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      Why do some adults with PiMZ α 1-antitrypsin develop bronchiectasis?

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          Abstract

          Bronchiectasis is an aetiologically heterogeneous, chronic and often progressive disease resulting in the permanent dilatation of one or more bronchi or bronchioli. Several reports indicated an association among inherited α 1-antitrypsin deficiency (α 1-ATD), pulmonary infections and bronchiectasis, with a frequency up to 10% [1, 2]. It has been postulated that in α 1-ATD individuals repeated episodes of ordinary bronchitis, of whatever cause, may lead to the development of bronchiectasis [3]. Most reported α 1-ATD cases with bronchiectasis are elderly homozygous PiZZ (Glu342Lys) smokers with emphysema. There are only a few historical case reports with bronchiectasis and α 1-ATD in the absence of emphysema [4, 5]. Whether there is an increased risk of pulmonary diseases, including bronchiectasis, in heterozygous PiMZ α 1-ATD carriers is a matter of debate [6, 7].

          Abstract

          Recurrent infections of the upper airways in early life may be a warning sign of inherited α 1-antitrypsin deficiency http://ow.ly/iJsF300kbyV

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          A Comprehensive Analysis of the Impact of Pseudomonas aeruginosa Colonization on Prognosis in Adult Bronchiectasis.

          Simon Finch, Melissa J McDonnell, Hani Abo-Leyah … (2015)
          Eradication and suppression of Pseudomonas aeruginosa is a key priority in national guidelines for bronchiectasis and is a major focus of drug development and clinical trials. An accurate estimation of the clinical impact of P. aeruginosa in bronchiectasis is therefore essential.
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            Antielastases of the human alveolar structures. Implications for the protease-antiprotease theory of emphysema.

            Deena Zimmerman, J E Gadek, S Rennard … (1981)
            The current concepts of the pathogenesis of emphysema hold that progressive, chronic destruction of the alveolar structures occurs because there was in imbalance between the proteases and antiproteases in the lower respiratory tract. In this context, proteases, particularly neutrophil elastase, work unimpeded to destroy the alveolar structures. This concept has evolved from consideration of patients with alpha 1-antitrypsin deficiency, who have decreased levels of serum alpha 1-antitrypsin and who have progressive panacinar emphysema. To directly assess the antiprotease side of this equation, the lower respiratory tract of non-smoking individuals with normal serum antiproteases and individuals with PiZ homozygous alpha 1-antitrypsin deficiency underwent bronchoalveolar lavage to evaluate the antiprotease screen of their lower respiratory tract. These studies demonstrated that: (a) alpha 1-antitrypsin is the major antielastase of the normal human lower respiratory tract; (b) alpha 2-macroglobulin, a large serum antielastase, and the bronchial mucous inhibitor, an antielastase of the central airways, do not contribute to the antielastase protection of the human alveolar structures; (c) individuals with PiZ alpha 1-antitrypsin deficiency have little or no alpha 1-antitrypsin in their lower respiratory tract and have no alternative antiprotease protection against neutrophil elastase; and (d) the lack of antiprotease protection of the lower respiratory tract of PiZ individuals is a chronic process, suggesting their vulnerability to neutrophil elastase is always present.
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              Chronic obstructive pulmonary disease in alpha1-antitrypsin PI MZ heterozygotes: a meta-analysis.

              C P Hersh, C K Hersh, Cameron N Ly … (2004)
              Severe alpha(1)-antitrypsin deficiency, usually related to homozygosity for the protease inhibitor (PI) Z allele, is a proven genetic risk factor for chronic obstructive pulmonary disease (COPD). The risk of COPD in PI MZ heterozygous individuals is controversial. A search of MEDLINE from January 1966 to May 2003 identified studies that examined the risk of COPD in PI MZ individuals and studies that measured forced expiratory volume in 1 second (FEV(1)) in heterozygotes. In 16 studies that reported COPD as a categorical outcome, the combined odds ratio (OR) for PI MZ versus PI MM (normal genotype) was 2.31 (95% CI 1.60 to 3.35). The summary OR was higher in case-control studies (OR 2.97; 95% CI 2.08 to 4.26) than in cross sectional studies (OR 1.50; 95% CI 0.97 to 2.31) and was attenuated in studies that adjusted for cigarette smoking (OR 1.61; 95% CI 0.92 to 2.81). In seven studies that reported FEV(1) as a continuous outcome there was no difference in mean FEV(1) between PI MM and PI MZ individuals. Case-control studies showed increased odds of COPD in PI MZ individuals, but this finding was not confirmed in cross sectional studies. Variability in study design and quality limits the interpretation. These results are consistent with a small increase in risk of COPD in all PI MZ individuals or a larger risk in a subset. Future studies that adjust for smoking and include other COPD related phenotypes are required to conclusively determine the risk of COPD in PI MZ heterozygotes.
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                Author and article information

                Journal
                Journal ID (nlm-ta): ERJ Open Res
                Journal ID (iso-abbrev): ERJ Open Res
                Journal ID (publisher-id): ERJOR
                Journal ID (hwp): erjor
                Title: ERJ Open Research
                Publisher: European Respiratory Society
                ISSN (Electronic): 2312-0541
                Publication date Collection: April 2016
                Publication date (Electronic): 06 June 2016
                Volume: 2
                Issue: 2
                Electronic Location Identifier: 00021-2016
                Affiliations
                [1 ]Dept of Respiratory Medicine, University Children's Hospital, Hannover Medical School, Endstage and Obstructive Lung Disease Hannover (BREATH), Member of the German Center for Lung Research (DZL), Hannover, Germany
                [2 ]Molecular Genetics Unit, Instituto de Investigación de Enfermedades Raras (IIER), Instituto de Salud Carlos III (ISCIII), Madrid, Spain
                [3 ]Dept of Pediatric Pneumology, Allergy and Neonatology, University Children's Hospital, Hannover Medical School, Endstage and Obstructive Lung Disease Hannover (BREATH), Member of the German Center for Lung Research (DZL), Hannover, Germany
                Author notes
                Sabina Janciauskiene, Dept of Respiratory Medicine, Hannover Medical School, Feodor-Lynen Str. 23, Hannover 30625, Germany. E-mail: SabinaJanciauskiene@ 123456gmail.com
                Author information
                http://orcid.org/0000-0001-6834-350X
                Article
                Publisher ID: 00021-2016
                DOI: 10.1183/23120541.00021-2016
                PMC ID: 5005170
                PubMed ID: 27730187
                SO-VID: 6665f06a-b632-404a-afd9-c099ec7cb88b
                Copyright © Copyright ©ERS 2016
                License:

                This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.

                History
                Date received : 15 February 2016
                Date accepted : 23 April 2016
                Categories
                Subject: Original Research Letters
                Subject: 3
                Subject: 16

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