PURPOSE: HHT is an autosomal dominant, multi-system vascular disorder, with a prevalence of about 1/5000. Hemoptysis, epistaxis, GI bleeding, and iron deficiency anemia occur secondary to telangiectasias, though patients often undergo endoscopy to investigate other etiologies. Outcomes of bronchoscopy, colonoscopy, and gastroscopy have not been studied in HHT patients. Our objective was to determine the indications and complications of these three procedures in HHT.
METHODS: Participants were invited to complete a web-based questionnaire, electronically mailed to 2000 members of the International HHT Foundation. Inclusion criteria were: 1) physician diagnosis of HHT and 2) age ≥ 18 years. Data on demographics, clinical manifestations (AVMs, GI bleeding, transfusions), endoscopic procedures and their indications and complications, were collected retrospectively.
RESULTS: 857(43%) people with HHT completed the questionnaire. Mean age was 55[18-87]years, two-thirds were female. Prevalence of pulmonary (51%), cerebral (10%) and liver(17%) AVMs, GI bleeding (32%) and blood transfusions (40%) was observed. Bronchoscopy was performed in 75(9%), with the most common indications being abnormal chest imaging (26%), hemoptysis/cough (23%), and infection (15%). Complications included hospitalization (5.3%), infection(8%), hemoptysis (7%), and prolonged nose/mouth bleeding(2.7%). Colonoscopy and gastroscopy was performed in 606(71%) and 344(40%), respectively. Main indications for colonoscopy were: routine cancer screening (48%), anemia (18%), and GI bleeding (16%). Main indications for gastroscopy were: acid reflux/dyspepsia (33%) and anemia (30%). Complications from colonoscopy and gastroscopy were, respectively: admission to hospital (2% & 2.3%), GI bleeding (3.3% & 4%), infection (2.2% & 0.6%), prolonged nose/mouth bleeding(1.7% & 2.3%) and perforation(0.7% & 0.6%).
CONCLUSIONS: Endoscopy is frequently performed in HHT patients. The indications and complications are similar to the general population, based on the literature. Despite low complication rates, HHT patients had a ten-fold greater rate than the general population, primarily due to infection and bleeding post-procedure, with prolonged nasal/oral bleeding unique to HHT. Verification of these results with prospective studies is important, especially to reduce recall bias.
CLINICAL IMPLICATIONS: Bronchoscopy and GI endoscopy are relatively safe in HHT patients though clinicians should be aware of an increased risk of infection and bleeding.
DISCLOSURE: The following authors have nothing to disclose: Dmitry Rozenberg, Marie Faughnan
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