Serum and bronchoalveolar fluid KL-6 levels in patients with pulmonary alveolar proteinosis.
Pulmonary alveolar proteinosis (PAP) is a disease characterized by the filling of alveolar spaces with periodic acid-Schiff-positive proteinaceous material and by the hypertrophy of type II pneumocytes in the alveolar interstitium. To examine if KL-6, a mucinlike glycoprotein, is useful for the diagnosis of PAP and the estimation of its activity, serum KL-6 levels in patients with PAP were measured by an enzyme-linked immunosorbent assay and compared with those of patients with other lung diseases. Furthermore, to estimate the origin of KL-6 in some patients, measurements of KL-6 levels in bronchoalveolar lavage (BAL) fluid and immunohistochemical staining of the lung tissues with a monoclonal antibody to KL-6 antigen were performed. Serum KL-6 levels in patients with PAP were extremely high and were significantly higher than those in patients with interstitial lung diseases in which elevation of serum KL-6 has been recognized. BAL-fluid KL-6 levels in patients with PAP were higher than serum levels. Both serum and BAL-fluid KL-6 levels in patients with PAP correlated well with the disease activity. Immunohistochemically, positive staining was observed in proliferating type II pneumocytes. These results suggest the usefulness of KL-6 measurement in the diagnosis and estimation of disease activity of PAP.
First Department of Medicine, School of Medicine, Hokkaido University, Sapporo, Japan.
T
T
Takahashi
M
M
Munakata
I
I
Suzuki
Y
Y
Kawakami
D000911:Antibodies, Monoclonal; D000941:Antigens; D000951:Antigens, Neoplasm; D015415:Biomarkers / Q000032:analysis / Q000097:blood; D001992:Bronchoalveolar Lavage Fluid / Q000737:chemistry*; D004396:Coloring Agents; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006023:Glycoproteins; D006801:Humans; D006984:Hypertrophy; D007150:Immunohistochemistry; D008168:Lung / Q000473:pathology; D017563:Lung Diseases, Interstitial / Q000097:blood / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D018396:Mucin-1; D009077:Mucins / Q000032:analysis* / Q000097:blood; D010446:Peptide Fragments / Q000032:analysis* / Q000097:blood; D010503:Periodic Acid-Schiff Reaction; D011347:Procollagen / Q000032:analysis* / Q000097:blood; D011649:Pulmonary Alveolar Proteinosis / Q000097:blood / Q000378:metabolism* / Q000473:pathology; D011650:Pulmonary Alveoli / Q000473:pathology
D000911:Antibodies, Monoclonal; D000941:Antigens; D000951:Antigens, Neoplasm; D015415:Biomarkers; D004396:Coloring Agents; D006023:Glycoproteins; C060192:MUC1 protein, human; D018396:Mucin-1; D009077:Mucins; D010446:Peptide Fragments; D011347:Procollagen; C041876:procollagen Type III-N-terminal peptide