Low Prevalence of Idiopathic Mast Cell Activation Syndrome Among 703 Patients With Suspected Mast Cell Disorders
Idiopathic mast cell activation syndrome (iMCAS) is characterized by severe, episodic systemic mast cell (MC) activation and mediator-related symptoms, an event-related increase in serum tryptase levels, and response to MC-targeted therapies in the absence of underlying IgE-mediated allergy or clonal MC disorder. Studies indicating its prevalence using evidence-based diagnostic criteria are lacking.