Mast cell activation syndrome: Importance of consensus criteria and call for research
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After deliberations and shared practical experience, the faculty produced a consensus proposal and introduced the 20% + 2 formula at the Working Conference.
“After in-depth deliberations and based on shared practical experience, the faculty did come up with a consensus proposal and put forth the 20% + 2 formula during the Working Conference [21].”
From Why the 20% + 2 Tryptase Formula Is a Diagnostic Gold Standard for Severe Systemic Mast Cell Activation and Mast Cell Activation Syndrome (2019) · cites this paper as [21]
The paper’s main text says an international consensus group met and introduced MCAS diagnostic criteria, including a serum tryptase increase of 20% over baseline plus 2 ng/mL. It does not say the formula was put forth at a Working Conference or describe the group’s deliberations and shared practical experience. No page numbers are provided in our copy. (LLM, conf=0.86)
Experimental — accuracy not yet measuredMCAS is diagnosed when there is documented recurrent episodic occurrence of typical systemic symptoms produced by mast cell mediators and involving at least two organ systems.
“documented recurrent episodic occurrence of typical systemic symptoms that are produced by MC mediators and involve at least 2 organ systems”
From Why the 20% + 2 Tryptase Formula Is a Diagnostic Gold Standard for Severe Systemic Mast Cell Activation and Mast Cell Activation Syndrome (2019) · cites this paper as [21]
The paper’s diagnostic-criteria passage identifies episodic mast-cell-related symptoms affecting two or more organ systems as the first criterion. It also requires a specified rise in serum tryptase and improvement with mast-cell-targeting treatment, so the symptoms alone do not establish an MCAS diagnosis. (LLM, conf=0.86)
Experimental — accuracy not yet measuredAt the 2010 Working Conference, one issue discussed was the smallest rise in serum tryptase above baseline that would count as significant systemic mast cell activation and as a diagnostic marker of MCAS.
“one of the deliberation points was the minimal increase in serum tryptase over the individual’s baseline that would meet the criterion of a significant systemic MC activation and would thus count as a diagnostic marker of MCAS [21].”
From Why the 20% + 2 Tryptase Formula Is a Diagnostic Gold Standard for Severe Systemic Mast Cell Activation and Mast Cell Activation Syndrome (2019) · cites this paper as [21]
The main text (page unavailable) says a consensus group met to establish MCAS diagnostic criteria and identifies a serum tryptase rise of 20% above baseline plus 2 ng/mL as one criterion. It does not identify a 2010 Working Conference or say that determining the minimum rise was a specific deliberation point. (LLM, conf=0.84)
Experimental — accuracy not yet measuredAccording to consensus criteria for MCAS diagnosis, sAT should be greater than sBT -1.2 + 2 ng/mL to confirm MCAS.
“According to the consensus criteria for the diagnosis of MCAS, sAT should be greater than sBT -1.2 þ 2 ng/mL to con fi rm the presence of MCAS.”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [42]
The diagnostic-criteria passage [4] specifies an acute serum tryptase increase of 20% over baseline plus 2 ng/mL. The claim prints subtraction of 1.2 rather than multiplication by 1.2; moreover, the paper requires characteristic symptoms and a response to treatment as additional criteria, so the tryptase threshold alone does not confirm MCAS. Page numbers are not available in our copy. (LLM, conf=0.88)
Experimental — accuracy not yet measuredThe 20% + 2 formula was put forward by a consensus group in 2012.
“the 20% + 2 formula was proposed by the consensus group in 2012.”
From Why the 20% + 2 Tryptase Formula Is a Diagnostic Gold Standard for Severe Systemic Mast Cell Activation and Mast Cell Activation Syndrome (2019) · cites this paper as [21]
MCAS is diagnosed when there is a documented clinically meaningful response to drugs that target mast cell mediators and/or suppress mast cell activation.
“a documented clinically meaningful response to drugs that either target MC-derived mediators (e.g., H1 antihistamines or antileukotrienes) and/or suppress MC activation (e.g., cromoglycate, ketotifen, or omalizumab) [19, 21, 22].”
From Why the 20% + 2 Tryptase Formula Is a Diagnostic Gold Standard for Severe Systemic Mast Cell Activation and Mast Cell Activation Syndrome (2019) · cites this paper as [21]
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- Authors
- Valent, P., Akin, C., Bonadonna, P., Hartmann, K., Broesby-Olsen, S., Brockow, K., Butterfield, J. H., Reiter, A., Gotlib, J., Castells, M., Milner, J. D., Carter, M. C., Komarow, H., Radia, D., Pardanani, A., Sotlar, K., Triggiani, M., Horny, H.-P., Arock, M., Schwartz, L. B., & Metcalfe, D. D.
- Affiliations
- Medical University of Vienna (AT)
- University of Michigan (US)
- Azienda Ospedaliera Universitaria Integrata Verona (IT)
- University of Lübeck (DE)
- Technical University of Munich (DE)
- Mayo Clinic (US)
- Heidelberg University (DE)
- Stanford Cancer Institute (US)
- Brigham and Women's Hospital (US)
- National Institutes of Health (US)
- Guy's Hospital (GB)
- Paracelsus Medical University (AT)
- University of Salerno (IT)
- Pathologisches Institut (DE)
- Centre National de la Recherche Scientifique (FR)
- Virginia Commonwealth University (US)
- Corresponding author
- Valent, Peter
- ORCID
- Venue
- Journal of Allergy and Clinical Immunology
- Publisher
- Elsevier BV
- Published
- 2018-09
- Volume
- 142
- Issue
- 3
- Pages
- 1008-1010
- ISSN
- 0091-6749 (ISSN-L)
- PMC
- PMC7115848
- OpenAlex
- W2809032396
- Cited by
- 34
- References
- 10
- Citations by year
- 2026: 3 · 2025: 4 · 2023: 1 · 2022: 3 · 2021: 5 · 2020: 4 · 2019: 13 · 2018: 1
- FWCI
- 2.47
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Journal of Allergy and Clinical Immunology (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- University of Southern Denmark Research Portal (University of Southern Denmark) (submittedVersion, cc-by-nc-nd)
- edoc (University of Basel) (submittedVersion)
- mediaTUM – the media and publications repository of the Technical University Munich (Technical University Munich) (submittedVersion)
- Funders
- Virginia Commonwealth University
- Austrian Science Fund (#F4704-B20, F4701, F4701-B20)
- National Institute of Allergy and Infectious Diseases
- NHLBI Division of Intramural Research
- Topics
- Life Sciences › Immunology and Microbiology › Immunology › Mast cells and histamine
- Health Sciences › Medicine › Immunology and Allergy › Food Allergy and Anaphylaxis Research
- Health Sciences › Medicine › Physiology › Asthma and respiratory diseases
- Keywords
- mast cell activation syndrome, mast cell activation, mast cell disease, diagnostic validity, overdiagnosis, referral centers, mast cell mediators
- MeSH terms
- Consensus Statements as Topic, Humans, Mast Cells, Mastocytosis, Consensus, Biomedical Research
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