Department of Respiratory Medicine and Allergy, K85, Karolinska University Hospital, Huddinge, SE-141 86, Stockholm, Sweden.
Immunology and Allergy Unit, Department of Medicine Solna, Karolinska Institutet and Karolinska University Hospital, Stockholm, Sweden.
Curr Allergy Asthma Rep. 2021 Feb 9;21(2):11. doi: 10.1007/s11882-021-00988-y.
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.
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.
本系统综述旨在介绍特发性过敏反应（IA）发病机制的理论，讨论其分类、诊断方法和管理。
IA 是一个重大的诊断挑战，当排除过敏反应的可能诱因时即可诊断。然而，目前的研究表明，某些情况包括肥大细胞增多症、肥大细胞激活综合征和遗传性α-胰蛋白酶血症，可能会伪装并与其症状重叠。此外，最近新发现的半乳糖-α-1,3-半乳糖哺乳动物红肉过敏已被认为是某些以前被认为是 IA 的过敏反应的潜在原因。IA 包含一组异质条件，在某些情况下，固有功能失调的肥大细胞在发病机制中起作用。标准的避免触发策略无效，且发作不可预测。因此，快速识别和治疗以及预防至关重要。患者应始终携带肾上腺素自动注射器。
