Medical Director Respiratory, Novartis Pharmaceuticals Corp, 1 Health Plaza, East Hanover, NJ, United States.
Gastroenterology Fellow, The George Washington University, 2121 I St NW, Washington, DC, United States.
Clin Res Hepatol Gastroenterol. 2021 Jul;45(4):101702. doi: 10.1016/j.clinre.2021.101702. Epub 2021 Apr 14.
Angioedema can be either mast cell-(histamine-)mediated or bradykinin-mediated. Treatment approaches for the two types are very different, making differential diagnosis critical. Severe acute abdominal pain caused by intestinal angioedema is commonly misdiagnosed, especially when associated with bradykinin-mediated angioedema. After describing a typical clinical scenario and diagnostic journey of a patient with recurrent, undiagnosed abdominal pain due to hereditary angioedema (HAE), a rare variant of bradykinin-mediated angioedema, we delve into the classification and differential diagnosis of the various types of angioedema and provide an overview of appropriate management with an emphasis on the bradykinin-mediated types. Bradykinin-induced angioedema may be inherited or acquired and is infrequent compared to mast cell-mediated angioedema. HAE is a rare disease characterized by recurrent attacks of non-urticarial, nonpruritic edema usually affecting the face, respiratory tract, extremities, gastrointestinal tract, and genitalia. Unlike mast cell-mediated angioedema, painful abdominal symptoms are prevalent in bradykinin-mediated angioedema and are sometimes the only manifestation of an attack, increasing the likelihood of initial misdiagnosis as appendicitis or other forms of acute abdomen. It is important for gastroenterologists to be vigilant for the possibility of angioedema pathology in patients presenting with undiagnosed, recurrent, abdominal symptoms to facilitate accurate diagnosis and effective treatment.
血管性水肿可由肥大细胞(组胺)介导或缓激肽介导。这两种类型的治疗方法非常不同,因此鉴别诊断至关重要。由肠血管性水肿引起的严重急性腹痛常被误诊,尤其是当与缓激肽介导的血管性水肿相关时。在描述了一位因遗传性血管性水肿(HAE)而反复出现未确诊腹痛的典型临床情况和诊断过程(一种罕见的缓激肽介导的血管性水肿变异)后,我们深入探讨了各种类型的血管性水肿的分类和鉴别诊断,并概述了适当的管理方法,重点介绍了缓激肽介导的类型。缓激肽诱导的血管性水肿可能是遗传性的或获得性的,与肥大细胞介导的血管性水肿相比,其发病率较低。HAE 是一种罕见疾病,其特征是反复发作的非荨麻疹性、非瘙痒性水肿,通常影响面部、呼吸道、四肢、胃肠道和生殖器。与肥大细胞介导的血管性水肿不同,缓激肽介导的血管性水肿中常见腹痛症状,且有时是发作的唯一表现,这增加了最初误诊为阑尾炎或其他类型的急腹症的可能性。对于出现未确诊、反复出现的腹部症状的患者,胃肠病学家要警惕血管性水肿病理的可能性,以便进行准确的诊断和有效的治疗。