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Cited by 101Mast cells and histamineAsthma and respiratory diseasesEosinophilic Disorders and Syndromes

Characterization of Mast Cell Activation Syndrome

Open access
Afrin, Lawrence B., Self, Sally, Menk, Jeremiah, Lazarchick, John·2017·The American Journal of the Medical Sciences
DOI 10.1016/j.amjms.2016.12.013PMC PMC5341697
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Abstract

Background Mast cell activation syndrome (MCAS), a recently recognized nonneoplastic mast cell disease driving chronic multisystem inflammation and allergy, appears prevalent and thus important. We report the first systematic characterization of a large MCAS population. Method Demographics, comorbidities, symptoms, family histories, physical examination and laboratory findings were reviewed in 298 retrospective and 115 prospective patients with MCAS. Blood samples from prospective subjects were examined by flow cytometry for clonal mast cell disease and tested for cytokines potentially driving the monocytosis frequent in MCAS. Results Demographically, white females dominated. Median ages at symptom onset and diagnosis were 9 and 49 years, respectively (range: 0-88 and 16-92, respectively) and median time from symptom onset to diagnosis was 30 years (range: 1-85). Median numbers of comorbidities, symptoms, and family medical issues were 11, 20, and 4, respectively (range: 1-66, 2-84, and 0-33, respectively). Gastroesophageal reflux, fatigue and dermatographism were the most common comorbidity, symptom and examination finding. Abnormalities in routine laboratories were common and diverse but typically modest. The most useful diagnostic markers were heparin, prostaglandin D 2, histamine and chromogranin A. Flow cytometric and cytokine assessments were unhelpful. Conclusions Our study highlights MCAS׳s morbidity burden and challenging heterogeneity. Recognition is important given good survival and treatment prospects.

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Crossref · 10.1016/j.amjms.2016.12.013

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  • There have not yet been any large-scale studies of any aspects of MCAS, though a few small-scale studies are beginning to emerge.

    “there have not yet been any large-scale studies of any aspects of the disease, though a few small-scale such studies are beginning to emerge [12,33,91].”

    From Diagnosis of mast cell activation syndrome: a global "consensus-2" (2021) · cites this paper as [12]

    The Introduction says earlier MCAS reports included a few small series, but describes this paper as the first systematic objective characterization of a large adult MCAS population. The Methods report 413 patients (298 retrospective and 115 prospective). Thus, the source supports the existence of earlier small studies but contradicts the claim that no large-scale study of any aspect of MCAS had yet appeared. (LLM, conf=0.89)

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  • MCAD is challenging to learn to recognize and challenging to diagnose.

    “MCAD, too, is challenging to learn to recognize and challenging to diagnose [12,34,53].”

    From Diagnosis of mast cell activation syndrome: a global "consensus-2" (2021) · cites this paper as [12]

    The Introduction says MCAS is difficult to recognize clinically, and the Discussion describes delays in its recognition and diagnosis and contention over diagnostic criteria. The paper identifies MCAS as a major part of MCAD, but these passages do not establish that MCAD as a whole is challenging to learn to recognize or diagnose. (LLM, conf=0.82)

    Experimental — accuracy not yet measured

  • Most MCAS patients, regardless of illness duration prior to diagnosis, can eventually identify treatment yielding sustained improvement.

    “most MCAS patients, regardless of illness duration prior to diagnosis, can eventually identify treatment yielding sustained improvement”

    From Diagnosis of mast cell activation syndrome: a global "consensus-2" (2021) · cites this paper as [12]

    The Discussion (page unavailable) says that, in the authors’ experience, most patients eventually find regimens that make them feel significantly better than their pre-treatment baseline the majority of the time. The Results report long and varied delays before diagnosis, but the paper does not establish that treatment improvement occurs regardless of illness duration; the claim also presents an experiential observation more definitively than the source does. (LLM, conf=0.84)

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Cites

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1 of the 47 references couldn’t be matched to a known paper.

Research works printed but not linked: 1

  1. [35]Pejler G Rönnberg E Waern I Mast cell proteases: multifaceted regulators of inflammatory disease Blood 2010 115 4981 4990 [PMID: 20233968] 20233968

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Authors
Afrin, L. B., Self, S., Menk, J., & Lazarchick, J.
Affiliations
  • University of Minnesota (US)
  • Medical University of South Carolina (US)
Corresponding author
Afrin, Lawrence B.
ORCID
Venue
The American Journal of the Medical Sciences
Publisher
Elsevier BV
Published
2017-03
Volume
353
Issue
3
Pages
207-215
ISSN
0002-9629 (ISSN-L)
DOI
10.1016/j.amjms.2016.12.013
PMC
PMC5341697
OpenAlex
W2566134933
Cited by
101
References
47
Citations by year
2026: 11 · 2025: 7 · 2024: 8 · 2023: 23 · 2022: 12 · 2021: 15 · 2020: 10 · 2019: 8 · 2018: 5 · 2017: 2
FWCI
3.03
Type
journal article
Language
en
Open access
Licence
www.elsevier.com/tdm/userlicense/1.0/ (tdm)
Locations
  • The American Journal of the Medical Sciences (publishedVersion)
  • PubMed (publishedVersion)
  • Europe PMC (PubMed Central) (acceptedVersion)
  • PubMed Central (acceptedVersion)
Funders
National Institutes of Health (UL1TR000114)
Topics
  • Life Sciences › Immunology and Microbiology › Immunology › Mast cells and histamine
  • Health Sciences › Medicine › Physiology › Asthma and respiratory diseases
  • Health Sciences › Medicine › Rheumatology › Eosinophilic Disorders and Syndromes
Keywords
mast cell activation syndrome, allergy, comorbidities, gastroesophageal reflux, fatigue, dermatographism, heparin, prostaglandin D2, histamine, chromogranin A, monocytosis, diagnostic biomarkers, disease heterogeneity, patient demographics
MeSH terms
Adolescent, Adult, Aged, Aged, 80 and over, Child, Child, Preschool, Female, Heparin, Histamine, Humans, Infant, Infant, Newborn, Male, Mastocytosis, Middle Aged, Prospective Studies, Retrospective Studies, Syndrome, Prostaglandin D2, Biomarkers, Chromogranin A, Young Adult

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