{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T12:11:00Z","timestamp":1790856660043,"version":"4.1.0"},"reference-count":170,"publisher":"Georg Thieme Verlag KG","issue":"04","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Semin Respir Crit Care Med"],"published-print":{"date-parts":[[2026,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease characterized by abnormal accumulation of surfactant in alveoli. Pathogenetically, in aPAP, the presence of granulocyte macrophage-colony stimulating factor (GM-CSF) autoantibodies hinders physiological binding of GM-CSF to its receptor, disrupting terminal differentiation of alveolar macrophages and the activation GM-CSF\u2013PU.1\u2013PPARG1\u2013ABCG1 axis, resulting in a primary reduction in cholesterol efflux from alveolar macrophages and a secondary reduction in surfactant clearance through macrophages from the alveolar surface. APAP is the most common, accounting for more than 90 to 95% of all patients included under the PAP term, which encompasses and classifies all forms of PAP according to etiopathogenetic mechanisms, as primary, secondary, congenital, and unclassified. APAP is worldwide distributed with an estimated prevalence fluctuating between 7.0 and 9.7 cases\/million and an annual incidence of 1.65, affecting middle-aged men and women. Clinical manifestation may be gradual and insidious, mainly manifesting with progressive dyspnea, but the natural history is variable, since some patients stabilize for a long period, while others progress to respiratory failure and death; in a minority, spontaneous resolution may be observed, while some develop lung and\/or systemic infections, and rarely pulmonary fibrosis. Until recently, whole lung lavage (WLL) was universally accepted as the gold-standard therapeutic modality in aPAP. However, after considerable progress in the past 25 years and the publication of several positive studies, replacing the use of inhaled-GM-CSF as the standard of care for aPAP and conceding WLL a rescue option is becoming more and more concrete. In conclusion, aPAP is the classic paradigm of a scientific disease progressing from the \u201cbench-to-bedside,\u201d since several discoveries made in the laboratory setting have become necessary to clarify its pathogenetic mechanisms, define diagnostic tools, and implement new therapeutic modalities, which established the disease as treatable and fully reversible, literally, moving patients from \u201chell to heaven.\u201d<\/jats:p>","DOI":"10.1055\/a-2737-7719","type":"journal-article","created":{"date-parts":[[2025,11,7]],"date-time":"2025-11-07T00:08:48Z","timestamp":1762474128000},"page":"359-372","source":"Crossref","is-referenced-by-count":1,"title":["Autoimmune Pulmonary Alveolar Proteinosis"],"prefix":"10.1055","volume":"47","author":[{"given":"Maurizio","family":"Zompatori","sequence":"first","affiliation":[{"name":"Radiologia, Villa Erbosa, Gruppo San Donato, Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah H.","family":"Forde","sequence":"first","affiliation":[{"name":"School of Medicine, University College Dublin, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Spyridon","family":"Prountzos","sequence":"first","affiliation":[{"name":"2nd Department of Radiology, General University Hospital \u201cAttikon,\u201d Medical School, National and Kapodistrian University of Athens, Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Spyros A.","family":"Papiris","sequence":"additional","affiliation":[{"name":"Athens Medical School, National and Kapodistrian University of Athens, Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Kallieri","sequence":"additional","affiliation":[{"name":"2nd Pulmonary Medicine Department, General University Hospital \u201cAttikon,\u201d Medical School, National and Kapodistrian University of Athens, Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lykourgos","family":"Kolilekas","sequence":"additional","affiliation":[{"name":"7th Pulmonary Department, Athens Chest Hospital \u201cSotiria,\u201d Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andriana I.","family":"Papaioannou","sequence":"additional","affiliation":[{"name":"1st Respiratory Medicine Department, National and Kapodistrian University of Athens, Medical School, Sotiria Chest Diseases Hospital, Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias","family":"Griese","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, Ludwig-Maximilians-Universit\u00e4t Munich, Munich, Germany"},{"name":"German Center for Lung Research (DZL), Comprehensive Pneumology Center Munich, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Effrosyni 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Med"},{"issue":"5159","key":"ref3","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1126\/science.8171324","article-title":"Involvement of granulocyte-macrophage colony-stimulating factor in pulmonary homeostasis","volume":"264","author":"G Dranoff","year":"1994","journal-title":"Science"},{"issue":"12","key":"ref4","doi-asserted-by":"crossref","first-page":"5592","DOI":"10.1073\/pnas.91.12.5592","article-title":"Granulocyte\/macrophage colony-stimulating factor-deficient mice show no major perturbation of hematopoiesis but develop a characteristic pulmonary pathology","volume":"91","author":"E Stanley","year":"1994","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1016\/S0014-5793(98)01668-8","article-title":"Lungs of patients with idiopathic pulmonary alveolar proteinosis express a factor which neutralizes granulocyte-macrophage colony stimulating factor","volume":"442","author":"N Tanaka","year":"1999","journal-title":"FEBS Lett"},{"issue":"06","key":"ref6","doi-asserted-by":"crossref","first-page":"875","DOI":"10.1084\/jem.190.6.875","article-title":"Idiopathic pulmonary alveolar proteinosis as an autoimmune disease with neutralizing antibody against granulocyte\/macrophage colony-stimulating factor","volume":"190","author":"T Kitamura","year":"1999","journal-title":"J Exp Med"},{"issue":"26","key":"ref7","doi-asserted-by":"crossref","first-page":"2527","DOI":"10.1056\/NEJMra023226","article-title":"Pulmonary alveolar proteinosis","volume":"349","author":"B C Trapnell","year":"2003","journal-title":"N Engl J Med"},{"issue":"01","key":"ref8","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1038\/s41572-019-0066-3","article-title":"Pulmonary alveolar proteinosis","volume":"5","author":"B C Trapnell","year":"2019","journal-title":"Nat Rev Dis Primers"},{"issue":"06","key":"ref9","first-page":"428","article-title":"Pulmonary alveolar proteinosis. Review of the literature with follow-up studies and report of two new cases","volume":"104","author":"J E Summers","year":"1966","journal-title":"Calif Med"},{"issue":"01","key":"ref10","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/S0007-0971(69)80040-9","article-title":"Pulmonary alveolar proteinosis","volume":"63","author":"J M Davidson","year":"1969","journal-title":"Br J Dis Chest"},{"issue":"25","key":"ref11","doi-asserted-by":"crossref","first-page":"1924","DOI":"10.1056\/NEJM199612193352513","article-title":"Efficacy of granulocyte-macrophage colony-stimulating factor in acquired alveolar proteinosis","volume":"335","author":"J F Seymour","year":"1996","journal-title":"N Engl J Med"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"1143","DOI":"10.1164\/ajrccm.161.4.9906044","article-title":"Exogenous granulocyte-macrophage colony-stimulating factor administration for pulmonary alveolar proteinosis","volume":"161","author":"M S Kavuru","year":"2000","journal-title":"Am J Respir Crit Care Med"},{"issue":"02","key":"ref13","doi-asserted-by":"crossref","first-page":"524","DOI":"10.1164\/ajrccm.163.2.2003146","article-title":"Therapeutic efficacy of granulocyte-macrophage colony-stimulating factor in patients with idiopathic acquired alveolar proteinosis","volume":"163","author":"J F Seymour","year":"2001","journal-title":"Am J Respir Crit Care Med"},{"issue":"07","key":"ref14","doi-asserted-by":"crossref","first-page":"752","DOI":"10.1164\/rccm.200708-1271OC","article-title":"Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan","volume":"177","author":"Y Inoue","year":"2008","journal-title":"Am J Respir Crit Care Med"},{"issue":"01","key":"ref15","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1186\/s13023-018-0846-y","article-title":"Prevalence and healthcare burden of pulmonary alveolar proteinosis","volume":"13","author":"C McCarthy","year":"2018","journal-title":"Orphanet J Rare Dis"},{"issue":"01","key":"ref16","doi-asserted-by":"crossref","first-page":"00666-2024","DOI":"10.1183\/23120541.00666-2024","article-title":"Epidemiology of pulmonary alveolar proteinosis: a descriptive study using a Japanese national administrative claims database","volume":"11","author":"Y Kimura","year":"2025","journal-title":"ERJ Open Res"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"292","DOI":"10.7326\/0003-4819-62-2-292","article-title":"Pulmonary alveolar proteinosis. report of six cases, review of the literature, and formulation of a new theory","volume":"62","author":"R K Larson","year":"1965","journal-title":"Ann Intern Med"},{"issue":"02","key":"ref18","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1001\/archinte.1974.00320140122013","article-title":"Pulmonary alveolar proteinosis","volume":"133","author":"J B McClenahan","year":"1974","journal-title":"Arch Intern Med"},{"issue":"02","key":"ref19","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1136\/thx.30.2.121","article-title":"Diagnosis and management of alveolar proteinosis: the r\u00f4le of electron microscopy","volume":"30","author":"J F Costello","year":"1975","journal-title":"Thorax"},{"issue":"01","key":"ref20","first-page":"82","article-title":"Pulmonary alveolar proteinosis. Staining for surfactant apoprotein in alveolar proteinosis and in conditions simulating it","volume":"83","author":"G Singh","year":"1983","journal-title":"Chest"},{"issue":"07","key":"ref21","doi-asserted-by":"crossref","first-page":"881","DOI":"10.1164\/rccm.201901-0086OC","article-title":"Quantitative Lipidomics in pulmonary alveolar proteinosis","volume":"200","author":"M Griese","year":"2019","journal-title":"Am J Respir Crit Care Med"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"S183","DOI":"10.1016\/S0091-6749(96)70065-9","article-title":"Roles of JAK kinases in human GM-CSF receptor signal transduction","volume":"98","author":"S Watanabe","year":"1996","journal-title":"J Allergy Clin Immunol"},{"issue":"28","key":"ref23","doi-asserted-by":"crossref","first-page":"17450","DOI":"10.1074\/jbc.272.28.17450","article-title":"The cytoplasmic domain of granulocyte-macrophage colony-stimulating factor (GM-CSF) receptor alpha subunit is essential for both GM-CSF-mediated growth and differentiation","volume":"272","author":"T Matsuguchi","year":"1997","journal-title":"J Biol Chem"},{"issue":"03","key":"ref24","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1016\/S0301-472X(99)00159-9","article-title":"A model for assembly and activation of the GM-CSF, IL-3 and IL-5 receptors: insights from activated mutants of the common beta subunit","volume":"28","author":"R J D'Andrea","year":"2000","journal-title":"Exp Hematol"},{"issue":"03","key":"ref25","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1189\/jlb.71.3.511","article-title":"Granulocyte-macrophage colony-stimulating factor (GM-CSF)-induced STAT5 activation and target-gene expression during human monocyte\/macrophage differentiation","volume":"71","author":"A Lehtonen","year":"2002","journal-title":"J Leukoc Biol"},{"issue":"04","key":"ref26","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1016\/S1074-7613(01)00218-7","article-title":"GM-CSF regulates alveolar macrophage differentiation and innate immunity in the lung through PU.1","volume":"15","author":"Y Shibata","year":"2001","journal-title":"Immunity"},{"issue":"11","key":"ref27","doi-asserted-by":"crossref","first-page":"1026","DOI":"10.1038\/ni.3005","article-title":"Induction of the nuclear receptor PPAR-\u03b3 by the cytokine GM-CSF is critical for the differentiation of fetal monocytes into alveolar macrophages","volume":"15","author":"C Schneider","year":"2014","journal-title":"Nat Immunol"},{"key":"ref28","first-page":"L650","article-title":"Surfactant metabolism in transgenic mice after granulocyte macrophage-colony stimulating factor ablation","volume":"270","author":"M Ikegami","year":"1996","journal-title":"Am J Physiol"},{"issue":"03","key":"ref29","doi-asserted-by":"crossref","first-page":"L379","DOI":"10.1152\/ajplung.2001.280.3.L379","article-title":"GM-CSF regulates protein and lipid catabolism by alveolar macrophages","volume":"280","author":"M Yoshida","year":"2001","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"issue":"44","key":"ref30","doi-asserted-by":"crossref","first-page":"45980","DOI":"10.1074\/jbc.M408652200","article-title":"Expression and regulation of multiple murine ATP-binding cassette transporter G1 mRNAs\/isoforms that stimulate cellular cholesterol efflux to high density lipoprotein","volume":"279","author":"K Nakamura","year":"2004","journal-title":"J Biol Chem"},{"issue":"02","key":"ref31","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/j.cmet.2005.01.002","article-title":"ABCG1 has a critical role in mediating cholesterol efflux to HDL and preventing cellular lipid accumulation","volume":"1","author":"M A Kennedy","year":"2005","journal-title":"Cell Metab"},{"issue":"10","key":"ref32","doi-asserted-by":"crossref","first-page":"2295","DOI":"10.1161\/01.ATV.0000237629.29842.4c","article-title":"Macrophage ABCG1 deletion disrupts lipid homeostasis in alveolar macrophages and moderately influences atherosclerotic lesion development in LDL receptor-deficient mice","volume":"26","author":"R Out","year":"2006","journal-title":"Arterioscler Thromb Vasc Biol"},{"issue":"39","key":"ref33","doi-asserted-by":"crossref","first-page":"29401","DOI":"10.1074\/jbc.M606597200","article-title":"Deletion of the transmembrane transporter ABCG1 results in progressive pulmonary lipidosis","volume":"281","author":"A Bald\u00e1n","year":"2006","journal-title":"J Biol Chem"},{"issue":"05","key":"ref34","doi-asserted-by":"crossref","first-page":"941","DOI":"10.1194\/jlr.M075101","article-title":"ABCG1 regulates pulmonary surfactant metabolism in mice and men","volume":"58","author":"T Q de Aguiar Vallim","year":"2017","journal-title":"J Lipid Res"},{"issue":"01","key":"ref35","doi-asserted-by":"crossref","first-page":"10211","DOI":"10.1038\/s41598-017-10879-w","article-title":"Targeting cholesterol homeostasis in lung diseases","volume":"7","author":"A Sallese","year":"2017","journal-title":"Sci Rep"},{"issue":"09","key":"ref36","doi-asserted-by":"crossref","first-page":"1016","DOI":"10.1164\/rccm.202112-2742SO","article-title":"Autoimmune pulmonary alveolar proteinosis","volume":"205","author":"C McCarthy","year":"2022","journal-title":"Am J Respir Crit Care Med"},{"issue":"03","key":"ref37","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1136\/thorax.58.3.252","article-title":"Relationship of anti-GM-CSF antibody concentration, surfactant protein A and B levels, and serum LDH to pulmonary parameters and response to GM-CSF therapy in patients with idiopathic alveolar proteinosis","volume":"58","author":"J F Seymour","year":"2003","journal-title":"Thorax"},{"issue":"01","key":"ref38","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1164\/rccm.201001-0008OC","article-title":"Patient-derived granulocyte\/macrophage colony-stimulating factor autoantibodies reproduce pulmonary alveolar proteinosis in nonhuman primates","volume":"182","author":"T Sakagami","year":"2010","journal-title":"Am J Respir Crit Care Med"},{"key":"ref39","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1186\/1750-1172-8-40","article-title":"Assessment and management of pulmonary alveolar proteinosis in a reference center","volume":"8","author":"I Campo","year":"2013","journal-title":"Orphanet J Rare Dis"},{"issue":"12","key":"ref40","doi-asserted-by":"crossref","first-page":"1908","DOI":"10.1016\/j.rmed.2011.08.018","article-title":"Pulmonary alveolar proteinosis: new insights from a single-center cohort of 70 patients","volume":"105","author":"F Bonella","year":"2011","journal-title":"Respir Med"},{"issue":"02","key":"ref41","doi-asserted-by":"crossref","first-page":"450","DOI":"10.1016\/j.chest.2018.11.002","article-title":"Blood testing for differential diagnosis of pulmonary alveolar proteinosis syndrome","volume":"155","author":"C McCarthy","year":"2019","journal-title":"Chest"},{"issue":"173","key":"ref42","doi-asserted-by":"crossref","first-page":"240064","DOI":"10.1183\/16000617.0064-2024","article-title":"Pathogenesis-driven treatment of primary pulmonary alveolar proteinosis","volume":"33","author":"S Lettieri","year":"2024","journal-title":"Eur Respir Rev"},{"issue":"07","key":"ref43","doi-asserted-by":"crossref","first-page":"554","DOI":"10.1016\/S2213-2600(18)30043-2","article-title":"Pulmonary alveolar proteinosis in adults: pathophysiology and clinical approach","volume":"6","author":"A Kumar","year":"2018","journal-title":"Lancet Respir Med"},{"issue":"05","key":"ref44","doi-asserted-by":"crossref","first-page":"514","DOI":"10.1055\/s-2007-991525","article-title":"Bronchoalveolar lavage in other interstitial lung diseases","volume":"28","author":"U Costabel","year":"2007","journal-title":"Semin Respir Crit Care Med"},{"issue":"05","key":"ref45","doi-asserted-by":"crossref","first-page":"2400725","DOI":"10.1183\/13993003.00725-2024","article-title":"European respiratory society guidelines for the diagnosis and management of pulmonary alveolar proteinosis","volume":"64","author":"C McCarthy","year":"2024","journal-title":"Eur Respir J"},{"issue":"05","key":"ref46","doi-asserted-by":"crossref","first-page":"464","DOI":"10.1016\/j.pulmoe.2021.01.001","article-title":"Hypersensitivity pneumonitis in a patient with pulmonary alveolar proteinosis","volume":"27","author":"M Costa E Silva","year":"2021","journal-title":"Pulmonology"},{"issue":"03","key":"ref47","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1093\/qjmed\/hcx235","article-title":"Pulmonary alveolar proteinosis","volume":"111","author":"O J McElvaney","year":"2018","journal-title":"QJM"},{"issue":"02","key":"ref48","doi-asserted-by":"crossref","first-page":"400","DOI":"10.1016\/j.chest.2016.09.002","article-title":"Transbronchial cryobiopsy in diffuse parenchymal lung disease: retrospective analysis of 74 cases","volume":"151","author":"K Ussavarungsi","year":"2017","journal-title":"Chest"},{"issue":"05","key":"ref49","first-page":"e60530","article-title":"Cryobiopsy for secondary pulmonary alveolar proteinosis","volume":"16","author":"H Kobe","year":"2024","journal-title":"Cureus"},{"issue":"02","key":"ref50","doi-asserted-by":"crossref","first-page":"240224","DOI":"10.1183\/20734735.0224-2024","article-title":"Summary for clinicians: ERS guidelines on pulmonary alveolar proteinosis","volume":"21","author":"T Alfaro","year":"2025","journal-title":"Breathe (Sheff)"},{"issue":"05","key":"ref51","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1164\/rccm.200912-1800ED","article-title":"Pulmonary alveolar proteinosis associated with dust inhalation: not secondary but autoimmune?","volume":"181","author":"U Costabel","year":"2010","journal-title":"Am J Respir Crit Care Med"},{"issue":"05","key":"ref52","doi-asserted-by":"crossref","first-page":"458","DOI":"10.1164\/rccm.200907-1022CR","article-title":"Pulmonary alveolar proteinosis in workers at an indium processing facility","volume":"181","author":"K J Cummings","year":"2010","journal-title":"Am J Respir Crit Care Med"},{"issue":"04","key":"ref53","doi-asserted-by":"crossref","first-page":"724","DOI":"10.3109\/14397595.2016.1153443","article-title":"Two cases of autoimmune and secondary pulmonary alveolar proteinosis during immunosuppressive therapy in dermatomyositis with interstitial lung disease","volume":"28","author":"Y Imura","year":"2018","journal-title":"Mod Rheumatol"},{"issue":"01","key":"ref54","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1136\/thorax-2023-220040","article-title":"Infections in autoimmune pulmonary alveolar proteinosis: a large retrospective cohort","volume":"79","author":"A Mabo","year":"2023","journal-title":"Thorax"},{"issue":"06","key":"ref55","doi-asserted-by":"crossref","first-page":"ofae269","DOI":"10.1093\/ofid\/ofae269","article-title":"Nocardia infection in patients with anti-granulocyte-macrophage colony-stimulating factor autoantibodies: a prospective multicenter French study","volume":"11","author":"T Kerdiles","year":"2024","journal-title":"Open Forum Infect Dis"},{"issue":"01","key":"ref56","doi-asserted-by":"crossref","first-page":"1032","DOI":"10.1038\/s41467-021-21011-y","article-title":"Genetic determinants of risk in autoimmune pulmonary alveolar proteinosis","volume":"12","author":"S Sakaue","year":"2021","journal-title":"Nat Commun"},{"issue":"158","key":"ref57","doi-asserted-by":"crossref","first-page":"190187","DOI":"10.1183\/16000617.0187-2019","article-title":"Alveolar proteinosis of genetic origins","volume":"29","author":"A Hadchouel","year":"2020","journal-title":"Eur Respir Rev"},{"issue":"01","key":"ref58","doi-asserted-by":"crossref","first-page":"00701-2021","DOI":"10.1183\/23120541.00701-2021","article-title":"Autoimmune pulmonary alveolar proteinosis in children","volume":"8","author":"M Griese","year":"2022","journal-title":"ERJ Open Res"},{"issue":"06","key":"ref59","doi-asserted-by":"crossref","first-page":"00703-2023","DOI":"10.1183\/23120541.00703-2023","article-title":"CSF2RB mutation-related hereditary pulmonary alveolar proteinosis: the \u201clong and winding road\u201d into adulthood","volume":"9","author":"S A Papiris","year":"2023","journal-title":"ERJ Open Res"},{"issue":"01","key":"ref60","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1186\/s12890-017-0382-x","article-title":"Elderly-onset hereditary pulmonary alveolar proteinosis and its cytokine profile","volume":"17","author":"M Ito","year":"2017","journal-title":"BMC Pulm Med"},{"issue":"04","key":"ref61","first-page":"229","article-title":"Pulmonary alveolar proteinosis. What is the role of GM-CSF in disease pathogenesis and treatment?","volume":"3","author":"J F Seymour","year":"2004","journal-title":"Treat Respir Med"},{"issue":"06","key":"ref62","doi-asserted-by":"crossref","first-page":"914","DOI":"10.1189\/jlb.1204723","article-title":"Disruption of granulocyte macrophage-colony stimulating factor production in the lungs severely affects the ability of mice to control Mycobacterium tuberculosis infection","volume":"77","author":"M Gonzalez-Juarrero","year":"2005","journal-title":"J Leukoc Biol"},{"issue":"06","key":"ref63","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1056\/NEJMoa062505","article-title":"GM-CSF autoantibodies and neutrophil dysfunction in pulmonary alveolar proteinosis","volume":"356","author":"K Uchida","year":"2007","journal-title":"N Engl J Med"},{"issue":"02","key":"ref64","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1016\/j.jinf.2012.03.020","article-title":"Opportunistic infections in patients with pulmonary alveolar proteinosis","volume":"65","author":"A D Punatar","year":"2012","journal-title":"J Infect"},{"key":"ref65","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1016\/0002-9343(60)90103-0","article-title":"Pulmonary alveolar proteinosis with unusual complicating infections; a report of two cases","volume":"29","author":"C C Jones","year":"1960","journal-title":"Am J Med"},{"key":"ref66","doi-asserted-by":"crossref","first-page":"266","DOI":"10.7326\/0003-4819-60-2-266","article-title":"The association of nocardiosis and pulmonary alveolar proteinosis. A case study","volume":"60","author":"V T Andriole","year":"1964","journal-title":"Ann Intern Med"},{"key":"ref67","doi-asserted-by":"crossref","first-page":"275","DOI":"10.7326\/0003-4819-60-2-275","article-title":"Nocardiosis and pulmonary alveolar proteinosis","volume":"60","author":"E T Carlsen","year":"1964","journal-title":"Ann Intern Med"},{"key":"ref68","doi-asserted-by":"crossref","first-page":"1002","DOI":"10.1016\/0002-9343(60)90210-2","article-title":"Pulmonary alveolar proteinosis and nocardiosis","volume":"28","author":"B Burbank","year":"1960","journal-title":"Am J Med"},{"issue":"03","key":"ref69","first-page":"469","article-title":"Septic arthritis due to Nocardia asteroides in association with pulmonary alveolar proteinosis","volume":"9","author":"H W Clague","year":"1982","journal-title":"J Rheumatol"},{"issue":"11","key":"ref70","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1157\/13128332","article-title":"[Streptococcus pneumoniae infection: cause or consequence of pulmonary alveolar proteinosis?]","volume":"44","author":"S S\u00e1nchez-Cu\u00e9llar","year":"2008","journal-title":"Arch Bronconeumol"},{"issue":"01","key":"ref71","doi-asserted-by":"crossref","first-page":"e1","DOI":"10.1016\/j.rce.2013.08.010","article-title":"[Alveolar proteinosis in an immunocompetent patient with previous Legionella and Nocardia infections]","volume":"214","author":"P Garmilla Ezquerra","year":"2014","journal-title":"Rev Clin Esp (Barc)"},{"issue":"02","key":"ref72","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1097\/00005792-199403000-00003","article-title":"Isolation of mycobacteria in patients with pulmonary alveolar proteinosis","volume":"73","author":"L A Witty","year":"1994","journal-title":"Medicine (Baltimore)"},{"issue":"04","key":"ref73","first-page":"452","article-title":"Pulmonary alveolar proteinosis. Treatment of a case complicated by tuberculosis","volume":"59","author":"S R Lathan Jr","year":"1971","journal-title":"Chest"},{"issue":"08","key":"ref74","doi-asserted-by":"crossref","first-page":"801","DOI":"10.1097\/00007611-199608000-00009","article-title":"Pulmonary alveolar proteinosis: an unusual association with Mycobacterium avium-intracellulare infection and lymphocytic interstitial pneumonia","volume":"89","author":"R Bakhos","year":"1996","journal-title":"South Med J"},{"issue":"10","key":"ref75","doi-asserted-by":"crossref","first-page":"641","DOI":"10.1016\/S0954-6111(96)90027-X","article-title":"Pulmonary alveolar proteinosis and disseminated Mycobacterium avium infection","volume":"90","author":"L J Couderc","year":"1996","journal-title":"Respir Med"},{"issue":"04","key":"ref76","doi-asserted-by":"crossref","first-page":"478","DOI":"10.1093\/ajcp\/74.4.478","article-title":"Association of pulmonary alveolar lipoproteinosis with mycobacterial infection","volume":"74","author":"J M Reyes","year":"1980","journal-title":"Am J Clin Pathol"},{"issue":"01","key":"ref77","doi-asserted-by":"crossref","first-page":"135","DOI":"10.2169\/naika.88.135","article-title":"Case of alveolar proteinosis associated with pulmonary aspergillosis [in Japanese]","volume":"88","author":"T Kobayashi","year":"1999","journal-title":"Nihon Naika Gakkai Zasshi"},{"issue":"04","key":"ref78","doi-asserted-by":"crossref","first-page":"375","DOI":"10.3109\/00365548609032350","article-title":"Cerebellar aspergilloma","volume":"18","author":"B Bj\u00f6rkholm","year":"1986","journal-title":"Scand J Infect Dis"},{"key":"ref79","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1111\/j.1699-0463.1961.tb00403.x","article-title":"Cryptococcosis as a cause of pulmonary alveolar proteinosis","volume":"53","author":"F Bergman","year":"1961","journal-title":"Acta Pathol Microbiol Scand"},{"issue":"04","key":"ref80","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1007\/BF00432306","article-title":"Pulmonary alveolar proteinosis and histoplasmosis: report of three cases","volume":"368","author":"M Hartung","year":"1975","journal-title":"Virchows Arch A Pathol Anat Histol"},{"issue":"04","key":"ref81","doi-asserted-by":"crossref","first-page":"801","DOI":"10.1378\/chest.98.4.801","article-title":"Pulmonary alveolar proteinosis associated with Pneumocystis carinii. Ultrastructural identification in bronchoalveolar lavage in AIDS and immunocompromised non-AIDS patients","volume":"98","author":"J Tran Van Nhieu","year":"1990","journal-title":"Chest"},{"key":"ref82","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1186\/1742-6413-3-22","article-title":"Alveolar proteinosis in a patient recovering from Pneumocystis carinii infection: a case report with a review of literature","volume":"3","author":"P V Kotov","year":"2006","journal-title":"Cytojournal"},{"issue":"05","key":"ref83","doi-asserted-by":"crossref","first-page":"1214","DOI":"10.1183\/09031936.00001910","article-title":"Pulmonary alveolar proteinosis associated with Epstein-Barr virus infection","volume":"36","author":"C Edwards","year":"2010","journal-title":"Eur Respir J"},{"issue":"03","key":"ref84","first-page":"139","article-title":"Pulmonary alveolar proteinosis and cytomegalovirus infection","volume":"103","author":"M Ranchod","year":"1979","journal-title":"Arch Pathol Lab Med"},{"issue":"02","key":"ref85","doi-asserted-by":"crossref","first-page":"235","DOI":"10.5858\/2003-127-235-HPVGCP","article-title":"Human parainfluenza virus giant cell pneumonia following cord blood transplant associated with pulmonary alveolar proteinosis","volume":"127","author":"K J Butnor","year":"2003","journal-title":"Arch Pathol Lab Med"},{"issue":"04","key":"ref86","doi-asserted-by":"crossref","first-page":"989","DOI":"10.1378\/chest.111.4.989","article-title":"Pulmonary alveolar proteinosis: high-resolution CT, chest radiographic, and functional correlations","volume":"111","author":"K N Lee","year":"1997","journal-title":"Chest"},{"issue":"05","key":"ref87","doi-asserted-by":"crossref","first-page":"1287","DOI":"10.2214\/ajr.176.5.1761287","article-title":"CT features of pulmonary alveolar proteinosis","volume":"176","author":"J M Holbert","year":"2001","journal-title":"AJR Am J Roentgenol"},{"issue":"01","key":"ref88","doi-asserted-by":"crossref","first-page":"21","DOI":"10.3348\/kjr.2009.10.1.21","article-title":"\u201cCrazy-paving\u201d patterns on high-resolution CT scans in patients with pulmonary complications after hematopoietic stem cell transplantation","volume":"10","author":"E Marchiori","year":"2009","journal-title":"Korean J Radiol"},{"issue":"05","key":"ref89","doi-asserted-by":"crossref","first-page":"1348","DOI":"10.1378\/chest.09-0097","article-title":"Comparative study of high-resolution CT findings between autoimmune and secondary pulmonary alveolar proteinosis","volume":"136","author":"H Ishii","year":"2009","journal-title":"Chest"},{"issue":"12","key":"ref90","doi-asserted-by":"crossref","first-page":"5394","DOI":"10.21037\/qims-22-205","article-title":"Quantitative chest CT assessment of pulmonary alveolar proteinosis with deep learning: a real-world longitudinal study","volume":"12","author":"S Shi","year":"2022","journal-title":"Quant Imaging Med Surg"},{"key":"ref91","doi-asserted-by":"crossref","first-page":"1058001","DOI":"10.3389\/fmed.2023.1058001","article-title":"Updated severity and prognosis score of pulmonary alveolar proteinosis: a multi-center cohort study in China","volume":"10","author":"J W Bai","year":"2023","journal-title":"Front Med (Lausanne)"},{"issue":"05","key":"ref92","doi-asserted-by":"crossref","first-page":"450","DOI":"10.1097\/MCP.0000000000000601","article-title":"Shaping the future of an ultra-rare disease: unmet needs in the diagnosis and treatment of pulmonary alveolar proteinosis","volume":"25","author":"C McCarthy","year":"2019","journal-title":"Curr Opin Pulm Med"},{"issue":"04","key":"ref93","doi-asserted-by":"crossref","first-page":"633","DOI":"10.1016\/j.ccm.2025.07.005","article-title":"Pulmonary alveolar proteinosis","volume":"46","author":"F Bonella","year":"2025","journal-title":"Clin Chest Med"},{"issue":"06","key":"ref94","doi-asserted-by":"crossref","first-page":"1509","DOI":"10.1148\/rg.236035101","article-title":"\u201cCrazy-paving\u201d pattern at thin-section CT of the lungs: radiologic-pathologic overview","volume":"23","author":"S E Rossi","year":"2003","journal-title":"Radiographics"},{"issue":"03","key":"ref95","doi-asserted-by":"crossref","first-page":"593","DOI":"10.1016\/j.ccm.2004.04.002","article-title":"Pulmonary alveolar proteinosis","volume":"25","author":"J J Presneill","year":"2004","journal-title":"Clin Chest Med"},{"issue":"06","key":"ref96","first-page":"1059","article-title":"Pulmonary alveolar proteinosis: shunt fraction and lactic acid dehydrogenase concentration as aids to diagnosis","volume":"117","author":"R J Martin","year":"1978","journal-title":"Am Rev Respir Dis"},{"issue":"17","key":"ref97","doi-asserted-by":"crossref","first-page":"1635","DOI":"10.1056\/NEJMoa1913590","article-title":"Inhaled molgramostim therapy in autoimmune pulmonary alveolar proteinosis","volume":"383","author":"B C Trapnell","year":"2020","journal-title":"N Engl J Med"},{"issue":"10","key":"ref98","doi-asserted-by":"crossref","first-page":"923","DOI":"10.1056\/NEJMoa1816216","article-title":"Inhaled GM-CSF for pulmonary alveolar proteinosis","volume":"381","author":"R Tazawa","year":"2019","journal-title":"N Engl J Med"},{"issue":"12","key":"ref99","doi-asserted-by":"crossref","first-page":"1345","DOI":"10.1164\/rccm.200906-0978OC","article-title":"Inhaled granulocyte\/macrophage-colony stimulating factor as therapy for pulmonary alveolar proteinosis","volume":"181","author":"R Tazawa","year":"2010","journal-title":"Am J Respir Crit Care Med"},{"issue":"01","key":"ref100","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1186\/2049-6958-7-4","article-title":"Pulmonary alveolar proteinosis: diagnostic and therapeutic challenges","volume":"7","author":"I Campo","year":"2012","journal-title":"Multidiscip Respir Med"},{"issue":"03","key":"ref101","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1183\/09031936.93.04030377","article-title":"Pulmonary alveolar proteinosis: prolonged spontaneous remission in two patients","volume":"4","author":"M A Mart\u00ednez-L\u00f3pez","year":"1991","journal-title":"Eur Respir J"},{"issue":"03","key":"ref102","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1586\/17476348.2015.1035259","article-title":"Pulmonary alveolar proteinosis: time to shift?","volume":"9","author":"S A Papiris","year":"2015","journal-title":"Expert Rev Respir Med"},{"issue":"02","key":"ref103","doi-asserted-by":"crossref","first-page":"e20170610","DOI":"10.1542\/peds.2017-0610","article-title":"Pulmonary alveolar proteinosis: a comprehensive clinical perspective","volume":"140","author":"M Griese","year":"2017","journal-title":"Pediatrics"},{"issue":"05","key":"ref104","doi-asserted-by":"crossref","first-page":"1357","DOI":"10.1378\/chest.114.5.1357","article-title":"Pulmonary alveolar proteinosis: clinical features and outcomes","volume":"114","author":"L S Goldstein","year":"1998","journal-title":"Chest"},{"issue":"01","key":"ref105","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1136\/thorax.55.1.67","article-title":"Pulmonary alveolar proteinosis: clinical aspects and current concepts on pathogenesis","volume":"55","author":"P L Shah","year":"2000","journal-title":"Thorax"},{"key":"ref106","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/j.jim.2013.11.011","article-title":"Standardized serum GM-CSF autoantibody testing for the routine clinical diagnosis of autoimmune pulmonary alveolar proteinosis","volume":"402","author":"K Uchida","year":"2014","journal-title":"J Immunol Methods"},{"issue":"10","key":"ref107","doi-asserted-by":"crossref","first-page":"1292","DOI":"10.1164\/rccm.201002-0271OC","article-title":"Hereditary pulmonary alveolar proteinosis: pathogenesis, presentation, diagnosis, and therapy","volume":"182","author":"T Suzuki","year":"2010","journal-title":"Am J Respir Crit Care Med"},{"issue":"07","key":"ref108","first-page":"1493","article-title":"Elevated tumor markers in patients with pulmonary alveolar proteinosis","volume":"51","author":"S C Fang","year":"2013","journal-title":"Clin Chem Lab Med"},{"issue":"01","key":"ref109","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1186\/s12890-021-01795-x","article-title":"CYFRA21-1 is a more sensitive biomarker to assess the severity of pulmonary alveolar proteinosis","volume":"22","author":"J W Bai","year":"2022","journal-title":"BMC Pulm Med"},{"key":"ref110","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1186\/1750-1172-8-53","article-title":"Serum KL-6 is a predictor of outcome in pulmonary alveolar proteinosis","volume":"8","author":"F Bonella","year":"2013","journal-title":"Orphanet J Rare Dis"},{"issue":"12","key":"ref111","doi-asserted-by":"crossref","first-page":"1344","DOI":"10.4065\/83.12.1344","article-title":"Clinical importance of bronchoalveolar lavage fluid and blood cytokines, surfactant protein D, and Kerbs von Lungren 6 antigen in idiopathic pulmonary alveolar proteinosis","volume":"83","author":"F C Lin","year":"2008","journal-title":"Mayo Clin Proc"},{"key":"ref112","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1186\/s13023-016-0430-2","article-title":"MUC1 gene polymorphisms are associated with serum KL-6 levels and pulmonary dysfunction in pulmonary alveolar proteinosis","volume":"11","author":"F Bonella","year":"2016","journal-title":"Orphanet J Rare Dis"},{"issue":"07","key":"ref113","doi-asserted-by":"crossref","first-page":"1371","DOI":"10.1111\/resp.13082","article-title":"Serum YKL-40 is a reliable biomarker for pulmonary alveolar proteinosis","volume":"22","author":"F Bonella","year":"2017","journal-title":"Respirology"},{"issue":"05","key":"ref114","doi-asserted-by":"crossref","first-page":"589","DOI":"10.2174\/138161206775474387","article-title":"Pulmonary surfactant proteins A and D: innate immune functions and biomarkers for lung diseases","volume":"12","author":"H Takahashi","year":"2006","journal-title":"Curr Pharm Des"},{"issue":"07","key":"ref115","doi-asserted-by":"crossref","first-page":"5167","DOI":"10.4049\/jimmunol.181.7.5167","article-title":"YKL-40 is elevated in patients with chronic obstructive pulmonary disease and activates alveolar macrophages","volume":"181","author":"S L\u00e9tuv\u00e9","year":"2008","journal-title":"J Immunol"},{"issue":"04","key":"ref116","doi-asserted-by":"crossref","first-page":"610","DOI":"10.1016\/j.resinv.2024.04.019","article-title":"Cytokine profiles associated with disease severity and prognosis of autoimmune pulmonary alveolar proteinosis","volume":"62","author":"K Akasaka","year":"2024","journal-title":"Respir Investig"},{"issue":"06","key":"ref117","doi-asserted-by":"crossref","first-page":"568","DOI":"10.5858\/arpa.2024-0136-OA","article-title":"Napsin-A immunohistochemistry in the diagnosis of pulmonary alveolar proteinosis","volume":"149","author":"C Larose","year":"2025","journal-title":"Arch Pathol Lab Med"},{"key":"ref118","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1001\/archinte.1963.03860030173021","article-title":"Pulmonary alveolar proteinosis: a new technique and rationale for treatment","volume":"112","author":"J Ramirez","year":"1963","journal-title":"Arch Intern Med"},{"key":"ref119","doi-asserted-by":"crossref","first-page":"429","DOI":"10.7326\/0003-4819-63-3-429","article-title":"Pulmonary alveolar proteinosis. endobronchial treatment","volume":"63","author":"J Ramirez","year":"1965","journal-title":"Ann Intern Med"},{"issue":"06","key":"ref120","doi-asserted-by":"crossref","first-page":"581","DOI":"10.1378\/chest.50.6.581","article-title":"Bronchopulmonary lavage. New techniques and observations","volume":"50","author":"J Ramirez","year":"1966","journal-title":"Dis Chest"},{"key":"ref121","first-page":"225","article-title":"Simplified technique of lavage of the lung. Results in dogs","volume":"9","author":"J A Kylstra","year":"1960","journal-title":"Acta Physiol Pharmacol Neerl"},{"issue":"05","key":"ref122","doi-asserted-by":"crossref","first-page":"631","DOI":"10.1513\/AnnalsATS.201502-089PS","article-title":"Historical perspectives of bronchoscopy. 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Cheng","year":"2002","journal-title":"Chest"},{"issue":"04","key":"ref126","doi-asserted-by":"crossref","first-page":"1123","DOI":"10.1378\/chest.122.4.1123","article-title":"Therapeutic whole lung lavage: a stop-gap therapy for alveolar proteinosis","volume":"122","author":"M S Kavuru","year":"2002","journal-title":"Chest"},{"issue":"04","key":"ref127","doi-asserted-by":"crossref","first-page":"526","DOI":"10.1183\/09031936.04.00102704","article-title":"Long-term durable benefit after whole lung lavage in pulmonary alveolar proteinosis","volume":"23","author":"M Beccaria","year":"2004","journal-title":"Eur Respir J"},{"issue":"04","key":"ref128","doi-asserted-by":"crossref","first-page":"503","DOI":"10.1183\/09031936.04.00016704","article-title":"The benefits of whole lung lavage in pulmonary alveolar proteinosis","volume":"23","author":"C Morgan","year":"2004","journal-title":"Eur Respir J"},{"issue":"06","key":"ref129","doi-asserted-by":"crossref","first-page":"1468","DOI":"10.1183\/09031936.00017612","article-title":"Wash-out kinetics and efficacy of a modified lavage technique for alveolar proteinosis","volume":"40","author":"F Bonella","year":"2012","journal-title":"Eur Respir J"},{"issue":"03","key":"ref130","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1097\/LBR.0000000000000741","article-title":"Increased efficacy of whole lung lavage treatment in alveolar proteinosis using a new modified lavage technique","volume":"28","author":"L A Grutters","year":"2021","journal-title":"J Bronchology Interv Pulmonol"},{"issue":"01","key":"ref131","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1378\/chest.130.1.227","article-title":"An open-label trial of granulocyte macrophage colony stimulating factor therapy for moderate symptomatic pulmonary alveolar proteinosis","volume":"130","author":"S B Venkateshiah","year":"2006","journal-title":"Chest"},{"issue":"04","key":"ref132","first-page":"L556","article-title":"Aerosolized GM-CSF ameliorates pulmonary alveolar proteinosis in GM-CSF-deficient mice","volume":"276","author":"J A Reed","year":"1999","journal-title":"Am J Physiol"},{"key":"ref133","first-page":"A889","article-title":"Aerosolized GM-CSF improves pulmonary function in idiopathic pulmonary alveolar proteinosis","volume":"161","author":"M ETR Wylam","year":"2000","journal-title":"Am J Respir Crit Care Med"},{"issue":"10","key":"ref134","doi-asserted-by":"crossref","first-page":"1142","DOI":"10.1164\/rccm.200406-716OC","article-title":"Granulocyte-macrophage colony-stimulating factor and lung immunity in pulmonary alveolar proteinosis","volume":"171","author":"R Tazawa","year":"2005","journal-title":"Am J Respir Crit Care Med"},{"issue":"03","key":"ref135","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1183\/09031936.06.00058305","article-title":"Aerosol granulocyte-macrophage colony-stimulating factor for pulmonary alveolar proteinosis","volume":"27","author":"M E Wylam","year":"2006","journal-title":"Eur Respir J"},{"issue":"04","key":"ref136","doi-asserted-by":"crossref","first-page":"729","DOI":"10.1378\/chest.13-0603","article-title":"Duration of benefit in patients with autoimmune pulmonary alveolar proteinosis after inhaled granulocyte-macrophage colony-stimulating factor therapy","volume":"145","author":"R Tazawa","year":"2014","journal-title":"Chest"},{"issue":"08","key":"ref137","doi-asserted-by":"crossref","first-page":"553","DOI":"10.1007\/s40261-014-0208-z","article-title":"Long-term inhaled granulocyte macrophage-colony-stimulating factor in autoimmune pulmonary alveolar proteinosis: effectiveness, safety, and lowest effective dose","volume":"34","author":"S A Papiris","year":"2014","journal-title":"Clin Drug 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GM-CSF","volume":"41","author":"A Price","year":"2006","journal-title":"Pediatr Pulmonol"},{"issue":"03","key":"ref141","doi-asserted-by":"crossref","first-page":"842","DOI":"10.1378\/chest.08-1317","article-title":"Quantitative analysis of longitudinal response to aerosolized granulocyte-macrophage colony-stimulating factor in two adolescents with autoimmune pulmonary alveolar proteinosis","volume":"135","author":"T E Robinson","year":"2009","journal-title":"Chest"},{"issue":"03","key":"ref142","first-page":"150","article-title":"[Response to inhaled granulocyte-macrophage colony-stimulating factor in a patient with alveolar proteinosis]","volume":"45","author":"J A Rodr\u00edguez Portal","year":"2009","journal-title":"Arch Bronconeumol"},{"issue":"08","key":"ref143","doi-asserted-by":"crossref","first-page":"828","DOI":"10.1002\/ppul.20856","article-title":"A combination therapy of whole lung lavage and GM-CSF inhalation in pulmonary alveolar proteinosis","volume":"43","author":"H Yamamoto","year":"2008","journal-title":"Pediatr Pulmonol"},{"issue":"01","key":"ref144","doi-asserted-by":"crossref","first-page":"2301982","DOI":"10.1183\/13993003.01982-2023","article-title":"Will inhalational GM-CSF replace whole lung lavage as a treatment for autoimmune pulmonary alveolar proteinosis? 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