OBJECTIVES: Health state utility values (HSUVs) are essential parameters in model-based economic evaluations. This study systematically identifies HSUVs in Huntington's disease (HD).

METHODS: A systematic review was conducted using three major biomedical databases to identify English language studies published from database inception to May 2022. Search terms included different combinations of HD along with utility or quality-of-life measures. Each study was reviewed by two independent reviewers; disagreements were resolved by a third reviewer.

RESULTS: The systematic search yielded 1,241 publications of which 13 studies (USA=4; Europe=9) were included. The sample size varied from 60 to 5560 patients. Eight studies each reported mean age (46-56 years) and proportion of males (32-49%). Most studies used validated utility instruments like EQ-5D (n=9) and SF-6D (n=2) to assess utility values. One study used the time trade-off technique, while one used both EQ-5D and SF-6D instruments. The average HSUV of HD patients in the USA was 0.34. Utility values in USA were further reported by HD chorea severity (severe [0.07], moderate/severe [0.26], moderate/mild [0.48], and mild [0.64] and by HD stage (prodromal [0.89], early-HD [0.80], late-HD [0.71] suggesting poor health status with increasing disease severity.

CONCLUSIONS: Despite the large variation in HSUVs, a consistent decline in health status can be seen as the disease progresses. These findings indicate substantial impairment in individuals' quality of life and suggest value in early detection.