Epidemiological and clinical features of idiopathic pulmonary alveolar proteinosis in Japan.
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.
Inoue
Yoshikazu
Y
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
Nakata
Koh
K
Arai
Toru
T
Tazawa
Ryushi
R
Hamano
Emi
E
Nukiwa
Toshihiro
T
Kudo
Koichiro
K
Keicho
Naoto
N
Hizawa
Nobuyuki
N
Yamaguchi
Etsuro
E
Eda
Ryosuke
R
Oishi
Kazunori
K
Maeda
Yuuka
Y
Koreeda
Yukiko
Y
Kodo
Naohiko
N
Sakatani
Mitsunori
M
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
D001323:Autoantibodies; D015415:Biomarkers; D016178:Granulocyte-Macrophage Colony-Stimulating Factor