{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T23:24:26Z","timestamp":1787095466844,"version":"build-2736575974"},"reference-count":116,"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>Pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by progressive accumulation of surfactant in pulmonary alveoli, resulting in hypoxemic respiratory insufficiency and an increased risk of secondary infections and pulmonary fibrosis. Secondary PAP (sPAP) occurs because of an underlying disease that reduces the number and\/or functions of alveolar macrophages, with hematological disorders being the commonest underlying cause. sPAP accounts for 4% of PAP cases, often occurs in the fourth decade of life, and has a slight male predominance. Patients with sPAP often present in the context of their underlying clinical condition. The prognosis of sPAP is considerably worse than aPAP, with an estimated median survival of less than 20 months. Given the nonspecific clinical presentation of PAP, its diagnosis requires appropriate serological, radiological, and bronchoscopic evaluation. The characteristic \u201ccrazy-paving\u201d appearance described in aPAP might not always be present in sPAP. Ground glass opacifications in sPAP typically show a more diffuse pattern compared with a patchy geographic pattern seen in aPAP. The only proven therapy for sPAP is the treatment of the underlying disease, with whole lung lavage demonstrating efficacy in a small number of cases. In this review, we discuss the presentation, prognosis, and treatment of sPAP.<\/jats:p>","DOI":"10.1055\/a-2741-2079","type":"journal-article","created":{"date-parts":[[2025,11,11]],"date-time":"2025-11-11T00:46:01Z","timestamp":1762821961000},"page":"373-383","source":"Crossref","is-referenced-by-count":0,"title":["Secondary Pulmonary Alveolar Proteinosis"],"prefix":"10.1055","volume":"47","author":[{"given":"Jayleigh","family":"Lim","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, St Vincent's University Hospital, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Niamh","family":"Boyle","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, St Vincent's University Hospital, Dublin, Ireland"},{"name":"School of Medicine, University College Dublin, Dublin 4, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cormac","family":"McCarthy","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, St Vincent's University Hospital, Dublin, Ireland"},{"name":"School of Medicine, University College Dublin, Dublin 4, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2025,11,24]]},"reference":[{"issue":"23","key":"ref1","doi-asserted-by":"crossref","first-page":"1123","DOI":"10.1056\/NEJM195806052582301","article-title":"Pulmonary alveolar proteinosis","volume":"258","author":"S H Rosen","year":"1958","journal-title":"N Engl J Med"},{"issue":"01","key":"ref2","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":"02","key":"ref3","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1164\/rccm.2109105","article-title":"Pulmonary alveolar proteinosis: progress in the first 44 years","volume":"166","author":"J F Seymour","year":"2002","journal-title":"Am J Respir Crit Care Med"},{"issue":"05","key":"ref4","doi-asserted-by":"crossref","first-page":"64","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":"09","key":"ref5","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"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1186\/1471-2466-14-37","article-title":"Secondary pulmonary alveolar proteinosis complicating myelodysplastic syndrome results in worsening of prognosis: a retrospective cohort study in Japan","volume":"14","author":"H Ishii","year":"2014","journal-title":"BMC Pulm Med"},{"issue":"02","key":"ref7","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1183\/09031936.00092910","article-title":"Clinical features of secondary pulmonary alveolar proteinosis: pre-mortem cases in Japan","volume":"37","author":"H Ishii","year":"2011","journal-title":"Eur Respir J"},{"issue":"05","key":"ref8","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":"26","key":"ref9","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":"ref10","doi-asserted-by":"crossref","first-page":"11","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"},{"issue":"10","key":"ref11","doi-asserted-by":"crossref","first-page":"1061","DOI":"10.1016\/j.jfma.2023.04.002","article-title":"Pulmonary alveolar proteinosis in Taiwan","volume":"122","author":"C H Chuang","year":"2023","journal-title":"J Formos Med Assoc"},{"issue":"03","key":"ref12","doi-asserted-by":"crossref","first-page":"364","DOI":"10.1016\/j.resinv.2023.02.011","article-title":"Nationwide survey of adult patients with pulmonary alveolar proteinosis using the National Database of designated intractable diseases of Japan","volume":"61","author":"K Miyashita","year":"2023","journal-title":"Respir Investig"},{"issue":"01","key":"ref13","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1186\/s12890-020-1074-5","article-title":"Pulmonary alveolar proteinosis in Korea: analysis of prevalence and incidence via a nationwide population-based study","volume":"20","author":"H Y Yoon","year":"2020","journal-title":"BMC Pulm Med"},{"issue":"01","key":"ref14","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1183\/23120541.00190-2018","article-title":"Incidence of autoimmune pulmonary alveolar proteinosis estimated using Poisson distribution","volume":"5","author":"N Kitamura","year":"2019","journal-title":"ERJ Open Res"},{"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"},{"key":"ref16","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":"ref17","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":"05","key":"ref18","doi-asserted-by":"crossref","first-page":"761","DOI":"10.1111\/j.1440-1843.2009.01539.x","article-title":"Pulmonary alveolar proteinosis in China: a systematic review of 241 cases","volume":"14","author":"Z Xu","year":"2009","journal-title":"Respirology"},{"issue":"07","key":"ref19","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":"02","key":"ref20","first-page":"75","article-title":"Pulmonary alveolar proteinosis in Israel: ethnic clustering","volume":"1","author":"I Ben-Dov","year":"1999","journal-title":"Isr Med Assoc J"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1007\/978-3-319-67144-4_24","article-title":"Epidemiology of rare lung diseases: the challenges and opportunities to improve research and knowledge","volume":"1031","author":"C McCarthy","year":"2017","journal-title":"Adv Exp Med Biol"},{"issue":"03","key":"ref22","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1097\/00043764-198903000-00008","article-title":"Pulmonary alveolar proteinosis and cement dust: a case report","volume":"31","author":"R J McCunney","year":"1989","journal-title":"J Occup Med"},{"issue":"02","key":"ref23","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1164\/arrd.1984.130.2.312","article-title":"Pulmonary alveolar proteinosis and aluminum dust exposure","volume":"130","author":"R R Miller","year":"1984","journal-title":"Am Rev Respir Dis"},{"issue":"01","key":"ref24","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1378\/chest.108.1.277","article-title":"Pulmonary alveolar proteinosis in a painter with elevated pulmonary concentrations of titanium","volume":"108","author":"C A Keller","year":"1995","journal-title":"Chest"},{"issue":"05","key":"ref25","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":"06","key":"ref26","doi-asserted-by":"crossref","first-page":"1813","DOI":"10.1378\/chest.117.6.1813","article-title":"Pulmonary alveolar proteinosis in association with household exposure to fibrous insulation material","volume":"117","author":"J W McDonald","year":"2000","journal-title":"Chest"},{"issue":"120","key":"ref27","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1183\/09059180.00001311","article-title":"Pulmonary alveolar proteinosis","volume":"20","author":"R Borie","year":"2011","journal-title":"Eur Respir Rev"},{"key":"ref28","first-page":"166","article-title":"Groupe D'etudes Et de Recherche Sur Les Maladies Orphelines P","volume":"19","author":"E Briens","year":"2002","journal-title":"Rev Mal Respir"},{"issue":"01","key":"ref29","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1186\/s12890-017-0493-4","article-title":"Clinical significance of cigarette smoking and dust exposure in pulmonary alveolar proteinosis: a Korean national survey","volume":"17","author":"J A Hwang","year":"2017","journal-title":"BMC Pulm Med"},{"issue":"05","key":"ref30","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":"03","key":"ref31","first-page":"132","article-title":"Pulmonary surfactant pathophysiology: current models and open questions","volume":"25","author":"J Perez-Gil","year":"2010","journal-title":"Physiology (Bethesda)"},{"key":"ref32","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1016\/S0925-4439(98)00061-1","article-title":"The role of lipids in pulmonary surfactant","volume":"1408","author":"R Veldhuizen","year":"1998","journal-title":"Biochim Biophys Acta"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1016\/S0925-4439(98)00060-X","article-title":"Pulmonary surfactant: functions and molecular composition","volume":"1408","author":"J Goerke","year":"1998","journal-title":"Biochim Biophys Acta"},{"key":"ref34","doi-asserted-by":"crossref","first-page":"1676","DOI":"10.1016\/j.bbamem.2008.05.003","article-title":"Structure of pulmonary surfactant membranes and films: the role of proteins and lipid-protein interactions","volume":"1778","author":"J P\u00e9rez-Gil","year":"2008","journal-title":"Biochim Biophys Acta"},{"key":"ref35","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1146\/annurev.med.60.041807.123500","article-title":"Alveolar surfactant homeostasis and the pathogenesis of pulmonary disease","volume":"61","author":"J A Whitsett","year":"2010","journal-title":"Annu Rev Med"},{"issue":"02","key":"ref36","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1165\/ajrcmb.19.2.140","article-title":"Collectins and pulmonary host defense","volume":"19","author":"E C Crouch","year":"1998","journal-title":"Am J Respir Cell Mol Biol"},{"key":"ref37","doi-asserted-by":"crossref","first-page":"495","DOI":"10.1146\/annurev.physiol.63.1.495","article-title":"The pulmonary collectins and surfactant metabolism","volume":"63","author":"S Hawgood","year":"2001","journal-title":"Annu Rev Physiol"},{"issue":"10","key":"ref38","doi-asserted-by":"crossref","first-page":"1453","DOI":"10.1172\/JCI200318650","article-title":"Pulmonary surfactant: a front line of lung host defense","volume":"111","author":"J R Wright","year":"2003","journal-title":"J Clin Invest"},{"issue":"02","key":"ref39","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1152\/japplphysiol.00995.2006","article-title":"Alveolar macrophage depletion is associated with increased surfactant pool sizes in adult rats","volume":"103","author":"A Forbes","year":"2007","journal-title":"J Appl Physiol"},{"issue":"05","key":"ref40","doi-asserted-by":"crossref","first-page":"826","DOI":"10.1016\/j.ajhg.2015.03.010","article-title":"Biallelic mutations of methionyl-tRNA synthetase cause a specific type of pulmonary alveolar proteinosis prevalent on R\u00e9union Island","volume":"96","author":"A Hadchouel","year":"2015","journal-title":"Am J Hum Genet"},{"key":"ref41","doi-asserted-by":"crossref","first-page":"788","DOI":"10.1164\/ajrccm.149.3.8118651","article-title":"Secondary alveolar proteinosis is a reversible cause of respiratory failure in leukemic patients","volume":"149","author":"C Cordonnier","year":"1994","journal-title":"Am J Respir Crit Care Med"},{"issue":"03","key":"ref42","first-page":"374","article-title":"[An autopsy case of acute lymphocytic leukemia associated with secondary pulmonary alveolar proteinosis and systemic aspergillosis]","volume":"31","author":"H Kita","year":"1993","journal-title":"Nihon Kyobu Shikkan Gakkai Zasshi"},{"issue":"02","key":"ref43","doi-asserted-by":"crossref","first-page":"187","DOI":"10.2169\/internalmedicine.42.187","article-title":"Secondary pulmonary alveolar proteinosis associated with myelodysplastic syndrome","volume":"42","author":"T Ohnishi","year":"2003","journal-title":"Intern Med"},{"issue":"07","key":"ref44","doi-asserted-by":"crossref","first-page":"710","DOI":"10.1002\/ppul.20818","article-title":"Recurrent pulmonary alveolar proteinosis secondary to agammaglobulinemia","volume":"43","author":"T Patiroglu","year":"2008","journal-title":"Pediatr Pulmonol"},{"key":"ref45","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1186\/s12890-015-0083-2","article-title":"GATA2 deficiency in children and adults with severe pulmonary alveolar proteinosis and hematologic disorders","volume":"15","author":"M Griese","year":"2015","journal-title":"BMC Pulm Med"},{"issue":"06","key":"ref46","doi-asserted-by":"crossref","first-page":"1187","DOI":"10.1016\/0002-9343(86)90683-2","article-title":"Secondary pulmonary alveolar proteinosis occurring in two patients with acquired immune deficiency syndrome","volume":"80","author":"F L Ruben","year":"1986","journal-title":"Am J Med"},{"issue":"02","key":"ref47","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1055\/s-0039-3402727","article-title":"Pulmonary alveolar proteinosis syndrome","volume":"41","author":"A Kelly","year":"2020","journal-title":"Semin Respir Crit Care Med"},{"issue":"221","key":"ref48","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1136\/adc.42.221.40","article-title":"Thymic alymphoplasia","volume":"42","author":"J C Haworth","year":"1967","journal-title":"Arch Dis Child"},{"issue":"04","key":"ref49","doi-asserted-by":"crossref","first-page":"434","DOI":"10.1097\/SHK.0b013e3181673543","article-title":"Granulocyte-macrophage-colony-stimulating factor-dependent peritoneal macrophage responses determine survival in experimentally induced peritonitis and sepsis in mice","volume":"30","author":"D Spight","year":"2008","journal-title":"Shock"},{"issue":"09","key":"ref50","doi-asserted-by":"crossref","first-page":"911","DOI":"10.1016\/S0046-8177(85)80130-1","article-title":"Pathologic pulmonary alterations in long-term human heart-lung transplantation","volume":"16","author":"S A Yousem","year":"1985","journal-title":"Hum Pathol"},{"issue":"02","key":"ref51","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1183\/13993003.01369-2016","article-title":"Secondary pulmonary alveolar proteinosis after lung transplantation: a single-centre series","volume":"49","author":"Q Philippot","year":"2017","journal-title":"Eur Respir J"},{"issue":"11","key":"ref52","doi-asserted-by":"crossref","first-page":"939","DOI":"10.1136\/thx.43.11.939","article-title":"Pulmonary alveolar lipoproteinosis complicating juvenile dermatomyositis","volume":"43","author":"M P Samuels","year":"1988","journal-title":"Thorax"},{"issue":"05","key":"ref53","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1111\/j.1440-1843.2006.00905.x","article-title":"Pulmonary alveolar proteinosis associated with a disease-modifying antirheumatoid arthritis drug","volume":"11","author":"N R Wardwell Jr","year":"2006","journal-title":"Respirology"},{"issue":"02","key":"ref54","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1111\/j.1440-1843.2008.01450.x","article-title":"Pulmonary alveolar proteinosis in a patient with Behcet's disease","volume":"14","author":"M Uchiyama","year":"2009","journal-title":"Respirology"},{"key":"ref55","volume-title":"Lysinuric Protein Intolerance","author":"V Nunes","year":"1993"},{"key":"ref56","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1186\/1750-1172-2-14","article-title":"Successful whole lung lavage in pulmonary alveolar proteinosis secondary to lysinuric protein intolerance: a case report","volume":"2","author":"M Ceruti","year":"2007","journal-title":"Orphanet J Rare Dis"},{"key":"ref57","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1186\/1750-1172-9-85","article-title":"Pulmonary alveolar proteinosis in children on La R\u00e9union Island: a new inherited disorder?","volume":"9","author":"L Enaud","year":"2014","journal-title":"Orphanet J Rare Dis"},{"key":"ref58","first-page":"102090","article-title":"Unusual cause of dyspnea in patient with myelofibrosis: the ruxolitinib lung","volume":"51","author":"A El Kik","year":"2024","journal-title":"Respir Med Case Rep"},{"issue":"08","key":"ref59","doi-asserted-by":"crossref","first-page":"1081","DOI":"10.2169\/internalmedicine.3770-19","article-title":"Secondary pulmonary alveolar proteinosis following treatment with azacitidine for myelodysplastic syndrome","volume":"59","author":"M Hashimoto","year":"2020","journal-title":"Intern Med"},{"key":"ref60","first-page":"101566","article-title":"Sirolimus-induced secondary pulmonary alveolar proteinosis","volume":"35","author":"S Wang","year":"2021","journal-title":"Respir Med Case Rep"},{"issue":"01","key":"ref61","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1016\/j.transproceed.2021.12.006","article-title":"Sirolimus-induced pulmonary alveolar proteinosis in the context of hematopoietic stem cells transplant rejection: a case report","volume":"54","author":"L Marchasson","year":"2022","journal-title":"Transplant Proc"},{"issue":"03","key":"ref62","doi-asserted-by":"crossref","first-page":"252","DOI":"10.4103\/lungindia.lungindia_484_19","article-title":"Reversible sirolimus-induced pulmonary alveolar proteinosis in a renal transplant patient","volume":"37","author":"D E Hinojosa-Gonz\u00e1lez","year":"2020","journal-title":"Lung India"},{"issue":"04","key":"ref63","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":"11","key":"ref64","first-page":"e74669","article-title":"Pulmonary alveolar proteinosis during intensive immunosuppressive treatment for acute exacerbation of interstitial pneumonia: a case report and literature review","volume":"16","author":"A Yanagisawa","year":"2024","journal-title":"Cureus"},{"issue":"02","key":"ref65","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1097\/RTI.0b013e3181930ed6","article-title":"Secondary pulmonary alveolar proteinosis: a confusing and potentially serious complication of hematologic malignancy","volume":"24","author":"J H Chung","year":"2009","journal-title":"J Thorac Imaging"},{"issue":"03","key":"ref66","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1002\/ajh.10410","article-title":"Disseminated Mycobacterium kansasii infection with pulmonary alveolar proteinosis in a patient with chronic myelogenous leukemia","volume":"74","author":"N Goldschmidt","year":"2003","journal-title":"Am J Hematol"},{"issue":"01","key":"ref67","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1186\/s13023-025-03617-3","article-title":"Phenotypic heterogeneity in mortality and prognosis of pulmonary alveolar proteinosis: a large-scale, global pooled analysis of individual-level data","volume":"20","author":"J Huang","year":"2025","journal-title":"Orphanet J Rare Dis"},{"issue":"03","key":"ref68","doi-asserted-by":"crossref","first-page":"544","DOI":"10.2214\/AJR.15.14982","article-title":"Pulmonary fibrosis on high-resolution CT of patients with pulmonary alveolar proteinosis","volume":"207","author":"M Akira","year":"2016","journal-title":"AJR Am J Roentgenol"},{"issue":"05","key":"ref69","doi-asserted-by":"crossref","first-page":"e230040","DOI":"10.1148\/ryct.230040","article-title":"Pulmonary alveolar proteinosis-associated pulmonary fibrosis: evolutional changes and radiologic-pathologic correlation","volume":"5","author":"E Hu-Wang","year":"2023","journal-title":"Radiol Cardiothorac Imaging"},{"issue":"06","key":"ref70","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1183\/23120541.00314-2024","article-title":"Pulmonary fibrosis in patients with autoimmune pulmonary alveolar proteinosis: a retrospective nationwide cohort study","volume":"10","author":"Y Guirriec","year":"2024","journal-title":"ERJ Open Res"},{"issue":"05","key":"ref71","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":"03","key":"ref72","doi-asserted-by":"crossref","first-page":"883","DOI":"10.1148\/rg.283075219","article-title":"From the archives of the AFIP: pulmonary alveolar proteinosis","volume":"28","author":"A A Frazier","year":"2008","journal-title":"Radiographics"},{"key":"ref74","doi-asserted-by":"crossref","first-page":"A6088","DOI":"10.1164\/ajrccm.2025.211.Abstracts.A6088","article-title":"Pulmonary alveolar proteinosis with associated fibrosis: a case series from a national referral centre","volume":"211","author":"M Penugonda","year":"2025","journal-title":"Am J Respir Crit Care Med"},{"issue":"10","key":"ref75","doi-asserted-by":"crossref","first-page":"5774","DOI":"10.21037\/jtd.2018.09.86","article-title":"CT findings in pulmonary alveolar proteinosis: serial changes and prognostic implications","volume":"10","author":"B Da Nam","year":"2018","journal-title":"J Thorac Dis"},{"issue":"01","key":"ref76","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1186\/s12890-018-0590-z","article-title":"Secondary pulmonary alveolar proteinosis: a single-center retrospective study (a case series and literature review)","volume":"18","author":"D Zhang","year":"2018","journal-title":"BMC Pulm Med"},{"issue":"04","key":"ref77","first-page":"274","article-title":"Acute silico-proteinosis. A new pathologic variant of acute silicosis in sandblasters, characterized by histologic features resembling alveolar proteinosis","volume":"55","author":"H A Buechner","year":"1969","journal-title":"Dis Chest"},{"issue":"03","key":"ref78","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1177\/0192623316688959","article-title":"Particle-induced pulmonary alveolar proteinosis and subsequent inflammation and fibrosis: a toxicologic and pathologic review","volume":"45","author":"E M Bomhard","year":"2017","journal-title":"Toxicol Pathol"},{"issue":"05","key":"ref79","doi-asserted-by":"crossref","first-page":"1510","DOI":"10.1183\/13993003.00252-2016","article-title":"Association of pulmonary alveolar proteinosis and fibrosis: patient with GATA2 deficiency","volume":"48","author":"A Ballerie","year":"2016","journal-title":"Eur Respir J"},{"issue":"05","key":"ref80","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1183\/13993003.00178-2017","article-title":"Haemodynamically proven pulmonary hypertension in a patient with GATA2 deficiency-associated pulmonary alveolar proteinosis and fibrosis","volume":"49","author":"S Sanges","year":"2017","journal-title":"Eur Respir J"},{"issue":"02","key":"ref81","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1016\/S1092-9134(02)91214-1","article-title":"Pulmonary alveolar proteinosis mimicking idiopathic pulmonary fibrosis","volume":"7","author":"Z K Arbiser","year":"2003","journal-title":"Ann Diagn Pathol"},{"key":"ref82","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"},{"issue":"05","key":"ref83","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":"04","key":"ref84","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":"03","key":"ref85","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1148\/radiology.169.3.3186983","article-title":"Pulmonary alveolar proteinosis: CT findings","volume":"169","author":"J D Godwin","year":"1988","journal-title":"Radiology"},{"issue":"01","key":"ref86","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1148\/radiology.211.1.r99ap10155","article-title":"Crazy-paving appearance at thin-section CT: spectrum of disease and pathologic findings","volume":"211","author":"T Johkoh","year":"1999","journal-title":"Radiology"},{"issue":"03","key":"ref87","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":"ref88","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"},{"key":"ref89","first-page":"4021928","article-title":"The clinical clues of pulmonary alveolar proteinosis: a report of 11 cases and literature review","volume":"2016","author":"Q Mo","year":"2016","journal-title":"Can Respir J"},{"key":"ref90","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"},{"key":"ref91","doi-asserted-by":"crossref","first-page":"658","DOI":"10.1164\/ajrccm.162.2.9910032","article-title":"Serological diagnosis of idiopathic pulmonary alveolar proteinosis","volume":"162","author":"T Kitamura","year":"2000","journal-title":"Am J Respir Crit Care Med"},{"issue":"03","key":"ref92","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1136\/thorax.57.3.277","article-title":"BAL findings in a patient with pulmonary alveolar proteinosis successfully treated with GM-CSF","volume":"57","author":"O D Schoch","year":"2002","journal-title":"Thorax"},{"issue":"06","key":"ref93","doi-asserted-by":"crossref","first-page":"1330","DOI":"10.1164\/ajrccm\/144.6.1330","article-title":"Bronchoalveolar lavage cell data in alveolar proteinosis","volume":"144","author":"B J Milleron","year":"1991","journal-title":"Am Rev Respir Dis"},{"issue":"03","key":"ref94","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1016\/j.ccm.2016.04.006","article-title":"Pulmonary alveolar proteinosis syndrome","volume":"37","author":"T Suzuki","year":"2016","journal-title":"Clin Chest Med"},{"issue":"04","key":"ref95","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":"01","key":"ref96","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1016\/j.chest.2016.04.030","article-title":"A global survey on whole lung lavage in pulmonary alveolar proteinosis","volume":"150","author":"I Campo","year":"2016","journal-title":"Chest"},{"issue":"01","key":"ref97","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1186\/s13023-016-0497-9","article-title":"Whole lung lavage therapy for pulmonary alveolar proteinosis: a global survey of current practices and procedures","volume":"11","author":"I Campo","year":"2016","journal-title":"Orphanet J Rare Dis"},{"issue":"04","key":"ref98","doi-asserted-by":"crossref","first-page":"1480","DOI":"10.1378\/chest.122.4.1480","article-title":"Pulmonary alveolar proteinosis: treatment by bronchofiberscopic lobar lavage","volume":"122","author":"S L Cheng","year":"2002","journal-title":"Chest"},{"issue":"02","key":"ref99","first-page":"224","article-title":"Alveolar proteinosis. Lobar lavage by fiberoptic bronchoscopic technique","volume":"69","author":"B B Brach","year":"1976","journal-title":"Chest"},{"issue":"10","key":"ref100","doi-asserted-by":"crossref","first-page":"871","DOI":"10.47102\/annals-acadmedsg.V36N10p871","article-title":"Treatment of a primary pulmonary alveolar proteinosis case with severe hypoxaemia by using segmental lavage technique","volume":"36","author":"E C Edis","year":"2007","journal-title":"Ann Acad Med Singap"},{"issue":"04","key":"ref101","first-page":"48","article-title":"Segmental lung lavage with fiberoptic bronchoscopy in a patient with special presentation of pulmonary alveolar proteinosis","volume":"12","author":"H A Sadeghi","year":"2013","journal-title":"Tanaffos"},{"issue":"03","key":"ref102","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1007\/s12185-009-0404-4","article-title":"Successful allogeneic bone marrow transplantation for myelodysplastic syndrome complicated by severe pulmonary alveolar proteinosis","volume":"90","author":"S Tabata","year":"2009","journal-title":"Int J Hematol"},{"issue":"05","key":"ref103","doi-asserted-by":"crossref","first-page":"642","DOI":"10.1007\/s12185-023-03603-0","article-title":"Successful allogeneic hematopoietic stem cell transplantation for myelodysplastic neoplasms complicated with secondary pulmonary alveolar proteinosis and Beh\u00e7et's disease harboring GATA2 mutation","volume":"118","author":"Y Sato","year":"2023","journal-title":"Int J Hematol"},{"key":"ref104","doi-asserted-by":"crossref","first-page":"e2021382","DOI":"10.4322\/acr.2021.382","article-title":"Pulmonary alveolar proteinosis due to familial myelodysplastic syndrome with resolution after stem cell transplant","volume":"12","author":"A Basheer","year":"2022","journal-title":"Autops Case Rep"},{"key":"ref105","doi-asserted-by":"crossref","first-page":"108522","DOI":"10.1016\/j.clim.2020.108522","article-title":"Allogeneic hematopoietic cell transplantation in the management of GATA2 deficiency and pulmonary alveolar proteinosis","volume":"218","author":"Y F van Lier","year":"2020","journal-title":"Clin Immunol"},{"issue":"05","key":"ref106","doi-asserted-by":"crossref","first-page":"610","DOI":"10.1007\/s12185-017-2375-1","article-title":"Hematopoietic stem cell transplantation for pulmonary alveolar proteinosis associated with primary immunodeficiency disease","volume":"107","author":"M Tanaka-Kubota","year":"2018","journal-title":"Int J Hematol"},{"issue":"09","key":"ref107","doi-asserted-by":"crossref","first-page":"1644","DOI":"10.12998\/wjcc.v12.i9.1644","article-title":"Pulmonary alveolar proteinosis induced by X-linked agammaglobulinemia: a case report","volume":"12","author":"T Zhang","year":"2024","journal-title":"World J Clin Cases"},{"key":"ref108","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1186\/1752-1947-5-46","article-title":"Unsuspected pulmonary alveolar proteinosis in a patient with acquired immunodeficiency syndrome: a case report","volume":"5","author":"D Tejwani","year":"2011","journal-title":"J Med Case Rep"},{"issue":"S 03","key":"ref109","doi-asserted-by":"crossref","first-page":"e246","DOI":"10.1016\/j.ijid.2009.10.004","article-title":"Resolution of secondary pulmonary alveolar proteinosis following treatment of rhinocerebral aspergillosis","volume":"14","author":"K P Lin","year":"2010","journal-title":"Int J Infect Dis"},{"issue":"02","key":"ref110","doi-asserted-by":"crossref","first-page":"e13659","DOI":"10.1111\/petr.13659","article-title":"Improvement of native pulmonary alveolar proteinosis after contralateral single living-donor lobar lung transplantation: a case report","volume":"24","author":"K Kobayashi","year":"2020","journal-title":"Pediatr Transplant"},{"key":"ref111","first-page":"101108","article-title":"Secondary pulmonary alveolar proteinosis treated by lung transplant: a case report","volume":"30","author":"D Lawi","year":"2020","journal-title":"Respir Med Case Rep"},{"issue":"07","key":"ref112","doi-asserted-by":"crossref","first-page":"1317","DOI":"10.1016\/j.ajt.2024.03.003","article-title":"Successful lung transplantation in genetic methionyl-tRNA synthetase-related alveolar proteinosis\/lung fibrosis without recurrence under methionine supplementation: medium-term outcome in 4 cases","volume":"24","author":"C Roy","year":"2024","journal-title":"Am J Transplant"},{"issue":"07","key":"ref113","first-page":"599","article-title":"[Secondary pulmonary alveolar proteinosis associated with myelodysplastic syndrome]","volume":"40","author":"K Yokomura","year":"2002","journal-title":"Nihon Kokyuki Gakkai Zasshi"},{"issue":"01","key":"ref114","doi-asserted-by":"crossref","first-page":"308","DOI":"10.1002\/ppul.25718","article-title":"Successful whole lung lavage in a child with pulmonary alveolar proteinosis secondary to hematologic malignancy","volume":"57","author":"K Tsukahara","year":"2022","journal-title":"Pediatr Pulmonol"},{"issue":"04","key":"ref115","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1183\/13993003.01554-2021","article-title":"Methionine supplementation for multi-organ dysfunction in MetRS-related pulmonary alveolar proteinosis","volume":"59","author":"A Hadchouel","year":"2022","journal-title":"Eur Respir J"},{"issue":"11","key":"ref116","doi-asserted-by":"crossref","first-page":"3057","DOI":"10.1002\/ppul.25031","article-title":"Rescue of respiratory failure in pulmonary alveolar proteinosis due to pathogenic MARS1 variants","volume":"55","author":"D Lenz","year":"2020","journal-title":"Pediatr Pulmonol"},{"issue":"05","key":"ref117","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1183\/13993003.02594-2021","article-title":"Methionine supplementation: potential for improving alveolar macrophage function through reverse cholesterol transport?","volume":"59","author":"M O'Callaghan","year":"2022","journal-title":"Eur Respir J"}],"container-title":["Seminars in Respiratory and Critical Care Medicine"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/a-2741-2079.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T22:41:34Z","timestamp":1787092894000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/a-2741-2079"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,11,10]]},"references-count":116,"journal-issue":{"issue":"04","published-online":{"date-parts":[[2026,8,18]]},"published-print":{"date-parts":[[2026,8]]}},"URL":"https:\/\/doi.org\/10.1055\/a-2741-2079","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["1069-3424","1098-9048"],"issn-type":[{"value":"1069-3424","type":"print"},{"value":"1098-9048","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2025,11,10]]},"published":{"date-parts":[[2025,11,10]]}}}