eMedicine Specialties > Radiology > Chest

Alveolar Proteinosis: Imaging

Author: Michael B Gotway, MD, Clinical Associate Professor of Diagnostic Radiology and Pulmonary/Critical Care Medicine, University of California at San Francisco School of Medicine; Consulting Staff, Scottsdale Medical Imaging, Ltd
Coauthor(s): Theodore J Lee, MD, Chief, Thoracic Radiology, San Francisco General Hospital; Assistant Professor of Clinical Radiology, University of California at San Francisco
Contributor Information and Disclosures

Updated: Dec 30, 2008

Radiography



Frontal chest radiograph from a patient with pulm...

Frontal chest radiograph from a patient with pulmonary alveolar proteinosis. This image demonstrates bilateral perihilar and infrahilar ground-glass opacity without evidence of mediastinal widening, pleural effusion, or adenopathy.

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Frontal chest radiograph from a patient with pulm...

Frontal chest radiograph from a patient with pulmonary alveolar proteinosis. This image demonstrates bilateral perihilar and infrahilar ground-glass opacity without evidence of mediastinal widening, pleural effusion, or adenopathy.


Frontal chest radiograph in a patient with pulmon...

Frontal chest radiograph in a patient with pulmonary alveolar proteinosis. This image reveals bilateral air-space opacity without evidence of effusion or mediastinal widening. A faintly reticular pattern is present, representing thickened, interlobular septa.

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Frontal chest radiograph in a patient with pulmon...

Frontal chest radiograph in a patient with pulmonary alveolar proteinosis. This image reveals bilateral air-space opacity without evidence of effusion or mediastinal widening. A faintly reticular pattern is present, representing thickened, interlobular septa.


Frontal chest radiograph in a patient with pulmon...

Frontal chest radiograph in a patient with pulmonary alveolar proteinosis that was subsequently treated with bronchoalveolar lavage. This image reveals bilateral symmetric air-space opacity without pleural effusion or mediastinal widening.

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Frontal chest radiograph in a patient with pulmon...

Frontal chest radiograph in a patient with pulmonary alveolar proteinosis that was subsequently treated with bronchoalveolar lavage. This image reveals bilateral symmetric air-space opacity without pleural effusion or mediastinal widening.


Chest radiograph in a patient after bronchoalveol...

Chest radiograph in a patient after bronchoalveolar lavage. This image demonstrates improvement in the bilateral air-space opacity. These opacities subsequently cleared completely, and the patient has remained in remission.

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Chest radiograph in a patient after bronchoalveol...

Chest radiograph in a patient after bronchoalveolar lavage. This image demonstrates improvement in the bilateral air-space opacity. These opacities subsequently cleared completely, and the patient has remained in remission.


Frontal chest radiograph in a patient with the di...

Frontal chest radiograph in a patient with the diffuse form of bronchioloalveolar carcinoma. This image shows the presence of bilateral hazy, ground-glass attenuation, a normal mediastinal width, and no pleural effusion. This appearance resembles alveolar proteinosis.

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Frontal chest radiograph in a patient with the di...

Frontal chest radiograph in a patient with the diffuse form of bronchioloalveolar carcinoma. This image shows the presence of bilateral hazy, ground-glass attenuation, a normal mediastinal width, and no pleural effusion. This appearance resembles alveolar proteinosis.


Findings

The classic chest radiographic finding of PAP is bilateral symmetric air-space opacity, which appears either as ground-glass or frank consolidation, with a perihilar or basilar predominance.

  • Air bronchograms are uncommon.
  • Radiographic opacities are often vaguely nodular and may be accompanied by fine linear opacities or reticulation. Serial radiographs may demonstrate persistence of this pattern over time, resulting in a more limited differential diagnosis of chronic air-space opacity.
  • Unlike in hydrostatic pulmonary edema, the mediastinum is not widened, the heart is not enlarged, and pleural effusions are not common.
  • Adenopathy is not a feature of the disease.
  • Atypical manifestations include a linear or reticular pattern that is unaccompanied by air-space opacity, lower lobe predominant consolidation, or poorly defined nodules as the primary radiographic manifestation of disease.

The differential diagnosis of pulmonary alveolar proteinosis (PAP) on chest radiographs includes all entities that result in diffuse air-space disease, such as hydrostatic and noncardiogenic pulmonary edema, diffuse infections (particularly Pneumocystis pneumonia), pulmonary hemorrhage, interstitial pneumonia, the diffuse form of bronchioloalveolar carcinoma, toxic lung injuries and hypersensitivity pneumonitis, eosinophilic pneumonia, drug or radiation-induced pulmonary disease, and lipoid pneumonia.

Chest radiograph60 and CT scan61 findings of treated PAP have also been described.

  • The initial radiographs after bronchoalveolar lavage (BAL) reveal increased opacity in the lobes in which the BAL fluid was instilled; this opacity is transient, clearing in hours to a few days.
  • After BAL, the aeration of the washed lung gradually improves. By 6 weeks after the BAL procedure, chest radiographs usually demonstrate improved aeration over preprocedure radiographs.
  • Rarely, in the course of resolution, new opacities may develop in either the washed lung or the contralateral lung. Occasionally, these opacities are related to endobronchial obstruction, usually representing atelectasis. Areas of decreased lung opacity, which represent hyperinflation, may also occur by the same mechanism.
  • Opacity in the contralateral lung may result from spillage of BAL fluid from the washed lung or atelectasis after the BAL procedure.
  • Complications of the BAL procedure, including pneumomediastinum and pneumothorax, may be evident on immediate postprocedure radiographs.

Degree of Confidence

Radiographic findings of PAP are not specific, and as stated above (see Differentials and Radiograph, Findings), a large differential diagnosis must be considered. Similar to the case with other diffuse lung diseases, HRCT scanning provides a more specific morphologic evaluation of the disease pattern and may provide a more limited differential diagnosis. HRCT scanning is also useful for targeting the optimal site for tissue sampling.

Computed Tomography



High-resolution computed tomography scan (window ...

High-resolution computed tomography scan (window width = 1000 Hounsfield units [HU], level = -700 HU) in a patient with pulmonary alveolar proteinosis. The image reveals bilateral ground-glass opacity that is associated with septal thickening, which is consistent with the crazy-paving pattern.

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High-resolution computed tomography scan (window ...

High-resolution computed tomography scan (window width = 1000 Hounsfield units [HU], level = -700 HU) in a patient with pulmonary alveolar proteinosis. The image reveals bilateral ground-glass opacity that is associated with septal thickening, which is consistent with the crazy-paving pattern.


High-resolution computed tomography scan (window ...

High-resolution computed tomography scan (window width = 1000 Hounsfield units [HU], level = -700 HU) in a patient with pulmonary alveolar proteinosis who received bronchoalveolar lavage. This image reveals regression of the ground-glass opacity, representing partial resolution of the disease.

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High-resolution computed tomography scan (window ...

High-resolution computed tomography scan (window width = 1000 Hounsfield units [HU], level = -700 HU) in a patient with pulmonary alveolar proteinosis who received bronchoalveolar lavage. This image reveals regression of the ground-glass opacity, representing partial resolution of the disease.


High-resolution computed tomography scan (window ...

High-resolution computed tomography scan (window width = 1000 Hounsfield units [HU], level = -700 HU) in a patient with pulmonary alveolar proteinosis (PAP) (same patient as in Image above). This image reveals slight interval worsening of the bilateral ground-glass opacity and interlobular septal thickening that is consistent with progression of PAP.

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High-resolution computed tomography scan (window ...

High-resolution computed tomography scan (window width = 1000 Hounsfield units [HU], level = -700 HU) in a patient with pulmonary alveolar proteinosis (PAP) (same patient as in Image above). This image reveals slight interval worsening of the bilateral ground-glass opacity and interlobular septal thickening that is consistent with progression of PAP.


Findings

Routine CT scans (7-10 mm collimation) may reveal bilateral areas of consolidation and reticulation in the lungs of patients with pulmonary alveolar proteinosis (PAP).

  • Occasionally, an underlying linear abnormality that represents thickened interlobular septa may be appreciated.
  • The disease distribution of PAP is usually bilateral and patchy.

HRCT scanning is superior to both conventional CT scanning and chest radiography for demonstrating the morphologic characteristics of PAP.

  • Crazy paving is the characteristic finding of PAP on high-resolution CT (HRCT) scanning and consists of patchy, bilateral, geographic areas of ground-glass opacity that are associated with interlobular septal thickening.59,62 The disease is often distributed uniformly from the lung apex to the base. Interlobular septal thickening may be encountered more frequently in the lower lung zones. Although the ground-glass opacity in PAP is usually patchy, centrilobular nodules have been described in pediatric patients with PAP, as well as in adult patients with acute silicoproteinosis.63
  • Although findings of the interlobular septa are commonly abnormal for pathologic specimens from patients with PAP, septa findings may occasionally be pathologically normal, despite a thickened appearance on HRCT scans. This apparent septal thickening on HRCT scans presumably reflects the aggregation of PAS-positive lipoproteinaceous material immediately adjacent to the interlobular septa.
  • Classically, the abnormal pulmonary parenchyma is demarcated sharply from normal lung areas without a discernible anatomic boundary.

Pleural effusions and adenopathy are uncommon in PAP, and the clinician should suspect a superimposed infection or malignancy if these are present. Occasionally, pleural effusions may be encountered in uncomplicated cases of PAP shortly after bronchoalveolar lavage (BAL).

Degree of Confidence

Although the appearance of crazy paving is highly suggestive of PAP, this finding is not pathognomonic for PAP. Many other entities may present with a pattern similar to crazy paving on HRCT scans, including hydrostatic pulmonary edema, diffuse alveolar damage from any number of causes, pulmonary hemorrhage, diffuse pulmonary infections (including M tuberculosis, M pneumoniae, and other bacterial pneumonias), the diffuse form of bronchioloalveolar carcinoma, ARDS, drug-induced pneumonitis, radiation pneumonitis, bronchiolitis obliterans organizing pneumonia, chronic eosinophilic pneumonia, obstructive pneumonitis, acute interstitial pneumonia, and lipoid pneumonia. In addition, atypical manifestations of PAP also occur. Do not expect all instances of PAP to present as crazy paving on HRCT scans.

Magnetic Resonance Imaging

Findings

Few data are available regarding the MRI appearance of PAP. Because of magnetic susceptibility effects and the short echo times of lung parenchyma, lung tissue is challenging to image with MRI.

A study by Moore et al addressed the appearances of diffuse air-space disease on MRI and found that air-space opacity in PAP has a short T1 value, and this opacity demonstrated relatively little increased signal intensity with T2-weighted imaging.64 Presumably, the short T1 signal of PAP reflects the relative lack of water within the lipoproteinaceous material that fills the air spaces in this disease.

More on Alveolar Proteinosis

Overview: Alveolar Proteinosis
Imaging: Alveolar Proteinosis
Follow-up: Alveolar Proteinosis
Multimedia: Alveolar Proteinosis
References
Further Reading
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References

  1. Brasch F, Birzele J, Ochs M, et al. Surfactant proteins in pulmonary alveolar proteinosis in adults. Eur Respir J. Sep 2004;24(3):426-35. [Medline]. [Full Text].

  2. Seymour JF, Presneill JJ. Pulmonary alveolar proteinosis: Progress in the first 44 years. Am J Respir Crit Care Med. July 2002;166:215-35. [Medline]. [Full Text].

  3. Shah PL, Hansell D, Lawson PR, et al. Pulmonary alveolar proteinosis: clinical aspects and current concepts on pathogenesis. Thorax. Jan 2000;55(1):67-77. [Medline]. [Full Text].

  4. Mu XD, Nie LG, Wang GF, Que CL, Li HC. [Idiopathic pulmonary alveolar proteinosis: report of three cases and literature review]. Beijing Da Xue Xue Bao. Oct 18 2008;40(5):551-4. [Medline].

  5. Birsak CA, van Rossem RN, Nijhuis-Heddes JM, Maartense E. Pulmonary alveolar proteinosis: a complication in patients with hematologic malignancy. Neth J Med. May 2000;56(5):193-7. [Medline].

  6. Gacouin A, Le Tulzo Y, Suprin E, et al. Acute respiratory failure caused by secondary alveolar proteinosis in a patient with acute myeloid leukemia: a case report. Intensive Care Med. Mar 1998;24(3):265-7. [Medline].

  7. Ladeb S, Fleury-Feith J, Escudier E, et al. Secondary alveolar proteinosis in cancer patients. Support Care Cancer. Nov 1996;4(6):420-6. [Medline].

  8. 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. Mar 1994;149(3 Pt 1):788-94. [Medline].

  9. Hildebrand FL Jr, Rosenow EC 3rd, Habermann TM, Tazelaar HD. Pulmonary complications of leukemia. Chest. Nov 1990;98(5):1233-9. [Medline]. [Full Text].

  10. Lakshminarayan S, Schwarz MI, Stanford RE. Unsuspected pulmonary alveolar proteinosis complicating acute myelogenous leukemia. Chest. Mar 1976;69(3):433-5. [Medline]. [Full Text].

  11. Lugassy G, Michaeli J, Hurwitz N, et al. Pulmonary alveolar proteinosis associated with recurrence of Hodgkin's disease in the lung. Isr J Med Sci. Aug 1990;26(8):446-9. [Medline].

  12. Miller RR, Churg AM, Hutcheon M, Lom S. Pulmonary alveolar proteinosis and aluminum dust exposure. Am Rev Respir Dis. Aug 1984;130(2):312-5. [Medline].

  13. Keller CA, Frost A, Cagle PT, Abraham JL. Pulmonary alveolar proteinosis in a painter with elevated pulmonary concentrations of titanium. Chest. Jul 1995;108(1):277-80. [Medline]. [Full Text].

  14. Lee KP, Trochimowicz HJ, Reinhardt CF. Pulmonary response of rats exposed to titanium dioxide (TiO2) by inhalation for two years. Toxicol Appl Pharmacol. Jun 30 1985;79(2):179-92. [Medline].

  15. McCunney RJ, Godefroi R. Pulmonary alveolar proteinosis and cement dust: a case report. J Occup Med. Mar 1989;31(3):233-7. [Medline].

  16. McDonald JW, Alvarez F, Keller CA. Pulmonary alveolar proteinosis in association with household exposure to fibrous insulation material. Chest. Jun 2000;117(6):1813-7. [Medline]. [Full Text].

  17. Dawkins SA, Gerhard H, Nevin M. Pulmonary alveolar proteinosis: a possible sequel of NO2 exposure. J Occup Med. May 1991;33(5):638-41. [Medline].

  18. Friemann J, Pott F, Wilk KB, et al. Pulmonary alveolar proteinosis in rats after administration of quartz: its possible role in morphogenesis of lung cancer. J Cancer Res Clin Oncol. 1994;120(6):348-53. [Medline].

  19. 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. Apr 1969;55(4):274-8. [Medline].

  20. Heppleston AG. Animal model of human disease. Pulmonary alveolar lipo-proteinosis. Animal model: Silica-induced pulmonary alveolar lipo-proteinosis. Am J Pathol. Jan 1975;78(1):171-4. [Medline]. [Full Text].

  21. Martinez-Maldonado M, Ramirez de Arellano G. Pulmonary alveolar proteinosis, nocardiosis and chronic granulocytic leukemia. South Med J. Aug 1966;59(8):901-5. [Medline].

  22. Ruben FL, Talamo TS. Secondary pulmonary alveolar proteinosis occurring in two patients with acquired immune deficiency syndrome. Am J Med. Jun 1986;80(6):1187-90. [Medline].

  23. Reyes JM, Putong PB. Association of pulmonary alveolar lipoproteinosis with mycobacterial infection. Am J Clin Pathol. Oct 1980;74(4):478-85. [Medline].

  24. Bakhos R, Gattuso P, Arcot C, Reddy VB. Pulmonary alveolar proteinosis: an unusual association with Mycobacterium avium-intracellulare infection and lymphocytic interstitial pneumonia. South Med J. Aug 1996;89(8):801-2. [Medline].

  25. Owens MW, Kinasewitz GT, Gonzalez E. Sandblaster's lung with mycobacterial infection. Am J Med Sci. Jun 1988;295(6):554-7. [Medline].

  26. Ranchod M, Bissell M. Pulmonary alveolar proteinosis and cytomegalovirus infection. Arch Pathol Lab Med. Mar 1979;103(3):139-42. [Medline].

  27. Hartung M, Salfelder K. Pulmonary alveolar proteinosis and histoplasmosis: report of three cases. Virchows Arch A Pathol Anat Histol. Nov 28 1975;368(4):281-7. [Medline].

  28. Lee YC, Chew GT, Robinson BW. Pulmonary and meningeal cryptococcosis in pulmonary alveolar proteinosis. Aust N Z J Med. Dec 1999;29(6):843-4. [Medline].

  29. Tran Van Nhieu J, Vojtek AM, Bernaudin JF, et al. Pulmonary alveolar proteinosis associated with Pneumocystis carinii. Ultrastructural identification in bronchoalveolar lavage in AIDS and immunocompromised non-AIDS patients. Chest. Oct 1990;98(4):801-5. [Medline]. [Full Text].

  30. Israel RH, Magnussen CR. Are AIDS patients at risk for pulmonary alveolar proteinosis?. Chest. Sep 1989;96(3):641-2. [Medline]. [Full Text].

  31. Williams GD, Christodoulou J, Stack J, et al. Surfactant protein B deficiency: clinical, histological and molecular evaluation. J Paediatr Child Health. Apr 1999;35(2):214-20. [Medline].

  32. Wallot M, Wagenvoort C, deMello D, et al. Congenital alveolar proteinosis caused by a novel mutation of the surfactant protein B gene and misalignment of lung vessels in consanguineous kindred infants. Eur J Pediatr. Jun 1999;158(6):513-8. [Medline].

  33. Dirksen U, Nishinakamura R, Groneck P, et al. Human pulmonary alveolar proteinosis associated with a defect in GM-CSF/IL-3/IL-5 receptor common beta chain expression. J Clin Invest. Nov 1 1997;100(9):2211-7. [Medline]. [Full Text].

  34. Huffman JA, Hull WM, Dranoff G, et al. Pulmonary epithelial cell expression of GM-CSF corrects the alveolar proteinosis in GM-CSF-deficient mice. J Clin Invest. Feb 1 1996;97(3):649-55. [Medline]. [Full Text].

  35. Dranoff G, Mulligan RC. Activities of granulocyte-macrophage colony-stimulating factor revealed by gene transfer and gene knockout studies. Stem Cells. 1994;12 Suppl 1:173-82; discussion 182-4. [Medline].

  36. Ioachimescu OC, Kavuru MS. Pulmonary alveolar proteinosis. Chron Respir Dis. 2006;3(3):149-59. [Medline]. [Full Text].

  37. Tchou-Wong KM, Harkin TJ, Chi C, et al. GM-CSF gene expression is normal but protein release is absent in a patient with pulmonary alveolar proteinosis. Am J Respir Crit Care Med. Dec 1997;156(6):1999-2002. [Medline]. [Full Text].

  38. Danel C, Israel-Biet D, Costabel U, et al. The clinical role of BAL in alveolar proteinosis. Eur Respir J. Sep 1990;3(8):950-1, 961-9. [Medline].

  39. Kitamura T, Tanaka N, Watanabe J, et al. Idiopathic pulmonary alveolar proteinosis as an autoimmune disease with neutralizing antibody against granulocyte/macrophage colony-stimulating factor. J Exp Med. Sep 20 1999;190(6):875-80. [Medline]. [Full Text].

  40. Sánchez-Cuéllar S, García-Pérez FJ, Reina MT. Streptococcus pneumoniae Infection: Cause or Consequence of Pulmonary Alveolar Proteinosis?. Arch Bronconeumol. Nov 2008;44(11):645-646. [Medline].

  41. Suzuki T, Sakagami T, Rubin BK, Nogee LM, Wood RE, Zimmerman SL, et al. Familial pulmonary alveolar proteinosis caused by mutations in CSF2RA. J Exp Med. Nov 24 2008;205(12):2703-10. [Medline].

  42. Martinez-Moczygemba M, Doan ML, Elidemir O, Fan LL, Cheung SW, Lei JT, et al. Pulmonary alveolar proteinosis caused by deletion of the GM-CSFRalpha gene in the X chromosome pseudoautosomal region 1. J Exp Med. Nov 24 2008;205(12):2711-6. [Medline].

  43. Ben-Dov I, Kishinevski Y, Roznman J, et al. Pulmonary alveolar proteinosis in Israel: ethnic clustering. Isr Med Assoc J. Oct 1999;1(2):75-8. [Medline].

  44. Kariman K, Kylstra JA, Spock A. Pulmonary alveolar proteinosis: prospective clinical experience in 23 patients for 15 years. Lung. 1984;162(4):223-31. [Medline].

  45. Wilson DO, Rogers RM. Prolonged spontaneous remission in a patient with untreated pulmonary alveolar proteinosis. Am J Med. May 1987;82(5):1014-6. [Medline].

  46. Haddad PG, Pankey GA. Pulmonary alveolar proteinosis: a case with spontaneous resolution. J La State Med Soc. Dec 1969;121(12):365-76. [Medline].

  47. Parker LA, Novotny DB. Recurrent alveolar proteinosis following double lung transplantation. Chest. May 1997;111(5):1457-8. [Medline]. [Full Text].

  48. Ganguli PC, Lynne-Davies P, Sproule BJ. Pulmonary alveolar proteinosis, bronchiectasis and secondary amyloidosis: a case report. Can Med Assoc J. Mar 4 1972;106(5):569 passim. [Medline].

  49. Steens RD, Summers QA, Tarala RA. Pulmonary alveolar proteinosis in association with Fanconi's anemia and psoriasis. A possible common pathogenetic mechanism. Chest. Aug 1992;102(2):637-8. [Medline]. [Full Text].

  50. Kaplan AI, Sabin S. Case report: interstitial fibrosis after uncomplicated pulmonary alveolar proteinosis. Postgrad Med. May 1977;61(5):263, 265. [Medline].

  51. Goldstein LS, Kavuru MS, Curtis-McCarthy P, et al. Pulmonary alveolar proteinosis: clinical features and outcomes. Chest. Nov 1998;114(5):1357-62. [Medline]. [Full Text].

  52. Sieracki JC, Horn RC Jr, Kay S. Pulmonary alveolar proteinosis: report of three cases. Ann Intern Med. Oct 1959;51:728-39. [Medline].

  53. Wang BM, Stern EJ, Schmidt RA, Pierson DJ. Diagnosing pulmonary alveolar proteinosis. A review and an update. Chest. Feb 1997;111(2):460-6. [Medline]. [Full Text].

  54. Lin FC, Chen YC, Chang SC. Clinical importance of bronchoalveolar lavage fluid and blood cytokines, surfactant protein D, and Kerbs von Lungren 6 antigen in idiopathic pulmonary alveolar proteinosis. Mayo Clin Proc. Dec 2008;83(12):1344-9. [Medline].

  55. Martin RJ, Rogers RM, Myers NM. PUlmonary alveolar proteinosis: shunt fraction and lactic acid dehydrogenase concentration as aids to diagnosis. Am Rev Respir Dis. Jun 1978;117(6):1059-62. [Medline].

  56. Takahashi T, Munakata M, Suzuki I, Kawakami Y. Serum and bronchoalveolar fluid KL-6 levels in patients with pulmonary alveolar proteinosis. Am J Respir Crit Care Med. Oct 1998;158(4):1294-8. [Medline]. [Full Text].

  57. Nagasaka Y, Takahashi M, Ueshima H, et al. Bronchoalveolar lavage with trypsin in pulmonary alveolar proteinosis. Thorax. Jul 1996;51(7):769-70. [Medline].

  58. Bonfield TL, Russell D, Burgess S, et al. Autoantibodies against granulocyte macrophage colony-stimulating factor are diagnostic for pulmonary alveolar proteinosis. Am J Respir Cell Mol Biol. Oct 2002;27(4):481-6. [Medline]. [Full Text].

  59. Johkoh T, Itoh H, Muller NL, et al. Crazy-paving appearance at thin-section CT: spectrum of disease and pathologic findings. Radiology. Apr 1999;211(1):155-60. [Medline]. [Full Text].

  60. Gale ME, Karlinsky JB, Robins AG. Bronchopulmonary lavage in pulmonary alveolar proteinosis: chest radiograph observations. AJR Am J Roentgenol. May 1986;146(5):981-5. [Medline]. [Full Text].

  61. Zontsich T, Helbich TH, Wojnarovsky C, et al. Pulmonary alveolar proteinosis in a child: HRCT findings before and after bronchoalveolar lavage. Eur Radiol. 1998;8(9):1680-2. [Medline].

  62. Rossi SE, Erasmus JJ, Volpacchio M, et al. "Crazy-paving" pattern at thin-section CT of the lungs: radiologic-pathologic overview. Radiographics. Nov-Dec 2003;23(6):1509-19. [Medline]. [Full Text].

  63. Marchiori E, Ferreira A, Muller NL. Silicoproteinosis: high-resolution CT and histologic findings. J Thorac Imaging. Apr 2001;16(2):127-9. [Medline].

  64. Moore EH, Webb WR, Muller N, Sollitto R. MRI of pulmonary airspace disease: experimental model and preliminary clinical results. AJR Am J Roentgenol. Jun 1986;146(6):1123-8. [Medline].

  65. McCook TA, Kirks DR, Merten DF, et al. Pulmonary alveolar proteinosis in children. AJR Am J Roentgenol. Nov 1981;137(5):1023-7. [Medline].

  66. Bonfield TL, Kavuru MS, Thomassen MJ. Anti-GM-CSF titer predicts response to GM-CSF therapy in pulmonary alveolar proteinosis. Clin Immunol. Dec 2002;105(3):342-50. [Medline].

  67. Claypool WD, Rogers RM, Matuschak GM. Update on the clinical diagnosis, management, and pathogenesis of pulmonary alveolar proteinosis (phospholipidosis). Chest. Apr 1984;85(4):550-8. [Medline].

  68. Conces DJ Jr, Tarver RD. Noninfectious and nonmalignant pulmonary disease in AIDS. J Thorac Imaging. Sep 1991;6(4):53-9. [Medline].

  69. Dee P, Suratt P, Winn W. The radiographic findings in acute silicosis. Radiology. Feb 1978;126(2):359-63. [Medline].

  70. Godwin JD, Muller NL, Takasugi JE. Pulmonary alveolar proteinosis: CT findings. Radiology. Dec 1988;169(3):609-13. [Medline].

  71. Green D, Criner GJ. Twenty-five-year follow-up of a patient treated with lung lavage for pulmonary alveolar proteinosis. N Engl J Med. Sep 24 1987;317(13):839-40. [Medline].

  72. Herman TE, Nogee LM, McAlister WH, Dehner LP. Surfactant protein B deficiency: radiographic manifestations. Pediatr Radiol. 1993;23(5):373-5. [Medline].

  73. Holbert JM, Costello P, Li W, et al. CT features of pulmonary alveolar proteinosis. AJR Am J Roentgenol. May 2001;176(5):1287-94. [Medline].

  74. Honda Y, Takahashi H, Shijubo N, Kuroki Y, Akino T. Surfactant protein-A concentration in bronchoalveolar lavage fluids of patients with pulmonary alveolar proteinosis. Chest. Feb 1993;103(2):496-9. [Medline]. [Full Text].

  75. Kadota J, Nakamura Y, Iwashita T, et al. Pulmonary alveolar proteinosis with lung squamous cell carcinoma. Respir Med. Feb 1999;93(2):138-40. [Medline].

  76. Kronauer CM, Marincek B, Russi EW. Bilateral focal pulmonary densities. Chest. Dec 1989;96(6):1413-4. [Medline].

  77. Kuroki Y, Takahashi H, Chiba H, Akino T. Surfactant proteins A and D: disease markers. Biochim Biophys Acta. Nov 19 1998;1408(2-3):334-45. [Medline].

  78. Lee KN, Levin DL, Webb WR, et al. Pulmonary alveolar proteinosis: high-resolution CT, chest radiographic, and functional correlations. Chest. Apr 1997;111(4):989-95. [Medline].

  79. Masuda T, Shimura S, Sasaki H, Takishima T. Surfactant apoprotein-A concentration in sputum for diagnosis of pulmonary alveolar proteinosis. Lancet. Mar 9 1991;337(8741):580-2. [Medline].

  80. Mikami T, Yamamoto Y, Yokoyama M, Okayasu I. Pulmonary alveolar proteinosis: diagnosis using routinely processed smears of bronchoalveolar lavage fluid. J Clin Pathol. Dec 1997;50(12):981-4. [Medline]. [Full Text].

  81. Milleron BJ, Costabel U, Teschler H, et al. Bronchoalveolar lavage cell data in alveolar proteinosis. Am Rev Respir Dis. Dec 1991;144(6):1330-2. [Medline].

  82. Murch CR, Carr DH. Computed tomography appearances of pulmonary alveolar proteinosis. Clin Radiol. May 1989;40(3):240-3. [Medline].

  83. Pascual J, Gomez Aguinaga MA, Vidal R, et al. Alveolar proteinosis and nocardiosis: a patient treated by bronchopulmonary lavage. Postgrad Med J. Sep 1989;65(767):674-7. [Medline].

  84. Paul K, Muller KM, Oppermann HC, Nutzenadel W. Pulmonary alveolar lipoproteinosis in a seven-year-old girl. A follow- up over six years. Acta Paediatr Scand. Apr 1991;80(4):477-81. [Medline].

  85. Popovsky J, Reydman M, Abellera M, Stockfish H. Pulmonary Alveolar proteinosis. Lobar localization and surgical excision. Int Surg. Mar 1976;61(3):146-7. [Medline].

  86. Santamaria F, Parenti G, Guidi G, et al. Early detection of lung involvement in lysinuric protein intolerance: role of high-resolution computed tomography and radioisotopic methods. Am J Respir Crit Care Med. Feb 1996;153(2):731-5. [Medline].

  87. Schumacher RE, Marrogi AJ, Heidelberger KP. Pulmonary alveolar proteinosis in a newborn. Pediatr Pulmonol. 1989;7(3):178-82. [Medline].

  88. Seymour JF, Doyle IR, Nakata K, et al. 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. Thorax. Mar 2003;58(3):252-7. [Medline]. [Full Text].

  89. Trapnell BC, Whitsett JA, Nakata K. Pulmonary alveolar proteinosis. N Engl J Med. Dec 25 2003;349(26):2527-39. [Medline].

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Keywords

alveolar proteinosis, pulmonary alveolar phospholipoproteinosis, alveolar lipoproteinosis, PAP, surfactant protein, SP-B, granulocyte macrophage-colony stimulating factor, GM-CSF, bronchoalveolar lavage, BAL, whole-lung lavage, diffuse air-space disease

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Contributor Information and Disclosures

Author

Michael B Gotway, MD, Clinical Associate Professor of Diagnostic Radiology and Pulmonary/Critical Care Medicine, University of California at San Francisco School of Medicine; Consulting Staff, Scottsdale Medical Imaging, Ltd
Michael B Gotway, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, Radiological Society of North America, and Society of Thoracic Radiology
Disclosure: Nothing to disclose.

Coauthor(s)

Theodore J Lee, MD, Chief, Thoracic Radiology, San Francisco General Hospital; Assistant Professor of Clinical Radiology, University of California at San Francisco
Theodore J Lee, MD is a member of the following medical societies: American Roentgen Ray Society, Radiological Society of North America, and Society of Thoracic Radiology
Disclosure: Nothing to disclose.

Medical Editor

Jeffrey A Miller, MD, Associate Professor of Clinical Radiology, University of Medicine and Dentistry of New Jersey; Associate Chief of Service, Department of Radiology, Veterans Affairs of New Jersey Health Care System
Jeffrey A Miller, MD is a member of the following medical societies: North American Society for Cardiac Imaging, Society for Health Services Research in Radiology, and Society of Thoracic Radiology
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

John D Newell, Jr, MD, FACR, FCCP, FASER, Co-Director of Thoracic Imaging, UCDHSC; Director of Lung Imaging Center, Professor of Radiology and Professor of Medicine, Department of Radiology, University of Colorado Health Sciences Center, National Jewish Medical and Research Center; Univ. Colorado Hospital
John D Newell, Jr, MD, FACR, FCCP, FASER is a member of the following medical societies: American College of Chest Physicians, American College of Radiology, American Roentgen Ray Society, American Thoracic Society, Association of University Radiologists, Radiological Society of North America, and Society of Thoracic Radiology
Disclosure: Siemens Medical Grant/research funds Consulting; Forevision Technologies Ownership interest Consulting; Vida Corporation Ownership interest Board membership; TeraRecon Grant/research funds Consulting; eMedicine Honoraria Consulting

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

Barry H Gross, MD, Professor, Department of Radiology, University of Michigan Medical School; Professor, University of Michigan Cancer Center
Barry H Gross, MD is a member of the following medical societies: American College of Chest Physicians, American College of Radiology, American Roentgen Ray Society, Association of University Radiologists, Michigan State Medical Society, Physicians for Social Responsibility, Radiological Society of North America, and Society of Thoracic Radiology
Disclosure: Nothing to disclose.

 
 
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