Theo Gulen1,2,3, Cem Akin4. 1. Department of Respiratory Medicine and Allergy, K85, Karolinska University Hospital, Huddinge, SE-141 86, Stockholm, Sweden. Theo.Gulen@ki.se. 2. Immunology and Allergy Unit, Department of Medicine Solna, Karolinska Institutet and Karolinska University Hospital, Stockholm, Sweden. Theo.Gulen@ki.se. 3. Mastocytosis Center Karolinska, Karolinska Institutet and Karolinska University Hospital, Stockholm, Sweden. Theo.Gulen@ki.se. 4. Division of Allergy and Clinical Immunology, Department of Internal Medicine, University of Michigan Health System, Ann Arbor, MI, USA.
Abstract
PURPOSE OF REVIEW: The aim of this systematic review is to present the proposed theories of pathogenesis for idiopathic anaphylaxis (IA), to discuss its classification, its diagnostic approach, and management. RECENT FINDINGS: IA represents a major diagnostic challenge and is diagnosed when excluding the possible identifiable triggers of anaphylaxis. The current research, however, revealed that certain conditions including mastocytosis, mast cell activation syndromes, and hereditary alpha tryptasemia can masquerade and overlap with its symptomatology. Also, newly identified galactose-alpha-1,3-galactose mammalian red meat allergy has recently been recognized as underlying cause of anaphylaxis in some cases that were previously considered as IA. IA comprises a heterogenous group of conditions where, in some cases, inherently dysfunctional mast cells play a role in pathogenesis. The standard trigger avoidance strategies are ineffective, and episodes are unpredictable. Therefore, prompt recognition and treatment as well as prophylaxis are critical. The patients should always carry an epinephrine autoinjector.
PURPOSE OF REVIEW: The aim of this systematic review is to present the proposed theories of pathogenesis for idiopathic anaphylaxis (IA), to discuss its classification, its diagnostic approach, and management. RECENT FINDINGS: IA represents a major diagnostic challenge and is diagnosed when excluding the possible identifiable triggers of anaphylaxis. The current research, however, revealed that certain conditions including mastocytosis, mast cell activation syndromes, and hereditary alpha tryptasemia can masquerade and overlap with its symptomatology. Also, newly identified galactose-alpha-1,3-galactosemammalian red meat allergy has recently been recognized as underlying cause of anaphylaxis in some cases that were previously considered as IA. IA comprises a heterogenous group of conditions where, in some cases, inherently dysfunctional mast cells play a role in pathogenesis. The standard trigger avoidance strategies are ineffective, and episodes are unpredictable. Therefore, prompt recognition and treatment as well as prophylaxis are critical. The patients should always carry an epinephrine autoinjector.
Authors: Héloïse Beutier; Caitlin M Gillis; Bruno Iannascoli; Ophélie Godon; Patrick England; Riccardo Sibilano; Laurent L Reber; Stephen J Galli; Mark S Cragg; Nico Van Rooijen; David A Mancardi; Pierre Bruhns; Friederike Jönsson Journal: J Allergy Clin Immunol Date: 2016-04-26 Impact factor: 10.793