Aipha1-antitrypsin phenotypes in patients with cryptogenic fibrosing alveolitis: A case-control study

R. Hubbard, Y. Baoku, N. Kalsheker, J. Britton, I. Johnston

Research output: Journal PublicationArticlepeer-review

15 Citations (Scopus)


Cryptogenic fibrosing alveolitis (CFA) is an interstitial lung disease, which by definition is of unknown aetiology. Recent evidence has suggested that smoking and occupational exposure to dusts may be environmental risk factors for the disease, but there has been little research into potential host risk factors. One previous study has suggested that the prevalence of abnormal alpha1-antitrypsin phenotypes may be increased in patients with CFA. Since alpha1-antitrypsin is important in regulating inflammation within the lung in response to environmental exposures, such abnormalities may be of aetiological importance in this disease. We have compared the alpha1-antitrypsin phenotypes of 189 patients with CFA with 189 age-, sex-, and community-matched controls. This sample size was sufficient to provide more than 95% power to detect an odds ratio (OR) of 2.5. Alpha1-antitrypsin phenotype was established by isoelectric focusing, and the prevalence of abnormal phenotypes in cases and controls was compared by conditional logistic regression, personal smoking histories were obtained by postal questionnaire. The prevalence of abnormal alpha1-antitrypsin phenotypes was similar in cases and controls (12.7 versus 15.3%; OR 0.88; 95% confidence interval 0.49-1.57; p = 0.66). No interaction was found between the presence of abnormal alpha1-antitrypsin phenotypes and a history of smoking. We conclude that cryptogenic fibrosing alveolitis is not associated with abnormal alpha1-antitrypsin phenotypes.

Original languageEnglish
Pages (from-to)2881-2883
Number of pages3
JournalEuropean Respiratory Journal
Issue number12
Publication statusPublished - Dec 1997
Externally publishedYes


  • Alpha-antitrypsin
  • Cryptogenic fibrosing alveolitis
  • Epidemiology
  • Idiopathic pulmonary fibrosis

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine


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