Posts Tagged ‘itchy skin dry skin skin rashes hives’

Mast Cell Activation Syndrome!

Friday, June 15th, 2018

 

Does histamine control your life?   Are you even aware that it might?  You might have MCAS!!

Much of this information comes from Dr Tania Dempsey and Dr Jill Carnahan in the States….  but it is a Syndrome worth thinking about if you’re simply not getting on top of your pain, allergies, nervous system stresses etc….  Many doctors in Australia are totally unaware of this condition.

What is Mast Cell Activation Syndrome?

Mast cells, a type of blood cell, play an important role in the body’s immune system. They reside in all body tissues and form part of the body’s initial defence system. Mast cells react to foreign bodies and injury by releasing a variety of potent chemical mediators, such as histamine, when activated. In a healthy person these chemicals will act beneficially to protect and heal the body, but in a person with MCAS these same chemicals are inappropriately triggered and released and have a negative effect on the body. Amongst the triggers are a variety of different foods, exercise, chemicals, fragrances and stress. Many sufferers struggle to identify their triggers and continue to discover new triggers for many years after diagnosis.

MCAS forms part of a spectrum of mast cell disorders involving proliferation and/or excessive sensitivity of mast cells, it has been identified since 2007. It features inappropriate mast cell activation with little or no increase in the number of mast cells, unlike in Mastocytosis*.  MCAS causes a wide range of unpleasant, sometimes debilitating, symptoms in any of the different systems of the body, frequently affecting several systems at the same time. The onset of MCAS is often sudden, affecting both children and adults, sometimes in family groups, mimicking many other conditions and presenting a wide-range of different symptoms that can be baffling for both the patient and their physician. Often there are no obvious clinical signs since MCAS confounds the anatomy-based structure underpinning the traditional diagnostic approach. Very often Mast Cell Activation Syndrome is hiding in plain sight.

Mastocystosis:  very rare and NOT what we’re talking about here…

Mastocytosis involves inappropriate mast cell activation AS WELL AS an increased number of mast cells. It is a rare but relatively well known mast cell disorder and is currently easier to diagnose than MCAS.

Mast Cell Activation Syndrome (MCAS): When Histamine Goes Haywire…

Mast cells are present in most tissues throughout the human body, especially connective tissue, skin, intestinal lining cardiovascular system, nervous system, and reproductive organs. They are part of the allergic response designed to protect us from threat and injury.  When the body is exposed to a perceived threat, the mast cells secrete chemical mediators, such as histamine, interleukins, prostaglandins, cytokines, chemokine and various other chemicals stored in the cytoplasm of the cell.  These chemical messengers produce both local and systemic effects, such as increased permeability of blood vessels (inflammation and swelling), contraction of smooth muscle (stomach cramps and heart palpitations), and increase mucous production (congestion, sneezing, etc).   Historically, we thought of mast cells only in relation to an allergic or anaphylactic response.  We now know they play a profound role in immune activation, development of autoimmunity and many other disorders, such as POTS (postural orthostatic tachycardia syndrome).  Sadly we are seeing a large increase in patients presenting with mast cell disorders and MCAS.  I believe it is in part do to the onslaught of more pervasive environmental toxins, moulds and chemicals.

Withouts mast cells, we would not be able to heal from a wound.  They protect us from injury and help the body to heal.  Unfortunately, overactive mast cells can cause a variety of serious symptoms.

Symptoms of overactive mast cells may include:

  • skin rashes/hives
  • swelling/oedema
  • flushing
  • asthma
  • itching
  • abdominal pain
  • nausea/vomiting
  • diarrhoea
  • wheezing
  • shortness of breath
  • heart palpitations
  • anxiety, difficulty concentrating
  • headaches
  • brain fog
  • low blood pressure
  • fatigue

Mast cell activation syndrome (MCAS) is a condition symptoms involving the skin, gastrointestinal, cardiovascular, respiratory, and neurologic systems. It can be classified into primary (clonal proliferation or mastocytosis), secondary (due to a specific stimulus), and idiopathic (no identifiable cause). Proposed criteria for the diagnosis of MCAS included episodic symptoms consistent with mast cell mediator release affecting two or more organ systems with hives, swelling, flushing, nausea, vomiting, diarrhea, abdominal pain, low blood pressure, fainting, heart palpitations, wheezing, red eyes, itching, and/or nasal congestion.  For a diagram of all of the varied symptoms histamine can cause, click here.

Triggers may be medications, foods, supplements, hormones, opioids, stressors (physical or emotional), cold temperature, heat, pressure, noxious odors, chemicals, insect bites, trauma or environmental toxins.

We commonly see mast cell activation syndromes associated with CIRS (chronic inflammatory response syndrome) in response to biotoxins, such as mould, inflammagens, and lyme-related toxins.

Low MSH and Mast Cell Disorders?

As mentioned above, we frequently see histamine intolerance and MCAS in patients with mold-related CIRS (chronic inflammatory response syndrome).  It is interesting to note that a common finding in CIRS is low MSH.  According to this study in the Journal of Investigative Dermatology, alpha-MSH plays an immunomodulatory role during inflammatory and allergic reactions of the skin.  In addition, there is evidence that MSH induces mast-cell apoptosis(cell death).

Definition of Mast Cell Activation Syndrome (MCAS)

  1. Typical clinical symptoms as listed above
  2. Increase in serum tryptase level or an increase in other mast cell derived mediators, such as histamine or prostaglandins (PGD2), or their urinary metabolites,
  3. Response of symptoms to treatment
Mast Cell Activation Syndrome (MCAS)

Mast cells can be activated by both direct and indirect mechanisms as a result of exposure of the host to pathogens.

Diseases Associated with Mast Cell Activation Syndrome (MCAS)

  • Allergies and Asthma
  • Autism
  • Autoimmune diseases (Hashimoto’s thyroiditis,  systemic lupus, multiple sclerosis, bullous pemphigoid, rheumatoid arthritis and others.Eczema
  • Celiac Disease
  • Chronic Fatigue Syndrome
  • CIRS (chronic inflammatory response syndrome)
  • Eosinophilic Esophagitis
  • Fibromyalgia
  • Food Allergy and Intolerances
  • Gastroesophageal reflux (GERD)
  • Infertility (mast cells in endometrium may contribute to endometriosis)
  • Interstitial Cystitis
  • Irritable Bowel Syndrome (IBS)
  • Migraine Headaches
  • Mood disorders – anxiety, depression, and insomnia
  • Multiple Chemical Sensitivities
  • POTS (postural orthostatic tachycardia syndrome)
Mast cells are known to be the primary responders in allergic reactions, orchestrating strong responses to minute amounts of allergens. Several recent observations indicate that they may also have a key role in coordinating the early phases of autoimmune diseases, particularly those involving auto-antibodies.

Mast cells are known to be the primary responders in allergic reactions, orchestrating strong responses to minute amounts of allergens. Several recent observations indicate that they may also have a key role in coordinating the early phases of autoimmune diseases, particularly those involving auto-antibodies.

Lab Tests for Mast Cell Activation Syndrome (MCAS) – many of these are as yet unavailable in Australia …  Possibly histamine is as yet the only available, but chat to Georgia about what our Naturopathic Labs can offer….

  • Lab tests specific to mast cell activation for suspected MCAS may include:
    • Serum tryptase (most famous mast cell mediator)
    • Serum chromogranin A
    • Plasma histamine
    • Plasma PGD2 (chilled)
    • Plasma heparin (chilled)
    • Urine for PGD2 (chilled)
    • PGF2a
    • N-methylhistamine
  • Tryptase is the most famous mast cell mediator. Serum tryptase value is usually normal in MCAS patients, but sometimes it is elevated.  Tryptase values that show an increase of 20% + 2 ng/ml above the baseline level are considered diagnostic for MCAS.
  • Chromogranin A is a heat-stable mast cell mediator.  High levels can suggest MCAS, but other sources must first be ruled out, such as heart failure, renal insufficiency, neuroendocrine tumors and proton pump inhibitor (PPI) use.
  • Heparin is a very sensitive and specific marker of mast cell activation.  However, due to its quick metabolism in the body, it is very difficult to measure reliably.
  • N-methylhistamine is usually measured in a 24 hour urine test to account for the variability in release over the course of the day.
  • Prostaglandin D2 is produced by several other cell types, but mast cell release is responsible for the dominant amount found in the body.  PGD2 is less stable than histamine and metabolized completely in 30 minutes.
  • Other less specific mast cell mediators that are sometimes abnormal in MCAS patients include Factor VIII, plasma free norepinephrine, tumor necrosis factor alpha, and interleukin-6.

Treatments to reduce MCAS symptoms and lower histamine

  • H1 Blockers
    1. hydroxyzine, doxepine, diphenhydramine, cetirizine, loratadine, fexofenadine
  • H2 Blockers
    1. Famotidine (Pepcid, Pepcid AC)
    2. Cimetidine (Tagamet, Tagamet HB)
    3. Ranitidine (Zantac)
  • Leukotriene inhibitors
    1. Montelukast (Singulair)
    2. Zafirlukast (Accolate)
  • Mast cell stabilizers –
    1. Cromolyn
    2. Ketotifen
    3. Hyroxyurea
  • Tyrosine kinase inhibitors – imatinib
  • Natural anti-histamines and mast-cell stabilizers
    • Ascorbic Acid
    • Quercetin
    • Vitamin B6 (pyridoxal-5-phosphate)
    • Omega-3 fatty acids (fish oil, krill oil)
    • Alpha Lipoic Acid
    • N-acetylcysteine (NAC)
    • Methylation donors (SAMe, B12, methyl-folate, riboflavin)
  • Certain probiotics decrease histamine production
    • Lactobacillus rhamnosus and bifidobacter species 
  • DAO Enzymes with meals – UmbrelluxDAO
  • Decrease consumption of high histamine foods (more on histamine-restricted diet)
    • Avoid alcoholic beverages
    • Avoid raw and cured sausage products such as salami.
    • Avoid processed or smoked fish products. Use freshly caught seafood instead.
    • Avoid pickles
    • Avoid citrus fruits.
    • Avoid chocolate
    • Avoid nuts
    • Avoid products made with yeast and yeast extracts
    • Avoid soy sauce and fermented soy products
    • Avoid black tea and instant coffee
    • Avoid aged cheese
    • Avoid spinach in large quantities
    • Avoid tomatoes, ketchup and tomato sauces
    • Avoid artificial food colorings & preservatives
    • Avoid certain spices: cinnamon, chili powder, cloves, anise, nutmeg, curry powder, cayenne pepper

References

  1. Mast Cell Activation Syndrome, A Review
  2. Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options
  3. Presentation, Diagnosis and Management of Mast Cell Activation Syndrome by Dr. Afrin
  4. Histamine and Gut Immune Mucosal Regulation
  5. Dr. Theoharides presents “Mast Cell Disorders”
  6. Diagram of Histamine Symptoms
  7. Mast Cell Aware
  8. A Tale of Two Syndromes
  9. Mold Histamine Connection