Idiopathic Anaphylaxis: A Form of Mast Cell Activation Syndrome.
Idiopathic anaphylaxis is a condition caused by paroxysmal episodes of sudden-onset multiorgan involvement variably including laryngeal edema, urticaria, bronchoconstriction, dyspnea, hypoxia, abdominal pain, nausea, vomiting, diarrhea, and hypotension. Rarely, the episodes can lead to cardiovascular collapse and death in the absence of a clear trigger, especially in the presence of other cardiovascular comorbidities. Elevated mast cell mediators such as tryptase and histamine have been reported during episodes, and mast cells are considered the primary cells responsible for driving anaphylaxis in humans. Basophils also secrete histamine and LTC4 when activated and theoretically can contribute to symptoms. As our understanding of mast cell disorders continue to grow, the classification for these disorders evolves. The purpose of this article was 2-fold: to review the epidemiology, clinical manifestations, and diagnosis of idiopathic anaphylaxis and to discuss the classification of idiopathic anaphylaxis within the broader context of mast cell activation disorders.
['Giannetti|Matthew P|MP|', 'Akin|Cem|C|', 'Castells|Mariana|M|']
D000707:Anaphylaxis D001491:Basophils D006801:Humans D008407:Mast Cells D008415:Mastocytosis D053802:Tryptases
Idiopathic anaphylaxis Anaphylaxis Mast cell activation syndrome Mast cells Tryptase Mastocytosis