Secondary Pulmonary Alveolar Proteinosis

Abstract

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 “crazy-paving” 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.

Keywords

secondary PAP - proteinosis - pulmonary alveolar proteinosis - lipoproteinosis - macrophage

Publikationsverlauf

Eingereicht: 28. Juli 2025

Angenommen: 31. Oktober 2025

Accepted Manuscript online:
10. November 2025

Artikel online veröffentlicht:
24. November 2025

• References

• 1 Rosen SH, Castleman B, Liebow AA. Pulmonary alveolar proteinosis. N Engl J Med 1958; 258 (23) 1123-1142
• 2 Trapnell BC, Nakata K, Bonella F. et al. Pulmonary alveolar proteinosis. Nat Rev Dis Primers 2019; 5 (01) 16
• 3 Seymour JF, Presneill JJ. Pulmonary alveolar proteinosis: progress in the first 44 years. Am J Respir Crit Care Med 2002; 166 (02) 215-235
• 4 McCarthy C, Bonella F, O'Callaghan M. et al. European Respiratory Society guidelines for the diagnosis and management of pulmonary alveolar proteinosis. Eur Respir J 2024; 64 (05) 64
• 5 McCarthy C, Carey BC, Trapnell BC. Autoimmune pulmonary alveolar proteinosis. Am J Respir Crit Care Med 2022; 205 (09) 1016-1035
• 6 Ishii H, Seymour JF, Tazawa R. et al. Secondary pulmonary alveolar proteinosis complicating myelodysplastic syndrome results in worsening of prognosis: a retrospective cohort study in Japan. BMC Pulm Med 2014; 14: 37
• 7 Ishii H, Tazawa R, Kaneko C. et al. Clinical features of secondary pulmonary alveolar proteinosis: pre-mortem cases in Japan. Eur Respir J 2011; 37 (02) 465-468
• 8 Ishii H, Trapnell BC, Tazawa R. et al; Japanese Center of the Rare Lung Disease Consortium. Comparative study of high-resolution CT findings between autoimmune and secondary pulmonary alveolar proteinosis. Chest 2009; 136 (05) 1348-1355
• 9 Trapnell BC, Whitsett JA, Nakata K. Pulmonary alveolar proteinosis. N Engl J Med 2003; 349 (26) 2527-2539
• 10 Kimura Y, Jo T, Hashimoto Y. et al. Epidemiology of pulmonary alveolar proteinosis: a descriptive study using a Japanese national administrative claims database. ERJ Open Res 2025; 11 (01) 11
• 11 Chuang CH, Cheng CH, Tsai YC, Tsai MJ, Sheu CC, Chong IW. Pulmonary alveolar proteinosis in Taiwan. J Formos Med Assoc 2023; 122 (10) 1061-1068
• 12 Miyashita K, Hozumi H, Inoue Y, Suzuki T, Suda T. Nationwide survey of adult patients with pulmonary alveolar proteinosis using the National Database of designated intractable diseases of Japan. Respir Investig 2023; 61 (03) 364-370
• 13 Yoon HY, Kim JH, Kim YJ, Song JW. Pulmonary alveolar proteinosis in Korea: analysis of prevalence and incidence via a nationwide population-based study. BMC Pulm Med 2020; 20 (01) 34
• 14 Kitamura N, Ohkouchi S, Tazawa R. et al. Incidence of autoimmune pulmonary alveolar proteinosis estimated using Poisson distribution. ERJ Open Res 2019; 5 (01) 5
• 15 McCarthy C, Avetisyan R, Carey BC, Chalk C, Trapnell BC. Prevalence and healthcare burden of pulmonary alveolar proteinosis. Orphanet J Rare Dis 2018; 13 (01) 129
• 16 Campo I, Mariani F, Rodi G. et al. Assessment and management of pulmonary alveolar proteinosis in a reference center. Orphanet J Rare Dis 2013; 8: 40
• 17 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) 1908-1916
• 18 Xu Z, Jing J, Wang H, Xu F, Wang J. Pulmonary alveolar proteinosis in China: a systematic review of 241 cases. Respirology 2009; 14 (05) 761-766
• 19 Inoue Y, Trapnell BC, Tazawa R. et al; Japanese Center of the Rare Lung Diseases Consortium. Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan. Am J Respir Crit Care Med 2008; 177 (07) 752-762
• 20 Ben-Dov I, Kishinevski Y, Roznman J. et al. Pulmonary alveolar proteinosis in Israel: ethnic clustering. Isr Med Assoc J 1999; 1 (02) 75-78
• 21 McCarthy C, Lara Gallego B, Trapnell BC, McCormack FX. Epidemiology of rare lung diseases: the challenges and opportunities to improve research and knowledge. Adv Exp Med Biol 2017; 1031: 419-442
• 22 McCunney RJ, Godefroi R. Pulmonary alveolar proteinosis and cement dust: a case report. J Occup Med 1989; 31 (03) 233-237
• 23 Miller RR, Churg AM, Hutcheon M, Lom S. Pulmonary alveolar proteinosis and aluminum dust exposure. Am Rev Respir Dis 1984; 130 (02) 312-315
• 24 Keller CA, Frost A, Cagle PT, Abraham JL. Pulmonary alveolar proteinosis in a painter with elevated pulmonary concentrations of titanium. Chest 1995; 108 (01) 277-280
• 25 Cummings KJ, Donat WE, Ettensohn DB, Roggli VL, Ingram P, Kreiss K. Pulmonary alveolar proteinosis in workers at an indium processing facility. Am J Respir Crit Care Med 2010; 181 (05) 458-464
• 26 McDonald JW, Alvarez F, Keller CA. Pulmonary alveolar proteinosis in association with household exposure to fibrous insulation material. Chest 2000; 117 (06) 1813-1817
• 27 Borie R, Danel C, Debray MP. et al. Pulmonary alveolar proteinosis. Eur Respir Rev 2011; 20 (120) 98-107
• 28 Briens E, Delaval P, Mairesse MP. et al. Groupe D'etudes Et de Recherche Sur Les Maladies Orphelines P. Rev Mal Respir 2002; 19: 166-182
• 29 Hwang JA, Song JH, Kim JH. et al. Clinical significance of cigarette smoking and dust exposure in pulmonary alveolar proteinosis: a Korean national survey. BMC Pulm Med 2017; 17 (01) 147
• 30 Costabel U, Nakata K. Pulmonary alveolar proteinosis associated with dust inhalation: not secondary but autoimmune?. Am J Respir Crit Care Med 2010; 181 (05) 427-428
• 31 Perez-Gil J, Weaver TE. Pulmonary surfactant pathophysiology: current models and open questions. Physiology (Bethesda) 2010; 25 (03) 132-141
• 32 Veldhuizen R, Nag K, Orgeig S, Possmayer F. The role of lipids in pulmonary surfactant. Biochim Biophys Acta 1998; 1408 (2–3): 90-108
• 33 Goerke J. Pulmonary surfactant: functions and molecular composition. Biochim Biophys Acta 1998; 1408 (2–3): 79-89
• 34 Pérez-Gil J. Structure of pulmonary surfactant membranes and films: the role of proteins and lipid-protein interactions. Biochim Biophys Acta 2008; 1778 (7–8): 1676-1695
• 35 Whitsett JA, Wert SE, Weaver TE. Alveolar surfactant homeostasis and the pathogenesis of pulmonary disease. Annu Rev Med 2010; 61: 105-119
• 36 Crouch EC. Collectins and pulmonary host defense. Am J Respir Cell Mol Biol 1998; 19 (02) 177-201
• 37 Hawgood S, Poulain FR. The pulmonary collectins and surfactant metabolism. Annu Rev Physiol 2001; 63: 495-519
• 38 Wright JR. Pulmonary surfactant: a front line of lung host defense. J Clin Invest 2003; 111 (10) 1453-1455
• 39 Forbes A, Pickell M, Foroughian M, Yao LJ, Lewis J, Veldhuizen R. Alveolar macrophage depletion is associated with increased surfactant pool sizes in adult rats. J Appl Physiol 2007; 103 (02) 637-645
• 40 Hadchouel A, Wieland T, Griese M. et al. Biallelic mutations of methionyl-tRNA synthetase cause a specific type of pulmonary alveolar proteinosis prevalent on Réunion Island. Am J Hum Genet 2015; 96 (05) 826-831
• 41 Cordonnier C, Fleury-Feith J, Escudier E, Atassi K, Bernaudin JF. Secondary alveolar proteinosis is a reversible cause of respiratory failure in leukemic patients. Am J Respir Crit Care Med 1994; 149 (3 Pt 1): 788-794
• 42 Kita H, Muro S, Nakano Y. et al. [An autopsy case of acute lymphocytic leukemia associated with secondary pulmonary alveolar proteinosis and systemic aspergillosis]. Nihon Kyobu Shikkan Gakkai Zasshi 1993; 31 (03) 374-378
• 43 Ohnishi T, Yamada G, Shijubo N. et al. Secondary pulmonary alveolar proteinosis associated with myelodysplastic syndrome. Intern Med 2003; 42 (02) 187-190
• 44 Patiroglu T, Akyildiz B, Patiroglu TE, Gulmez IY. Recurrent pulmonary alveolar proteinosis secondary to agammaglobulinemia. Pediatr Pulmonol 2008; 43 (07) 710-713
• 45 Griese M, Zarbock R, Costabel U. et al. GATA2 deficiency in children and adults with severe pulmonary alveolar proteinosis and hematologic disorders. BMC Pulm Med 2015; 15: 87
• 46 Ruben FL, Talamo TS. Secondary pulmonary alveolar proteinosis occurring in two patients with acquired immune deficiency syndrome. Am J Med 1986; 80 (06) 1187-1190
• 47 Kelly A, McCarthy C. Pulmonary alveolar proteinosis syndrome. Semin Respir Crit Care Med 2020; 41 (02) 288-298
• 48 Haworth JC, Hoogstraten J, Taylor H. Thymic alymphoplasia. Arch Dis Child 1967; 42 (221) 40-54
• 49 Spight D, Trapnell B, Zhao B, Berclaz P, Shanley TP. Granulocyte-macrophage-colony-stimulating factor-dependent peritoneal macrophage responses determine survival in experimentally induced peritonitis and sepsis in mice. Shock 2008; 30 (04) 434-442
• 50 Yousem SA, Burke CM, Billingham ME. Pathologic pulmonary alterations in long-term human heart-lung transplantation. Hum Pathol 1985; 16 (09) 911-923
• 51 Philippot Q, Cazes A, Borie R. et al. Secondary pulmonary alveolar proteinosis after lung transplantation: a single-centre series. Eur Respir J 2017; 49 (02) 49
• 52 Samuels MP, Warner JO. Pulmonary alveolar lipoproteinosis complicating juvenile dermatomyositis. Thorax 1988; 43 (11) 939-940
• 53 Wardwell Jr NR, Miller R, Ware LB. Pulmonary alveolar proteinosis associated with a disease-modifying antirheumatoid arthritis drug. Respirology 2006; 11 (05) 663-665
• 54 Uchiyama M, Nagao T, Hattori A. et al. Pulmonary alveolar proteinosis in a patient with Behcet's disease. Respirology 2009; 14 (02) 305-308
• 55 Nunes V, Niinikoski H. Lysinuric Protein Intolerance. In: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Amemiya A. editors. GeneReviews((R)). Seattle (WA): 1993
• 56 Ceruti M, Rodi G, Stella GM. et al. Successful whole lung lavage in pulmonary alveolar proteinosis secondary to lysinuric protein intolerance: a case report. Orphanet J Rare Dis 2007; 2: 14
• 57 Enaud L, Hadchouel A, Coulomb A. et al. Pulmonary alveolar proteinosis in children on La Réunion Island: a new inherited disorder?. Orphanet J Rare Dis 2014; 9: 85
• 58 El Kik A, Vander Kuylen M, Bailly B, Fallas J, Bondue B. Unusual cause of dyspnea in patient with myelofibrosis: the ruxolitinib lung. Respir Med Case Rep 2024; 51: 102090
• 59 Hashimoto M, Itonaga H, Nannya Y. et al. Secondary pulmonary alveolar proteinosis following treatment with azacitidine for myelodysplastic syndrome. Intern Med 2020; 59 (08) 1081-1086
• 60 Wang S, Lee E, Lau R, Wang T. Sirolimus-induced secondary pulmonary alveolar proteinosis. Respir Med Case Rep 2021; 35: 101566
• 61 Marchasson L, Dutilh J, Jouneau S, Bironneau V. Sirolimus-induced pulmonary alveolar proteinosis in the context of hematopoietic stem cells transplant rejection: a case report. Transplant Proc 2022; 54 (01) 180-184
• 62 Hinojosa-González DE, Dávila-González D, Salgado-Garza G, Flores-Villalba E. Reversible sirolimus-induced pulmonary alveolar proteinosis in a renal transplant patient. Lung India 2020; 37 (03) 252-256
• 63 Imura Y, Yukawa N, Handa T. et al. Two cases of autoimmune and secondary pulmonary alveolar proteinosis during immunosuppressive therapy in dermatomyositis with interstitial lung disease. Mod Rheumatol 2018; 28 (04) 724-729
• 64 Yanagisawa A, Konaka H, Tanaka M, Ihara S, Tachibana I. Pulmonary alveolar proteinosis during intensive immunosuppressive treatment for acute exacerbation of interstitial pneumonia: a case report and literature review. Cureus 2024; 16 (11) e74669
• 65 Chung JH, Pipavath SJ, Myerson DH, Godwin D. Secondary pulmonary alveolar proteinosis: a confusing and potentially serious complication of hematologic malignancy. J Thorac Imaging 2009; 24 (02) 115-118
• 66 Goldschmidt N, Nusair S, Gural A, Amir G, Izhar U, Laxer U. Disseminated Mycobacterium kansasii infection with pulmonary alveolar proteinosis in a patient with chronic myelogenous leukemia. Am J Hematol 2003; 74 (03) 221-223
• 67 Huang J, Xie S, Gao Y. 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 (01) 102
• 68 Akira M, Inoue Y, Arai T. et al; Osaka Respiratory Diseases Symposia Group. Pulmonary fibrosis on high-resolution CT of patients with pulmonary alveolar proteinosis. AJR Am J Roentgenol 2016; 207 (03) 544-551
• 69 Hu-Wang E, Chelala L, Landeras L. et al. Pulmonary alveolar proteinosis-associated pulmonary fibrosis: evolutional changes and radiologic-pathologic correlation. Radiol Cardiothorac Imaging 2023; 5 (05) e230040
• 70 Guirriec Y, Luque-Paz D, Bernard G. et al; OrphaLung network. Pulmonary fibrosis in patients with autoimmune pulmonary alveolar proteinosis: a retrospective nationwide cohort study. ERJ Open Res 2024; 10 (06) 10
• 71 Holbert JM, Costello P, Li W, Hoffman RM, Rogers RM. CT features of pulmonary alveolar proteinosis. AJR Am J Roentgenol 2001; 176 (05) 1287-1294
• 72 Frazier AA, Franks TJ, Cooke EO, Mohammed TL, Pugatch RD, Galvin JR. From the archives of the AFIP: pulmonary alveolar proteinosis. Radiographics 2008; 28 (03) 883-899 , quiz 915
• 73 Mariani F, Infusino C, Lettieri S. et al. Lung fibrosis in Pulmonary Alveolar Proteinosis (PAP): different stages of a syndrome or distinct diseases?: European Respiratory Society; 2021.
• 74 Penugonda M, Lim J, O'Callaghan M. et al. Pulmonary alveolar proteinosis with associated fibrosis: a case series from a national referral centre. Am J Respir Crit Care Med 2025; 211: A6088-A6088
• 75 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) 5774-5783
• 76 Zhang D, Tian X, Feng R. et al. Secondary pulmonary alveolar proteinosis: a single-center retrospective study (a case series and literature review). BMC Pulm Med 2018; 18 (01) 15
• 77 Buechner HA, Ansari A. Acute silico-proteinosis. A new pathologic variant of acute silicosis in sandblasters, characterized by histologic features resembling alveolar proteinosis. Dis Chest 1969; 55 (04) 274-278
• 78 Bomhard EM. Particle-induced pulmonary alveolar proteinosis and subsequent inflammation and fibrosis: a toxicologic and pathologic review. Toxicol Pathol 2017; 45 (03) 389-401
• 79 Ballerie A, Nimubona S, Meunier C. et al. Association of pulmonary alveolar proteinosis and fibrosis: patient with GATA2 deficiency. Eur Respir J 2016; 48 (05) 1510-1514
• 80 Sanges S, Prévotat A, Fertin M. et al. Haemodynamically proven pulmonary hypertension in a patient with GATA2 deficiency-associated pulmonary alveolar proteinosis and fibrosis. Eur Respir J 2017; 49 (05) 49
• 81 Arbiser ZK, Guidot DM, Pine JR, Giltman LI, Gal AA. Pulmonary alveolar proteinosis mimicking idiopathic pulmonary fibrosis. Ann Diagn Pathol 2003; 7 (02) 82-86
• 82 Jones CC. Pulmonary alveolar proteinosis with unusual complicating infections; a report of two cases. Am J Med 1960; 29: 713-722
• 83 Goldstein LS, Kavuru MS, Curtis-McCarthy P, Christie HA, Farver C, Stoller JK. Pulmonary alveolar proteinosis: clinical features and outcomes. Chest 1998; 114 (05) 1357-1362
• 84 Lee KN, Levin DL, Webb WR, Chen D, Storto ML, Golden JA. Pulmonary alveolar proteinosis: high-resolution CT, chest radiographic, and functional correlations. Chest 1997; 111 (04) 989-995
• 85 Godwin JD, Müller NL, Takasugi JE. Pulmonary alveolar proteinosis: CT findings. Radiology 1988; 169 (03) 609-613
• 86 Johkoh T, Itoh H, Müller NL. et al. Crazy-paving appearance at thin-section CT: spectrum of disease and pathologic findings. Radiology 1999; 211 (01) 155-160
• 87 Presneill JJ, Nakata K, Inoue Y, Seymour JF. Pulmonary alveolar proteinosis. Clin Chest Med 2004; 25 (03) 593-613, viii
• 88 Martin RJ, Rogers RM, Myers NM. Pulmonary alveolar proteinosis: shunt fraction and lactic acid dehydrogenase concentration as aids to diagnosis. Am Rev Respir Dis 1978; 117 (06) 1059-1062
• 89 Mo Q, Wang B, Dong N. et al. The clinical clues of pulmonary alveolar proteinosis: a report of 11 cases and literature review. Can Respir J 2016; 2016: 4021928
• 90 Uchida K, Nakata K, Carey B. et al. Standardized serum GM-CSF autoantibody testing for the routine clinical diagnosis of autoimmune pulmonary alveolar proteinosis. J Immunol Methods 2014; 402 (1–2): 57-70
• 91 Kitamura T, Uchida K, Tanaka N. et al. Serological diagnosis of idiopathic pulmonary alveolar proteinosis. Am J Respir Crit Care Med 2000; 162 (2 Pt 1): 658-662
• 92 Schoch OD, Schanz U, Koller M. et al. BAL findings in a patient with pulmonary alveolar proteinosis successfully treated with GM-CSF. Thorax 2002; 57 (03) 277-280
• 93 Milleron BJ, Costabel U, Teschler H. et al. Bronchoalveolar lavage cell data in alveolar proteinosis. Am Rev Respir Dis 1991; 144 (06) 1330-1332
• 94 Suzuki T, Trapnell BC. Pulmonary alveolar proteinosis syndrome. Clin Chest Med 2016; 37 (03) 431-440
• 95 Beccaria M, Luisetti M, Rodi G. et al. Long-term durable benefit after whole lung lavage in pulmonary alveolar proteinosis. Eur Respir J 2004; 23 (04) 526-531
• 96 Campo I, Luisetti M, Griese M. et al; WLL International Study Group. A global survey on whole lung lavage in pulmonary alveolar proteinosis. Chest 2016; 150 (01) 251-253
• 97 Campo I, Luisetti M, Griese M. et al; WLL International Study Group. Whole lung lavage therapy for pulmonary alveolar proteinosis: a global survey of current practices and procedures. Orphanet J Rare Dis 2016; 11 (01) 115
• 98 Cheng SL, Chang HT, Lau HP, Lee LN, Yang PC. Pulmonary alveolar proteinosis: treatment by bronchofiberscopic lobar lavage. Chest 2002; 122 (04) 1480-1485
• 99 Brach BB, Harrell JH, Moser KM. Alveolar proteinosis. Lobar lavage by fiberoptic bronchoscopic technique. Chest 1976; 69 (02) 224-227
• 100 Edis EC, Tabakoglu E, Caglar T, Hatipoglu ON, Cevirme L, Alagol A. Treatment of a primary pulmonary alveolar proteinosis case with severe hypoxaemia by using segmental lavage technique. Ann Acad Med Singap 2007; 36 (10) 871-872
• 101 Sadeghi HA. Segmental lung lavage with fiberoptic bronchoscopy in a patient with special presentation of pulmonary alveolar proteinosis. Tanaffos 2013; 12 (04) 48-52
• 102 Tabata S, Shimoji S, Murase K. et al. Successful allogeneic bone marrow transplantation for myelodysplastic syndrome complicated by severe pulmonary alveolar proteinosis. Int J Hematol 2009; 90 (03) 407-412
• 103 Sato Y, Fukatsu M, Suzuki T. et al. Successful allogeneic hematopoietic stem cell transplantation for myelodysplastic neoplasms complicated with secondary pulmonary alveolar proteinosis and Behçet's disease harboring GATA2 mutation. Int J Hematol 2023; 118 (05) 642-646
• 104 Basheer A, Padrao EMH, Huh K, Parker S, Shah T, Gerardi DA. Pulmonary alveolar proteinosis due to familial myelodysplastic syndrome with resolution after stem cell transplant. Autops Case Rep 2022; 12: e2021382
• 105 van Lier YF, de Bree GJ, Jonkers RE. et al. Allogeneic hematopoietic cell transplantation in the management of GATA2 deficiency and pulmonary alveolar proteinosis. Clin Immunol 2020; 218: 108522
• 106 Tanaka-Kubota M, Shinozaki K, Miyamoto S. et al. Hematopoietic stem cell transplantation for pulmonary alveolar proteinosis associated with primary immunodeficiency disease. Int J Hematol 2018; 107 (05) 610-614
• 107 Zhang T, Li M, Tan L, Li X. Pulmonary alveolar proteinosis induced by X-linked agammaglobulinemia: a case report. World J Clin Cases 2024; 12 (09) 1644-1648
• 108 Tejwani D, Delacruz AE, Niazi M, Diaz-Fuentes G. Unsuspected pulmonary alveolar proteinosis in a patient with acquired immunodeficiency syndrome: a case report. J Med Case Rep 2011; 5: 46
• 109 Lin KP, Sheng WH, Wang CP, Chang YL, Chang SC. Resolution of secondary pulmonary alveolar proteinosis following treatment of rhinocerebral aspergillosis. Int J Infect Dis 2010; 14 (Suppl. 03) e246-e249
• 110 Kobayashi K, Ohkouchi S, Sasahara Y. et al. Improvement of native pulmonary alveolar proteinosis after contralateral single living-donor lobar lung transplantation: a case report. Pediatr Transplant 2020; 24 (02) e13659
• 111 Lawi D, Dubruc E, Gonzalez M, Aubert JD, Soccal PM, Janssens JP. Secondary pulmonary alveolar proteinosis treated by lung transplant: a case report. Respir Med Case Rep 2020; 30: 101108
• 112 Roy C, Allou N, Coulomb A. et al. Successful lung transplantation in genetic methionyl-tRNA synthetase-related alveolar proteinosis/lung fibrosis without recurrence under methionine supplementation: medium-term outcome in 4 cases. Am J Transplant 2024; 24 (07) 1317-1322
• 113 Yokomura K, Chida K, Suda T. et al. [Secondary pulmonary alveolar proteinosis associated with myelodysplastic syndrome]. Nihon Kokyuki Gakkai Zasshi 2002; 40 (07) 599-604
• 114 Tsukahara K, Lindell RB, Newman H, Lerman BJ, Kersun LS, Piccione J. Successful whole lung lavage in a child with pulmonary alveolar proteinosis secondary to hematologic malignancy. Pediatr Pulmonol 2022; 57 (01) 308-310
• 115 Hadchouel A, Drummond D, Pontoizeau C. et al. Methionine supplementation for multi-organ dysfunction in MetRS-related pulmonary alveolar proteinosis. Eur Respir J 2022; 59 (04) 59
• 116 Lenz D, Stahl M, Seidl E. et al. Rescue of respiratory failure in pulmonary alveolar proteinosis due to pathogenic MARS1 variants. Pediatr Pulmonol 2020; 55 (11) 3057-3066
• 117 O'Callaghan M, Helly F, Tarling E, Keane MP, McCarthy C. Methionine supplementation: potential for improving alveolar macrophage function through reverse cholesterol transport?. Eur Respir J 2022; 59 (05) 59