RESUMEN

La proteinosis alveolar pulmonar (PAP) es una entidad poco frecuente, donde más del 90% de los casos en los adultos son primarios, las causas secundarias son hematológicas o inmunodeficiencias en general. Esta enfermedad muestra alteraciones morfológicas muy características, en donde el acúmulo anormal de surfactante intra-alveolar se manifiesta con trastornos en el intercambio gaseoso, un proceso restrictivo de gravedad variable y los hallazgos de imagen son muy específicos, sobre todo en tomografía computada de alta resolución (TCAR), lo que contrasta con la casi ausencia de datos clínicos; la respuesta clínica en general es satisfactoria con remisión del cuadro posterior al lavado pulmonar total y en ocasiones en forma espontánea, con sobrevidas casi del 100% en los últimos años, las complicaciones son poco frecuentes y se presentan en el primer año del diagnóstico y éstas son principalmente de índole infeccioso donde los patógenos oportunistas no son infrecuentes, todo ello al parecer asociadas a la disfunción macrofágica mediadas por alteraciones en el factor estimulante de colonias granulocito/macrófago (GM-CSF).

PALABRAS CLAVE

Proteinosis alveolar pulmonar (PAP), surfactante, tomografía computada de alta resolución (TCAR), factor estimulante de colonias granulocito/macrófago (GM-CSF).

REFERENCIAS

1. Schwarz MI, King TE. Miscellaneous interstitial lung diseases: in Interstitial Lung Diseases. 3rd edition. BC Decker, Hamilton: 1998: 725-728.

2. Persson A. Pulmonary alveolar proteinosis. In: Fishman´s pulmonary diseases and disorders. 3rd edition, McGraw-Hill, New York. Vol 1: 1998: 1225-1230.

3. Trapnell BC, Whitsett JA, Nakata K. Pulmonary alveolar proteinosis, mechanisms of disease. N Engl J Med 2003; 349: 2527-39.

4. Seymour JF, Presneill JJ. Pulmonary alveolar proteinosis, progress in the first 44 years. Am J Respir Crit Care Med 2002; 166: 215-235.

5. Ben-Dov I, Kishinevski Y, Roznman J, Soliman A, Bishara H, Zelligson E, et al. Pulmonary alveolar proteinosis in Israel: ethnic clustering. Isr Med Assoc J 1998; 1: 75-78.

6. Presneill J, Nakata K, Inoue Y, Seymour JF. Pulmonary alveolar proteinosis. Clin Chest Med 2004; 25: 593-613.

7. Buechner H, Ansari A. Acute silicoproteinosis: a new pathologic variant of acute silicosis in sandblasters, characterized by histologic features resembling alveolar proteinosis. Dis Chest 1969; 55: 274-278.

8. Ruben F, Talamo T. Secondary pulmonary alveolar proteinosis occurring in two patients with acquired immune deficiency syndrome. Am J Med 1986; 80: 1187-1190.

9. Doyle A, Balcerzak S, Wells C, Crittenden J. Pulmonary alveolar proteinosis with hematologic disorders. Arch Intern Med 1963; 112: 940-946.

10. Dirksen U, Hattenhorst U, Schneider P, et al. Defective expression of granulocyte macrophage colony stimulating factor/interleukin – 3/interleukin – 5 receptor common beta chain in children with acute myeloid leukemia associated with respiratory failure. Blood 1998; 92: 1097-1103.

11. 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 1997; 100: 2211-2217.

12. Nogee L, deMello D, Dehner L, Colten H. Deficiency of pulmonary surfactant protein B in congenital alveolar proteinosis. N Engl J Med 1993; 328: 406-410.

13. Mazzone P, Thomassen M, Kavuru M. Pulmonary alveolar proteinosis: Recent advances. Sem Respir Crit Care Med 2002; 23: 115-126.

14. Lee K, Levin D, Webb R, Chen D, Storto M, Golden J. Pulmonary alveolar proteinosis: High resolution CT, Chest radiography, and functional correlations. Chest 1997; 111: 989-995.

15. Webb R, Muller N, Naidich D. Diseases Characterized primarily by parenchymal opacification. High resolution CT of the lung. 3rd edition, Lippincott, Williams & Wilkins, Philadelphia: 2001: 390-393.

16. Johkoh T, Itoh H, Muller N, Ichikado K, Nakamura H, Ikesoe J, et al. Crazy-paving appearance at thin section CT: spectrum of disease and pathologic findings. Radiology 1999; 211: 155-160.

17. Rossi S, Erasmus J, Vopacchio M, Franquet T, Castiglioni T, McAdams H. Crazy paving pattern at thin section CT of the lungs: radiologic-pathologic overview. Radiographics 2003; 23: 1509-1519.

18. Wang B, Stern E, Schmidt R, Pierson D. Diagnosing pulmonary alveolar proteinosis: a review and an update. Chest 1997; 111: 460-466.

19. Travis W, Rosado M, Colby T. Diffuse parenchymal lung diseases in: Non neoplastic disorders of the lower respiratory tract. Atlas of non tumor pathology. Published by the American registry of pathology and the armed forces institute of pathology; 2002; 2: 169-176.

20. Shah P, Hansell D, Lawson P, Reid K, Morgan C. Pulmonary alveolar proteinosis: clinical aspects and current concepts on pathogenesis. Thorax 2000; 55: 67-77.

21. Chesnutt M, Nuckton T, Golden J, Folkesson H, Matthay M. Rapid alveolar epithelial fluid clearance following lung lavage in pulmonary alveolar proteinosis. Chest 2001; 120: 271-274.

22. Cheng S, Chang H, Lau H, Lee L, Yang P. Pulmonary alveolar proteinosis: treatment by bronchofiberscopic lobar lavage. Chest 2002; 122: 1480-1485.

23. Burgess A, Camakaris J, Metcalf D. Purification and properties of colony-stimulating factor from mouse lung-conditioned medium. J Biol Chem 1977; 252: 1998-2003.

24. Evans C, Ariffin S, Pierce A, Whetton A. Identification of primary structural features that define the differential actions of IL-3 and GM-SCF receptors. Blood 2002; 100: 3164-3174.

25. Stanley E, Lieschke G, Grail D, Metcalf D, Hodgson G, Gall J, et al. Granulocyte/macrophage colony-stimulating factor-deficient mice show no major perturbation of hematopoiesis but develop a characteristic pulmonary pathologic. Proc Natl Acad Sci USA 1994; 91: 5592-5596.

26. Seymour JF, Presneill JJ, Schoch OD, Downie GH, Moore PE, Doyle IR. Therapeutic efficacy of granulocyte-macrophage colony-stimulating factor in patients with idiopathic acquired alveolar proteinosis. Am J Respir Crit Care Med 2001; 163: 524-531.