{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,14]],"date-time":"2026-08-14T19:19:44Z","timestamp":1786735184266,"version":"build-2736575974"},"reference-count":113,"publisher":"Georg Thieme Verlag KG","issue":"06","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Semin Respir Crit Care Med"],"published-print":{"date-parts":[[2025,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>In sarcoidosis, pulmonary manifestations are almost universal; however, their severity ranges from asymptomatic to respiratory failure and death. Approximately 20% of patients progress to advanced pulmonary sarcoidosis (APS), a disease phenotype that is driven mostly by pulmonary fibrosis and associated complications, including bronchiectasis, chronic pulmonary aspergillosis, and pulmonary hypertension, which may result from multiple mechanisms. APS may be burnt out but is often active as confirmed by 18F-fluorodeoxyglucose-PET. APS is a major cause of morbidity and mortality in patients with sarcoidosis, but its natural history is largely unknown and evidence-based treatment guidelines are lacking. Here, we review the major patterns of APS with emphasis on clinical manifestations, pathophysiology, and management, although this is based on expert opinion and may include, based on the predominant disease phenotype, anti-inflammatory\/immunosuppressive treatment, antifibrotics, pulmonary rehabilitation, antibiotics, vasodilators, and, in highly selected patients, lung transplantation.<\/jats:p>","DOI":"10.1055\/a-2768-2520","type":"journal-article","created":{"date-parts":[[2025,12,12]],"date-time":"2025-12-12T00:12:25Z","timestamp":1765498345000},"page":"564-575","source":"Crossref","is-referenced-by-count":5,"title":["Advanced Pulmonary Sarcoidosis"],"prefix":"10.1055","volume":"46","author":[{"given":"Paolo","family":"Spagnolo","sequence":"additional","affiliation":[{"name":"Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Respiratory Disease Unit, University of Padova, Padova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zehra","family":"Dhanani","sequence":"additional","affiliation":[{"name":"Department of Thoracic Medicine and Surgery, Temple University Hospital, Philadelphia, Pennsylvania, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Cameli","sequence":"additional","affiliation":[{"name":"Respiratory Diseases Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Siena, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giordano","family":"Fiorent\u00f9","sequence":"additional","affiliation":[{"name":"Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Respiratory Disease Unit, University of Padova, Padova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rohit","family":"Gupta","sequence":"additional","affiliation":[{"name":"Deaprtment of Thoracic Medicine and Surgery, Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2026,1,5]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"103247","DOI":"10.1016\/j.jaut.2024.103247","article-title":"The immunopathogenesis of sarcoidosis","volume":"149","author":"J Miedema","year":"2024","journal-title":"J Autoimmun"},{"issue":"02","key":"ref2","doi-asserted-by":"crossref","first-page":"438","DOI":"10.1378\/chest.14-1120","article-title":"Racial difference in sarcoidosis mortality in the United States","volume":"147","author":"M Mirsaeidi","year":"2015","journal-title":"Chest"},{"issue":"01","key":"ref3","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/j.chest.2017.07.008","article-title":"Predictors of mortality in pulmonary sarcoidosis","volume":"153","author":"G Kirkil","year":"2018","journal-title":"Chest"},{"key":"ref4","first-page":"372","article-title":"Pathological studies on sarcoidosis autopsy. 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