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Mast cell activation syndrome (MCAS) is a term applied to several clinical entities that have gained increased attention from patients and medical providers. Although several descriptive publications about MCAS exist, there are many gaps in knowledge, resulting in confusion about this clinical syndrome. Whether MCAS is a primary syndrome or exists as a constellation of symptoms in the context of known inflammatory, allergic, or clonal disorders associated with systemic mast cell activation is not well understood. More importantly, the underlying mechanisms and pathways that lead to mast cell activation in MCAS patients remain to be elucidated. Here we summarize the known literature, identify gaps in knowledge, and highlight research needs. Covered topics include contextualization of MCAS and MCAS-like endotypes and related diagnostic evaluations; mechanistic research; management of typical and refractory symptoms; and MCAS-specific education for patients and health care providers.

Original publication

DOI

10.1016/j.jaci.2024.05.025

Type

Journal article

Journal

J allergy clin immunol

Publication Date

08/2024

Volume

154

Pages

255 - 263

Keywords

Mast cell, Mast cell activation syndrome, hereditary alpha-tryptasemia, mast cell activation–related disorders, mastocytosis, tryptase, Humans, Mast Cells, Mastocytosis, Syndrome, Animals