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Diagnosis of mast cell activation syndrome: a global “consensus-2”

Authors
  • Afrin, Lawrence B.1
  • Ackerley, Mary B.2
  • Bluestein, Linda S.3
  • Brewer, Joseph H.4
  • Brook, Jill B.5
  • Buchanan, Ariana D.6
  • Cuni, Jill R.7
  • Davey, William P.8
  • Dempsey, Tania T.1
  • Dorff, Shanda R.9
  • Dubravec, Martin S.10
  • Guggenheim, Alena G.11
  • Hindman, Kimberly J.
  • Hoffman, Bruce12
  • Kaufman, David L.13
  • Kratzer, Stephanie J.
  • Lee, Theodore M.6
  • Marantz, Mindy S.14
  • Maxwell, Andrew J.15
  • McCann, Kelly K.16
  • And 23 more
  • 1 Armonk Integrative Medicine, USA , (United States)
  • 2 My Passion 4 Health, AZ , (United States)
  • 3 Medical College of Wisconsin, WI , (United States)
  • 4 Plaza Infectious Disease, MO , (United States)
  • 5 Alaska Internal Medicine and Pediatrics, AK , (United States)
  • 6 Peachtree Allergy and Asthma, GA , (United States)
  • 7 Beaver Medical Group, CA , (United States)
  • 8 University of Kentucky, KY , (United States)
  • 9 Complex Cares, LLC, MN , (United States)
  • 10 Allergy and Asthma Specialists of Cadillac, MI , (United States)
  • 11 Oregon Health and Science University, OR , (United States)
  • 12 Hoffman Centre for Integrative and Functional Medicine, Alberta , (Canada)
  • 13 Center for Complex Diseases, CA , (United States)
  • 14 Healthwell Physical Therapy Group, CA , (United States)
  • 15 Heart of the Valley Pediatric Cardiology, CA , (United States)
  • 16 The Spring Center, CA , (United States)
  • 17 National College of Natural Medicine, OR , (United States)
  • 18 University of Utah, UT , (United States)
  • 19 Elevate Health, OR , (United States)
  • 20 Restoration Healthcare, CA , (United States)
  • 21 Haven Medical, NC , (United States)
  • 22 Royal College of General Practitioners, UK , (United Kingdom)
  • 23 University of California San Francisco, CA , (United States)
  • 24 University of Colorado, CO , (United States)
  • 25 Johns Hopkins University, MD , (United States)
  • 26 Center for Healing Neurology, WA , (United States)
  • 27 Center for Complex Neurology, EDS and POTS, AZ , (United States)
  • 28 Patient Navigator, LLC, VA , (United States)
  • 29 University of Colorado School of Medicine, CO , (United States)
  • 30 Washington University, MO , (United States)
  • 31 University Hospital Lübeck, Germany , (Germany)
  • 32 Westaway Medical, Queensland , (Australia)
  • 33 Keck School of Medicine, University of Southern California, CA , (United States)
  • 34 University Hospital of Bonn, Germany , (Germany)
Type
Published Article
Journal
Diagnosis
Publisher
De Gruyter
Publication Date
Apr 22, 2020
Volume
8
Issue
2
Pages
137–152
Identifiers
DOI: 10.1515/dx-2020-0005
Source
De Gruyter
Keywords
License
Green

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.

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