AAAAI Mast Cell Disorders Committee Work Group Report: Mast cell activation syndrome (MCAS) diagnosis and management

Authors

• Catherine R. Weiler, Department of Internal Medicine, Division of Allergy, Mayo Clinic, Rochester, Minn.
• K Frank Austen, Department of Medicine, Division of Rheumatology, Immunology and Allergy, Brigham and Women's Hospital, Boston, Mass.
• Cem Akin, Division of Allergy and Clinical Immunology, University of Michigan, Ann Arbor, Mich.
• Marla S. Barkoff, Private Practice, Endocrinology, Chicago, Ill.
• Jonathan A. Bernstein, Internal Medicine, Immunology and Allergy, University of Cincinnati College of Medicine and Partner of Bernstein Allergy Group, and Bernstein Clinical Research Center, Cincinnati, Ohio.
• Patrizia Bonadonna, Allergy Unit, Multidisciplinary Mastocytosis Clinic, Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy.
• Joseph H. Butterfield, Department of Internal Medicine, Division of Allergy, Mayo Clinic, Rochester, Minn.
• Melody Carter, National Institutes of Health Clinical Center, Bethesda, Md.
• Charity C. Fox, Department of Otolaryngology, Division of Allergy and Immunology, Ohio State University Wexner Medical Center, Columbus, Ohio.
• Anne Maitland, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY.
• Thanai Pongdee, Department of Internal Medicine, Division of Allergy, Mayo Clinic, Rochester, Minn.
• S Shahzad Mustafa, Rochester Regional HealthFollow
• Anupama Ravi, Department of Pediatrics, Division of Pediatric Allergy and Immunology, Mayo Clinic, Rochester, Minn.
• Mary C. Tobin, Department of Internal Medicine, Allergy/Immunology Division, Rush University Medical Center, Chicago, Ill.
• Harissios Vliagoftis, Department of Medicine, University of Alberta, Edmonton, Alberta, Canada.
• Lawrence B. Schwartz, Department of Internal Medicine, Division of Rheumatology, Allergy and Immunology, Virginia Commonwealth University, Richmond, Va.

Department

Allergy and Immunology

Document Type

Article

Publication Title

The Journal of allergy and clinical immunology

Abstract

Our current recommendations for diagnosing and treating primary mast cell (MC) activation syndrome make use of the latest studies and consensus guidelines for clinically recognizing systemic anaphylaxis in real time, regardless of whether allergen-triggered or other pathways are involved; our current understanding of the biomarkers secreted by activated MCs that best discriminate this disorder from other conditions; and the therapeutic drugs that might selectively affect those mediators or MCs themselves. Finding familial or somatic mutations of genes that cause MCs to be hyperactivatable would extend our diagnostic tools and potentially indicate new therapeutic interventions, targeting either the mutated gene product or the associated molecular pathway. In conclusion, we trust that the clinical, laboratory, and therapeutic criteria for primary MC activation syndromes described herein will provide clinicians with practical criteria of sufficient sensitivity and specificity to diagnose most cases without overdiagnosing the disorder in patients who likely have other conditions.

First Page

883

Last Page

896

DOI

10.1016/j.jaci.2019.08.023

Volume

144

Issue

4

Publication Date

9-3-2019

Recommended Citation

Weiler, C. R., Austen, K. F., Akin, C., Barkoff, M. S., Bernstein, J. A., Bonadonna, P., Butterfield, J. H., Carter, M., Fox, C. C., Maitland, A., Pongdee, T., Mustafa, S. S., Ravi, A., Tobin, M. C., Vliagoftis, H., & Schwartz, L. B. (2019). AAAAI Mast Cell Disorders Committee Work Group Report: Mast cell activation syndrome (MCAS) diagnosis and management. The Journal of allergy and clinical immunology, 144 (4), 883-896. https://doi.org/10.1016/j.jaci.2019.08.023