Skip to main content
Log in

Pulmonary alveolar proteinosis: prospective clinical experience in 23 patients for 15 years

  • Original Articles
  • Published:
Lung Aims and scope Submit manuscript

Abstract

Twenty-three patients, including three children, with pulmonary alveolar proteinosis (PAP) were studied over a 15 year period. Based on the overall course and outcome they were divided into two groups. Group I (24%) had spontaneous remission and received no treatment. Group II (76%), because of progressive dyspnea and deterioration of pulmonary function tests, underwent lung lavage. Of those who underwent lung lavage and were followed, 76% had a favorable response (Group II-a) and a significant number of them developed a complete remission, while 21% (Group II-b) had no response to lung lavage. Group I had higher arterial PO2 and lower alveolar-arterial oxygen partial pressure difference [P (A-a)O2] than Group II (72±5 and 38±3 vs 57±4 and 51±3,p<0.05). Similarly, the values of arterial PO2 and P(A-a)O2 were better in Group II-a than Group II-b (57±4 and 51±3 vs 40±6 and 65±11). In lavage material of 16 patients studied, macrophages appeared unusually enlarged and their number (% of differential count) was significantly lower (60±4%) than normal (90–95%). In contrast to previous reports, there was no evidence of active infection related to unusual or opportunistic organisms. No death related to PAP was observed. This prospective long term follow-up of large series of patients with PAP documents the overall course, prognosis and management of this illness.

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

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Bedrossian CWM, Luna MA, Conklin RH, Miller WC (1980) Alveolar proteinosis as a consequence of immuno-suppression. A hypothesis based on clinical and pathological observations. Hum Pathol 11:527–534

    PubMed  CAS  Google Scholar 

  2. Bell D, Hook GER (1979) Pulmonary alveolar proteinosis: analysis of airway and alveolar proteins. Am Rev Respir Dis 119:979–989

    PubMed  CAS  Google Scholar 

  3. Blenkarn GD, Lanning DF, Kylstra JA (1975) Anaesthetic management of volume-controlled unilateral lung lavage. Can Anaesth Soc J 22:154–163

    PubMed  CAS  Google Scholar 

  4. Davidson JM, MacLeod WM (1969) Pulmonary alveolar proteinosis. Br J Dis Chest 63:13–28

    Article  PubMed  CAS  Google Scholar 

  5. DuBois RM, McAllister WA, Branthwaite MA (1983) Alveolar proteinosis: diagnosis and treatment over a 10-year period. Thorax 30:360–363

    Article  Google Scholar 

  6. Golde DW, Territo M, Finley TN, Cline MJ (1976) Defective lung macrophages in pulmonary alveolar proteinosis. Ann Intern Med 45:304–309

    Google Scholar 

  7. Harris JO, Swenson EW, Johnson JE III (1970) Human alveolar macrophages: comparison of phagocytic ability, glucose utilization and ultrastructure in smokers and non-smokers. J Clin Invest 49:2086–2096

    Article  PubMed  CAS  Google Scholar 

  8. Kylstra JA, Rausch DC, Hall KD, Spock A (1971) Volume-controlled lung lavage in the treatment of asthma, bronchiectasis, and mucoviscidosis. Am Rev Respir Dis 103:651–665

    PubMed  CAS  Google Scholar 

  9. Muggenburg BA, Mauderly JL (1975) Lung lavage using a single-lumen endotracheal tube. J Appl Physiol 38:922–926

    PubMed  CAS  Google Scholar 

  10. Ramirez RJ (1971) Alveolar proteinosis: importance of pulmonary lavage. Am Rev Respir Dis 103:666–678

    PubMed  CAS  Google Scholar 

  11. Ramirez RJ, Kieffer FR Jr, Ball WC Jr (1965) Bronchopulmonary lavage in man. Ann Intern Med 63:819–828

    PubMed  CAS  Google Scholar 

  12. Ramirez RJ, Savard EV, Hawkins JE (1966) Biological effects of pulmonary washings from cases of alveolar proteinosis. Am Rev Respir Dis 94:244–246

    PubMed  CAS  Google Scholar 

  13. Reynolds HY, Newball HH (1974) Analysis of proteins and respiratory cells obtained from human lungs by bronchial lavage. J Lab Clin Med 84:559–573

    PubMed  CAS  Google Scholar 

  14. Rogers RM, Braunstein MS, Shuman JF (1972) Role of bronchopulmonary lavage in the treatment of respiratory failure: a review. Chest 62:95–106s

    Google Scholar 

  15. Rogers RM, Levin DC, Gray BA, Mosely LW Jr (1978) Physiologic effects of bronchopulmonary lavage in alveolar proteinosis. Am Rev Respir Dis 118:255–264

    PubMed  CAS  Google Scholar 

  16. Rosen SH (1960) Pulmonary alveolar proteinosis. US Armed Forces Med J 11:1507–1515

    CAS  Google Scholar 

  17. Rosen SH, Castleman B, Liebow AA (1958) Pulmonary alveolar proteinosis. N Engl J Med 258:1123–1142

    Article  PubMed  CAS  Google Scholar 

  18. Selecky PA, Wasserman K, Benfield JR, Pippman M (1977) The clinical and physiological effect of whole-lung lavage in pulmonary alveolar proteinosis: a ten-year experience. Ann Thorac Surg 24:451–461

    Article  PubMed  CAS  Google Scholar 

  19. Singh G, Katyal SL, Bedrossian CWM, Rogers RM (1983) Pulmonary alveolar proteinosis. Staining for surfactant apoprotein in alveolar proteinosis and in conditions simulating it. Chest 83:82–86

    PubMed  CAS  Google Scholar 

  20. Spock A (1981) State of the art of lung lavage in patients with cystic fibrosis. In: Warwick WJ (ed) 100 years of cystic fibrosis. Collected Papers. University of Minnesota, pp 113–117

  21. Spock A (1977) Characteristics of macrophages obtained by bronchial lavage in humans. Scand J Respir Dis 58:159–163

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Additional information

Supported by the General Research Centers Program (RR-30)

Rights and permissions

Reprints and permissions

About this article

Cite this article

Kariman, K., Kylstra, J.A. & Spock, A. Pulmonary alveolar proteinosis: prospective clinical experience in 23 patients for 15 years. Lung 162, 223–231 (1984). https://doi.org/10.1007/BF02715650

Download citation

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02715650

Key words

Navigation