Pulmonary alveolar proteinosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mahda Alihashemi M.D. [2] [3] [4]

Synonyms and keywords:Pulmonary alveolar phospholipoproteinosis

Overview

Pulmonary alveolar proteinosis ( PAP) was first discovered by Samuel Rosen, Benjamin Castleman, and Averill Liebow, in 1958. Pulmonary alveolar proteinosis ( PAP) may be classified into 2 subtypes, primary and secondary pulmonary alveolar proteinosis. The pathogenesis of pulmonary alveolar proteinosis is characterized by the intraalveolar accumulation of surfactant phospholipid and apoproteins. Pulmonary alveolar proteinosis develop because of reduced granulocyte-macrophage colony-stimulating factor (GM-CSF) levels or function and/or impaired alveolar macrophage function. On gross pathology after lung washings, fluid with the milky composition are the characteristic finding of pulmonary alveolar proteinosis. On microscopic histopathological analysis, terminal bronchioles and alveoli are filled with a lipoproteinaceous material that will be pink after periodic acid-Schiff (PAS) stain. The exact etiology of pulmonary alveolar proteinosis (PAP) is unknown, but it may be associated with exposure to insecticides, hematologic malignancies, or HIV infection. Pulmonary alveolar proteinosis must be differentiated from other diseases that cause ground glass opacities in radiography. Pulmonary alveolar proteinosis is more commonly observed among patients aged 40- 50 years old. Men are more commonly affected with pulmonary alveolar proteinosis than women. Common risk factors in the development of pulmonary alveolar proteinosis are current or former cigarette smokers and HIV infection. If left untreated, patients with pulmonary alveolar proteinosis may progress to develop pulmonary fibrosis, cor pulmonale. Symptoms of pulmonary alveolar proteinosis may include dyspnea on exertion, cough. The physical examination is often normal. Laboratory findings are serum anti-granulocyte-macrophage colony-stimulating factor (GM-CSF) antibodies in autoimmune pulmonary alveolar proteinosis and elevated serum level of GM-CSF in hereditary pulmonary alveolar proteinosis. Imaging studies include chest x-ray and high resolution computed tomography (HRCT). Treatment include whole-lung lavage (WLL), recombinant GM-CSF and lung transplantation.

Historical Perspective

  • Pulmonary alveolar proteinosis ( PAP) was first discovered by Samuel Rosen, Benjamin Castleman, and Averill Liebow, in 1958, during pathologic investigations of material filling the alveoli. [1]
  • In 1960, the first therapeutic bronchoalveolar lavage by repeated segmental flooding was developed by Dr. Jose Ramirez-Rivera to treat pulmonary alveolar proteinosis. [2]

Classification

Pulmonary alveolar proteinosis ( PAP) may be classified into 2 subtypes:

Primary Pulmonary alveolar proteinosis:
Secondary pulmonary alveolar proteinosis:

Pathophysiology

Causes

The exact etiology of pulmonary alveolar proteinosis (PAP) is unknown, but it may be associated with:

Differentiating pulmonary alveolar proteinosis from other Diseases

  • Pulmonary alveolar proteinosis must be differentiated from other diseases that cause ground glass opacities in radiography such as:[17]

Epidemiology and Demographics

  • The prevalence of pulmonary alveolar proteinosis is approximately 1 per 100,000 individuals worldwide.
  • The incidence of pulmonary alveolar proteinosis is estimated to be 0.33 cases per 100,000 individuals in united states. [18]

Age

  • Pulmonary alveolar proteinosis is more commonly observed among patients aged 40- 50 years old.[19]

Gender

  • Men are more commonly affected with pulmonary alveolar proteinosis than women. The men to women ratio is approximately 2 to 1.

Race

  • There is no racial predilection for pulmonary alveolar protoeinosis.

Risk Factors

  • Common risk factors in the development of pulmonary alveolar proteinosis are:
    • Current or former cigarette smokers[20]
    • HIV infection[15]

Natural History, Complications and Prognosis

  • Prognosis is generally good for primary pulmonary alveolar proteinosis with whole lung lavage. Congenital pulmonary alveolar proteinosis respond well to lung transplantation. Prognosis for secondary pulmonary alveolar proteinosis is related to underlying etiologies.

Diagnosis

Symptoms

  • One third of patients are usually asymptomatic.[22]
  • Symptoms of pulmonary alveolar proteinosis may include the following:[23]

Physical Examination

  • The physical examination is often normal. [24]
  • Physical examination may be remarkable for:[25][26]

Laboratory Findings

Imaging Findings

Chest X-ray:
High resolution computed tomography (HRCT):
  • Ground-glass opacification with crazy-paving pattern (interlobular septal thickening).[34][35]

Other Diagnostic Studies

Pulmonary function test:
 Bronchoalveolar lavage (BAL):
  • It is done via flexible bronchoscopy.
  • Milky or opaque appearance because of abundant lipoproteinaceous material.[38]
Surgical lung biopsy:
  • It is done via video-assisted thoracoscopic biopsy.[39]

Treatment

Interventional therapy

  • Whole-lung lavage (WLL) with normal saline under general anesthesia: [40]
    • Indication:[41]
      • Resting PaO2 <65 mmHg
      • Alveolar-arterial O2 gradient ≥40 mmHg at rest
      • Severe dyspnea
      • Hypoxemia 
    • Contraindication:[42]
      • Clotting disorders
      • Anesthetic risks
      • Cardiopulmonary instability

Medical therapy

  • Recombinant GM-CSF ( sargramostim) by inhaled or subcutenous injections:[43]
    • For patients with failed whole-lung lavage (WLL)
  • Rituximab ( anti-CD20 monoclonal antibody): for refractory autoimmune pulmonary alveolar proteinosis.[44]
  • Therapeutic plasma exchange: for patients with failed whole lung lavage.[45]

Surgery

Prevention

  • Avoiding further exposure is the effective measure for the secondary prevention of pulmonary alveolar proteinosis due to inorganic dust or insecticides.[47]

References

  1. ↑ Rosen, Samuel H.; Castleman, Benjamin; Liebow, Averill A.; Enzinger, Frank M.; Hunt, Richard T. N. (1958). "Pulmonary Alveolar Proteinosis". New England Journal of Medicine. 258 (23): 1123–1142. doi:10.1056/NEJM195806052582301. ISSN 0028-4793.
  2. ↑ Ramirez-R., Jose; Nyka, Walenty; McLaughlin, Joseph (1963). "Pulmonary Alveolar Proteinosis". New England Journal of Medicine. 268 (4): 165–171. doi:10.1056/NEJM196301242680401. ISSN 0028-4793.
  3. ↑ Suzuki T, Trapnell BC (September 2016). "Pulmonary Alveolar Proteinosis Syndrome". Clin. Chest Med. 37 (3): 431–40. doi:10.1016/j.ccm.2016.04.006. PMID 27514590.
  4. ↑ Brasch F, Birzele J, Ochs M, Guttentag SH, Schoch OD, Boehler A, Beers MF, Müller KM, Hawgood S, Johnen G (September 2004). "Surfactant proteins in pulmonary alveolar proteinosis in adults". Eur. Respir. J. 24 (3): 426–35. doi:10.1183/09031936.04.00076403. PMID 15358702.
  5. ↑ Mulugeta S, Gray JM, Notarfrancesco KL, Gonzales LW, Koval M, Feinstein SI, Ballard PL, Fisher AB, Shuman H (June 2002). "Identification of LBM180, a lamellar body limiting membrane protein of alveolar type II cells, as the ABC transporter protein ABCA3". J. Biol. Chem. 277 (25): 22147–55. doi:10.1074/jbc.M201812200. PMID 11940594.
  6. ↑ Salerno T, Peca D, Menchini L, Schiavino A, Petreschi F, Occasi F, Cogo P, Danhaive O, Cutrera R (March 2014). "Respiratory insufficiency in a newborn with congenital hypothyroidism due to a new mutation of TTF-1/NKX2.1 gene". Pediatr. Pulmonol. 49 (3): E42–4. doi:10.1002/ppul.22788. PMID 23997037.
  7. ↑ "GeneReviews® - NCBI Bookshelf".
  8. ↑ Hadchouel A, Wieland T, Griese M, Baruffini E, Lorenz-Depiereux B, Enaud L, Graf E, Dubus JC, Halioui-Louhaichi S, Coulomb A, Delacourt C, Eckstein G, Zarbock R, Schwarzmayr T, Cartault F, Meitinger T, Lodi T, de Blic J, Strom TM (May 2015). "Biallelic Mutations of Methionyl-tRNA Synthetase Cause a Specific Type of Pulmonary Alveolar Proteinosis Prevalent on Réunion Island". Am. J. Hum. Genet. 96 (5): 826–31. doi:10.1016/j.ajhg.2015.03.010. PMC 4570277. PMID 25913036.
  9. ↑ 9.0 9.1 Buechner HA, Ansari A (April 1969). "Acute silico-proteinosis. A new pathologic variant of acute silicosis in sandblasters, characterized by histologic features resembling alveolar proteinosis". Dis Chest. 55 (4): 274–8. PMID 5775743.
  10. ↑ 10.0 10.1 Cordonnier C, Fleury-Feith J, Escudier E, Atassi K, Bernaudin JF (March 1994). "Secondary alveolar proteinosis is a reversible cause of respiratory failure in leukemic patients". Am. J. Respir. Crit. Care Med. 149 (3 Pt 1): 788–94. doi:10.1164/ajrccm.149.3.8118651. PMID 8118651.
  11. ↑ Sharma S, Nadrous HF, Peters SG, Tefferi A, Litzow MR, Aubry MC, Afessa B (September 2005). "Pulmonary complications in adult blood and marrow transplant recipients: autopsy findings". Chest. 128 (3): 1385–92. doi:10.1378/chest.128.3.1385. PMID 16162733.
  12. ↑ Carey B, Trapnell BC (May 2010). "The molecular basis of pulmonary alveolar proteinosis". Clin. Immunol. 135 (2): 223–35. doi:10.1016/j.clim.2010.02.017. PMC 2866141. PMID 20338813.
  13. ↑ Mikami T, Yamamoto Y, Yokoyama M, Okayasu I (December 1997). "Pulmonary alveolar proteinosis: diagnosis using routinely processed smears of bronchoalveolar lavage fluid". J. Clin. Pathol. 50 (12): 981–4. PMC 500376. PMID 9516877.
  14. ↑ Maygarden SJ, Iacocca MV, Funkhouser WK, Novotny DB (June 2001). "Pulmonary alveolar proteinosis: a spectrum of cytologic, histochemical, and ultrastructural findings in bronchoalveolar lavage fluid". Diagn. Cytopathol. 24 (6): 389–95. PMID 11391819.
  15. ↑ 15.0 15.1 Juvet SC, Hwang D, Waddell TK, Downey GP (2008). "Rare lung disease II: pulmonary alveolar proteinosis". Can. Respir. J. 15 (4): 203–10. PMC 2677953. PMID 18551202.
  16. ↑ Wardwell NR, Miller R, Ware LB (September 2006). "Pulmonary alveolar proteinosis associated with a disease-modifying antirheumatoid arthritis drug". Respirology. 11 (5): 663–5. doi:10.1111/j.1440-1843.2006.00905.x. PMID 16916345.
  17. ↑ Patel SM, Sekiguchi H, Reynolds JP, Krowka MJ (2012). "Pulmonary alveolar proteinosis". Can. Respir. J. 19 (4): 243–5. PMC 3411387. PMID 22891182.
  18. ↑ Hunt S, Miller AL, Schissel S, Ross JJ (December 2010). "A crazy cause of dyspnea". N. Engl. J. Med. 363 (25): e38. doi:10.1056/NEJMimc1008281. PMID 21158654.
  19. ↑ Inoue Y, Trapnell BC, Tazawa R, Arai T, Takada T, Hizawa N, Kasahara Y, Tatsumi K, Hojo M, Ichiwata T, Tanaka N, Yamaguchi E, Eda R, Oishi K, Tsuchihashi Y, Kaneko C, Nukiwa T, Sakatani M, Krischer JP, Nakata K (April 2008). "Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan". Am. J. Respir. Crit. Care Med. 177 (7): 752–62. doi:10.1164/rccm.200708-1271OC. PMC 2720118. PMID 18202348.
  20. ↑ Suzuki T, Trapnell BC (September 2016). "Pulmonary Alveolar Proteinosis Syndrome". Clin. Chest Med. 37 (3): 431–40. doi:10.1016/j.ccm.2016.04.006. PMID 27514590.
  21. ↑ Abdul Rahman JA, Moodley YP, Phillips MJ (August 2004). "Pulmonary alveolar proteinosis associated with psoriasis and complicated by mycobacterial infection: successful treatment with granulocyte-macrophage colony stimulating factor after a partial response to whole lung lavage". Respirology. 9 (3): 419–22. PMID 15497254.
  22. ↑ Inoue Y, Trapnell BC, Tazawa R, Arai T, Takada T, Hizawa N, Kasahara Y, Tatsumi K, Hojo M, Ichiwata T, Tanaka N, Yamaguchi E, Eda R, Oishi K, Tsuchihashi Y, Kaneko C, Nukiwa T, Sakatani M, Krischer JP, Nakata K (April 2008). "Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan". Am. J. Respir. Crit. Care Med. 177 (7): 752–62. doi:10.1164/rccm.200708-1271OC. PMC 2720118. PMID 18202348.
  23. ↑ Suzuki T, Trapnell BC (September 2016). "Pulmonary Alveolar Proteinosis Syndrome". Clin. Chest Med. 37 (3): 431–40. doi:10.1016/j.ccm.2016.04.006. PMID 27514590.
  24. ↑ Suzuki T, Trapnell BC (September 2016). "Pulmonary Alveolar Proteinosis Syndrome". Clin. Chest Med. 37 (3): 431–40. doi:10.1016/j.ccm.2016.04.006. PMID 27514590.
  25. ↑ Goldstein LS, Kavuru MS, Curtis-McCarthy P, Christie HA, Farver C, Stoller JK (November 1998). "Pulmonary alveolar proteinosis: clinical features and outcomes". Chest. 114 (5): 1357–62. PMID 9824014.
  26. ↑ Inoue Y, Trapnell BC, Tazawa R, Arai T, Takada T, Hizawa N, Kasahara Y, Tatsumi K, Hojo M, Ichiwata T, Tanaka N, Yamaguchi E, Eda R, Oishi K, Tsuchihashi Y, Kaneko C, Nukiwa T, Sakatani M, Krischer JP, Nakata K (April 2008). "Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan". Am. J. Respir. Crit. Care Med. 177 (7): 752–62. doi:10.1164/rccm.200708-1271OC. PMC 2720118. PMID 18202348.
  27. ↑ Suzuki T, Trapnell BC (September 2016). "Pulmonary Alveolar Proteinosis Syndrome". Clin. Chest Med. 37 (3): 431–40. doi:10.1016/j.ccm.2016.04.006. PMID 27514590.
  28. ↑ Suzuki T, Trapnell BC (September 2016). "Pulmonary Alveolar Proteinosis Syndrome". Clin. Chest Med. 37 (3): 431–40. doi:10.1016/j.ccm.2016.04.006. PMID 27514590.
  29. ↑ Martin RJ, Rogers RM, Myers NM (June 1978). "PUlmonary alveolar proteinosis: shunt fraction and lactic acid dehydrogenase concentration as aids to diagnosis". Am. Rev. Respir. Dis. 117 (6): 1059–62. doi:10.1164/arrd.1978.117.6.1059. PMID 666104.
  30. ↑ Suzuki T, Trapnell BC (September 2016). "Pulmonary Alveolar Proteinosis Syndrome". Clin. Chest Med. 37 (3): 431–40. doi:10.1016/j.ccm.2016.04.006. PMID 27514590.
  31. ↑ Patel SM, Sekiguchi H, Reynolds JP, Krowka MJ (2012). "Pulmonary alveolar proteinosis". Can. Respir. J. 19 (4): 243–5. PMC 3411387. PMID 22891182.
  32. ↑ Prakash UB, Barham SS, Carpenter HA, Dines DE, Marsh HM (June 1987). "Pulmonary alveolar phospholipoproteinosis: experience with 34 cases and a review". Mayo Clin. Proc. 62 (6): 499–518. PMID 3553760.
  33. ↑ Hudson AR, Halprin GM, Miller JA, Kilburn KH (June 1974). "Pulmonary interstitial fibrosis following alveolar proteinosis". Chest. 65 (6): 700–2. PMID 4832278.
  34. ↑ Patel SM, Sekiguchi H, Reynolds JP, Krowka MJ (2012). "Pulmonary alveolar proteinosis". Can. Respir. J. 19 (4): 243–5. PMC 3411387. PMID 22891182.
  35. ↑ Godwin JD, Müller NL, Takasugi JE (December 1988). "Pulmonary alveolar proteinosis: CT findings". Radiology. 169 (3): 609–13. doi:10.1148/radiology.169.3.3186983. PMID 3186983.
  36. ↑ Inoue Y, Trapnell BC, Tazawa R, Arai T, Takada T, Hizawa N, Kasahara Y, Tatsumi K, Hojo M, Ichiwata T, Tanaka N, Yamaguchi E, Eda R, Oishi K, Tsuchihashi Y, Kaneko C, Nukiwa T, Sakatani M, Krischer JP, Nakata K (April 2008). "Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan". Am. J. Respir. Crit. Care Med. 177 (7): 752–62. doi:10.1164/rccm.200708-1271OC. PMC 2720118. PMID 18202348.
  37. ↑ Seymour JF, Presneill JJ (July 2002). "Pulmonary alveolar proteinosis: progress in the first 44 years". Am. J. Respir. Crit. Care Med. 166 (2): 215–35. doi:10.1164/rccm.2109105. PMID 12119235.
  38. ↑ Bonella F, Bauer PC, Griese M, Ohshimo S, Guzman J, Costabel U (December 2011). "Pulmonary alveolar proteinosis: new insights from a single-center cohort of 70 patients". Respir Med. 105 (12): 1908–16. doi:10.1016/j.rmed.2011.08.018. PMID 21900000.
  39. ↑ Inoue Y, Trapnell BC, Tazawa R, Arai T, Takada T, Hizawa N, Kasahara Y, Tatsumi K, Hojo M, Ichiwata T, Tanaka N, Yamaguchi E, Eda R, Oishi K, Tsuchihashi Y, Kaneko C, Nukiwa T, Sakatani M, Krischer JP, Nakata K (April 2008). "Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan". Am. J. Respir. Crit. Care Med. 177 (7): 752–62. doi:10.1164/rccm.200708-1271OC. PMC 2720118. PMID 18202348.
  40. ↑ Zhao YY, Huang H, Liu YZ, Song XY, Li S, Xu ZJ (October 2015). "Whole Lung Lavage Treatment of Chinese Patients with Autoimmune Pulmonary Alveolar Proteinosis: A Retrospective Long-term Follow-up Study". Chin. Med. J. 128 (20): 2714–9. doi:10.4103/0366-6999.167295. PMC 4736873. PMID 26481735.
  41. ↑ Campo, Ilaria; Luisetti, Maurizio; Griese, Matthias; Trapnell, Bruce C.; Bonella, Francesco; Grutters, Jan; Nakata, Koh; Van Moorsel, Coline H. M.; Costabel, Ulrich; Cottin, Vincent; Ichiwata, Toshio; Inoue, Yoshikazu; Braschi, Antonio; Bonizzoni, Giacomo; Iotti, Giorgio A.; Tinelli, Carmine; Rodi, Giuseppe (2016). "Whole lung lavage therapy for pulmonary alveolar proteinosis: a global survey of current practices and procedures". Orphanet Journal of Rare Diseases. 11 (1). doi:10.1186/s13023-016-0497-9. ISSN 1750-1172.
  42. ↑ Michaud, Gaëtane; Reddy, Chakravarthy; Ernst, Armin (2009). "Whole-Lung Lavage for Pulmonary Alveolar Proteinosis". Chest. 136 (6): 1678–1681. doi:10.1378/chest.09-2295. ISSN 0012-3692.
  43. ↑ Kavuru MS, Sullivan EJ, Piccin R, Thomassen MJ, Stoller JK (April 2000). "Exogenous granulocyte-macrophage colony-stimulating factor administration for pulmonary alveolar proteinosis". Am. J. Respir. Crit. Care Med. 161 (4 Pt 1): 1143–8. doi:10.1164/ajrccm.161.4.9906044. PMID 10764303.
  44. ↑ Borie R, Debray MP, Laine C, Aubier M, Crestani B (June 2009). "Rituximab therapy in autoimmune pulmonary alveolar proteinosis". Eur. Respir. J. 33 (6): 1503–6. doi:10.1183/09031936.00160908. PMID 19483052.
  45. ↑ Luisetti M, Rodi G, Perotti C, Campo I, Mariani F, Pozzi E, Trapnell BC (May 2009). "Plasmapheresis for treatment of pulmonary alveolar proteinosis". Eur. Respir. J. 33 (5): 1220–2. doi:10.1183/09031936.00097508. PMID 19407056.
  46. ↑ Patel SM, Sekiguchi H, Reynolds JP, Krowka MJ (2012). "Pulmonary alveolar proteinosis". Can. Respir. J. 19 (4): 243–5. PMC 3411387. PMID 22891182.
  47. ↑ Patel SM, Sekiguchi H, Reynolds JP, Krowka MJ (2012). "Pulmonary alveolar proteinosis". Can. Respir. J. 19 (4): 243–5. PMC 3411387. PMID 22891182.


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