Journal of gastroenterology · 2026
Healthcare burden and clinical spectrum of symptomatic polycystic liver disease in Japan: a nationwide epidemiological survey.
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Tsuruya Kota, Ohfuji Satoko, Suwabe Tatsuya, Mishima Yusuke, Arase Yoshitaka, Hirose Shunji, Ieda Satsuki, Hoshino Junichi, Yagi Shintaro, Hasegawa Kiyoshi, Tanaka Atsushi, Kagawa Tatehiro
Аннотация
BACKGROUND: Polycystic liver disease (PLD) is often asymptomatic, but some patients develop symptoms or complications requiring care or intervention. The nationwide burden of symptomatic PLD remains poorly defined. We conducted a nationwide survey to estimate the number and prevalence of patients with symptomatic PLD in Japan and characterize clinical features, treatment patterns, healthcare utilization, and outcomes.
METHODS: A nationwide two-stage questionnaire survey was conducted. Symptomatic PLD was defined as 10 or more hepatic cysts with cyst-related symptoms or complications. In the first-stage survey, 3127 departments were selected by stratified random sampling to estimate the number of patients treated or followed up during 2021-2023. In the second-stage survey, clinical data were collected for patients treated or followed up in 2023.
RESULTS: The estimated number of patients was 6500 during 2021-2023 and 3570 in 2023, corresponding to an annual prevalence of 28.6 per million. Among 557 patients with clinical data, 62% were female and 67.2% met the diagnostic criteria for polycystic kidney disease. Abdominal distension, abdominal pain, and cyst infection were the most frequent manifestations. More than half underwent hepatic cyst treatment, and 60.4% required consultation with other departments or institutions. Cyst infection and liver failure accounted for a subset of deaths. Mortality differed significantly among Gigot classification groups and was highest in type III.
CONCLUSIONS: This nationwide survey provides the first comprehensive epidemiological and clinical characterization of symptomatic PLD in Japan and establishes a benchmark for future disease severity classification, clinical management, and prospective studies.
METHODS: A nationwide two-stage questionnaire survey was conducted. Symptomatic PLD was defined as 10 or more hepatic cysts with cyst-related symptoms or complications. In the first-stage survey, 3127 departments were selected by stratified random sampling to estimate the number of patients treated or followed up during 2021-2023. In the second-stage survey, clinical data were collected for patients treated or followed up in 2023.
RESULTS: The estimated number of patients was 6500 during 2021-2023 and 3570 in 2023, corresponding to an annual prevalence of 28.6 per million. Among 557 patients with clinical data, 62% were female and 67.2% met the diagnostic criteria for polycystic kidney disease. Abdominal distension, abdominal pain, and cyst infection were the most frequent manifestations. More than half underwent hepatic cyst treatment, and 60.4% required consultation with other departments or institutions. Cyst infection and liver failure accounted for a subset of deaths. Mortality differed significantly among Gigot classification groups and was highest in type III.
CONCLUSIONS: This nationwide survey provides the first comprehensive epidemiological and clinical characterization of symptomatic PLD in Japan and establishes a benchmark for future disease severity classification, clinical management, and prospective studies.