{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T15:33:55Z","timestamp":1792596835718,"version":"4.5.0"},"reference-count":149,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2024,5,12]],"date-time":"2024-05-12T00:00:00Z","timestamp":1715472000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Pulmonary manifestations of vasculitis are associated with significant morbidity and mortality in affected individuals. They result from a complex interplay between immune dysregulation, which leads to vascular inflammation and tissue damage. This review explored the underlying pathogenesis of pulmonary involvement in vasculitis, encompassing various forms such as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and anti-GBM disease. Mechanisms involving ANCA and anti-GBM autoantibodies, neutrophil activation, and neutrophil extracellular trap (NETs) formation are discussed, along with the role of the complement system in inducing pulmonary injury. Furthermore, the impact of genetic predisposition and environmental factors on disease susceptibility and severity was considered, and the current treatment options were presented. Understanding the mechanisms involved in the pathogenesis of pulmonary vasculitis is crucial for developing targeted therapies and improving clinical outcomes in affected individuals.<\/jats:p>","DOI":"10.3390\/ijms25105278","type":"journal-article","created":{"date-parts":[[2024,5,14]],"date-time":"2024-05-14T08:03:08Z","timestamp":1715673788000},"page":"5278","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":21,"title":["Pathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome"],"prefix":"10.3390","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4456-8987","authenticated-orcid":false,"given":"Evangelia","family":"Fouka","sequence":"first","affiliation":[{"name":"Department of Respiratory Medicine, General Hospital G. Papanikolaou, Medical School, Aristotle University of Thessaloniki, 57010 Thessaloniki, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4408-291X","authenticated-orcid":false,"given":"Fotios","family":"Drakopanagiotakis","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Medical School, Democritus University of Thrace, 68100 Alexandroupolis, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7121-6253","authenticated-orcid":false,"given":"Paschalis","family":"Steiropoulos","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Medical School, Democritus University of Thrace, 68100 Alexandroupolis, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,5,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1325","DOI":"10.1136\/bmj.320.7245.1325","article-title":"ABC of arterial and vascular disease: Vasculitis","volume":"320","author":"Savage","year":"2000","journal-title":"BMJ"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1016\/j.berh.2018.10.003","article-title":"Introduction, epidemiology and classification of vasculitis","volume":"32","author":"Watts","year":"2018","journal-title":"Best Pract. 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