Mast cell activation syndrome: Proposed diagnostic criteria

J Allergy Clin Immunol. 2010 Dec;126(6):1099-104.e4. doi: 10.1016/j.jaci.2010.08.035. Epub 2010 Oct 28.

Abstract

The term mast cell activation syndrome (MCAS) is finding increasing use as a diagnosis for subjects who present with signs and symptoms involving the dermis, gastrointestinal track, and cardiovascular system frequently accompanied by neurologic complaints. Such patients often have undergone multiple extensive medical evaluations by different physicians in varied disciplines without a definitive medical diagnosis until the diagnosis of MCAS is applied. However, MCAS as a distinct clinical entity has not been generally accepted, nor do there exist definitive criteria for diagnosis. Based on current understanding of this disease "syndrome" and on what we do know about mast cell activation and resulting pathology, we will explore and propose criteria for its diagnosis. The proposed criteria will be discussed in the context of other disorders involving mast cells or with similar presentations and as a basis for further scientific study and validation.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, N.I.H., Intramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Abnormalities, Multiple / diagnosis*
  • Abnormalities, Multiple / immunology*
  • Abnormalities, Multiple / pathology
  • Abnormalities, Multiple / physiopathology
  • Cell Degranulation / immunology
  • Cytokines / genetics
  • Cytokines / metabolism
  • Diagnosis, Differential
  • Humans
  • Leukotriene C4 / genetics
  • Leukotriene C4 / metabolism
  • Mast Cells / immunology
  • Mast Cells / metabolism*
  • Mast Cells / pathology
  • Practice Guidelines as Topic
  • Prostaglandin D2 / genetics
  • Prostaglandin D2 / metabolism
  • Syndrome

Substances

  • Cytokines
  • Leukotriene C4
  • Prostaglandin D2