Epidemiology and Trends of Hereditary Hemorrhagic Telangiectasia in the United States

Andrew M. Ferry, Alex E. Wright, Gwen Baillargeon, Yong Fang Kuo, Mohamad R. Chaaban

Research output: Contribution to journalArticle

Abstract

Background: To our knowledge, no national studies have investigated the epidemiology of hereditary hemorrhagic telangiectasia (HHT) in the United States since the incorporation of the International Statistical Classification of Diseases and Related Health Problems, 10th Revision, Clinical Modification (ICD-10 CM). Objective: Our objective is to analyze the epidemiology of HHT from 2013 to 2017 and to determine the relationships between epistaxis and other associated complications of this rare disease. Methods: We analyzed the epidemiology of HHT between 2013 and 2017 by accessing data of 87 709 738 patients from the Clinformatics Data Mart using ICD-9 CM and ICD-10 CM codes. Variables analyzed included age, gender, region, clinical setting of diagnosis, hospitalizations, and complications. Bivariate analyses using generalized linear models were conducted to determine the likelihood of HHT patients with epistaxis enduring associated life-threatening complications such as cerebral hemorrhage, thrombosis, and pulmonary hemorrhage compared to HHT patients without epistaxis during the study period. Results: The prevalence of HHT increased from 6.1 to 12.1 per 100 000 persons, with patients of ages 18 to 29 years and those older than 60 years seeing the greatest percent increase. The prevalence of HHT in the Southern United States saw a 147% increase. Compared to HHT patients without epistaxis, HHT patients with epistaxis were 3.4 times more likely to experience pulmonary hemorrhage, 3.3 times more likely to have pulmonary emboli, 2.8 times more likely to experience cerebral hemorrhage, and 2.0 times more likely to have thrombosis during the study period. Conclusion: Our national study has provided the first incidence and prevalence rates of HHT in the United States since the incorporation of the ICD-10 CM. HHT patients with epistaxis require prompt multidisciplinary treatment of their condition due to their increased risk of life-threatening complications.

Original languageEnglish (US)
JournalAmerican Journal of Rhinology and Allergy
DOIs
StateAccepted/In press - Jan 1 2019

Fingerprint

Hereditary Hemorrhagic Telangiectasia
Epidemiology
Epistaxis
International Classification of Diseases
Cerebral Hemorrhage
Lung
Hemorrhage
Intracranial Thrombosis
Rare Diseases
Embolism
Linear Models
Hospitalization
Thrombosis

Keywords

  • complications
  • epidemiology
  • epistaxis
  • hemorrhage
  • hereditary hemorrhagic telangiectasia
  • ICD-10
  • incidence
  • prevalence
  • thrombosis

ASJC Scopus subject areas

  • Immunology and Allergy
  • Otorhinolaryngology

Cite this

Epidemiology and Trends of Hereditary Hemorrhagic Telangiectasia in the United States. / Ferry, Andrew M.; Wright, Alex E.; Baillargeon, Gwen; Kuo, Yong Fang; Chaaban, Mohamad R.

In: American Journal of Rhinology and Allergy, 01.01.2019.

Research output: Contribution to journalArticle

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abstract = "Background: To our knowledge, no national studies have investigated the epidemiology of hereditary hemorrhagic telangiectasia (HHT) in the United States since the incorporation of the International Statistical Classification of Diseases and Related Health Problems, 10th Revision, Clinical Modification (ICD-10 CM). Objective: Our objective is to analyze the epidemiology of HHT from 2013 to 2017 and to determine the relationships between epistaxis and other associated complications of this rare disease. Methods: We analyzed the epidemiology of HHT between 2013 and 2017 by accessing data of 87 709 738 patients from the Clinformatics Data Mart using ICD-9 CM and ICD-10 CM codes. Variables analyzed included age, gender, region, clinical setting of diagnosis, hospitalizations, and complications. Bivariate analyses using generalized linear models were conducted to determine the likelihood of HHT patients with epistaxis enduring associated life-threatening complications such as cerebral hemorrhage, thrombosis, and pulmonary hemorrhage compared to HHT patients without epistaxis during the study period. Results: The prevalence of HHT increased from 6.1 to 12.1 per 100 000 persons, with patients of ages 18 to 29 years and those older than 60 years seeing the greatest percent increase. The prevalence of HHT in the Southern United States saw a 147{\%} increase. Compared to HHT patients without epistaxis, HHT patients with epistaxis were 3.4 times more likely to experience pulmonary hemorrhage, 3.3 times more likely to have pulmonary emboli, 2.8 times more likely to experience cerebral hemorrhage, and 2.0 times more likely to have thrombosis during the study period. Conclusion: Our national study has provided the first incidence and prevalence rates of HHT in the United States since the incorporation of the ICD-10 CM. HHT patients with epistaxis require prompt multidisciplinary treatment of their condition due to their increased risk of life-threatening complications.",
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