Diagnosis of mast cell activation syndrome: a global "consensus-2".

Lawrence B Afrin, Mary B Ackerley, Linda S Bluestein, Joseph H Brewer, Jill B Brook, Ariana D Buchanan, Jill R Cuni, William P Davey, Tania T Dempsey, Shanda R Dorff, Martin S Dubravec, Alena G Guggenheim, Kimberly J Hindman, Bruce Hoffman, David L Kaufman, Stephanie J Kratzer, Theodore M Lee, Mindy S Marantz, Andrew J Maxwell, Kelly K McCann, Dwight L McKee, Laurie Menk Otto, Laura A Pace, Dahra D Perkins, Laurie Radovsky, Mary S Raleigh, Sonia A Rapaport, Emma J Reinhold, Mark L Renneker, William A Robinson, Aaron M Roland, E Scott Rosenbloom, Peter C Rowe, Ilene S Ruhoy, David S Saperstein, David A Schlosser, Jill R Schofield, Janet E Settle, Leonard B Weinstock, Martina Wengenroth, Mark Westaway, Shijun Cindy Xi, Gerhard J Molderings

Journal: Diagnosis (Berlin, Germany) 2021;8(2):137-152

PMID: 32324159

Abstract

The concept that disease rooted principally in chronic aberrant constitutive and reactive activation of mast cells (MCs), without the gross MC neoplasia in mastocytosis, first emerged in the 1980s, but only in the last decade has recognition of "mast cell activation syndrome" (MCAS) grown significantly. Two principal proposals for diagnostic criteria have emerged. One, originally published in 2012, is labeled by its authors as a "consensus" (re-termed here as "consensus-1"). Another sizable contingent of investigators and practitioners favor a different approach (originally published in 2011, newly termed here as "consensus-2"), resembling "consensus-1" in some respects but differing in others, leading to substantial differences between these proposals in the numbers of patients qualifying for diagnosis (and thus treatment). Overdiagnosis by "consensus-2" criteria has potential to be problematic, but underdiagnosis by "consensus-1" criteria seems the far larger problem given (1) increasing appreciation that MCAS is prevalent (up to 17% of the general population), and (2) most MCAS patients, regardless of illness duration prior to diagnosis, can eventually identify treatment yielding sustained improvement. We analyze these proposals (and others) and suggest that, until careful research provides more definitive answers, diagnosis by either proposal is valid, reasonable, and helpful.

©2020 Lawrence B. Afrin et al., published by De Gruyter, Berlin/Boston.

Address: Armonk Integrative Medicine, Purchase, NY, USA.; My Passion 4 Health, Tucson, AZ, USA.; Department of Anesthesiology, Medical College of Wisconsin, Wausau, WI, USA.; Plaza Infectious Disease, Kansas City, MO, USA.; Alaska Internal Medicine and Pediatrics, Anchorage, AK, USA.; Peachtree Allergy and Asthma, Atlanta, GA, USA.; Division of Pediatrics, Beaver Medical Group, Banning, CA, USA.; Department of Dermatology, University of Kentucky, Lexington, KY, USA.; Complex Cares, LLC, Shoreview, MN, USA.; Allergy and Asthma Specialists of Cadillac, Cadillac, MI, USA.; Comprehensive Pain Center, Oregon Health and Science University, Portland, OR, USA.; Healing Dragon, Portland, OR, USA.; Hoffman Centre for Integrative and Functional Medicine, Calgary, Alberta, Canada.; Center for Complex Diseases, Mountain View, CA, USA.; CHI Health St. Elizabeth, Lincoln, NE, USA.; Healthwell Physical Therapy Group, San Francisco, CA, USA.; Heart of the Valley Pediatric Cardiology, Pleasanton, CA, USA.; The Spring Center, Costa Mesa, CA, USA.; Integrative Cancer Consulting, Aptos, CA, USA.; Helfgott Research Institute, National College of Natural Medicine, Portland, OR, USA.; Division of Gastroenterology, Hepatology and Nutrition, University of Utah, Salt Lake City, UT, USA.; Elevate Health, Portland, OR, USA.; St. Paul, MN, USA.; Restoration Healthcare, Irvine, CA, USA.; Haven Medical, Chapel Hill, NC, USA.; Royal College of General Practitioners, London, UK.; Department of Family Medicine, University of California San Francisco, San Francisco, CA, USA.; Division of Hematology/Oncology, University of Colorado, Denver, CO, USA.; Sacramento, CA, USA.; Department of Pediatrics, Johns Hopkins University, Baltimore, MD, USA.; Center for Healing Neurology, Seattle, WA, USA.; Center for Complex Neurology, EDS and POTS, Phoenix, AZ, USA.; Patient Navigator, LLC, Reston, VA, USA.; Department of Internal Medicine, University of Colorado School of Medicine, Aurora, CO, USA.; Department of Psychiatry, University of Colorado, Denver, CO, USA.; Department of Medicine, Washington University, St. Louis, MO, USA.; Institute of Neuroradiology, University Hospital Lübeck, Lübeck, Germany.; Westaway Medical, Brisbane, Queensland, Australia.; Section of Allergy and Immunology, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.; Institute of Human Genetics, University Hospital of Bonn, Bonn, Germany.

Link outs

Bant logo

© Copyright 2026, Nutrition Evidence

NED wishes to thank the following organisations for their support:

We use cookies to improve your experience and analyze site traffic with Google Analytics. By continuing to use our site, you agree to our use of cookies. Learn more.