title: Plasma levels of alpha1-antichymotrypsin and secretory leukocyte proteinase inhibitor in healthy and chronic obstructive pulmonary disease (COPD) subjects with and without severe alpha1-antitrypsin deficiency.
abstract: BACKGROUND
  Individuals with severe Z alpha1-antitrypsin (AAT) deficiency have a considerably increased risk of developing chronic obstructive lung disease (COPD). It has been hypothesized that compensatory increases in levels of other protease inhibitors mitigate the effects of this AAT deficiency. We analysed plasma levels of AAT, alpha1-antichymotrypsin (ACT) and secretory leukocyte protease inhibitor (SLPI) in healthy (asymptomatic) and COPD subjects with and without AAT deficiency.
  
  
  METHODS
  Studied groups included: 71 asymptomatic AAT-deficient subjects (ZZ, n = 48 and SZ, n = 23, age 31 +/- 0.5) identified during Swedish neonatal screening for AAT deficiency between 1972 and 1974; age-matched controls (MM, n = 57, age 30.7 +/- 0.6); older asymptomatic ZZ (n = 10); healthy MM (n = 20, age 53 +/- 9.6); and COPD patients (ZZ, n = 10, age 47.4 +/- 11 and MM, n = 10, age 59.4 +/- 6.7). Plasma levels of SLPI, AAT and ACT were analysed using ELISA and immunoelectrophoresis.
  
  
  RESULTS
  No significant difference was found in plasma ACT and SLPI levels between the healthy MM and the ZZ or SZ subjects in the studied groups. Independent of the genetic variant, subjects with COPD (n = 19) had elevated plasma levels of SLPI and ACT relative to controls (n = 153) (49.5 +/- 7.2 vs 40.7 +/- 9.1 ng/ml, p < 0.001 and 0.52 +/- 0.19 vs 0.40 +/- 0.1 mg/ml, p < 0.05, respectively).
  
  
  CONCLUSIONS
  Our findings show that plasma levels of ACT and SLPI are not elevated in subjects with genetic AAT deficiency compared MM controls and do not appear to compensate for the deficiency of plasma AAT.
authors.affiliation: Department of Otolaryngology, Institution of Clinical Sciences, Lund University, University Hospital Malmö, SE-205 02 Malmö, Sweden. Camilla.Hollander@med.lu.se
authors.forename: Camilla
authors.initials: C
authors.lastname: Hollander
authors.forename: Ulla
authors.initials: U
authors.lastname: Westin
authors.forename: Anders
authors.initials: A
authors.lastname: Wallmark
authors.forename: Eeva
authors.initials: E
authors.lastname: Piitulainen
authors.forename: Tomas
authors.initials: T
authors.lastname: Sveger
authors.forename: Sabina M
authors.initials: SM
authors.lastname: Janciauskiene
mesh_terms: D000328:Adult; D000367:Age Factors; D000704:Analysis of Variance; D016022:Case-Control Studies; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011336:Probability; D011379:Prognosis; D029424:Pulmonary Disease, Chronic Obstructive / Q000097:blood* / Q000175:diagnosis; D012016:Reference Values; D053481:Secretory Leukocyte Peptidase Inhibitor / Q000097:blood*; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index; D012737:Sex Factors; D000514:alpha 1-Antichymotrypsin / Q000097:blood*; D019896:alpha 1-Antitrypsin Deficiency / Q000097:blood* / Q000175:diagnosis
chemical_list: D053481:Secretory Leukocyte Peptidase Inhibitor; D000514:alpha 1-Antichymotrypsin