An epistaxis severity score for hereditary hemorrhagic telangiectasia.
OBJECTIVE
Hereditary hemorrhagic telangiectasia (HHT)-related epistaxis leads to alterations in social functioning and quality of life. Although more than 95% experience epistaxis, there is considerable variability of severity. Because no standardized method exists to measure epistaxis severity, the purpose of this study was to determine factors associated with patient-reported severity to develop a severity score.


METHODS
Prospective, survey-based study.


METHODS
HHT care providers and a focus group of patients were interviewed to determine epistaxis-associated factors. From this, an electronic survey was developed and administered to patients with HHT. Descriptive analyses were performed with calculations of means and medians for continuous and proportions for categorical variables. Multiple ordinal logistic and linear regression models were developed to determine risk factors for epistaxis severity.


RESULTS
Nine hundred respondents from 21 countries were included. Eight hundred fifty-five (95%) subjects reported epistaxis. The mean (standard deviation) age was 52.1 (13.9) years, and 61.4% were female. Independently associated risk factors for self-reported epistaxis severity included epistaxis frequency (odds ratio [OR] 1.57), duration (OR 2.17), intensity (OR 2.45), need for transfusion (OR 2.74), anemia (OR 1.44), and aggressiveness of treatment required (OR 1.53, P < .001 for all).


CONCLUSIONS
Risk factors for increasing epistaxis severity in patients with HHT include frequency, duration, and intensity of episodes; invasiveness of prior therapy required to stop epistaxis; anemia; and the need for blood transfusion. From these factors, an epistaxis severity score will be presented.
Hoag
Jeffrey B
JB
Department of Medicine, Drexel University College of Medicine, Philadelphia, Pennsylvania 19102, USA. jhoag@drexelmed.edu
Terry
Peter
P
Mitchell
Sally
S
Reh
Douglas
D
Merlo
Christian A
CA
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D004844:Epistaxis / Q000175:diagnosis* / Q000209:etiology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D011788:Quality of Life; D012307:Risk Factors; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications* / Q000175:diagnosis; D055815:Young Adult