Α1-Antitrypsin PHENOTYPES IN FIBROSING ALVEOLITIS AND RHEUMATOID ARTHRITIS

Geddes, Duncan M.; Webley, M; Brewerton, D. A.; Turton, Charles; Turner-Warwick, M; Murphy, AJ; Ward, A. Milford

doi:10.1016/s0140-6736(77)91883-9

Cited by 108 publications

(50 citation statements)

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“…Our results provide no evidence of an association between the presence of abnormal alpha 1 -antitrypsin phenotypes and cryptogenic fibrosing alveolitis, findings which clearly contrast with those of GEDDES et al [10]. This negative study is not due to a lack of statistical power since we had sufficient cases, almost four times as many as GEDDES et al [10], and controls to detect an OR of 2.5, which is of similar size to the effect reported previously [10].…”

Section: Discussioncontrasting

confidence: 65%

“…Homozygote deficiency of alpha 1 -antitrypsin is known to cause emphysema, but there have been reports of increased heterozygote phenotype deficiency in patients with rheumatoid arthritis, juvenile polyarthritis and vasculitis [9]. In one widely cited study, patients with rheumatoid arthritis complicated by fibrosing alveolitis were found to be more likely to have abnormal alpha 1 -antitrypsin phenotypes than healthy controls [10]. Because of this finding, a further 49 patients with CFA were studied and found to be two and a half times more likely to have abnormal alpha 1 -antitrypsin phenotypes than healthy controls.…”

mentioning

confidence: 99%

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Alpha1-antitrypsin phenotypes in patients with cryptogenic fibrosing alveolitis: a case-control study

Hubbard

Baoku²,

Kalsheker³

et al. 1997

Eur Respir J

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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.

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Section: Discussioncontrasting

confidence: 65%

mentioning

confidence: 99%

Alpha1-antitrypsin phenotypes in patients with cryptogenic fibrosing alveolitis: a case-control study

Hubbard

Baoku²,

Kalsheker³

et al. 1997

Eur Respir J

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“…The most common phenotype in the PI loci encoding the a 1 -AT gene is MM, which results in normal serum levels of a 1 -AT, whereas the PI ZZ phenotype results in low serum levels of a 1 -AT. A highly significant increase in the frequency of a non-MM (MZ) phenotype was found among patients with pulmonary fibrosis with and without rheumatoid arthritis (RA), as compared with control subjects and patients with RA with no pulmonary fibrosis (54). These findings were replicated in patients with RA in a later study (55).…”

Section: Candidate Gene Studies In Pulmonary Fibrosismentioning

confidence: 92%

Genetic Analysis of Sporadic and Familial Interstitial Pneumonia

Schwartz

2008

Proceedings of the American Thoracic Society

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Although much progress has been made in understanding the biology and clinical course of interstitial pneumonia, the etiology of this disease remains elusive. Epidemiologic studies have consistently identified cigarette smoke as an important exposure; however, most smokers do not develop interstitial pneumonia and many individuals with interstitial pneumonia do not smoke cigarettes. Moreover, interstitial pneumonias have been reported to cluster in families. Thus, a more thorough understanding of the genetic etiology of interstitial pneumonia may prove critically important in defining the biology and clinical course of this complex human disease.

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“…Significant risk factors for developing lung disease in RA include presence of subcutaneous nodules, high titres of circulating rheumatoid factor or antinuclear antibodies (Gordon et al, 1973) and genetic factors, for example, the presence of non-MM alpha 1-antitrypsin phenotypes. (Geddes et al, 1977;Michalski et al, 1986) Common symptoms include exertional dyspnoea and nonproductive cough. Clinical examination reveals bibasal pulmonary crackles in most patients.…”

Section: Rheumatoid Arthritis (Ra)mentioning

confidence: 99%