Department of Biomedical and Clinical Science "Luigi Sacco", Università degli Studi di Milano, Ospedale Luigi Sacco, Milan, Italy.
J Intern Med. 2015 May;277(5):585-93. doi: 10.1111/joim.12304. Epub 2014 Sep 27.
The first classification of angioedema without wheals was recently reported and comprises different forms of the disease distinguished by aetiology, mediator of oedema and inheritance.
In total, 1725 consecutive patients with angioedema without wheals were examined at our centre between 1993 and 2012. We excluded from the analysis 667 patients because of incomplete data or because angioedema was related to a specific factor.
According to the new classification of angioedema, the 1058 patients included in this analysis were diagnosed with hereditary (HAE; n = 377) or acquired angioedema (AAE; n = 681). The former group included HAE with C1-inhibitor (C1-INH) deficiency (C1-INH-HAE; n = 353) and HAE with normal C1-INH levels (n = 24), of which six had a factor XII mutation (FXII-HAE) and 18 had disease of unknown origin (U-HAE). The AAE group included disease with C1-INH deficiency (C1-INH-AAE; n = 49), AAE related to angiotensin-converting enzyme inhibitor treatment (n = 183), idiopathic histaminergic (IH-AAE; n = 379) and idiopathic nonhistaminergic angioedema (InH-AAE; n = 70). We compared hereditary and AAE with uncertain aetiopathogenesis: the FXII-HAE and U-HAE groups pooled (FXII/U-HAE) versus InH-AAE. The median age at onset of FXII/U-HAE and InH-AAE was 26 and 38 years, respectively. In addition, 56% of patients with FXII/U-HAE and 81% of those with InH-AAE reported more than five attacks per year (median duration of 48 h). The location of angioedema in patients with FXII/U-HAE versus those with InH-AAE was the following: face, 70% versus 86%; tongue, oral cavity or larynx, 55% versus 68%; limbs, 70% versus 56%; and gastrointestinal mucosa, 50% versus 20%. Prophylaxis with tranexamic acid was effective in all six patients with U-HAE and in 37 of 38 with InH-AAE who were started on this treatment.
Our findings in this cohort of patients with angioedema provide new information on the clinical characteristics, diagnosis and treatment of this disease.
最近报道了一种无风团性血管性水肿的首次分类，该分类包括由病因、水肿介质和遗传决定的不同疾病形式。
在 1993 年至 2012 年间，我们中心共检查了 1725 例连续的无风团性血管性水肿患者。我们排除了 667 例因数据不完整或血管性水肿与特定因素有关而未进行分析的患者。
根据新的血管性水肿分类，本分析纳入的 1058 例患者被诊断为遗传性（HAE；n=377）或获得性血管性水肿（AAE；n=681）。前者包括 C1 抑制剂（C1-INH）缺乏的 HAE（C1-INH-HAE；n=353）和 C1-INH 水平正常的 HAE（n=24），其中 6 例存在因子 XII 突变（FXII-HAE），18 例病因不明（U-HAE）。AAE 组包括 C1-INH 缺乏的疾病（C1-INH-AAE；n=49）、与血管紧张素转换酶抑制剂治疗相关的 AAE（n=183）、特发性组胺能性（IH-AAE；n=379）和特发性非组胺能性血管性水肿（InH-AAE；n=70）。我们比较了遗传性和病因不明的 AAE：FXII/U-HAE 组（n=62）与 InH-AAE 组（n=70）。FXII/U-HAE 和 InH-AAE 组发病年龄的中位数分别为 26 岁和 38 岁。此外，56%的 FXII/U-HAE 患者和 81%的 InH-AAE 患者每年发作超过 5 次（中位持续时间为 48 小时）。FXII/U-HAE 患者与 InH-AAE 患者的血管性水肿部位如下：面部，70%比 86%；舌、口腔或喉部，55%比 68%；四肢，70%比 56%；胃肠道黏膜，50%比 20%。氨甲环酸预防治疗对 6 例 U-HAE 患者和开始治疗的 38 例 InH-AAE 患者中的 37 例均有效。
本研究中血管性水肿患者队列的发现提供了有关该疾病临床特征、诊断和治疗的新信息。
