OBJECTIVES: To conduct a systematic literature review to understand and measure the economic burden associated with retinitis pigmentosa (RP) and visual impairment in France.

METHODS: EMBASE and PubMed-Medline were searched from 2000 to November 17^th, 2022. Other relevant sources including congress proceedings were also searched for the 2019–2022 period. As a rare disease leading to visual impairment, paucity of evidence available on RP in France was expected. Therefore, the research was extended to any chronic ocular pathology associated with visual impairment.

RESULTS: Thirty-five articles of interest were identified. Eight articles on RP, 6 articles on visual impairment, 7 articles on common RP complications, and 14 articles on other ocular pathologies were included. Although no published guidelines for RP management in France were retrieved, management of RP primarily involves symptomatic and preventative treatments. The main categories of costs associated with visual impairment identified in the review include medical costs (e.g., visits, exams, and treatment) and non-medical costs mainly associated with disability (e.g., technical assistance, caregiver, home assistance, allowance, and loss of productivity). Non-medical cost accounted for most of the direct cost of visual impairment, especially in diseases such as RP, where few or no innovative treatments are available. Patients with blindness incurred greater non-medical annual costs than patients with low vision, which 48.0% and 29.7% of patients with RP have indicated to be affected by blindness and low vision, respectively. Furthermore, a decrease in visual capacity due to the progression of RP was associated with a decreased level of autonomy, increased disability, and decreased employment, potentially associated with costs as demonstrated in available evidence from other ocular diseases.

CONCLUSIONS: RP and visual impairment more generally, are associated with a substantial societal burden, mainly driven by non-medical costs. The exact quantification of the economic burden of RP will require further research.