title: Serum levels of KL-6 as a useful marker for evaluating pulmonary fibrosis in patients with systemic sclerosis.
abstract: OBJECTIVE
  KL-6 is a mucin-like glycoprotein that is strongly expressed on type II pneumocytes in the lung. Circulating KL-6 has been shown to be a sensitive marker of the disease activity of interstitial lung diseases. We determined the serum levels of KL-6 in patients with systemic sclerosis (SSc) and investigated whether these levels would serve as a useful marker of pulmonary fibrosis (PF) in patients with SSc.
  
  
  METHODS
  The serum KL-6 levels were determined using a specific ELISA in 91 patients with SSc, and in 38 healthy controls.
  
  
  RESULTS
  The serum levels of KL-6 were significantly higher in patients with SSc than in healthy controls (923+/-860 vs. 382+/-55 U/ml; p<0.0001). The serum KL-6 levels of the patients with diffuse cutaneous SSc (dSSc) tended to be higher than those with limited cutaneous SSc (ISSc) (1054+/-1000 vs. 800+/-694 U/ml), but there was no significant difference between these 2 groups. The serum KL-6 levels in the patients with PF were significantly elevated compared to those without PF (1283+/-1056 vs. 520+/-148 U/ml; p<0.0001). Moreover, DLCO and VC were also significantly decreased in the patients with elevated KL-6 levels compared to those with normal levels (62+/-22% vs. 72+/-17%, p<0.05; 87 +/-20% vs. 100+/-18%, p<0.01, respectively).
  
  
  CONCLUSIONS
  Serum KL-6 level may be a useful serum marker for evaluating pulmonary fibrosis in patients with SSc.
authors.affiliation: Department of Dermatology, Faculty of Medicine, University of Tokyo, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Yamane
authors.forename: H
authors.initials: H
authors.lastname: Ihn
authors.forename: M
authors.initials: M
authors.lastname: Kubo
authors.forename: N
authors.initials: N
authors.lastname: Yazawa
authors.forename: K
authors.initials: K
authors.lastname: Kikuchi
authors.forename: Y
authors.initials: Y
authors.lastname: Soma
authors.forename: K
authors.initials: K
authors.lastname: Tamaki
mesh_terms: D000328:Adult; D000941:Antigens / Q000097:blood*; D000951:Antigens, Neoplasm; D001323:Autoantibodies / Q000097:blood; D015415:Biomarkers; D004264:DNA Topoisomerases, Type I / Q000276:immunology; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006023:Glycoproteins / Q000097:blood*; D006801:Humans; D008168:Lung / Q000201:enzymology / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D018396:Mucin-1; D009077:Mucins; D011237:Predictive Value of Tests; D011658:Pulmonary Fibrosis / Q000097:blood / Q000175:diagnosis / Q000209:etiology; D012595:Scleroderma, Systemic / Q000150:complications*
chemical_list: D000941:Antigens; D000951:Antigens, Neoplasm; D001323:Autoantibodies; D015415:Biomarkers; D006023:Glycoproteins; C060192:MUC1 protein, human; D018396:Mucin-1; D009077:Mucins; D004264:DNA Topoisomerases, Type I