Pulmonary alveolar proteinosis: report of two cases and update
Keywords:
pulmonary alveolar proteinosis, lung diseases, therapyAbstract
Pulmonary alveolar proteinosis is a rare disease characterized by extensive radiological and tomographic pulmonary lesions and a variable clinical picture ranging from pulmonary insufficiency to spontaneous remission. Among its three described forms, the so called Idiopathic or Adult form is responsible for more than 80% of published cases. It’s physiopathology depends on an autoimmune process directed against the GM-CSF (Granulocite Macrophage- Colony Stimulating Factor) that induces a functional defect of the macrophage with consequent intraalveolar accumulation of surfactant. Pulmonary and extrapulmonary infections, are common, often with unusual pathogens. Diagnosis can be made from the combination of clinical, radiological and bronchoalveolar lavage data, although sometimes histopathologic material is needed for confirmation. Total pulmonary lavage is currently the treatment of choice. Mortality is usually low and related mostly to infectious complications. We hereby describe our experience with two patients.
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Ioachimescu OC, Kavuru MS. Pulmonary alveolar proteinosis. Chronic Respiratory Disease 2006; 3:149-159.3)
Trapnell BC, Whitsett JA, Nakata K. Pulmonary alveolar proteinosis. N Engl J Med 2003;349:2527-39.
Seymour JF, Presneill JJ. Pulmonary alveolar proteinosis: progress in the first 44 years. Am J Respir Crit Care Med 2002;166:215-35.
Holbert JM, Costello P, Li W, Hoffman RM, Rogers RM. CT features of pulmonary alveolar proteinosis. Am J Roentgenol 2001;176:1287-94.
Goldstein LS, Kavuru MS, Curtis-McCarthy P, Christie HA, Farver C, Stoller JK. Pulmonary alveolar proteinosis: clinical features and outcomes. Chest 1998;114:1357-62.
Lin FC, Chang GD, Chern MS, Chen YC, Chang SC. Clinical significance of anti-GM-CSF antibodies in idiopathic pulmonary alveolar proteinosis. Thorax 2006; 61:528-534.
Lee KN, Levin DL, Webb WR, Chen D, Storto ML, Golden JA. Pulmonary alveolar proteinosis: high-resolution CT, chest radiographic, and functional correlations. Chest 1997;111:989-95.
Dranoff G, Crawford AD, Sadelain M et al. Involvement of granulocyte-macrophage colony-stimulating factor in pulmonary homeostasis. Science 1994; 264: 713-16.
Stanley E, Lieschke GJ, Grail D et al. Granulocyte/macrophage colony-stimulating factor-deficient mice show no major perturbation of hematopoiesis but develop a characteristic pulmonary pathology. Proc Natl Acad Sci USA 1994;91:5592-96.
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 1999; 190:875-80.
Uchida K, Beck DC, Yamamoto T, Berclaz PY, Abe S, Staudt MK et al. GM-CSF autoantibodies and neutrophil dysfunction in pulmonary alveolar proteinosis. N Engl J Med 2007;356:567-79.
Ramirez J, Campbell GD. Pulmonary alveolar proteinosis. Endobronchial treatment. Ann Intern Med 1965;63:429-41.
Seymour JF, Ashley R, Vincent JM, Presneill JJ, Pain MC. Efficacy of Granulocyte–Macrophage Colony-Stimulating factor in acquired alveolar proteinosis.N Engl J Med 1996;335:1924-25.
Wylam ME, Katzmann JA. Aerosolized GM-CSF improves pulmonary function in idiopathic pulmonary alveolar proteinosis. Am J Respir Crit Care Med 2000;161:889.
Bonfield TL, Kavuru MS, Thomassen MJ. Anti-GM-CSF titer predicts response to GM-CSF therapy in pulmonary alveolar proteinosis. Clinical mmunology 2002; 105(3): 342-50
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