sensitive to certain fabrics.  Chemicals may be a trigger. Foods and medicines are common triggers. It may be the inert ingredients in pills that triggers the response and some patients have all their meds compounded.  All of the above may be absent. 
Herxheimer reactions may have a MCAS component and may respond to appropriate therapy. 
Are there lab tests? Iffy. Labs tend to positive only in severe forms of the disease, not the syndromes we are discussing.  Occasionally serum tryptase or histamine may be elevated.  24-hour urine tests are sometimes abnormal. The diagnosis is usually clinical. 
MCAS is treatable -- frequently with remarkable results and commonly used meds are extremely safe. 
Diet may help.  Certain foods are known to be high in histamine or trigger histamine release. Reducing intake of certain foods can help.  Well-known examples include tomatoes, strawberries, avocado, nuts and deli meats. You may only have to cut down on certain foods, not eliminate them completely. Food reactions vary a lot amongst individuals. 
MCAS is not an allergic reaction. For example, an allergy to peanuts is something different, mediated by different pathways in the immune system. Still – there is some overlap. 
Meds. In most cases patients respond to simple, safe meds. But may have to take a lot of them.  The treatment has 2 parts:  Blocking the effects of inflammatory substances released from mast cells and stabilizing mast cells so they don’t activate in the first place.  There are 2 kinds of histamine receptors called H1 and H2.  H1 blockers are the familiar antihistamines. MCAS requires higher doses, and multiple antihistamines. The H2 blocker are thought of as ulcer/heartburn drugs, they block the production of stomach acid. The H2 receptors have other functions and blocking the receptors helps. Blocking leukotrienes (Singulair) is helpful.  Prostaglandin blockers, anti-inflammatories like aspirin may help. Stabilization is more problematic.  Cromolyn would be great but has poor bioavailabity but may still be effective. Ketotifen is mast cell stabilizer with antihistaminic properties.  It is available through compounding pharmacies. Effective mast cell stabilizers may include benzodiazepines and cannabinoids. More difficult cases may be treated with the asthma/hives injectable Xolair and an array of immunosuppressive drugs.


lymie said...

Very interesting. Although I have had lyme a long time, it has only been in the last two years that MCAS and histamine problems became a problem and caused me to undertake a crash course in it. What is still a puzzle is in the situation where SIBO is present, if it is caused by MCAS, then is it even curable so normal digestion can take place? Apparently for many people SIBO is chronic too. Do we have a chronic infection bringing on a chronic mast cell syndrome which then produces chronic SIBO and a host of other symptoms, including some dangerous heart related ones?

As far as MCAS treatment is concerned, if you reduce the stomach acid with H2 blockers, then SIBO may worsen. This seems to be puzzle wrapped in an enigma, and one so complicated that mainstream doctors are apt to steer clear or do nothing but a lot of expensive testing with very little treatment resulting from it.

Will we ever get out from under this increasing pile of problems? Like dominoes, one falls and then more fall, triggered by the previous ones. It is mind boggling to try to figure out a solution.

Lyme report: Montgomery County, MD said...

OK. Acid blockade can be a problem. Each case is different. Proton pump inhibitors like Prilosec are of greater concern. Reduction of stomach acid also increases C. diff risk. SIBO is treated with specific meds, probiotics and ?glutamine. Antibiotics may need to be placed on hold. Certain antibiotics may treat Lyme and SIBO.

Some people want to blame Lyme for everything and some people want to blame MCAS. SIBO has immune system ramifications but I don't see a direct MCAS connection.

Patients: please don't be your own doctor. Be an advocate for yourself. Have informed conversations with a doctor who will listen and work with you.

lymie said...

Since it is hard to find doctors to treat SIBO, Mast cell activation, and lyme, the poor patients have to try and figure out some of this for themselves. Because one doctor is seldom going to treat them all, or understand the connections. My impression, maybe wrong, is that the nervous system infection of lyme can affect digestion and motility, producing SIBO. Then since there are many mast cells in the GI tract, they are activated. In theory, treating the lyme ought to make the rest of it better. Those who do a lot of SIBO treatment, mostly naturopaths and lesser numbers of medical doctors say that only a third of patients are cured of this. Probably because the underlying cause is still there? And lyme is only one of the possible underlying causes. But if it is found in a lyme patient, along with mast cell activation, it would all be related in most cases. Just trying to understand this puzzle.