MCAS

Hives in autonomic disorders: a cutaneous marker of a distinct symptom phenotype.

Postural Orthostatic Tachycardia Syndrome (POTS) and Neurally-Mediated Hypotension (NMH) are heterogeneous syndromes characterized by dysautonomia and multisystem symptoms. Mast cell activation, often manifesting as hives, has been proposed as a contributing mechanism, but its prevalence and clinica…

Urine mast cell mediators and anaphylaxis.

Anaphylaxis is a severe type 1 hypersensitivity reaction which is the result of mast cell degranulation causing multiorgan dysfunction. Currently, the most accepted biomarker measurement for mast cell mediated anaphylaxis is serum tryptase measurement. Serum tryptase measurement has limitations incl…

Non-immunoglobulin E-mediated mechanisms of anaphylaxis.

Anaphylaxis attributable to non-immunoglobulin E (IgE)-mediated mechanisms represents an increasingly recognized and clinically challenging subset of severe hypersensitivity reactions. This review synthesizes recent advances in the understanding of IgE-independent pathways, with emphasis on literatu…

Pathophysiological Drivers of Mast Cell Activation Syndrome and Implications for Treatment.

Mast Cell Activation Syndrome (MCAS) is an underrecognized multisystem disorder causing nonspecific and diverse symptoms, making it difficult for clinicians to diagnose and treat. This comprehensive review provides an overview of the signs and symptoms of MCAS while investigating the known pathophys…

Gastrointestinal Manifestations of Hypermobile Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorders: A Mentored Review.

Hypermobile Ehlers-Danlos Syndrome (hEDS) is a connective tissue disorder that is often diagnosed after diagnostic delays due to reliance on heightened clinical suspicion. Hypermobility spectrum disorders (HSD) bridge the gap between asymptomatic joint hypermobility and hEDS. Due to overlapping feat…

Hypermobility spectrum disorders and hypermobile Ehlers-Danlos syndrome: patient experiences, disability and implications for rehabilitation.

The diagnosis and management of hypermobility spectrum disorders and hypermobile Ehlers-Danlos syndrome (HSD/hEDS) are a healthcare challenge because of the wide array of symptoms, lack of diagnostic biomarkers, and evolving management guidelines. This study aimed to describe patient experiences, di…

Turbulence Ahead: Distinguishing Exercise-Induced Anaphylaxis from Mast Cell Activation Syndrome in a Military Pilot.

The REMA Score Revisited: An Optimized Algorithm for Early Prediction of Clonal Mast Cell Disease.

The Spanish Network on Mastocytosis (REMA) score was developed to predict clonal mast cell (MC) activation disorders (MCAD) in patients presenting with anaphylaxis without mastocytosis in the skin (MIS) and guide selection of patients for bone marrow (BM) examination. This study reassessed the diagn…