title: Airway hyperresponsiveness in a large group of subjects with alpha1-antitrypsin deficiency: a cross-sectional controlled study.
abstract: BACKGROUND
  It has been suggested that subjects with alpha-antitrypsin (AAT) deficiency, lacking a major antiprotease defence against airway inflammation, might be more susceptible of development of airway hyperresponsiveness (AHR). Moreover, lower AAT blood levels might also be able to influence the severity of AHR.
  
  
  OBJECTIVE
  This study was aimed to investigate the prevalence of AHR in a large group of subjects with AAT deficiency included in the Italian Registry and to evaluate the relationship between AAT blood levels and the severity of AHR in this population.
  
  
  METHODS
  Cross-sectional controlled study.
  
  
  METHODS
  Regional Reference Centre for AAT deficiency in Brescia, Italy.
  
  
  METHODS
  A total of 114 subjects with AAT deficiency underwent pulmonary function tests. Eighty-six were eligible to perform a bronchial provocation test with methacholine (MCh) (baseline FEV1 > 60% predicted) to assess the provocative dose producing a 20% fall of FEV1 (PD20FEV1). Similar measurements were performed in a control group of 27 age-matched normal subjects.
  
  
  RESULTS
  The prevalence of AHR (PD20FEV1 < 2000 microg MCh) was not different between AAT deficiency subjects and controls (16.3% and 11.1%, respectively; P = 0.66), and also amongst two subgroups of AAT deficiency subjects divided according to different protease inhibitor (Pi) phenotypes (PiMZ-MS, PiSZ-ZZ). Hyperresponsive subjects with AAT deficiency, however, showed a positive correlation between AAT blood levels and PD20FEV1 values (r = 0.71, P < 0.01).
  
  
  CONCLUSIONS
  These findings indicate that AAT deficiency subjects did not exhibit a greater prevalence of airway hyperresponsiveness as compared with control subjects, but suggest that, in the subset of AAT deficiency subjects hyperresponsive to MCh, lower levels of AAT are associated with a higher severity of AHR.
authors.affiliation: Institute of Internal Medicine, University of Brescia, Brescia, Italy. malerba@master.cci.unibs.it
authors.forename: M
authors.initials: M
authors.lastname: Malerba
authors.forename: A
authors.initials: A
authors.lastname: Radaeli
authors.forename: L
authors.initials: L
authors.lastname: Ceriani
authors.forename: C
authors.initials: C
authors.lastname: Tantucci
authors.forename: V
authors.initials: V
authors.lastname: Grassi
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D016535:Bronchial Hyperreactivity / Q000209:etiology*; D001985:Bronchial Provocation Tests; D016085:Bronchoconstrictor Agents; D002248:Carbon Monoxide; D003430:Cross-Sectional Studies; D005260:Female; D005541:Forced Expiratory Volume / Q000502:physiology; D006801:Humans; D008297:Male; D016210:Methacholine Chloride; D008875:Middle Aged; D014797:Vital Capacity / Q000502:physiology; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications*
chemical_list: D016085:Bronchoconstrictor Agents; D016210:Methacholine Chloride; D002248:Carbon Monoxide