title: Elevated serum concentrations of polymorphonuclear neutrophilic leukocyte elastase in systemic sclerosis: association with pulmonary fibrosis.
abstract: OBJECTIVE
  To determine the serum concentrations and clinical association of polymorphonuclear neutrophilic leukocyte (PMN) elastase in patients with systemic sclerosis (SSc).
  
  
  METHODS
  Serum PMN elastase levels from 21 patients with limited cutaneous SSc (lSSc) and 32 with diffuse cutaneous SSc (dSSc) were examined by ELISA.
  
  
  RESULTS
  Serum PMN elastase levels were elevated in patients with SSc, especially dSSc, compared to healthy controls. SSc patients with elevated serum PMN elastase levels had more frequent presence of pulmonary fibrosis, arthritis, contracture of phalanges, and diffuse pigmentation. Anticentromere antibody was detected less frequently in SSc patients with elevated serum PMN elastase levels than in controls. Consistently, serum PMN elastase levels also correlated positively with serum levels of KL-6 and surfactant protein-D, serological markers for pulmonary fibrosis. Serum PMN elastase levels were also associated with levels of serum 8-isoprostane, an oxidative stress marker in SSc.
  
  
  CONCLUSIONS
  Serum PMN elastase levels were elevated in patients with SSc, and it was more prominent in patients with pulmonary fibrosis, suggesting that serum PMN elastase is a novel serological marker for SSc-related pulmonary fibrosis.
authors.affiliation: Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences, 1-7-1 Sakamoto, Nagasaki 852-8501, Japan.
authors.forename: Toshihide
authors.initials: T
authors.lastname: Hara
authors.forename: Fumihide
authors.initials: F
authors.lastname: Ogawa
authors.forename: Koichi
authors.initials: K
authors.lastname: Yanaba
authors.forename: Yohei
authors.initials: Y
authors.lastname: Iwata
authors.forename: Eiji
authors.initials: E
authors.lastname: Muroi
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Komura
authors.forename: Motoi
authors.initials: M
authors.lastname: Takenaka
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Shimizu
authors.forename: Minoru
authors.initials: M
authors.lastname: Hasegawa
authors.forename: Manabu
authors.initials: M
authors.lastname: Fujimoto
authors.forename: Shinichi
authors.initials: S
authors.lastname: Sato
mesh_terms: D000328:Adult; D000368:Aged; D015415:Biomarkers / Q000097:blood*; D015237:Dinoprost / Q000031:analogs & derivatives / Q000097:blood; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D019272:Leukocyte Elastase / Q000097:blood*; D008297:Male; D008875:Middle Aged; D018396:Mucin-1 / Q000097:blood; D018384:Oxidative Stress; D011658:Pulmonary Fibrosis / Q000097:blood* / Q000175:diagnosis*; D037663:Pulmonary Surfactant-Associated Protein D / Q000097:blood; D012595:Scleroderma, Systemic / Q000097:blood* / Q000175:diagnosis*
chemical_list: D015415:Biomarkers; C060192:MUC1 protein, human; D018396:Mucin-1; D037663:Pulmonary Surfactant-Associated Protein D; C075750:8-epi-prostaglandin F2alpha; D015237:Dinoprost; D019272:Leukocyte Elastase