Department of Clinical Immunology, University Hospital Zurich, Zurich, Switzerland.
Allergy Unit, Department of Dermatology, University Hospital Zurich, Zurich, Switzerland.
Orphanet J Rare Dis. 2018 Jun 5;13(1):90. doi: 10.1186/s13023-018-0832-4.
Hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) is a rare inherited disease. In most HAE-affected subjects, defined trigger factors precede angioedema attacks. Mechanisms of how trigger factors stimulate the contact activation pathway with bradykinin generation are not well elucidated. In recent studies, hypersensitivity reactions and food were stated as relevant triggers. We investigated HAE affected people for possible hypersensitivity reactions or intolerances and their relation in triggering angioedema attacks.
A questionnaire was filled in, recording date of birth, gender, and self-reported angioedema attacks associated with the ingestion of foodstuffs, administration of drugs, hymenoptera stings and hypersensitivity reactions against inhalation allergens. All participants performed a skin prick test against inhalation allergens and food. In patients who stated an association of possible hypersensitivity with angioedema, a serological ImmunoCAP test was also performed.
From the 27 women and 15 men analyzed, 79% stated trigger factors. From those food was mentioned in 36%. The suspected food included tomato, green salad, fish, citrus fruits, apple, onion, garlic, cheese, chili, kiwi, milk, tree nuts, strawberry, pineapple, shrimps, bread, banana, leek, chicken and alcohol, and were associated with abdominal angioedema. Neither the skin prick test nor the ImmunoCAP-test turned out positive for the tested food allergens.
Food seems to be a relevant trigger factor, causing angioedema in HAE affected patients. The reason, however, is not IgE-mediated hypersensitivity, but most probably an intolerance reaction to food products.
C1 抑制剂缺乏症(C1-INH-HAE)所致遗传性血管性水肿是一种罕见的遗传性疾病。在大多数 HAE 患者中,特定的触发因素先于血管性水肿发作。触发因素如何刺激接触激活途径并产生缓激肽的机制尚未完全阐明。在最近的研究中,过敏反应和食物被认为是相关的触发因素。我们调查了 HAE 患者是否存在可能的过敏反应或不耐受,并研究它们与触发血管性水肿发作的关系。
填写问卷,记录出生日期、性别以及自我报告的与食物摄入、药物使用、蜂螫和吸入性过敏原过敏反应相关的血管性水肿发作。所有参与者均接受了吸入性过敏原和食物的皮肤点刺试验。对于报告可能与血管性水肿相关的过敏反应的患者,还进行了血清免疫 CAP 试验。
在分析的 27 名女性和 15 名男性中,79%的人报告了触发因素。其中 36%的人提到了食物。可疑食物包括番茄、生菜、鱼、柑橘类水果、苹果、洋葱、大蒜、奶酪、辣椒、猕猴桃、牛奶、坚果、草莓、菠萝、虾、面包、香蕉、韭菜、鸡肉和酒精,与腹部血管性水肿有关。皮肤点刺试验和免疫 CAP 试验均未对测试的食物过敏原呈阳性。
食物似乎是 HAE 患者血管性水肿的一个相关触发因素。然而,原因不是 IgE 介导的过敏反应,而是对食物产品的不耐受反应。