{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T03:02:58Z","timestamp":1790564578291,"version":"4.1.0"},"reference-count":33,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2022,1,19]],"date-time":"2022-01-19T00:00:00Z","timestamp":1642550400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000272","name":"National Institute for Health Research","doi-asserted-by":"publisher","award":["Academic Clinical Lectureship"],"award-info":[{"award-number":["Academic Clinical Lectureship"]}],"id":[{"id":"10.13039\/501100000272","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Air in the pleural cavity is termed pneumothorax. When this occurs in the absence of trauma or medical intervention, it is called spontaneous pneumothorax. Primary spontaneous pneumothorax typically occurs in young patients without known lung disease. However, the idea that these patients have \u201cnormal\u201d lungs is outdated. This article will review evidence of inflammation and respiratory bronchiolitis on surgical specimens, discuss the identification of emphysema-like change (i.e., blebs and bullae), the concept of pleural porosity and review recent data on the overexpression of matrix metalloproteinases in the lungs of patients who have had pneumothorax.<\/jats:p>","DOI":"10.3390\/jcm11030490","type":"journal-article","created":{"date-parts":[[2022,1,19]],"date-time":"2022-01-19T08:20:57Z","timestamp":1642580457000},"page":"490","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":34,"title":["Aetiology of Primary Spontaneous Pneumothorax"],"prefix":"10.3390","volume":"11","author":[{"given":"Rob","family":"Hallifax","sequence":"first","affiliation":[{"name":"Respiratory Trials Unit, Oxford Centre for Respiratory Medicine, Churchill Hospital, NHS Trust, Oxford OX3 7LJ, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,1,19]]},"reference":[{"key":"ref_1","first-page":"152","article-title":"Were Pneumothorax and Its Management Known in 15th-Century Anatolia?","volume":"36","author":"Kaya","year":"2009","journal-title":"Tex. Heart Inst. J."},{"key":"ref_2","unstructured":"Laennec, R., Laennec, M., and Andral, G. (1937). Trait\u00e9 de L\u2019auscultation M\u00e9diate, et des Maladies des Poumons et du Coeur, Soci\u00e9t\u00e9 Typographique Belge."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1183\/09031936.00219214","article-title":"ERS task force statement: Diagnosis and treatment of primary spontaneous pneumothorax","volume":"46","author":"Tschopp","year":"2015","journal-title":"Eur. Respir. J."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"578","DOI":"10.1016\/S2213-2600(15)00220-9","article-title":"Spontaneous pneumothorax: Time to rethink management?","volume":"3","author":"Bintcliffe","year":"2015","journal-title":"Lancet Respir. Med."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1136\/thx.52.9.805","article-title":"Recurrence of primary spontaneous pneumothorax","volume":"52","author":"Sadikot","year":"1997","journal-title":"Thorax"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"2","DOI":"10.2169\/internalmedicine1962.22.2","article-title":"Physical constitution and smoking habits of patients with idiopathic spontaneous pneumothorax","volume":"22","author":"Nakamura","year":"1983","journal-title":"Jpn. J. Med."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"899","DOI":"10.1378\/chest.116.4.899","article-title":"Physical development of surgically treated patients with primary spontaneous pneumothorax","volume":"116","author":"Fujino","year":"1999","journal-title":"Chest"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"772","DOI":"10.1016\/0002-9610(64)90030-3","article-title":"Spontaneous pneumothorax: Suggested etiology and comparison of treatment methods","volume":"108","author":"Withers","year":"1964","journal-title":"Am. J. Surg."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1378\/chest.103.2.433","article-title":"Nonsmoking, non-alpha 1-antitrypsin deficiency-induced emphysema in nonsmokers with healed spontaneous pneumothorax, identified by computed tomography of the lungs","volume":"103","author":"Bense","year":"1993","journal-title":"Chest"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1800864","DOI":"10.1183\/13993003.00864-2018","article-title":"Recurrence rates in primary spontaneous pneumothorax: A systematic review and meta-analysis","volume":"52","author":"Walker","year":"2018","journal-title":"Eur. Respir. J."},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"Porcel, J.M., and Lee, P. (2021). Thoracoscopy for Spontaneous Pneumothorax. J. Clin. Med., 10.","DOI":"10.3390\/jcm10173835"},{"key":"ref_12","unstructured":"Miller, W.S. (1947). The Pleura (The Lung), Charles C. Thomas."},{"key":"ref_13","unstructured":"Reid, L. (1967). Bullae (The Pathology of Emphysema), Lloyd-Luke Medical Books."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1590\/S1806-37562015000000230","article-title":"Etiology of primary spontaneous pneumothorax","volume":"42","author":"Lyra","year":"2016","journal-title":"J. Bras. de Pneumol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1140","DOI":"10.1378\/chest.07-0029","article-title":"The prevalence of blebs or bullae among young healthy adults: A thoracoscopic investigation","volume":"132","author":"Amjadi","year":"2007","journal-title":"Chest"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"697","DOI":"10.1148\/radiol.2462070712","article-title":"Fleischner Society: Glossary of Terms for Thoracic Imaging","volume":"246","author":"Hansell","year":"2008","journal-title":"Radiology"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"771","DOI":"10.1378\/chest.77.6.771","article-title":"Pathogenesis of spontaneous pneumothorax. With special reference to the ultrastructure of emphysematous bullae","volume":"77","author":"Ohata","year":"1980","journal-title":"Chest"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1136\/thx.26.4.409","article-title":"Spontaneous pneumothorax in young subjects. A clinical and pathological study","volume":"26","author":"Lichter","year":"1971","journal-title":"Thorax"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"702","DOI":"10.1183\/09031936.98.12030702","article-title":"Respiratory bronchiolitis in smokers with spontaneous pneumothorax","volume":"12","author":"Cottin","year":"1998","journal-title":"Eur. Respir. J."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1016\/S0003-4975(00)01411-9","article-title":"Long-term results after video-assisted thoracoscopic surgery for first-time and recurrent spontaneous pneumothorax","volume":"70","author":"Hatz","year":"2000","journal-title":"Ann. Thorac. Surg."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1164\/rccm.200602-259OC","article-title":"Fluorescein-enhanced autofluorescence thoracoscopy in patients with primary spontaneous pneumothorax and normal subjects","volume":"174","author":"Noppen","year":"2006","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"418","DOI":"10.1159\/000366065","article-title":"Overexpression of Matrix Metalloproteinases in Lung Tissue of Patients with Primary Spontaneous Pneumothorax","volume":"88","author":"Chen","year":"2014","journal-title":"Respiration"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"409","DOI":"10.2174\/1389203023380549","article-title":"Matrix metalloproteinases in lung diseases","volume":"3","author":"Ohbayashi","year":"2002","journal-title":"Curr. Protein Pept. Sci."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"17","DOI":"10.2165\/00151829-200403010-00003","article-title":"Matrix metalloproteinases in the pathogenesis of asthma and COPD: Implications for therapy","volume":"3","author":"Suzuki","year":"2004","journal-title":"Treat. Respir. Med."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/j.kjms.2016.10.007","article-title":"Association of MMP-2 and MMP-9 expression with recurrences in primary spontaneous pneumothorax","volume":"33","author":"Huang","year":"2017","journal-title":"Kaohsiung J. Med. Sci."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"496","DOI":"10.1016\/j.kjms.2017.06.001","article-title":"High Nrf2 expression in alveolar type I pneumocytes is associated with low recurrences in primary spontaneous pneumothorax","volume":"33","author":"Chen","year":"2017","journal-title":"Kaohsiung J. Med. Sci."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1002\/ajmg.1320400207","article-title":"On the inheritance of primary spontaneous pneumothorax","volume":"40","author":"Abolnik","year":"1991","journal-title":"Am. J. Med. Genet."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1111\/j.1399-0004.2008.01030.x","article-title":"Mutation analysis of the FLCN gene in Chinese patients with sporadic and familial isolated primary spontaneous pneumothorax","volume":"74","author":"Ren","year":"2008","journal-title":"Clin. Genet."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"476","DOI":"10.1136\/jmg.2009.072785","article-title":"The revised Ghent nosology for the Marfan syndrome","volume":"47","author":"Loeys","year":"2010","journal-title":"J. Med. Genet."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1016\/S1535-6108(02)00104-6","article-title":"Mutations in a novel gene lead to kidney tumors, lung wall defects, and benign tumors of the hair follicle in patients with the Birt-Hogg-Dube syndrome","volume":"2","author":"Nickerson","year":"2002","journal-title":"Cancer Cell"},{"key":"ref_31","doi-asserted-by":"crossref","unstructured":"The Alpha-1-Antitrypsin Deficiency Registry Study Group (1998). Survival and FEV1Decline in Individuals with Severe Deficiency of \u03b11-Antitrypsin. Am. J. Respir. Crit. Care Med., 158, 49\u201359.","DOI":"10.1164\/ajrccm.158.1.9712017"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"831","DOI":"10.1023\/A:1005384121649","article-title":"Spontaneous pneumothorax in association with pyridoxine-responsive homocystinuria","volume":"20","author":"Bass","year":"1997","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"270","DOI":"10.1136\/thoraxjnl-2017-211169","article-title":"Familial pneumothorax: Towards precision medicine","volume":"73","author":"Scott","year":"2017","journal-title":"Thorax"}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/11\/3\/490\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T22:03:44Z","timestamp":1760133824000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/11\/3\/490"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,1,19]]},"references-count":33,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2022,2]]}},"alternative-id":["jcm11030490"],"URL":"https:\/\/doi.org\/10.3390\/jcm11030490","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,1,19]]}}}