Serum antibody against granulocyte/macrophage colony-stimulating factor and KL-6 in idiopathic pulmonary alveolar proteinosis.
Here we describe a case of idiopathic pulmonary alveolar proteinosis (I-PAP), in which anti-granulocyte/macrophage colony-stimulating factor (GM-CSF) antibody and high level of KL-6 were found in the serum. Anti-GM-CSF antibody is responsible for I-PAP, and KL-6 is a serum marker for the activity of diffuse interstitial lung disease. A 38-year-old woman, who had no symptoms, was found to have an abnormal shadow on chest radiograph 5 years previously at a health check up. Chest radiograph showed a patchy shadow in the left lower lung field. Thoracoscopic biopsy was performed because the shadow had gradually expanded during the 5 years. Histological examination revealed proteinous material filling the alveoli and positive staining by the PAS method, suggesting PAP. Anti-GM-CSF antibody and a high level of KL-6 were detected in the serum at the time of diagnosis. Three years later, the shadow disappeared spontaneously. During this period, the level of KL-6 dramatically decreased, although that of GM-CSF antibody remained unchanged. The present case suggests that the serum level of the anti-GM-CSF antibody represents a useful marker for the diagnosis but not for follow-up of the clinical course. On the contrary, KL-6 is an excellent marker for the assessment of the clinical course of I-PAP.
Nara
Masayuki
M
Department of Infectious and Respiratory Diseases, Tohoku University School of Medicine, Sendai, Japan. nara@rid.med.tohoku.ac.jp
Sano
Kunio
K
Ogawa
Hiromasa
H
Tamada
Tsutomu
T
Nagaoka
Miyuki
M
Okada
Katsunori
K
Watanabe
Mika
M
Moriya
Takuya
T
Miki
Hiroshi
H
Nakata
Koh
K
Ichinose
Masakazu
M
Hattori
Toshio
T
D000328:Adult; D000951:Antigens, Neoplasm; D001323:Autoantibodies / Q000097:blood*; D015415:Biomarkers / Q000378:metabolism; D001706:Biopsy; D005260:Female; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000276:immunology* / Q000378:metabolism; D006801:Humans; D018396:Mucin-1; D009077:Mucins / Q000276:immunology* / Q000378:metabolism; D011649:Pulmonary Alveolar Proteinosis / Q000276:immunology* / Q000378:metabolism / Q000473:pathology; D011650:Pulmonary Alveoli / Q000473:pathology; D013902:Radiography, Thoracic; D013906:Thoracoscopy
D000951:Antigens, Neoplasm; D001323:Autoantibodies; D015415:Biomarkers; C060192:MUC1 protein, human; D018396:Mucin-1; D009077:Mucins; D016178:Granulocyte-Macrophage Colony-Stimulating Factor