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alpha 1-Proteinase inhibitor, elastase activity, and lung disease severity in cystic fibrosis.
Am Rev Respir Dis. 1993 Dec; 148(6 Pt 1):1665-70.AR

Abstract

The potential role of neutrophil elastase in exacerbating pulmonary infection and tissue damage in cystic fibrosis (CF) has led to proposals for treatment of lung disease in CF with the elastase inhibitor, alpha 1-proteinase inhibitor (alpha 1PI). Reports that alpha 1PI is inactivated in the CF lung suggest that the effectiveness of alpha 1PI therapy depends on the quantity of elastase present and the extent of alpha 1PI inactivation, both of which are expected to vary with disease severity. In this study we assessed the elastase-alpha 1PI profile in sputum and plasma from CF patients with various degrees of pulmonary involvement. Levels of active elastase in sputum samples increased with severity of pulmonary disease (F ratio = 5.63, p < 0.01), as did sputum levels of alpha 1PI (F ratio = 4.88, p < 0.01). A positive correlation was observed between sputum levels of active elastase and alpha 1PI (r = 0.68, p < 0.005). Plasma alpha 1PI levels were also elevated in CF patients compared with control subjects (p < 0.005), indicating a compensatory increase in plasma and sputum levels of alpha 1PI in response to increased elastase load. Molar levels of total immunogenic neutrophil elastase were, on average, 12 times higher than alpha 1PI in CF sputum. These results suggest that the major contributor to the elevated levels of active elastase observed in the CF lung is an increase in elastase release rather than inactivation of alpha 1PI.

Authors+Show Affiliations

Department of Medicine, University College Dublin, Ireland.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

8256917

Citation

O'Connor, C M., et al. "Alpha 1-Proteinase Inhibitor, Elastase Activity, and Lung Disease Severity in Cystic Fibrosis." The American Review of Respiratory Disease, vol. 148, no. 6 Pt 1, 1993, pp. 1665-70.
O'Connor CM, Gaffney K, Keane J, et al. Alpha 1-Proteinase inhibitor, elastase activity, and lung disease severity in cystic fibrosis. Am Rev Respir Dis. 1993;148(6 Pt 1):1665-70.
O'Connor, C. M., Gaffney, K., Keane, J., Southey, A., Byrne, N., O'Mahoney, S., & Fitzgerald, M. X. (1993). Alpha 1-Proteinase inhibitor, elastase activity, and lung disease severity in cystic fibrosis. The American Review of Respiratory Disease, 148(6 Pt 1), 1665-70.
O'Connor CM, et al. Alpha 1-Proteinase Inhibitor, Elastase Activity, and Lung Disease Severity in Cystic Fibrosis. Am Rev Respir Dis. 1993;148(6 Pt 1):1665-70. PubMed PMID: 8256917.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - alpha 1-Proteinase inhibitor, elastase activity, and lung disease severity in cystic fibrosis. AU - O'Connor,C M, AU - Gaffney,K, AU - Keane,J, AU - Southey,A, AU - Byrne,N, AU - O'Mahoney,S, AU - Fitzgerald,M X, PY - 1993/12/1/pubmed PY - 1993/12/1/medline PY - 1993/12/1/entrez SP - 1665 EP - 70 JF - The American review of respiratory disease JO - Am Rev Respir Dis VL - 148 IS - 6 Pt 1 N2 - The potential role of neutrophil elastase in exacerbating pulmonary infection and tissue damage in cystic fibrosis (CF) has led to proposals for treatment of lung disease in CF with the elastase inhibitor, alpha 1-proteinase inhibitor (alpha 1PI). Reports that alpha 1PI is inactivated in the CF lung suggest that the effectiveness of alpha 1PI therapy depends on the quantity of elastase present and the extent of alpha 1PI inactivation, both of which are expected to vary with disease severity. In this study we assessed the elastase-alpha 1PI profile in sputum and plasma from CF patients with various degrees of pulmonary involvement. Levels of active elastase in sputum samples increased with severity of pulmonary disease (F ratio = 5.63, p < 0.01), as did sputum levels of alpha 1PI (F ratio = 4.88, p < 0.01). A positive correlation was observed between sputum levels of active elastase and alpha 1PI (r = 0.68, p < 0.005). Plasma alpha 1PI levels were also elevated in CF patients compared with control subjects (p < 0.005), indicating a compensatory increase in plasma and sputum levels of alpha 1PI in response to increased elastase load. Molar levels of total immunogenic neutrophil elastase were, on average, 12 times higher than alpha 1PI in CF sputum. These results suggest that the major contributor to the elevated levels of active elastase observed in the CF lung is an increase in elastase release rather than inactivation of alpha 1PI. SN - 0003-0805 UR - https://www.unboundmedicine.com/medline/citation/8256917/alpha_1-Proteinase_inhibitor,_elastase_activity,_and_lung_disease_severity_in_cystic_fibrosis. L2 - https://www.atsjournals.org/doi/10.1164/ajrccm/148.6_Pt_1.1665?url_ver=Z39.88-2003&amp;rfr_id=ori:rid:crossref.org&amp;rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -