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

Author:

Afrin Lawrence B.1ORCID,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.1ORCID,Dorff Shanda R.9,Dubravec Martin S.10,Guggenheim Alena G.11,Hindman Kimberly J.12,Hoffman Bruce13,Kaufman David L.14,Kratzer Stephanie J.15,Lee Theodore M.6,Marantz Mindy S.16,Maxwell Andrew J.17,McCann Kelly K.18,McKee Dwight L.19,Menk Otto Laurie20,Pace Laura A.21,Perkins Dahra D.22,Radovsky Laurie23,Raleigh Mary S.24,Rapaport Sonia A.25,Reinhold Emma J.26,Renneker Mark L.27,Robinson William A.28,Roland Aaron M.27,Rosenbloom E. Scott29,Rowe Peter C.30,Ruhoy Ilene S.31,Saperstein David S.32,Schlosser David A.33,Schofield Jill R.34,Settle Janet E.35,Weinstock Leonard B.36,Wengenroth Martina37,Westaway Mark38,Xi Shijun Cindy39,Molderings Gerhard J.40

Affiliation:

1. Armonk Integrative Medicine , Purchase, NY , USA

2. My Passion 4 Health , Tucson , AZ , USA

3. Department of Anesthesiology , Medical College of Wisconsin , Wausau , WI , USA

4. Plaza Infectious Disease , Kansas City , MO , USA

5. Alaska Internal Medicine and Pediatrics , Anchorage , AK , USA

6. Peachtree Allergy and Asthma , Atlanta , GA , USA

7. Division of Pediatrics , Beaver Medical Group , Banning , CA , USA

8. Department of Dermatology , University of Kentucky , Lexington , KY , USA

9. Complex Cares, LLC , Shoreview , MN , USA

10. Allergy and Asthma Specialists of Cadillac , Cadillac , MI , USA

11. Comprehensive Pain Center , Oregon Health and Science University , Portland , OR , USA

12. Healing Dragon, Portland , OR , USA

13. Hoffman Centre for Integrative and Functional Medicine , Calgary , Alberta , Canada

14. Center for Complex Diseases , Mountain View , CA , USA

15. CHI Health St. Elizabeth, Lincoln , NE , USA

16. Healthwell Physical Therapy Group , San Francisco , CA , USA

17. Heart of the Valley Pediatric Cardiology , Pleasanton , CA , USA

18. The Spring Center , Costa Mesa , CA , USA

19. Integrative Cancer Consulting, Aptos , CA , USA

20. Helfgott Research Institute , National College of Natural Medicine , Portland , OR , USA

21. Division of Gastroenterology, Hepatology and Nutrition , University of Utah , Salt Lake City , UT , USA

22. Elevate Health , Portland , OR , USA

23. St. Paul , MN , USA

24. Restoration Healthcare , Irvine , CA , USA

25. Haven Medical , Chapel Hill , NC , USA

26. Royal College of General Practitioners , London , UK

27. Department of Family Medicine , University of California San Francisco , San Francisco , CA , USA

28. Division of Hematology/Oncology , University of Colorado , Denver , CO , USA

29. Sacramento , CA , USA

30. Department of Pediatrics , Johns Hopkins University , Baltimore , MD , USA

31. Center for Healing Neurology , Seattle , WA , USA

32. Center for Complex Neurology, EDS and POTS , Phoenix , AZ , USA

33. Patient Navigator, LLC , Reston , VA , USA

34. Department of Internal Medicine , University of Colorado School of Medicine , Aurora , CO , USA

35. Department of Psychiatry , University of Colorado , Denver , CO , USA

36. Department of Medicine , Washington University , St. Louis , MO , USA

37. Institute of Neuroradiology , University Hospital Lübeck , Lübeck , Germany

38. Westaway Medical , Brisbane , Queensland , Australia

39. Section of Allergy and Immunology , Keck School of Medicine, University of Southern California , Los Angeles , CA , USA

40. Institute of Human Genetics , University Hospital of Bonn , Bonn , Germany

Abstract

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.

Publisher

Walter de Gruyter GmbH

Subject

Biochemistry (medical),Clinical Biochemistry,Public Health, Environmental and Occupational Health,Health Policy,Medicine (miscellaneous)

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