Idiopathic Anaphylaxis: a Perplexing Diagnostic Challenge for Allergists

Purpose of ReviewThe 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 FindingsIA 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.SummaryIA 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.

Automated summary

The systematic review aims to present proposed theories of pathogenesis for idiopathic anaphylaxis (IA), discuss its classification, diagnostic approach, and management. Recent findings show that IA represents a major diagnostic challenge and certain conditions such as mastocytosis and red meat allergy may overlap with its symptomatology. Patients with IA should always carry an epinephrine autoinjector for prompt treatment and prophylaxis.