Abstract

Mast Cell Activation Syndrome (MCAS) is a hypersensitivity disorder, and subset of Mast Cell Disease, which has become increasingly recognized over the past three decades. Since the first case reports were published in the late 1980s, 1 Akin C Valent P Metcalfe DD. Mast cell activation syndrome: proposed diagnostic criteria. J Allergy Clin Immunol. 2010; 126 (e4): 1099-1104 Abstract Full Text Full Text PDF PubMed Scopus (229) Google Scholar , 2 Roberts LJ. Carcinoid syndrome and disorders of systemic mast-cell activation including systemic mastocytosis. Endocrinol Metab Clin North Am. 1988; 17: 415-436 Abstract Full Text PDF PubMed Google Scholar significant advancements have been made to better understand MCAS characteristics and determine effective treatment strategies. With improvements in recognition and modification of diagnostic criteria, it is estimated that up to 17% of the population has MCAS, 3 Buttgereit T Gu S Carneiro-Leão L et al. Idiopathic mast cell activation syndrome is more often suspected than diagnosed – a prospective real-life study. Allergy. 2022; 77: 2794-2802 Crossref Scopus (2) Google Scholar , 4 Afrin LB Ackerley MB Bluestein LS et al. Diagnosis of mast cell activation syndrome: a global “consensus-2”. Diagnosis (Berl). 2020; 8: 137-152 Crossref PubMed Scopus (34) Google Scholar highlighting the importance of clinician education regarding this recently identified disorder. To better understand the pathophysiology of MCAS—and the pharmacologic interventions effective in its management—it is important to first begin by discussing the normal activity of mast cells.

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