- Pulmonary alveolar proteinosis
Pulmonary alveolar proteinosis -(PAP) is a rare
lung diseasein which abnormal accumulation of surfactantoccurs within the alveoli, interfering with gas exchange. PAP can occur in a primary form or secondarily in the settings of malignancy (especially in myeloid leukemia), pulmonary infection, or environmental exposure to dusts or chemicals. Rare familial forms have also been recognized, suggesting a genetic component in some cases.
History and pathology
PAP was first described in 1958 by the physicians Samuel Rosen, Benjamin Castleman, and Averill Liebow. In their case series published in the New England Journal of Medicine on June 7th of that year, they described 27 patients with pathologic evidence of periodic acid Schiff positive material filling the alveoli. This
lipidrich material was subsequently recognized to be surfactant.
Although the cause of PAP remains obscure, a major breakthrough in the understanding of the etiology of the disease came by the chance observation that mice bred for experimental study to lack a hematologic
growth factorknown as granulocyte-macrophage colony stimulating factor (GM-CSF) developed a pulmonary syndromeof abnormal surfactant accumulation resembling human PAP. The implications of this finding are still being explored, but significant progress was reported in February, 2007. Researchers in that report discussed the presence of anti- GM-CSF autoantibodiesin patients with PAP, and duplicated that syndrome with the infusion of these autoantibodiesinto mice. [cite journal |author=Uchida K, Beck D, Yamamoto T, Berclaz P, Abe S, Staudt M, Carey B, Filippi M, Wert S, Denson L, Puchalski J, Hauck D, Trapnell B |title=GM-CSF autoantibodies and neutrophil dysfunction in pulmonary alveolar proteinosis |journal=N Engl J Med |volume=356 |issue=6 |pages=567–79 |year=2007 |pmid=17287477 |doi=10.1056/NEJMoa062505]
The disease is more common in males and in
symptomsof PAP include:
dyspnea(shortness of breath)
x-rays of affected individuals typically reveal nonspecific alveolar opacities. Diagnosisis generally made by surgical or endoscopic biopsyof the lung, revealing the distinctive pathologic finding.
The clinical course of PAP is unpredictable. Spontaneous remission is recognized; some patients have stable symptoms. Death may occur due to progression of PAP or due to the underlying disease associated with PAP. Individuals with PAP are more vulnerable to infection of the lung by bacteria or
The standard treatment for PAP is whole-lung lavage, in which sterile fluid is instilled into the lung and then removed, along with the abnormal surfactant material. This is generally effective at ameliorating symptoms, often for prolonged periods. Since the mouse discovery noted above, the use of GM-CSF injections has also been attempted, with variable success. Lung transplantation can be performed in refractory cases.
#Rosen SH, Castleman B, and Liebow AA. Pulmonary alveolar proteinosis. New England Journal of Medicine 1958; 258: 1123-1142.
#Seymour JF and Presneill JJ. Pulmonary alveolar proteinosis: progress in the first 44 years. American Journal of Respiratory and Critical Care Medicine 2002; 166: 215-235.
#Shah PL, Hansell D, Lawson PR, et al. Pulmonary alveolar proteinosis; clinical aspects and current concepts on pathogenesis. Thorax 2000; 55: 67-77.
#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: 5592-5596.
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