| アブストラクト | BACKGROUND: Using patient registries or limited regional hospitalization data may result in underestimation of the incidence and prevalence of rare diseases. Therefore, we used the national administrative database to estimate the incidence and prevalence of lymphangioleiomyomatosis over six years (2014-2019) and describe changes in clinical practice and mortality. METHODS: We extracted data from the National Database of Health Insurance Claims and Specific Health Checkups of Japan between January 2013 and December 2020. This database covers >/=99% of the population. We used the diagnostic code for lymphangioleiomyomatosis to estimate the incidence and prevalence from 2014 to 2019. Additionally, we examined the demographic characteristics, treatments, comorbidities, and mortality of the patients. RESULTS: In women, the incidence and prevalence of lymphangioleiomyomatosis in 2019 were approximately 3 per 1,000,000 person-years and 28.7 per 1,000,000 persons, respectively. While, in men, the incidence and prevalence of lymphangioleiomyomatosis were <0.2 per 1,000,000 person-years and 0.8 per 1,000,000 persons, respectively. From 2014 to 2019, the proportion of prescriptions of sirolimus and everolimus increased, while the use of home oxygen therapy, chest drainage, comorbid pneumothorax, and bloody phlegm decreased. The mortality rate remained stable at approximately 1%. CONCLUSIONS: The incidence and prevalence of lymphangioleiomyomatosis were higher in women than those reported previously. Although the incidence did not change during the 6-year period, the prevalence gradually increased. Moreover, lymphangioleiomyomatosis was observed to be rare in men. The practice of treating patients with lymphangioleiomyomatosis changed across the six years while mortality remained low, at approximately 1%. |
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| 投稿者 | Kimura, Yuya; Jo, Taisuke; Hashimoto, Yohei; Kumazawa, Ryosuke; Ishimaru, Miho; Matsui, Hiroki; Yokoyama, Akira; Tanaka, Goh; Yasunaga, Hideo |
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| 組織名 | Department of Clinical Epidemiology and Health Economics, School of Public;Health, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-0033, Japan;;Clinical Research Center, National Hospital Organization Tokyo Hospital, 3-1-1;Takeoka, Kiyose-shi, Tokyo, 204-8585, Japan.;Department of Health Services Research, Graduate School of Medicine, The;University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-0033, Japan; Department;of Respiratory Medicine, Graduate School of Medicine, The University of Tokyo,;7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-0033, Japan. Electronic address:;jo-taisuke@umin.ac.jp.;Save Sight Institute, The University of Sydney, South Block, Sydney Eye Hospital;8 Macquarie Street, Sydney, NSW, 2000, Australia.;Health, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-0033, Japan.;Institute of Education, Tokyo Medical and Dental University (TMDU), 1-5-45;Yushima, Bunkyo-ku, Tokyo, 113-8510, Japan.;Department of Respiratory Medicine, Graduate School of Medicine, The University;of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-0033, Japan. |
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