BACKGROUND Gender differences in organ involvement and clinical severity have been poorly described in hereditary hemorrhagic telangiectasia (HHT). The aim of this study was to describe differences in the severity of HHT manifestations according to gender. METHODS Severity was measured according to Epistaxis Severity Score (ESS), Simple Clinical Scoring Index for hepatic involvement, a general HHT-score, needing for invasive treatment (pulmonary or brain arteriovenous malformations -AVMs- embolization, liver transplantation or Young's surgery) or the presence of adverse outcomes (severe anemia, emergency department -ED- or hospital admissions and mortality). RESULTS One hundred forty-two (58.7%) women and 100 (41.3%) men were included with a mean age of 48.9 ± 16.6 and 49 ± 16.5 years, respectively. Women presented hepatic manifestations (7.1% vs 0%) and hepatic involvement (59.8% vs 47%), hepatic AVMs (28.2% vs 13%) and bile duct dilatation (4.9% vs 0%) at abdominal CT, and pulmonary AVMs at thoracic CT (35.2% vs 23%) more often than men. The Simple Clinical Scoring Index was higher in women (3.38 ± 1.2 vs 2.03 ± 1.2), and more men were considered at low risk of harboring clinically significant liver disease than women (61% vs 25.3%). These differences were mantained when considering HHT1 and HHT2 patients separetely. Duodenal telangiectasia were more frequent in men than women (21% vs 9.8%). Invasive treatments were more frequently needed in women (28.2% vs 16%) but men needed attention at the ED more often than women (48% vs 28.2%), with no differences in ESS, HHT-score, anemia hospital admissions or mortality. CONCLUSIONS HHT women showed more severe hepatic involvement than men, also among HHT1 and HHT2 patients. Women had higher prevalence of pulmonary AVMs and needed invasive procedures more frequently, while men needed attention at the ED more often. These data might help physicians to individualize HHT patients follow-up.

中文翻译：

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遗传性出血性毛细血管扩张严重程度的性别差异。

背景技术在遗传性出血性毛细血管扩张症（HHT）中，器官受累和临床严重程度的性别差异描述得很少。这项研究的目的是根据性别描述HHT表现的严重性差异。方法根据严重性评分（Episaxis Severity Score，ESS），肝累及的简单临床评分指数，一般HHT评分，需要侵入性治疗（肺或脑动静脉畸形-AVMs-栓塞，肝移植或杨氏手术）或不良后果的存在（严重贫血，急诊科-ED-或住院和死亡）。结果共纳入一百四十二名女性（58.7％）和一百名男性（41.3％），平均年龄分别为48.9±16.6岁和49±16.5岁。妇女表现出肝脏表现（7。1％vs 0％）和肝脏受累（59.8％vs 47％），腹部CT时肝AVM（28.2％vs 13％）和胆管扩张（4.9％vs 0％），胸腔CT时肺AVM（35.2％ vs 23％）。女性的简单临床评分指数较高（3.38±1.2对2.03±1.2），并且男性比女性具有更高的具有临床显着肝病风险的可能性也较低（61％对25.3％）。当分别考虑HHT1和HHT2患者时，这些差异得以维持。男性十二指肠毛细血管扩张比女性多（21％比9.8％）。女性更需要侵入性治疗（28.2％vs 16％），但男性比女性更需要急诊治疗（48％vs 28.2％），而ESS，HHT评分，贫血住院率或死亡率无差异。结论HHT女性的肝脏受累程度比男性高，在HHT1和HHT2患者中也是如此。女性的肺AVM患病率较高，需要更频繁地进行侵入性手术，而男性则更需要在急诊科就诊。这些数据可能有助于医师个性化HHT患者的随访。