{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,5]],"date-time":"2026-05-05T03:44:06Z","timestamp":1777952646048,"version":"3.51.4"},"reference-count":33,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2020,3,29]],"date-time":"2020-03-29T00:00:00Z","timestamp":1585440000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJERPH"],"abstract":"<jats:p>Alpha-1-antitrypsin deficiency (AATd) is a hereditary disease, mainly characterized by early onset and the lower lobes\u2019 predominant emphysema. Bronchiectasis is characterized by dilatation of the bronchial wall and a clinical syndrome whose features are a cough, sputum production and frequent respiratory exacerbations. In the literature, there are many papers concerning these two clinical entities, but there is still a lot of debate about a possible association between them, in particular about the frequency of their association and causal links. The aim of this short communication is to show the literature reports about the association between AATd and bronchiectasis to establish the state of the art and possible future developments in this research field.<\/jats:p>","DOI":"10.3390\/ijerph17072294","type":"journal-article","created":{"date-parts":[[2020,3,31]],"date-time":"2020-03-31T10:29:38Z","timestamp":1585650578000},"page":"2294","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Alpha-1-Antitrypsin Deficiency and Bronchiectasis: A Concomitance or a Real Association?"],"prefix":"10.3390","volume":"17","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0977-2643","authenticated-orcid":false,"given":"Alessandro","family":"Sanduzzi","sequence":"first","affiliation":[{"name":"Department of Clinical Medicine and Surgery, Section of Respiratory Disease, University Federico II, AORN dei Colli-Monaldi Hospital, 80138 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emanuele","family":"Ciasullo","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine and Surgery, Section of Respiratory Disease, University Federico II, AORN dei Colli-Monaldi Hospital, 80138 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ludovica","family":"Capitelli","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine and Surgery, Section of Respiratory Disease, University Federico II, AORN dei Colli-Monaldi Hospital, 80138 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefano","family":"Sanduzzi Zamparelli","sequence":"additional","affiliation":[{"name":"University Luigi Vanvitelli, U.O.C. Clinica Pneumologica SUN, AORN dei Colli-Monaldi Hospital, 80138 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marialuisa","family":"Bocchino","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine and Surgery, Section of Respiratory Disease, University Federico II, AORN dei Colli-Monaldi Hospital, 80138 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2020,3,29]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"190073","DOI":"10.1183\/16000617.0073-2019","article-title":"\u03b11-Antitrypsin deficiency and chronic respiratory disorders","volume":"29","author":"Cazzola","year":"2020","journal-title":"Eur. Respir. Rev."},{"key":"ref_2","first-page":"10006","article-title":"Diagnostic evaluation of bronchiectasis","volume":"1","author":"Hsieh","year":"2019","journal-title":"Respir. Med. X"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1055","DOI":"10.1056\/NEJM198704233161704","article-title":"Replacement therapy for alpha 1-antitrypsin deficiency associated with emphysema","volume":"316","author":"Wewers","year":"1987","journal-title":"N. Engl. J. Med."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"S189","DOI":"10.1097\/01.CCM.0000057842.90746.1E","article-title":"Proteases and lung injury","volume":"31","author":"Moraes","year":"2003","journal-title":"Crit. Care Med."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"S8","DOI":"10.1016\/S0002-9343(99)80304-0","article-title":"Role of inflammation in respiratory tract infections","volume":"99","author":"Stockley","year":"1995","journal-title":"Am. J. Med."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1165\/rcmb.2011-0011RT","article-title":"Innate immunity in the respiratory epithelium","volume":"45","author":"Parker","year":"2011","journal-title":"Am. J. Respir. Cell Mol. Biol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1155","DOI":"10.2353\/ajpath.2006.060058","article-title":"Alpha-1 antitrypsin inhibits caspase-3 activity, preventing lung endothelial cell apoptosis","volume":"169","author":"Petrache","year":"2006","journal-title":"Am. J. Pathol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1016\/S0167-5699(97)01243-7","article-title":"The molecular basis of leukocytosis","volume":"19","author":"Opdenakker","year":"1998","journal-title":"Immunol. Today"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"341","DOI":"10.3389\/fphar.2018.00341","article-title":"The Multifaceted Effects of Alpha1-Antitrypsin on Neutrophil Functions","volume":"9","author":"Janciauskiene","year":"2018","journal-title":"Front Pharmacol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"15007","DOI":"10.1073\/pnas.1309648110","article-title":"Anti-inflammatory and immunomodulatory properties of \u03b11-antitrypsin without inhibition of elastase","volume":"110","author":"Jonigk","year":"2013","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"4236","DOI":"10.1172\/JCI41196","article-title":"\u03b1-1 Antitrypsin regulates human neutrophil chemotaxis induced by soluble immune complexes and IL-8","volume":"120","author":"Bergin","year":"2010","journal-title":"J. Clin. Investig."},{"key":"ref_12","first-page":"3628","article-title":"The BLT1 Inhibitory Function of \u03b1-1 Antitrypsin Augmentation Therapy Disrupts Leukotriene B4 Neutrophil Signaling","volume":"15","author":"Wormald","year":"2015","journal-title":"J. Immunol."},{"key":"ref_13","first-page":"217","article-title":"The circulating proteinase inhibitor \u03b1-1 antitrypsin regulates neutrophil degranulation and autoimmunity","volume":"1","author":"Bergin","year":"2014","journal-title":"Sci. Transl. Med."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"680","DOI":"10.2119\/molmed.2016.00119","article-title":"\u03b1-Linoleic acid enhances the capacity of \u03b1-1 antitrypsin to inhibit lipopolysaccharide induced IL-1\u03b2 in human blood neutrophils","volume":"22","author":"Aggarwal","year":"2016","journal-title":"Mol. Med."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1177\/1535370217740852","article-title":"Alpha 1-antitrypsin ameliorates ventilator-induced lung injury in rats by inhibiting inflammatory responses and apoptosis","volume":"243","author":"Zhu","year":"2018","journal-title":"Exp. Biol. Med. (Maywood)"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"19","DOI":"10.3389\/fpubh.2013.00019","article-title":"Alpha-1 Antitrypsin Reduces Severity of Pseudomonas Pneumonia in Mice and Inhibits Epithelial Barrier Disruption and Pseudomonas Invasion of Respiratory Epithelial Cells","volume":"1","author":"Pott","year":"2013","journal-title":"Front. 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Dis."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"690","DOI":"10.1080\/00365540701225744","article-title":"Alpha-1-antitrypsin (AAT) anomalies are associated with lung disease due to rapidly growing mycobacteria and AAT inhibits Mycobacterium abscessus infection of macrophages","volume":"39","author":"Chan","year":"2007","journal-title":"Scand. J. Infect. Dis."},{"key":"ref_20","first-page":"1248","article-title":"Association between alpha 1 antitrypsin and bronchiectasis","volume":"46","author":"Araujo","year":"2015","journal-title":"Eur. Respir. J."},{"key":"ref_21","first-page":"4780","article-title":"Bronchiectasis but not emphysema is more prevalent in non-PiZ alpha-1 antitrypsin deficiency (AATD) COPD than in usual COPD","volume":"44","author":"Dandurand","year":"2014","journal-title":"Eur. Respir. J."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"59","DOI":"10.4103\/atm.ATM_234_17","article-title":"Description of a new rare alpha-1 antitrypsin mutation in Naples (Italy), PI*M S-Napoli","volume":"13","author":"Mosella","year":"2018","journal-title":"Ann. Thorac. Med."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"679","DOI":"10.1007\/s00408-017-0033-2","article-title":"A New SERPINA-1 Missense Mutation Associated with Alpha-1Antitrypsin Deficiency and Bronchiectasis","volume":"195","author":"Carpagnano","year":"2017","journal-title":"Lung"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1384","DOI":"10.1378\/chest.104.5.1384","article-title":"Bronchiectasis in patients with a1-antitrypsin deficiency: A rare occurrence?","volume":"104","author":"Shin","year":"1993","journal-title":"Chest"},{"key":"ref_25","first-page":"12","article-title":"Etiology of Non\u2013Cystic Fibrosis Bronchiectasis in Adults and Its Correlation to Disease Severity","volume":"12","author":"Lonni","year":"2015","journal-title":"AnnalsATS"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"1277","DOI":"10.1164\/ajrccm.162.4.9906120","article-title":"An investigation into causative factors in patients with bronchiectasis","volume":"162","author":"PastEur","year":"2000","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_27","first-page":"37","article-title":"Bronchiectasis in patients with alpha1-antitrypsin deficiency: Prevalence and characteristics","volume":"48","author":"Cortese","year":"2016","journal-title":"Eur. Respir. J."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1215","DOI":"10.1164\/rccm.200703-489OC","article-title":"Prevalence and impact of bronchiectasis in alpha1-antitrypsin deficiency","volume":"15","author":"Parr","year":"2007","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_29","first-page":"4359","article-title":"Bronchiectasis in alpha1-antitrypsin deficiency","volume":"54","author":"Costa","year":"2019","journal-title":"Eur. Respir. J."},{"key":"ref_30","first-page":"50","article-title":"European Respiratory Society statement: Diagnosis and treatment of pulmonary disease in \u03b11-antitrypsin deficiency","volume":"30","author":"Miravitlles","year":"2017","journal-title":"Eur. Respir. J."},{"key":"ref_31","first-page":"50","article-title":"European Respiratory Society guidelines for the management of adult bronchiectasis","volume":"9","author":"Polverino","year":"2017","journal-title":"Eur. Respir. J."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"e000348","DOI":"10.1136\/bmjresp-2018-000348","article-title":"British Thoracic Society guideline for bronchiectasis in adults","volume":"5","author":"Hill","year":"2018","journal-title":"BMJ Open Resp. Res."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"244","DOI":"10.1183\/13993003.00232-2016","article-title":"SIP bronchiectasis audit working group. Quality standards for the management of bronchiectasis in Italy: A national audit","volume":"48","author":"Aliberti","year":"2016","journal-title":"Eur. Respir. J."}],"container-title":["International Journal of Environmental Research and Public Health"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1660-4601\/17\/7\/2294\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T09:13:04Z","timestamp":1760173984000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1660-4601\/17\/7\/2294"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,3,29]]},"references-count":33,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2020,4]]}},"alternative-id":["ijerph17072294"],"URL":"https:\/\/doi.org\/10.3390\/ijerph17072294","relation":{},"ISSN":["1660-4601"],"issn-type":[{"value":"1660-4601","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,3,29]]}}}