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. McCannDwight 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

Research output: Contribution to journalReview articlepeer-review

26 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)137-152
Number of pages16
JournalDiagnosis
Volume8
Issue number2
DOIs
StatePublished - May 1 2021

Keywords

  • chronic illness
  • mast cell activation disease
  • mast cell activation syndrome
  • medical controversies
  • medically unexplained symptoms
  • misdiagnosis

ASJC Scopus subject areas

  • Medicine(all)

Fingerprint

Dive into the research topics of 'Diagnosis of mast cell activation syndrome: A global "consensus-2"'. Together they form a unique fingerprint.

Cite this