{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T23:17:14Z","timestamp":1793143034267,"version":"4.5.0"},"reference-count":68,"publisher":"Springer Science and Business Media LLC","issue":"10","license":[{"start":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T00:00:00Z","timestamp":1756252800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0"},{"start":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T00:00:00Z","timestamp":1756252800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0"}],"funder":[{"DOI":"10.13039\/501100018950","name":"Centre Hospitalier Universitaire de Rennes","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100018950","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Drugs"],"published-print":{"date-parts":[[2025,10]]},"DOI":"10.1007\/s40265-025-02228-3","type":"journal-article","created":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T17:43:00Z","timestamp":1756316580000},"page":"1193-1206","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Pharmacotherapy for Autoimmune Pulmonary Alveolar Proteinosis"],"prefix":"10.1007","volume":"85","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1949-3461","authenticated-orcid":false,"given":"St\u00e9phane","family":"Jouneau","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre","family":"Chauvin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mathieu","family":"Lederlin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beno\u00eet","family":"Painvin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mallorie","family":"Kerjouan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,8,27]]},"reference":[{"issue":"8","key":"2228_CR1","doi-asserted-by":"crossref","first-page":"816","DOI":"10.1111\/resp.13831","volume":"25","author":"S Jouneau","year":"2020","unstructured":"Jouneau S, Menard C, Lederlin M. Pulmonary alveolar proteinosis. Respirology. 2020;25(8):816\u201326.","journal-title":"Respirology"},{"issue":"1","key":"2228_CR2","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1038\/s41572-019-0066-3","volume":"5","author":"BC Trapnell","year":"2019","unstructured":"Trapnell BC, Nakata K, Bonella F, Campo I, Griese M, Hamilton J, et al. Pulmonary alveolar proteinosis. Nat Rev Dis Primers. 2019;5(1):16.","journal-title":"Nat Rev Dis Primers"},{"issue":"7","key":"2228_CR3","doi-asserted-by":"crossref","first-page":"752","DOI":"10.1164\/rccm.200708-1271OC","volume":"177","author":"Y Inoue","year":"2008","unstructured":"Inoue Y, Trapnell BC, Tazawa R, Arai T, Takada T, Hizawa N, et al. Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan. Am J Respir Crit Care Med. 2008;177(7):752\u201362.","journal-title":"Am J Respir Crit Care Med"},{"issue":"1","key":"2228_CR4","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1186\/s13023-018-0846-y","volume":"13","author":"C McCarthy","year":"2018","unstructured":"McCarthy C, Avetisyan R, Carey BC, Chalk C, Trapnell BC. Prevalence and healthcare burden of pulmonary alveolar proteinosis. Orphanet J Rare Dis. 2018;13(1):129.","journal-title":"Orphanet J Rare Dis"},{"issue":"5","key":"2228_CR5","doi-asserted-by":"crossref","first-page":"2400725","DOI":"10.1183\/13993003.00725-2024","volume":"64","author":"C McCarthy","year":"2024","unstructured":"McCarthy C, Bonella F, O\u2019Callaghan M, Dupin C, Alfaro T, Fally M, et al. European Respiratory Society guidelines for the diagnosis and management of pulmonary alveolar proteinosis. Eur Respir J. 2024;64(5):2400725.","journal-title":"Eur Respir J"},{"key":"2228_CR6","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1146\/annurev.med.60.041807.123500","volume":"61","author":"JA Whitsett","year":"2010","unstructured":"Whitsett JA, Wert SE, Weaver TE. Alveolar surfactant homeostasis and the pathogenesis of pulmonary disease. Annu Rev Med. 2010;61:105\u201319.","journal-title":"Annu Rev Med"},{"issue":"5","key":"2228_CR7","doi-asserted-by":"crossref","first-page":"L1132","DOI":"10.1152\/ajplung.00216.2003","volume":"285","author":"TL Bonfield","year":"2003","unstructured":"Bonfield TL, Raychaudhuri B, Malur A, Abraham S, Trapnell BC, Kavuru MS, et al. PU.1 regulation of human alveolar macrophage differentiation requires granulocyte-macrophage colony-stimulating factor. Am J Physiol Lung Cell Mol Physiol. 2003;285(5):L1132\u20136.","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"issue":"4","key":"2228_CR8","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1165\/rcmb.2002-0023OC","volume":"27","author":"TL Bonfield","year":"2002","unstructured":"Bonfield TL, Russell D, Burgess S, Malur A, Kavuru MS, Thomassen MJ. Autoantibodies against granulocyte macrophage colony-stimulating factor are diagnostic for pulmonary alveolar proteinosis. Am J Respir Cell Mol Biol. 2002;27(4):481\u20136.","journal-title":"Am J Respir Cell Mol Biol"},{"issue":"4","key":"2228_CR9","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1016\/S1074-7613(01)00218-7","volume":"15","author":"Y Shibata","year":"2001","unstructured":"Shibata Y, Berclaz PY, Chroneos ZC, Yoshida M, Whitsett JA, Trapnell BC. GM-CSF regulates alveolar macrophage differentiation and innate immunity in the lung through PU.1. Immunity. 2001;15(4):557\u201367.","journal-title":"Immunity"},{"issue":"12","key":"2228_CR10","doi-asserted-by":"crossref","first-page":"5592","DOI":"10.1073\/pnas.91.12.5592","volume":"91","author":"E Stanley","year":"1994","unstructured":"Stanley E, Lieschke GJ, Grail D, Metcalf D, Hodgson G, Gall JA, et al. Granulocyte\/macrophage colony-stimulating factor-deficient mice show no major perturbation of hematopoiesis but develop a characteristic pulmonary pathology. Proc Natl Acad Sci U S A. 1994;91(12):5592\u20136.","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"2","key":"2228_CR11","doi-asserted-by":"crossref","DOI":"10.1148\/radiol.232558","volume":"310","author":"AA Bankier","year":"2024","unstructured":"Bankier AA, MacMahon H, Colby T, Gevenois PA, Goo JM, Leung ANC, et al. Fleischner Society: glossary of terms for thoracic imaging. Radiology. 2024;310(2): e232558.","journal-title":"Radiology"},{"issue":"2","key":"2228_CR12","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1164\/rccm.2109105","volume":"166","author":"JF Seymour","year":"2002","unstructured":"Seymour JF, Presneill JJ. Pulmonary alveolar proteinosis: progress in the first 44 years. Am J Respir Crit Care Med. 2002;166(2):215\u201335.","journal-title":"Am J Respir Crit Care Med"},{"issue":"1","key":"2228_CR13","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1186\/s13023-025-03617-3","volume":"20","author":"J Huang","year":"2025","unstructured":"Huang J, Xie S, Gao Y, Lin Z, Xu Z, Lin J, et al. Phenotypic heterogeneity in mortality and prognosis of pulmonary alveolar proteinosis: a large-scale, global pooled analysis of individual-level data. Orphanet J Rare Dis. 2025;20(1):102.","journal-title":"Orphanet J Rare Dis"},{"issue":"2 Pt1","key":"2228_CR14","first-page":"166","volume":"19","author":"E Briens","year":"2002","unstructured":"Briens E, Delaval P, Mairesse MP, Valeyre D, Wallaert B, Lazor R, et al. Pulmonary alveolar proteinosis. Rev Mal Respir. 2002;19(2 Pt1):166\u201382.","journal-title":"Rev Mal Respir"},{"issue":"3","key":"2228_CR15","doi-asserted-by":"crossref","first-page":"544","DOI":"10.2214\/AJR.15.14982","volume":"207","author":"M Akira","year":"2016","unstructured":"Akira M, Inoue Y, Arai T, Sugimoto C, Tokura S, Nakata K, et al. Pulmonary fibrosis on high-resolution CT of patients with pulmonary alveolar proteinosis. AJR Am J Roentgenol. 2016;207(3):544\u201351.","journal-title":"AJR Am J Roentgenol"},{"issue":"7","key":"2228_CR16","doi-asserted-by":"crossref","first-page":"554","DOI":"10.1016\/S2213-2600(18)30043-2","volume":"6","author":"A Kumar","year":"2018","unstructured":"Kumar A, Abdelmalak B, Inoue Y, Culver DA. Pulmonary alveolar proteinosis in adults: pathophysiology and clinical approach. Lancet Respir Med. 2018;6(7):554\u201365.","journal-title":"Lancet Respir Med"},{"issue":"10","key":"2228_CR17","doi-asserted-by":"crossref","first-page":"5774","DOI":"10.21037\/jtd.2018.09.86","volume":"10","author":"B Da Nam","year":"2018","unstructured":"Da Nam B, Kim TJ, Chung MP, Chung MJ, Kim TS, Lee KS. CT findings in pulmonary alveolar proteinosis: serial changes and prognostic implications. J Thorac Dis. 2018;10(10):5774\u201383.","journal-title":"J Thorac Dis"},{"issue":"6","key":"2228_CR18","doi-asserted-by":"crossref","first-page":"00314","DOI":"10.1183\/23120541.00314-2024","volume":"10","author":"Y Guirriec","year":"2024","unstructured":"Guirriec Y, Luque-Paz D, Bernard G, Mabo A, Kerjouan M, M\u00e9nard C, et al. Pulmonary fibrosis in patients with auto-immune pulmonary alveolar proteinosis: a retrospective nationwide cohort study. ERJ Open Res. 2024;10(6):00314\u20132024.","journal-title":"ERJ Open Res."},{"issue":"2","key":"2228_CR19","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1016\/j.jinf.2012.03.020","volume":"65","author":"AD Punatar","year":"2012","unstructured":"Punatar AD, Kusne S, Blair JE, Seville MT, Vikram HR. Opportunistic infections in patients with pulmonary alveolar proteinosis. J Infect. 2012;65(2):173\u20139.","journal-title":"J Infect"},{"issue":"1","key":"2228_CR20","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1136\/thorax-2023-220040","volume":"79","author":"A Mabo","year":"2023","unstructured":"Mabo A, Borie R, Wemeau-Stervinou L, Uzunhan Y, Gomez E, Prevot G, et al. Infections in autoimmune pulmonary alveolar proteinosis: a large retrospective cohort. Thorax. 2023;79(1):68\u201374.","journal-title":"Thorax"},{"issue":"12","key":"2228_CR21","doi-asserted-by":"crossref","first-page":"1908","DOI":"10.1016\/j.rmed.2011.08.018","volume":"105","author":"F Bonella","year":"2011","unstructured":"Bonella F, Bauer PC, Griese M, Ohshimo S, Guzman J, Costabel U. Pulmonary alveolar proteinosis: new insights from a single-center cohort of 70 patients. Respir Med. 2011;105(12):1908\u201316.","journal-title":"Respir Med"},{"key":"2228_CR22","doi-asserted-by":"crossref","first-page":"292","DOI":"10.7326\/0003-4819-62-2-292","volume":"62","author":"RK Larson","year":"1965","unstructured":"Larson RK, Gordinier R. Pulmonary alveolar proteinosis. Report of six cases, review of the literature, and formulation of a new theory. Ann Intern Med. 1965;62:292\u2013312.","journal-title":"Ann Intern Med"},{"issue":"1","key":"2228_CR23","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1016\/j.chest.2016.04.030","volume":"150","author":"I Campo","year":"2016","unstructured":"Campo I, Luisetti M, Griese M, Trapnell BC, Bonella F, Grutters JC, et al. A global survey on whole lung lavage in pulmonary alveolar proteinosis. Chest. 2016;150(1):251\u20133.","journal-title":"Chest"},{"issue":"3","key":"2228_CR24","doi-asserted-by":"crossref","first-page":"198","DOI":"10.1159\/000455179","volume":"93","author":"P Gay","year":"2017","unstructured":"Gay P, Wallaert B, Nowak S, Yserbyt J, Anevlavis S, Hermant C, et al. Efficacy of whole-lung lavage in pulmonary alveolar proteinosis: a multicenter international study of GELF. Respiration. 2017;93(3):198\u2013206.","journal-title":"Respiration"},{"issue":"1","key":"2228_CR25","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1136\/thorax.55.1.67","volume":"55","author":"PL Shah","year":"2000","unstructured":"Shah PL, Hansell D, Lawson PR, Reid KB, Morgan C. Pulmonary alveolar proteinosis: clinical aspects and current concepts on pathogenesis. Thorax. 2000;55(1):67\u201377.","journal-title":"Thorax"},{"issue":"120","key":"2228_CR26","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1183\/09059180.00001311","volume":"20","author":"R Borie","year":"2011","unstructured":"Borie R, Danel C, Debray MP, Taille C, Dombret MC, Aubier M, et al. Pulmonary alveolar proteinosis. Eur Respir Rev. 2011;20(120):98\u2013107.","journal-title":"Eur Respir Rev"},{"issue":"4 Pt 1","key":"2228_CR27","doi-asserted-by":"crossref","first-page":"1143","DOI":"10.1164\/ajrccm.161.4.9906044","volume":"161","author":"MS Kavuru","year":"2000","unstructured":"Kavuru MS, Sullivan EJ, Piccin R, Thomassen MJ, Stoller JK. Exogenous granulocyte-macrophage colony-stimulating factor administration for pulmonary alveolar proteinosis. Am J Respir Crit Care Med. 2000;161(4 Pt 1):1143\u20138.","journal-title":"Am J Respir Crit Care Med"},{"issue":"2","key":"2228_CR28","doi-asserted-by":"crossref","first-page":"524","DOI":"10.1164\/ajrccm.163.2.2003146","volume":"163","author":"JF Seymour","year":"2001","unstructured":"Seymour JF, Presneill JJ, Schoch OD, Downie GH, Moore PE, Doyle IR, et al. Therapeutic efficacy of granulocyte-macrophage colony-stimulating factor in patients with idiopathic acquired alveolar proteinosis. Am J Respir Crit Care Med. 2001;163(2):524\u201331.","journal-title":"Am J Respir Crit Care Med"},{"issue":"1","key":"2228_CR29","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1378\/chest.130.1.227","volume":"130","author":"SB Venkateshiah","year":"2006","unstructured":"Venkateshiah SB, Yan TD, Bonfield TL, Thomassen MJ, Meziane M, Czich C, et al. An open-label trial of granulocyte macrophage colony stimulating factor therapy for moderate symptomatic pulmonary alveolar proteinosis. Chest. 2006;130(1):227\u201337.","journal-title":"Chest"},{"issue":"5","key":"2228_CR30","doi-asserted-by":"crossref","first-page":"1273","DOI":"10.1378\/chest.11-0951","volume":"141","author":"A Khan","year":"2012","unstructured":"Khan A, Agarwal R, Aggarwal AN. Effectiveness of granulocyte-macrophage colony-stimulating factor therapy in autoimmune pulmonary alveolar proteinosis: a meta-analysis of observational studies. Chest. 2012;141(5):1273\u201383.","journal-title":"Chest"},{"issue":"3","key":"2228_CR31","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1183\/09031936.06.00058305","volume":"27","author":"ME Wylam","year":"2006","unstructured":"Wylam ME, Ten R, Prakash UB, Nadrous HF, Clawson ML, Anderson PM. Aerosol granulocyte-macrophage colony-stimulating factor for pulmonary alveolar proteinosis. Eur Respir J. 2006;27(3):585\u201393.","journal-title":"Eur Respir J"},{"issue":"1","key":"2228_CR32","doi-asserted-by":"crossref","first-page":"2301233","DOI":"10.1183\/13993003.01233-2023","volume":"63","author":"I Campo","year":"2024","unstructured":"Campo I, Carey BC, Paracchini E, Kadija Z, De Silvestri A, Rodi G, et al. Inhaled recombinant GM-CSF reduces the need for whole lung lavage and improves gas exchange in autoimmune pulmonary alveolar proteinosis patients. Eur Respir J. 2024;63(1):2301233.","journal-title":"Eur Respir J"},{"issue":"4","key":"2228_CR33","doi-asserted-by":"crossref","first-page":"729","DOI":"10.1378\/chest.13-0603","volume":"145","author":"R Tazawa","year":"2014","unstructured":"Tazawa R, Inoue Y, Arai T, Takada T, Kasahara Y, Hojo M, et al. Duration of benefit in patients with autoimmune pulmonary alveolar proteinosis after inhaled granulocyte-macrophage colony-stimulating factor therapy. Chest. 2014;145(4):729\u201337.","journal-title":"Chest"},{"issue":"10","key":"2228_CR34","doi-asserted-by":"crossref","first-page":"923","DOI":"10.1056\/NEJMoa1816216","volume":"381","author":"R Tazawa","year":"2019","unstructured":"Tazawa R, Ueda T, Abe M, Tatsumi K, Eda R, Kondoh S, et al. Inhaled GM-CSF for pulmonary alveolar proteinosis. N Engl J Med. 2019;381(10):923\u201332.","journal-title":"N Engl J Med"},{"issue":"1","key":"2228_CR35","doi-asserted-by":"crossref","first-page":"174","DOI":"10.1186\/s13023-020-01450-4","volume":"15","author":"X Tian","year":"2020","unstructured":"Tian X, Yang Y, Chen L, Sui X, Xu W, Li X, et al. Inhaled granulocyte-macrophage colony stimulating factor for mild-to-moderate autoimmune pulmonary alveolar proteinosis: a six month phase II randomized study with 24 months of follow-up. Orphanet J Rare Dis. 2020;15(1):174.","journal-title":"Orphanet J Rare Dis"},{"key":"2228_CR36","doi-asserted-by":"publisher","unstructured":"Trapnell BC, Inoue Y, Bonella F, Wang T, McCarthy C, Arai T, et al. Phase 3 trial of inhaled molgramostim in autoimmune pulmonary alveolar proteinosis. N Engl J Med. 2025;393(8):764\u201373. https:\/\/doi.org\/10.1056\/NEJMoa2410542","DOI":"10.1056\/NEJMoa2410542"},{"issue":"17","key":"2228_CR37","doi-asserted-by":"crossref","first-page":"1635","DOI":"10.1056\/NEJMoa1913590","volume":"383","author":"BC Trapnell","year":"2020","unstructured":"Trapnell BC, Inoue Y, Bonella F, Morgan C, Jouneau S, Bendstrup E, et al. Inhaled molgramostim therapy in autoimmune pulmonary alveolar proteinosis. N Engl J Med. 2020;383(17):1635\u201344.","journal-title":"N Engl J Med"},{"issue":"170","key":"2228_CR38","doi-asserted-by":"crossref","DOI":"10.1183\/16000617.0080-2023","volume":"32","author":"M Munsif","year":"2023","unstructured":"Munsif M, Sweeney D, Leong TL, Stirling RG. Nebulised granulocyte-macrophage colony-stimulating factor (GM-CSF) in autoimmune pulmonary alveolar proteinosis: a systematic review and meta-analysis. Eur Respir Rev. 2023;32(170): 230080.","journal-title":"Eur Respir Rev"},{"issue":"6","key":"2228_CR39","doi-asserted-by":"crossref","first-page":"1361","DOI":"10.1183\/09031936.00197710","volume":"38","author":"MS Kavuru","year":"2011","unstructured":"Kavuru MS, Malur A, Marshall I, Barna BP, Meziane M, Huizar I, et al. An open-label trial of rituximab therapy in pulmonary alveolar proteinosis. Eur Respir J. 2011;38(6):1361\u20137.","journal-title":"Eur Respir J"},{"issue":"1","key":"2228_CR40","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1186\/s12931-018-0780-5","volume":"19","author":"B Soyez","year":"2018","unstructured":"Soyez B, Borie R, Menard C, Cadranel J, Chavez L, Cottin V, et al. Rituximab for auto-immune alveolar proteinosis, a real life cohort study. Respir Res. 2018;19(1):74.","journal-title":"Respir Res"},{"key":"2228_CR41","first-page":"CD007356","volume":"1","author":"MA Lopez-Olivo","year":"2015","unstructured":"Lopez-Olivo MA, Amezaga Urruela M, McGahan L, Pollono EN, Suarez-Almazor ME. Rituximab for rheumatoid arthritis. Cochrane Database Syst Rev. 2015;1:CD007356.","journal-title":"Cochrane Database Syst Rev"},{"issue":"1","key":"2228_CR42","doi-asserted-by":"crossref","DOI":"10.1183\/23120541.00701-2021","volume":"8","author":"M Griese","year":"2022","unstructured":"Griese M, Panagiotou P, Manali ED, Stahl M, Schwerk N, Costa V, et al. Autoimmune pulmonary alveolar proteinosis in children. ERJ Open Res. 2022;8(1): 00701\u20132021.","journal-title":"ERJ Open Res"},{"issue":"3","key":"2228_CR43","doi-asserted-by":"crossref","first-page":"342","DOI":"10.1006\/clim.2002.5301","volume":"105","author":"TL Bonfield","year":"2002","unstructured":"Bonfield TL, Kavuru MS, Thomassen MJ. Anti-GM-CSF titer predicts response to GM-CSF therapy in pulmonary alveolar proteinosis. Clin Immunol. 2002;105(3):342\u201350.","journal-title":"Clin Immunol"},{"issue":"2","key":"2228_CR44","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1007\/s00408-014-9678-2","volume":"193","author":"B Garber","year":"2015","unstructured":"Garber B, Albores J, Wang T, Neville TH. A plasmapheresis protocol for refractory pulmonary alveolar proteinosis. Lung. 2015;193(2):209\u201311.","journal-title":"Lung"},{"issue":"3","key":"2228_CR45","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1016\/j.rmr.2016.06.002","volume":"34","author":"A Jezequel","year":"2017","unstructured":"Jezequel A, Kerjouan M, Lederlin M, Laine-Caroff C, Camus C, Delaval P, et al. Plasmapheresis failure in the treatment of auto-immune pulmonary alveolar proteinosis. Rev Mal Respir. 2017;34(3):240\u20133.","journal-title":"Rev Mal Respir"},{"issue":"7","key":"2228_CR46","doi-asserted-by":"crossref","first-page":"1036","DOI":"10.1164\/ajrccm.167.7.950","volume":"167","author":"MS Kavuru","year":"2003","unstructured":"Kavuru MS, Bonfield TL, Thomassen MJ. Plasmapheresis, GM-CSF, and alveolar proteinosis. Am J Respir Crit Care Med. 2003;167(7):1036 (author reply 1037).","journal-title":"Am J Respir Crit Care Med"},{"key":"2228_CR47","doi-asserted-by":"crossref","first-page":"2104270","DOI":"10.1155\/2022\/2104270","volume":"2022","author":"A Keske","year":"2022","unstructured":"Keske A, Destrampe EM, Barksdale B, Rose WN. Pulmonary alveolar proteinosis refractory to plasmapheresis and rituximab despite GM-CSF antibody reduction. Case Reports Immunol. 2022;2022:2104270.","journal-title":"Case Reports Immunol."},{"issue":"5","key":"2228_CR48","doi-asserted-by":"crossref","first-page":"1220","DOI":"10.1183\/09031936.00097508","volume":"33","author":"M Luisetti","year":"2009","unstructured":"Luisetti M, Rodi G, Perotti C, Campo I, Mariani F, Pozzi E, et al. Plasmapheresis for treatment of pulmonary alveolar proteinosis. Eur Respir J. 2009;33(5):1220\u20132.","journal-title":"Eur Respir J"},{"key":"2228_CR49","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1186\/1471-2466-14-87","volume":"14","author":"H Yu","year":"2014","unstructured":"Yu H, Sun X, Wang Y, Xu Z, Huang H. Whole lung lavage combined with granulocyte-macrophage colony stimulating factor inhalation for an adult case of refractory pulmonary alveolar proteinosis. BMC Pulm Med. 2014;14:87.","journal-title":"BMC Pulm Med"},{"issue":"3","key":"2228_CR50","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1097\/LBR.0000000000000686","volume":"27","author":"DC Vis","year":"2020","unstructured":"Vis DC, Kelly MM, De Heuvel E, MacEachern PR. Reduction in alveolar macrophage size in refractory autoimmune pulmonary alveolar proteinosis after treatment with pioglitazone. J Bronchol Interv Pulmonol. 2020;27(3):219\u201322.","journal-title":"J Bronchol Interv Pulmonol"},{"issue":"1","key":"2228_CR51","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1002\/jca.21950","volume":"37","author":"V Coirier","year":"2022","unstructured":"Coirier V, Lesouhaitier M, Reizine F, Painvin B, Quelven Q, Maamar A, et al. Tolerance and complications of therapeutic plasma exchange by centrifugation: a single center experience. J Clin Apher. 2022;37(1):54\u201364.","journal-title":"J Clin Apher"},{"issue":"3","key":"2228_CR52","doi-asserted-by":"crossref","first-page":"342","DOI":"10.1111\/bjh.12629","volume":"164","author":"HM Reeves","year":"2014","unstructured":"Reeves HM, Winters JL. The mechanisms of action of plasma exchange. Br J Haematol. 2014;164(3):342\u201351.","journal-title":"Br J Haematol"},{"issue":"11","key":"2228_CR53","doi-asserted-by":"crossref","first-page":"1349","DOI":"10.1016\/j.healun.2021.07.005","volume":"40","author":"LE Leard","year":"2021","unstructured":"Leard LE, Holm AM, Valapour M, Glanville AR, Attawar S, Aversa M, et al. Consensus document for the selection of lung transplant candidates: an update from the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. 2021;40(11):1349\u201379.","journal-title":"J Heart Lung Transplant"},{"issue":"10","key":"2228_CR54","doi-asserted-by":"crossref","first-page":"855","DOI":"10.1016\/j.rmr.2022.10.005","volume":"39","author":"J Le Pavec","year":"2022","unstructured":"Le Pavec J, Pison C, Hirschi S, Bunel V, Mordant P, Brugiere O, et al. Updated indications and contraindications in 2022 for lung transplantation in France. Rev Mal Respir. 2022;39(10):855\u201372.","journal-title":"Rev Mal Respir"},{"key":"2228_CR55","volume":"83","author":"J Le Pavec","year":"2023","unstructured":"Le Pavec J, Pison C, Hirschi S, Bunel V, Mordant P, Brugiere O, et al. 2022 update of indications and contraindications for lung transplantation in France. Respir Med Res. 2023;83: 100981.","journal-title":"Respir Med Res"},{"issue":"7","key":"2228_CR56","first-page":"667","volume":"45","author":"YB Wang","year":"2022","unstructured":"Wang YB, Li FK, Ding ZD, Zhao K, Fang ZM, Feng M, et al. Lung transplantation for pulmonary alveolar proteinosis: a case report and literature review. Zhonghua Jie He He Hu Xi Za Zhi. 2022;45(7):667\u201370.","journal-title":"Zhonghua Jie He He Hu Xi Za Zhi"},{"key":"2228_CR57","volume":"74","author":"J Liang","year":"2022","unstructured":"Liang J, Chen Y, Zheng M, Ye S, Liu F, Chen J, et al. Single lung transplantation for idiopathic pulmonary alveolar proteinosis with intraoperative veno-venous extracorporeal membrane oxygenation support. Transpl Immunol. 2022;74: 101627.","journal-title":"Transpl Immunol"},{"issue":"1","key":"2228_CR58","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1016\/j.resinv.2024.12.001","volume":"63","author":"H Ishimoto","year":"2025","unstructured":"Ishimoto H, Sakamoto N, Yura H, Takazono T, Kido T, Matsumoto K, et al. Successful granulocyte-macrophage colony-stimulating factor inhalation therapy for recurrent autoimmune pulmonary alveolar proteinosis after lung transplantation: a case report. Respir Investig. 2025;63(1):180\u20132.","journal-title":"Respir Investig"},{"issue":"6","key":"2228_CR59","doi-asserted-by":"crossref","DOI":"10.1002\/rcr2.1160","volume":"11","author":"S Kawana","year":"2023","unstructured":"Kawana S, Miyoshi K, Tanaka S, Sugimoto S, Shimizu D, Matsubara K, et al. Pulmonary alveolar proteinosis after lung transplantation: two case reports and literature review. Respirol Case Rep. 2023;11(6): e01160.","journal-title":"Respirol Case Rep"},{"issue":"12","key":"2228_CR60","doi-asserted-by":"crossref","first-page":"1383","DOI":"10.1016\/S0046-8177(97)90228-8","volume":"28","author":"SA Yousem","year":"1997","unstructured":"Yousem SA. Alveolar lipoproteinosis in lung allograft recipients. Hum Pathol. 1997;28(12):1383\u20136.","journal-title":"Hum Pathol"},{"issue":"1","key":"2228_CR61","doi-asserted-by":"crossref","first-page":"3127","DOI":"10.1038\/s41467-018-05491-z","volume":"9","author":"C McCarthy","year":"2018","unstructured":"McCarthy C, Lee E, Bridges JP, Sallese A, Suzuki T, Woods JC, et al. Statin as a novel pharmacotherapy of pulmonary alveolar proteinosis. Nat Commun. 2018;9(1):3127.","journal-title":"Nat Commun"},{"issue":"4","key":"2228_CR62","first-page":"300060521101004","volume":"49","author":"S Shi","year":"2021","unstructured":"Shi S, Wang R, Chen L, Li Y, Zhang Y, Xin X, et al. Long-term follow-up and successful treatment of pulmonary alveolar proteinosis without hypercholesterolemia with statin therapy: a case report. J Int Med Res. 2021;49(4):3000605211010046.","journal-title":"J Int Med Res"},{"key":"2228_CR63","volume":"38","author":"T Takano","year":"2022","unstructured":"Takano T, Takeda K, Nakamura S, Akiyama G, Ando N, Komori M. A case of autoimmune pulmonary alveolar proteinosis with severe respiratory failure treated with segmental lung lavage and oral statin therapy. Respir Med Case Rep. 2022;38: 101684.","journal-title":"Respir Med Case Rep"},{"issue":"10","key":"2228_CR64","doi-asserted-by":"crossref","first-page":"1061","DOI":"10.1016\/j.jfma.2023.04.002","volume":"122","author":"CH Chuang","year":"2023","unstructured":"Chuang CH, Cheng CH, Tsai YC, Tsai MJ, Sheu CC, Chong IW. Pulmonary alveolar proteinosis in Taiwan. J Formos Med Assoc. 2023;122(10):1061\u20138.","journal-title":"J Formos Med Assoc"},{"issue":"1","key":"2228_CR65","volume":"2022","author":"S Shi","year":"2022","unstructured":"Shi S, Gui X, Ding J, Yang S, Xin X, Xu K, et al. Assessment of statin treatment for pulmonary alveolar proteinosis without hypercholesterolemia: a 12-month prospective, longitudinal, and observational study. BioMed Res Int. 2022;2022(1): 2022:1589660.","journal-title":"BioMed Res Int"},{"issue":"1","key":"2228_CR66","doi-asserted-by":"crossref","first-page":"10211","DOI":"10.1038\/s41598-017-10879-w","volume":"7","author":"A Sallese","year":"2017","unstructured":"Sallese A, Suzuki T, McCarthy C, Bridges J, Filuta A, Arumugam P, et al. Targeting cholesterol homeostasis in lung diseases. Sci Rep. 2017;7(1):10211.","journal-title":"Sci Rep"},{"key":"2228_CR67","volume":"78","author":"C Dupin","year":"2020","unstructured":"Dupin C, Hurtado M, Cazes A, Taille C, Debray MP, Guenee C, et al. Pioglitazone in pulmonary alveolar proteinosis: promising first clinical experience. Respir Med Res. 2020;78: 100756.","journal-title":"Respir Med Res"},{"issue":"10","key":"2228_CR68","doi-asserted-by":"crossref","first-page":"975","DOI":"10.1016\/j.rmr.2014.08.009","volume":"31","author":"S Jouneau","year":"2014","unstructured":"Jouneau S, Kerjouan M, Briens E, Lenormand JP, Meunier C, Letheulle J, et al. Pulmonary alveolar proteinosis. Rev Mal Respir. 2014;31(10):975\u201391.","journal-title":"Rev Mal Respir"}],"container-title":["Drugs"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s40265-025-02228-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s40265-025-02228-3\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s40265-025-02228-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,30]],"date-time":"2025-09-30T14:19:51Z","timestamp":1759241991000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s40265-025-02228-3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,8,27]]},"references-count":68,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2025,10]]}},"alternative-id":["2228"],"URL":"https:\/\/doi.org\/10.1007\/s40265-025-02228-3","relation":{},"ISSN":["0012-6667","1179-1950"],"issn-type":[{"value":"0012-6667","type":"print"},{"value":"1179-1950","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,8,27]]},"assertion":[{"value":"9 January 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"12 August 2025","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"27 August 2025","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Over the past 5 years, St\u00e9phane Jouneau declares conflicts of interest in relation to the subject of this article as he was a primary investigator in IMPALA trials and a co-author of the international guidelines on PAP management, and outside of patients with aPAP, St\u00e9phane Jouneau has declared links with the following pharmaceutical companies (or similar): clinical research: AIRB, Astra Zeneca, Biogen, BMS, Boehringer Ingelheim, Galacto, GSK, Pliant Therapeutics, Roche, and United Therapeutics; advisory boards, consultancy: AIRB, Boehringer Ingelheim, GSK, and Sanofi; courses, training: AIRB, Astra Zeneca, BMS, Boehringer Ingelheim, Chiesi, GSK, LVL, Novartis, Pfizer, Roche, and Sanofi; and research grants: AIRB and Boehringer Ingelheim. Over the past 5 years, Pierre Chauvin reports no conflicts of interest related to the subject of this article. However, he declares affiliations with the following pharmaceutical companies (or related entities): Boehringer Ingelheim, GSK, MSD, Pfizer, Takeda France, Fujifilm France, and Novatech SA. Over the past 5 years, Mathieu Lederlin reports no conflicts of interest related to the subject of this article. However, he declares affiliations with the following pharmaceutical companies (or related entities): Boehringer Ingelheim, Sanofi, Sophia Genetics, and AstraZeneca. Over the past 5 years, Beno\u00eet Painvin reports no conflicts of interest related to the subject of this article or outside of it. Over the past 5 years, Mallorie Kerjouan reports no conflicts of interest related to the subject of this article. However, she declares affiliations with the following pharmaceutical companies (or related entities): Boehringer Ingelheim, Grifols, CSL Behring, LFB, SOS, and Oxyg\u00e8ne.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflicts of Interest\/Competing Interests"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics Approval"}},{"value":"Not applicable.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent to Participate"}},{"value":"Not applicable.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for Publication"}},{"value":"Not applicable.","order":6,"name":"Ethics","group":{"name":"EthicsHeading","label":"Availability of Data and Material"}},{"value":"Not applicable.","order":7,"name":"Ethics","group":{"name":"EthicsHeading","label":"Code Availability"}},{"value":"SJ has written the first draft of the manuscript, all authors have read and amended the manuscript. All authors have read and approved the final version of the manuscript, and agree to be accountable for the work.","order":8,"name":"Ethics","group":{"name":"EthicsHeading","label":"Authors\u2019 Contributions"}}]}}