PDF(Pubmed)
Abstract：
BACKGROUND: 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.
METHODS: 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.
CONCLUSIONS: 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.
METHODS: 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.
CONCLUSIONS: 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.
摘要：
背景：过敏反应是一种急性，通常在暴露于触发器后发生的潜在威胁生命的过敏反应，而特发性过敏反应（IA）发生在没有触发的情况下。触发的过敏反应和IA的急性管理都依赖于肾上腺素的使用。在一些复发性IA患者中，用泼尼松预防糖皮质激素是有效的。虽然目前没有高质量的证据表明使用其他预防性选择来预防复发性IA，不断发展的数据支持对靶向IgE或Th2途径的生物制剂的考虑。
方法：我们介绍了一名28岁女性，没有特应性或自身免疫性病史，自童年以来IA反复发作，每周发生两次。使用第一代或第二代抗组胺药和/或肌内肾上腺素可改善急性症状。没有可识别的触发器，她被诊断为IA和频繁的特发性荨麻疹,治疗方案中加入了奥马珠单抗,症状频率得到改善.在失去跟进后，她在未接受奥马珠单抗治疗的情况下出现症状频率和严重程度的复发,随后就诊于我们的机构.她此时的检查对食物过敏呈阴性，alphagal综合征,系统性肥大细胞增多症,遗传性α类胰蛋白酶血症,类癌综合征,嗜铬细胞瘤,她接受了dupilumab的试验，在6个月的时间内症状频率几乎消失。
结论：复发性IA是一种与高发病率相关的排除性诊断。预防仍然是一个不确定的领域，尽管泼尼松在某些病例中有效。当泼尼松禁忌或无效预防IA时，靶向IgE或Th2途径的生物疗法可能是一个合理的考虑因素.这个案例增加了对dupilumab可能是预防复发性IA的逻辑标签外考虑的建议的支持。dupilumab在这种临床情况下的数据仍然非常有限，在提出任何建议之前，还需要进一步的研究。
方法：我们介绍了一名28岁女性，没有特应性或自身免疫性病史，自童年以来IA反复发作，每周发生两次。使用第一代或第二代抗组胺药和/或肌内肾上腺素可改善急性症状。没有可识别的触发器，她被诊断为IA和频繁的特发性荨麻疹,治疗方案中加入了奥马珠单抗,症状频率得到改善.在失去跟进后，她在未接受奥马珠单抗治疗的情况下出现症状频率和严重程度的复发,随后就诊于我们的机构.她此时的检查对食物过敏呈阴性，alphagal综合征,系统性肥大细胞增多症,遗传性α类胰蛋白酶血症,类癌综合征,嗜铬细胞瘤,她接受了dupilumab的试验，在6个月的时间内症状频率几乎消失。
结论：复发性IA是一种与高发病率相关的排除性诊断。预防仍然是一个不确定的领域，尽管泼尼松在某些病例中有效。当泼尼松禁忌或无效预防IA时，靶向IgE或Th2途径的生物疗法可能是一个合理的考虑因素.这个案例增加了对dupilumab可能是预防复发性IA的逻辑标签外考虑的建议的支持。dupilumab在这种临床情况下的数据仍然非常有限，在提出任何建议之前，还需要进一步的研究。
