Epidemiology of Angioid Streaks and Pseudoxanthoma Elasticum (2011–2020)

• DOI: 10.1016/j.xops.2023.100370
• Publication Date: 2023-07-20T03:32:54Z
• Authors: Saori Wada; Masahiro Miyake; Ai Kido; Takuro Kamei; Shusuke Hiragi; Hanako Ohashi Ikeda; Masayuki Hata; Hiroaki Ueshima; Akitaka Tsujikawa; Hiroshi Tamura
• Keywords: Pseudoxanthoma Elasticum; Angioid streaks

We aimed to describe the epidemiology of angioid streaks (AS) and pseudoxanthoma elasticum (PXE), which are rare diseases, using a national claims database.This was population-based longitudinal cohort study.A total 126 million individuals were covered by universal health coverage system in Japan.With permission from Ministry Health, Labor Welfare, we accessed all data National Database Health Insurance Claims Specific Checkups Japan, contains nationwide insurance for Japanese. identified with AS PXE between January 2011 December 2020. The incidence rates, prevalence, overlap PXE, mean age at death calculated.The rates prevalence PXE.A 6598 cases 1020 during 10-year study period. 0.52 (95% confidence interval, 0.48-0.56) 0.08 0.07-0.10) per 100 000 person-years, respectively. On October 1, 2020, 6.5 6.38-6.66) 0.83 0.78-0.89) persons, 363 patients. 79.3 ± 0.51 77.1 2.68 years, respectively.This is first elucidate PXE. both patients younger than life expectancy general Japanese population, thus, appropriate diagnosis management important avoid preventable death.Proprietary or commercial disclosure may be found Footnotes Disclosures end this article.