Skip to main content

Pulmonary Alveolar Proteinosis Versus Pulmonary Edema

  • Chapter
  • First Online:
Practical Lung Pathology

Part of the book series: Practical Anatomic Pathology ((PAP))

  • 606 Accesses

Abstract

Pulmonary alveolar proteinosis (PAP) and pulmonary edema are both acute to subacute pulmonary conditions that result in bilateral hazy opacities on chest X-ray and ground-glass opacities on chest computed tomography (CT). However, pulmonary edema is typically secondary to an underlying illness or condition such as congestive heart failure, while PAP is an autoimmune in nature in greater than 90% of cases. On histopathology, pulmonary edema can resemble PAP due to the presence of frothy, eosinophilic material within alveolar spaces. However, PAP can be readily distinguished by cytology or surgical biopsy based on the features including the periodic acid-Schiff (PAS)-positive nature of the proteinaceous material, lack of inflammatory infiltrate, and presence of cholesterol clefts. Distinguishing these two entities is important due to their vastly different treatment regimens with PAP requiring whole lung lavage in moderate to severe cases versus the use of diuretics and treatment of the underlying conditions in pulmonary edema.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

eBook
USD 16.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Inoue Y, Trapnell BC, Tazawa R, Arai T, Takada T, Hizawa N, et al. Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan. Am J Respir Crit Care Med. 2008;177(7):752–62.

    Article  PubMed  PubMed Central  Google Scholar 

  2. Seymour JF, Presneill JJ. Pulmonary alveolar proteinosis: progress in the first 44 years. Am J Respir Crit Care Med. 2002;166(2):215–35.

    Article  PubMed  Google Scholar 

  3. Shah PL, Hansell D, Lawson PR, Reid KB, Morgan C. Pulmonary alveolar proteinosis: clinical aspects and current concepts on pathogenesis. Thorax. 2000;55(1):67–77.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Holbert JM, Costello P, Li W, Hoffman RM, Rogers RM. CT features of pulmonary alveolar proteinosis. AJR Am J Roentgenol. 2001;176(5):1287–94.

    Article  CAS  PubMed  Google Scholar 

  5. Johkoh T, Itoh H, Muller NL, Ichikado K, Nakamura H, Ikezoe J, et al. Crazy-paving appearance at thin-section CT: spectrum of disease and pathologic findings. Radiology. 1999;211(1):155–60.

    Article  CAS  PubMed  Google Scholar 

  6. Chou CW, Lin FC, Tung SM, Liou RD, Chang SC. Diagnosis of pulmonary alveolar proteinosis: usefulness of papanicolaou-stained smears of bronchoalveolar lavage fluid. Arch Intern Med. 2001;161(4):562–6.

    Article  CAS  PubMed  Google Scholar 

  7. Prakash UB, Barham SS, Carpenter HA, Dines DE, Marsh HM. Pulmonary alveolar phospholipoproteinosis: experience with 34 cases and a review. Mayo Clin Proc. 1987;62(6):499–518.

    Article  CAS  PubMed  Google Scholar 

  8. Wang BM, Stern EJ, Schmidt RA, Pierson DJ. Diagnosing pulmonary alveolar proteinosis. A review and an update. Chest. 1997;111(2):460–6.

    Article  CAS  PubMed  Google Scholar 

  9. Alberti A, Luisetti M, Braschi A, Rodi G, Iotti G, Sella D, et al. Bronchoalveolar lavage fluid composition in alveolar proteinosis. Early changes after therapeutic lavage. Am J Respir Crit Care Med. 1996;154(3 Pt 1):817–20.

    Article  CAS  PubMed  Google Scholar 

  10. Poletti V, Costabel U, Casoni GL, Bigliazzi C, Drent M, Olivieri D. Rare infiltrative lung diseases: a challenge for clinicians. Respiration. 2004;71(5):431–43.

    Article  PubMed  Google Scholar 

  11. Uchida K, Nakata K, Trapnell BC, Terakawa T, Hamano E, Mikami A, et al. High-affinity autoantibodies specifically eliminate granulocyte-macrophage colony-stimulating factor activity in the lungs of patients with idiopathic pulmonary alveolar proteinosis. Blood. 2004;103(3):1089–98.

    Article  CAS  PubMed  Google Scholar 

  12. Kumar A, Abdelmalak B, Inoue Y, Culver DA. Pulmonary alveolar proteinosis in adults: pathophysiology and clinical approach. Lancet Respir Med. 2018;6(7):554–65.

    Article  CAS  PubMed  Google Scholar 

  13. Lin FC, Chang GD, Chern MS, Chen YC, Chang SC. Clinical significance of anti-GM-CSF antibodies in idiopathic pulmonary alveolar proteinosis. Thorax. 2006;61(6):528–34.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Rosen SH, Castleman B, Liebow AA. Pulmonary alveolar proteinosis. N Engl J Med. 1958;258(23):1123–42.

    Article  CAS  PubMed  Google Scholar 

  15. Dranoff G, Crawford AD, Sadelain M, Ream B, Rashid A, Bronson RT, et al. Involvement of granulocyte-macrophage colony-stimulating factor in pulmonary homeostasis. Science. 1994;264(5159):713–6.

    Article  CAS  PubMed  Google Scholar 

  16. Kitamura T, Tanaka N, Watanabe J, Uchida, Kanegasaki S, Yamada Y, et al. Idiopathic pulmonary alveolar proteinosis as an autoimmune disease with neutralizing antibody against granulocyte/macrophage colony-stimulating factor. J Exp Med. 1999;190(6):875–80.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. Chaulagain CP, Pilichowska M, Brinckerhoff L, Tabba M, Erban JK. Secondary pulmonary alveolar proteinosis in hematologic malignancies. Hematol Oncol Stem Cell Ther. 2014;7(4):127–35.

    Article  PubMed  Google Scholar 

  18. Leth S, Bendstrup E, Vestergaard H, Hilberg O. Autoimmune pulmonary alveolar proteinosis: treatment options in year 2013. Respirology. 2013;18(1):82–91.

    Article  PubMed  Google Scholar 

  19. Campo I, Luisetti M, Griese M, Trapnell BC, Bonella F, Grutters J, et al. Whole lung lavage therapy for pulmonary alveolar proteinosis: a global survey of current practices and procedures. Orphanet J Rare Dis. 2016;11(1):115.

    Article  PubMed  PubMed Central  Google Scholar 

  20. Takaki M, Tanaka T, Komohara Y, Tsuchihashi Y, Mori D, Hayashi K, et al. Recurrence of pulmonary alveolar proteinosis after bilateral lung transplantation in a patient with a nonsense mutation in CSF2RB. Respir Med Case Rep. 2016;19:89–93.

    PubMed  PubMed Central  Google Scholar 

  21. Santamaria F, Brancaccio G, Parenti G, Francalanci P, Squitieri C, Sebastio G, et al. Recurrent fatal pulmonary alveolar proteinosis after heart-lung transplantation in a child with lysinuric protein intolerance. J Pediatr. 2004;145(2):268–72.

    Article  PubMed  Google Scholar 

  22. Philippot Q, Cazes A, Borie R, Debray MP, Danel C, Hurtado Nedelec M, et al. Secondary pulmonary alveolar proteinosis after lung transplantation: a single-centre series. Eur Respir J. 2017;49(2):1601369.

    Article  PubMed  Google Scholar 

  23. Albores J, Seki A, Fishbein MC, Abtin F, Lynch JP III, Wang T, et al. A rare occurrence of pulmonary alveolar proteinosis after lung transplantation. Semin Respir Crit Care Med. 2013;34(3):431–8.

    Article  PubMed  Google Scholar 

  24. Rosen LB, Rocha Pereira N, Figueiredo C, Fiske LC, Ressner RA, Hong JC, et al. Nocardia-induced granulocyte macrophage colony-stimulating factor is neutralized by autoantibodies in disseminated/extrapulmonary nocardiosis. Clin Infect Dis. 2015;60(7):1017–25.

    Article  CAS  PubMed  Google Scholar 

  25. Akira M, Inoue Y, Arai T, Sugimoto C, Tokura S, Nakata K, et al. Pulmonary fibrosis on high-resolution CT of patients with pulmonary alveolar proteinosis. AJR Am J Roentgenol. 2016;207(3):544–51.

    Article  PubMed  Google Scholar 

  26. Goldstein LS, Kavuru MS, Curtis-McCarthy P, Christie HA, Farver C, Stoller JK. Pulmonary alveolar proteinosis: clinical features and outcomes. Chest. 1998;114(5):1357–62.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Chen Zhang .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2022 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Trevino, K.E., Zhang, C. (2022). Pulmonary Alveolar Proteinosis Versus Pulmonary Edema. In: Xu, H., Ricciotti, R.W., Mantilla, J.G. (eds) Practical Lung Pathology. Practical Anatomic Pathology. Springer, Cham. https://doi.org/10.1007/978-3-031-14402-8_45

Download citation

  • DOI: https://doi.org/10.1007/978-3-031-14402-8_45

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-031-14401-1

  • Online ISBN: 978-3-031-14402-8

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics