title: Epidemiological and clinical features of idiopathic pulmonary alveolar proteinosis in Japan.
abstract: Idiopathic pulmonary alveolar proteinosis (IPAP) is a rare disease characterized by excessive amounts of lipoproteinaceous material in the alveolus. This report presents an interim analysis of nationwide epidemiological data from Japanese patients with pulmonary alveolar proteinosis, and the roles of serum markers for IPAP. (i) The nationwide demographic data from 166 Japanese patients with IPAP are shown. The female to male ratio was 1:2, and the average age was 51 +/- 14 years old (age range: 15-79 years) at registration or diagnosis. A total of 30% of patients with IPAP have a poor clinical course. In total, 30% of patients were treated with whole lung lavage therapy (WLL). Under WLL, the patients significantly improved in the short term, but 40% of the patients who underwent WLL worsened again. A new strategy such as granulocyte-macrophage colony-stimulating factor (GM-CSF) therapy for intractable PAP is required. (ii) The correlation of serum KL-6, carcinoembryonic antigen, surfactant proteins D and A, and LDH with disease severity suggests their potential as disease markers. In contrast, serum anti-GM-CSF antibody did not correlate with disease severity, but is a specific marker for the diagnosis of IPAP. The combined measurements of the serum markers may well prove very useful for both the diagnosis and the management of IPAP patients.
authors.affiliation: Department of Diffuse Lung Diseases and Respiratory Failure, Clinical Research Center, National Hospital Organization (NHO) Kinki-Chuo Chest Medical Center, Sakai, Osaka, Japan. giichi@kch.hosp.go.jp
authors.forename: Yoshikazu
authors.initials: Y
authors.lastname: Inoue
authors.forename: Koh
authors.initials: K
authors.lastname: Nakata
authors.forename: Toru
authors.initials: T
authors.lastname: Arai
authors.forename: Ryushi
authors.initials: R
authors.lastname: Tazawa
authors.forename: Emi
authors.initials: E
authors.lastname: Hamano
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Nukiwa
authors.forename: Koichiro
authors.initials: K
authors.lastname: Kudo
authors.forename: Naoto
authors.initials: N
authors.lastname: Keicho
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Hizawa
authors.forename: Etsuro
authors.initials: E
authors.lastname: Yamaguchi
authors.forename: Ryosuke
authors.initials: R
authors.lastname: Eda
authors.forename: Kazunori
authors.initials: K
authors.lastname: Oishi
authors.forename: Yuuka
authors.initials: Y
authors.lastname: Maeda
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Koreeda
authors.forename: Naohiko
authors.initials: N
authors.lastname: Kodo
authors.forename: Mitsunori
authors.initials: M
authors.lastname: Sakatani
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood; D015415:Biomarkers / Q000097:blood*; D005260:Female; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000276:immunology; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011649:Pulmonary Alveolar Proteinosis / Q000097:blood* / Q000175:diagnosis / Q000453:epidemiology*; D012372:ROC Curve; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; D016178:Granulocyte-Macrophage Colony-Stimulating Factor