Pulmonary alveolar proteinosis represents a rare syndrome characterized by the accumulation of proteinaceous phospholipid-laden material in the alveoli. This leads to impaired gas exchange and arterial hypoxemia of varying degrees. The diagnosis is confirmed by lung biopsy. Sequential whole-lung lavage (WLL) first described in 1963 is the standard of care. We report a case of a male diagnosed of having pulmonary alveolar proteinosis (PAP) on transbroncial lung biopsy (TBLB). He was treated with sequential WLL (Left followed by right, Left being more involved on chest X-ray) followed by recombinant GM-CSF, with good result.
Ben-Dov I, Kishinevski Y, Roznman J, Soliman A, Bishara H, Zelligson, et al. Pulmonary alveolar proteinosis in Israel: Ethnic clustering. Isr Med Assoc J 1999;1:75-8.
Seymour JF, Presneill JJ. Pulmonary alveolar proteinosis: Progress in the first 44 years. Am J Respir Crit Care Med 2002;166:215-35.
Johkoh T, Itoh H, Muller NL, Ichikado K, Nakamura H, Ikezoe J, et al. Crazy-paving appearance at thin-section CT: Spectrum of disease and pathologic findings. Radiology 1999;211:155-60.
Wang BM, Stern EJ, Schmidt RA, Pierson DJ. Diagnosing pulmonary alveolar proteinosis: A review and an update. Chest 1997;111:460-6.
Trapnell BC, Whitsett JA, Nakata K. Pulmonary alveolar proteinosis. N Engl J Med 2003;349:2527-39.
Hamvas A, Nogee LM, Mallory GB Jr, Spray TL, Huddleston CB, August A, et al. Lung transplantation for treatment of infants with surfactant protein B deficiency. J Pediatr 1997;130:231-9.
Du Bois RM, McAllister WA, Branthwaite MA. Alveolar proteinosis: Diagnosis and treatment over a 10-year period. Thorax 1983;38:360-3.
Seymour JF, Presneill JJ, Schoch OD, Downie GH, Moore PE, Doyle IR, et al. Therapeutic efficacy of granulocyte-macrophage colony-stimulating factor in patients with idiopathic acquired alveolar proteinosis. Am J Respir Crit Care Med 2001;163:524-31.