TABLE 4

Summary of the latest epidemiology for autoimmune pulmonary alveolar proteinosis (APAP) in Japan

 Indices Estimated (95% CI) Estimated annual onset in Niigata Prefecture 3.9 (1.5–10.2)# Incidence per 106 population 1.65 (0.64–4.31)¶ Estimated annual cases in all of Japan 211 (81–549)+ Estimated survival period years 16.1 (15.1–17.1)§ Prevalence per 106 population 26.6 (9.6–73.6)ƒ Estimated number of patients in Japan 3387 (1226–9367)##

#: estimated using the Poisson distribution Z(0.975) as the 97.5% quantile of the standard normal distribution. The lower limit is calculated using the equation ((–1×Z(0.975)+(Z(0.975)2+4×event number)0.5)/2)2 and the upper limit using equation ((1×Z(0.975)+(Z(0.975)2+4×event number)0.5)/2)2; : calculated by dividing the Poisson estimate by the population of Niigata Prefecture; +: calculated by multiplying the incidence rate per million by the population of Japan; §: estimated using the restricted mean survival time; ƒ: when the occurrence rate of the disease is stable, the formula prevalence=incidence×mean disease duration (=estimated survival period) is used; ##: calculated by multiplying prevalence rate per million by the total population of Japan.