Pepper Elizabeth, Pittman Luke
Department of Internal Medicine, Dwight D Eisenhower Army Medical Center, 300 E Hospital Street, Fort Gordon, GA, 30905, USA.
Department of Allergy and Immunology, Dwight D Eisenhower Army Medical Center, 300 E Hospital Street, Fort Gordon, GA, 30905, USA.
Allergy Asthma Clin Immunol. 2023 Sep 9;19(1):82. doi: 10.1186/s13223-023-00838-8.
Anaphylaxis is an acute, potentially life-threatening allergic reaction that typically occurs after exposure to a trigger, while idiopathic anaphylaxis (IA) occurs in the absence of a trigger. Acute management of both triggered anaphylaxis and IA relies on the use of epinephrine. In some patients with recurrent IA, glucocorticoid prophylaxis with prednisone can be effective. While there is currently no high quality evidence for the use of other prophylactic options to prevent recurrent IA, evolving data exists to support the consideration of biologics that target IgE or the Th2 pathway.
We present the case of a 28 year old female with no atopic or autoimmune history with recurrent episodes of IA since childhood occurring up to twice weekly. There was improvement in acute symptoms with administration of first or second generation antihistamines and/or intramuscular epinephrine. Without an identifiable trigger, she was diagnosed with IA and frequent idiopathic urticaria and omalizumab was added to her treatment regimen with improvement in symptom frequency. After being lost to follow up, she had recurrence of symptom frequency and severity without omalizumab therapy and subsequently presented to our institution. Her workup at this point was negative for food allergy, alpha gal syndrome, systemic mastocytosis, hereditary alpha tryptasemia, carcinoid syndrome, and pheochromocytoma, and she was trialed on dupilumab with near resolution of her symptom frequency over a six month time period.
Recurrent IA is a diagnosis of exclusion that is associated with high morbidity. Prophylaxis remains an area of uncertainty, although prednisone has been effective in some cases. When prednisone is contraindicated or ineffective for the prevention of IA, biologic therapies that target IgE or the Th2 pathway may present a reasonable consideration. This case adds support to the suggestion that dupilumab may be a logical off-label consideration for prophylaxis of recurrent IA. The data for dupilumab in this clinical scenario is still very limited, and further research is required before any recommendation can be made.
过敏反应是一种急性、可能危及生命的过敏反应，通常在接触触发因素后发生，而特发性过敏反应（IA）则在无触发因素的情况下出现。触发型过敏反应和IA的急性处理均依赖于肾上腺素的使用。在一些复发性IA患者中，使用泼尼松进行糖皮质激素预防可能有效。虽然目前尚无高质量证据支持使用其他预防措施来预防复发性IA，但不断有新数据支持考虑使用靶向IgE或Th2途径的生物制剂。
我们报告一例28岁女性，无特应性或自身免疫病史，自幼反复发作IA，每周发作多达两次。使用第一代或第二代抗组胺药和/或肌内注射肾上腺素后，急性症状有所改善。由于未发现可识别的触发因素，她被诊断为IA和频繁特发性荨麻疹，治疗方案中加用奥马珠单抗后症状发作频率有所改善。在失访后，未使用奥马珠单抗治疗时，她的症状发作频率和严重程度复发，随后就诊于我们的机构。此时她的检查结果显示对食物过敏、α-半乳糖综合征、系统性肥大细胞增多症、遗传性α-胰蛋白酶血症、类癌综合征和嗜铬细胞瘤均为阴性，她试用了度普利尤单抗，在6个月的时间里症状发作频率几乎完全缓解。
复发性IA是一种排除性诊断，发病率较高。预防仍然是一个不确定的领域，尽管泼尼松在某些情况下有效。当泼尼松禁忌或对预防IA无效时，靶向IgE或Th2途径的生物治疗可能是一个合理的考虑。该病例支持了度普利尤单抗可能是预防复发性IA的合理非标签用药选择这一建议。度普利尤单抗在这种临床情况下的数据仍然非常有限，在做出任何推荐之前还需要进一步研究。
