Prevalence and Risk Factors for Bleeding in Hereditary Hemorrhagic Telangiectasia: A National Inpatient Sample Study.

Hereditary hemorrhagic telangiectasia (HHT) is a common bleeding disorder, but little is known regarding prevalence and risk factors for bleeding. Adult discharges with HHT and bleeding were identified by ICD-10 codes in the National Inpatient Sample (NIS), 2016-2018. Prevalence estimates were weighted using NIS-discharge-level weights to reflect national estimates. Categorical variables were compared by Rao-Scott chi-square test, and continuous variables by weighted simple linear regression. Risk factors for bleeding were determined by weighted multivariable logistic regression. Among 18,170,849 discharges 2016-2018, 2,528 (0.01%) had HHT, of whom 648 (25.6%) had bleeding. AV malformation, 31.9% vs 1.3%, angiodysplasia, 23.5% vs 2.3%, telangiectasia, 2.3% vs 0.2%, and epistaxis, 17.9% vs 0.6%, were more common in HHT than non-HHT, each p<0.001. By contrast, menstrual and postpartum bleeding were less common in reproductive-age HHT, 4.1% vs. 24.5%, than non-HHT females, 4.1% vs. 41.5%, each p<0.001. Iron deficiency anemia, equally common in HHT bleeders and non-bleeders, 15.7% vs. 16.0%, was significantly greater than in non-HHT bleeders, 7.5%, p<0.001. Comorbidities including gastroesophageal reflux, 25.9% vs 20.0%, and cirrhosis, 10.0% vs 3.6%, were greater in HHT than non-HHT bleeders, each p<0.001. In multivariable logistic regression among HHT, peptic ulcer disease, OR 8.86, p<0.001, portal vein thrombosis, OR 3.68, p=0.006, and hepatitis C, OR 2.13, p=0.017, were significantly associated with bleeding. In conclusion, AVM and angiodysplasia are more common, and HMB and PPH less common in HHT than non-HHT. Iron deficiency anemia prevalence is similar in bleeders and non-bleeders, suggesting ongoing blood loss and need for universal iron screening.