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Mast Cell Activation Syndrome: A Primer for the Gastroenterologist

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Abstract

Mast cell activation syndrome is thought to be a common, yet under-recognized, chronic multi-system disorder caused by inappropriate mast cell activation. Gastrointestinal symptoms are frequently reported by these patients and are often mistaken by physicians as functional gastrointestinal disorders. This syndrome can be diagnosed by the medical history and measurable biomarkers. Gastroenterologists manage diseases associated with active inflammatory cells including neutrophils, lymphocytes, macrophages, and eosinophils. The mast cell has only recently been recognized as a major player in our specialty. Gastrointestinal disorders from mast cell mediators often present with apparent irritable bowel syndrome, dyspepsia, chronic or cyclical nausea, and heartburn. Individuals with mast cell activation syndrome experience significant delays in diagnosis. The gastrointestinal symptoms are often refractory to symptom-targeted prescription medications. Beyond avoiding triggers, the best therapy is directed at modulating mast cell activation and the effects of the mediators. Many of these therapies are simple over-the-counter medications. In this article, we review mast cell function and dysfunction and the gastrointestinal symptoms, comorbid conditions, diagnosis, and management of mast cell activation syndrome. Gastroenterologists who become aware of this syndrome can dramatically improve the quality of life for their patients who previously have been labeled with a functional gastrointestinal disorder.

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Funding

This study was funded by LAP, Office of Research on Women’s Health of the NIH K12HD085852.

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Authors and Affiliations

  1. Specialists in Gastroenterology, 11525 Olde Cabin Rd, St. Louis, MO, 63141, USA

    Leonard B. Weinstock

  2. Division of Gastroenterology, Department of Internal Medicine, University of Utah, 30 N 1900 E, SOM 4R118, Salt Lake City, UT, 84132, USA

    Laura A. Pace

  3. Cedars-Sinai Medical Center, Gastroenterology, 8730 Alden Dr., Suite 204E, Los Angeles, CA, 90048, USA

    Ali Rezaie

  4. Armonk Integrative Medicine, Hematology/Oncology, 3010 Westchester Avenue, Suite 401, Armonk, NY, 10577, USA

    Lawrence B. Afrin

  5. Institute of Human Genetics, University Hospital Bonn, 53127, Bonn, Germany

    Gerhard J. Molderings

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  1. Leonard B. Weinstock
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Contributions

LBW, MD, FACG, is the guarantor of the article. LBW and LAP performed the literature search and co-wrote the manuscript. AR, LBA, and GJM provided significant intellectual input, critical review, and revisions to the manuscript. The final draft was reviewed and approved by LBW, LAP, AR, LBA, and GJM.

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Correspondence to Leonard B. Weinstock.

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Conflict of interest

Drs. Weinstock, Pace, Rezaie, and Afrin do not have any conflicts of interest. Dr. Molderings is the Chief Medical Officer of the startup company MC Sciences, Ltd.

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Weinstock, L.B., Pace, L.A., Rezaie, A. et al. Mast Cell Activation Syndrome: A Primer for the Gastroenterologist. Dig Dis Sci 66, 965–982 (2021). https://doi.org/10.1007/s10620-020-06264-9

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