What is Mast Cell Activation Syndrome?
Mast Cell Activation Syndrome (MCAS) forms part of a spectrum of mast cell disorders caused by the abnormal proliferation, accumulation, function and/or activation of mast cells. It causes a wide range of unpleasant, frequently debilitating allergic and inflammatory symptoms in many different systems of the body.
People with MCAS can react to a wide range of triggers and potentiating factors including foods, chemicals, drugs, fragrances, temperature, exercise, stress, hormonal fluctuations and infection.
The onset of MCAS can be unpredictable, affecting both children and adults, and presents with a wide range of symptoms in multiple body systems, ranging from hives and rashes, respiratory problems, syncope, gastrointestinal pain, diarrhoea, reflux, neurological problems including anxiety and brain fog, bone pain, and anaphylaxis.
The complex, multisystem nature of MCAS can make gaining a diagnosis particularly challenging. The ability to gain a diagnosis is also negatively impacted by a lack of universally accepted diagnostic criteria and access to diagnostic biomarkers.
There is an urgent need to give a voice to patients, support doctors and researchers, and inform policy-makers about this widely under-recognised condition.
With your support, we feel confident that we can make a substantial difference to the lives of those affected by MCAS and their families.
You can read our collection of patient stories here.
Symptoms of MCAS
The symptoms of MCAS are often unpleasant, sometimes debilitating and can include life-threatening anaphylaxis. These symptoms usually involve multiple systems of the body and can include skin (dermatologic) symptoms, gastrointestinal symptoms (such as abdominal pain, nausea and vomiting), respiratory symptoms, neurological problems and urinary issues.
MCAS symptoms are often episodic or wax and wane (meaning that they go and come back) and their severity can fluctuate over time. In some cases, people may suffer mild symptoms for a long time before experiencing a sudden increase in the frequency or severity of symptoms. Possibly after a particularly triggering incident for the immune system, such as a severe infection or a medical procedure or operation.
The extent of symptoms for people suffering MCAS can be substantial. The potential severity of symptoms paired with the unpredictability of triggers can mean that the everyday lives of people with MCAS, their families and carers are considerably impacted.
Mast Cell Mediators
Mast Cells are tissue white blood cells, found in all parts of the body
They are functionally and phenotypically heterogeneous. They are a key part of the innate immune system with a role in immune defence, patrolling linings of mucosal surfaces of organs: the host-environment interface
In response to triggers mast cells release a range of mast cell mediators. (See image to the left)
Comorbidities
Observational studies and patient-reported data show ‘clustering’ of MCAS with other conditions, meaning that people who have MCAS often also have at least one additional condition.
Common comorbidities include connective tissue disorders, e.g. Ehlers Danlos Syndrome (EDS) and Marfans, dysautonomia, such as postural orthostatic tachycardia syndrome (POTS), and type 2 diabetes. The relationship between MCAS and many of these comorbidities is yet to be fully understood, and more research is required.
Further information on comorbidities can be found in the Royal College of GPs Ehlers Danlos Syndromes Toolkit.
Link Between Mast Cell Activation Syndrome (MCAS) and Long COVID
As the medical community continues to unravel the complexities of Long COVID, emerging research suggests a significant link with Mast Cell Activation Syndrome (MCAS).
In recent studies, an increasing number of Long COVID patients exhibit symptoms that show similarities to MCAS, including persistent inflammation, gastrointestinal disturbances, and neurocognitive issues. We know that understanding this connection is likely to be crucial in developing effective diagnostic and therapeutic strategies for patients living with MCAS and/or long covid.
A study conducted in 2021, highlighted the similarities between the symptoms of those living with Long Covid and those with MCAS. 136 adults (mostly female) in a long covid focus group were recruited for an online assessment of symptoms pre & post COVID infection.
- 136 cases 89% F;
- 136 controls, 65% F;
- 80 MCAS cases 85% F
- Pre COVID LC subjects & controls similar symptoms
As you can see from the image on the left, Long COVID subjects & those with MCAS prior to treatment, had identical symptoms. Furthermore, MCAS symptoms increased in Long Covid patients, similar to those who have MCAS.
Conclusion: activation aberrant mast cells was induced by SARS-CoV-2 infection and may underlie part of the pathophysiology of long covid, suggesting routes to effective treatments
Mast cell activation symptoms are prevalent in long covid Weinstock L, Brook J, Afrin L, Molderings G. Int Journal of Infectious Diseases. 2021;112:217-226
Professional Network
If you are treating patients with MCAS or suspected MCAS or are interested in treating patients with MCAS, please register your details with us here. We are passionate about connecting healthcare professionals to share knowledge and collaborate. Mast Cell Action believes together we can enhance research, education, and awareness and ultimately improve the quality of life for those living with mast cell disease.
Join our professional network to:
- Receive important news and updates
- Get invites to dedicated HCP events
- Get updates about resources for patients
- Have optional listing on our database of HCP's - Opt in required
Useful presentations
Watch two presentations given by Dr Alex Croom and Dr Bethan Myers at the 2019 PoTs Masterclass in the UK for more information on MCAS.
Watch two presentations given by Dr. Anne Maitland from the ICAHN school of medicine at Mount Sinai in the US:
- EDS Virtual Summer Conference 2020: Treatment of Allergy and Immunology Issues
- Bobby Jones Chiari & Syringomyelia Foundation Educational Lecture Series 2019: Mast Cell Activation Syndrome
Listen to Dr. Theoharis Theoharides explain more about mast cells and the role of mast cell activation in the immune system here.
We are urgently looking for people with knowledge and understanding of the medical community, to help us with our communication and advocacy in that area.
If this is you – or you know someone who fits the bill – please do contact Joy directly.
Thank you
Further reading
Akin C. Mast cell activation syndromes. J Allergy Clin Immunol. 2017;140(2):349-355.
Molderings GJ, Haenisch B, Bogdanow M, Fimmers R, Nöthen MM. Familial occurrence of systemic mast cell activation disease. PLoS One. 2013;8(9):e76241. Published 2013 Sep 30.
Theoharides TC, Tsilioni I, Ren H. Recent advances in our understanding of mast cell activation - or should it be mast cell mediator disorders?. Expert Rev Clin Immunol. 2019;15(6):639-656.
Afrin LB, Ackerley MB, Bluestein LS, et al. Diagnosis of mast cell activation syndrome: a global "consensus-2". Diagnosis (Berl). 2020;/j/dx.ahead-of-print/dx-2020-0005/dx-2020-0005.xml.
Simons FER, Ardusso LR, Bilò MB, et al. International consensus on (ICON) anaphylaxis. World Allergy Organization Journal.
Resources to share with patients
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