TY - JOUR T1 - Establishment of the consecutive registration system for pulmonary alveolar proteinosis in Japan: Updated incidence, prevalence and surveillance for intractable cases JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.congress-2016.PA3873 VL - 48 IS - suppl 60 SP - PA3873 AU - Yoshikazu Inoue AU - Koh Nakata AU - Etsuro Yamaguchi AU - Toru Arai AU - Chikatoshi Sugimoto AU - Yasuhiro Setoguchi AU - Toshio Ichiwata AU - Masahito Ebina AU - Kazutoshi Cho AU - Ryushi Tazawa AU - Haruyuki Ishii AU - Takahiro Kasai AU - Masanori Akira AU - Kanji Uchida AU - Hiroshi Kida AU - Sakae Homma AU - Koichiro Tatsumi AU - Arata Azuma AU - Koichi Hagiwara AU - Keisuke Tomii AU - Masanori Kitaichi AU - Masaru Suzuki AU - Kohnosuke Morimoto AU - Toshinori Takada AU - Hideaki Nakayama AU - Shinya Ohkouchi AU - Takahiro Tanaka AU - Masaki Hirose AU - Akiko Matsumuro Y1 - 2016/09/01 UR - http://erj.ersjournals.com/content/48/suppl_60/PA3873.abstract N2 - In 2007, we reported a large cohort of autoimmune pulmonary alveolar proteinosis (n=248, PAP) in Japan, and showed the incidence and prevalence were 0.24 - 0.49/million/year and 2.04 - 6.2/million (AJRCCM 2007), and 1.4%, 1.4%, and 5.7% of patients had interstitial lung diseases (ILD), autoimmune diseases, and infections. The aims of this study are; (1) To establish a nationwide consecutive registration system for PAP, updating the incidence and prevalence, and (2) To show the usefulness of the system via a surveillance of intractable cases.Methods: (1) All patients, who received the serum anti-GM-CSF autoantibodies tests and were definitely diagnosed, were annually consecutively registered to our database (2011-2014). Three institutes (Niigata, Kinki-Chuo, Aichi) measured the antibodies. (2) Surveillance for the intractable cases (dead, complications such as ILD, infection, etc.) was conducted via the system.Results: (1) Total 903 cases were registered. 88 cases/year have been newly diagnosed in average. The incidence and the prevalence were 0.69/million/year and 6.11/million. 92.9, 6.1, 0.4, and 0.6% were autoimmune, secondary, congenital, and unclassifiable PAP. (2)50% of the institutes responded, providing the data of 579 cases (64% of total patients). 14, 11.2, 10.2, 3.8, 6.7, and 7.9% had dead, respiratory failure, ILD, autoimmune diseases, infections, extra-pulmonary complications.Conclusions: We established a simple consecutive nation-wide registration system for PAP in Japan, providing updated annual epidemiological data and effective surveillance data.This study was granted from AMED (#15652554 , YI). ER -