Pulmonary alveolar proteinosis

Pulmonary alveolar proteinosis

Pulmonary alveolar proteinosis -(PAP) is a rare lung disease in which abnormal accumulation of surfactant occurs 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 lipid rich 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 factor known as granulocyte-macrophage colony stimulating factor (GM-CSF) developed a pulmonary syndrome of 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 autoantibodies in patients with PAP, and duplicated that syndrome with the infusion of these autoantibodies into 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 tobacco smokers.


The symptoms of PAP include:
*dyspnea (shortness of breath)
*low grade fever
*weight lossChest x-rays of affected individuals typically reveal nonspecific alveolar opacities. Diagnosis is generally made by surgical or endoscopic biopsy of the lung, revealing the distinctive pathologic finding.

Natural History

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 fungi.


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.

Wikimedia Foundation. 2010.

Look at other dictionaries:

  • pulmonary alveolar proteinosis — n a chronic disease of the lungs characterized by the filling of the alveoli with proteinaceous material and by the progressive loss of lung function * * * a chronic lung disease characterized by dyspnea, productive cough, chest pain, weakness,… …   Medical dictionary

  • pulmonary alveolar proteinosis — ▪ pathology       respiratory disorder caused by the filling of large groups of alveoli with excessive amounts of surfactant, a complex mixture of protein and lipid (fat) molecules. The alveoli are air sacs, minute structures in the lungs in… …   Universalium

  • alveolar proteinosis — pulmonary alveolar p …   Medical dictionary

  • Alveolar lung disease — Alveolar lung disease, also known as airspace disease, is a general term that described edema and exudates in the airspaces of the lung (the acini and alveoli).CausesAlveolar lung disease may be divided into acute or chronic. Causes of acute… …   Wikipedia

  • Pulmonary edema — Classification and external resources Pulmonary edema with small pleural effusions on both sides. ICD 10 J …   Wikipedia

  • Pulmonary hypertension — Pulmonary arterial hypertension Classification and external resources The pulmonary artery receives blood (blue arrow) from the right ventricle of the heart; increased pressure on the artery can impair the function of the right ventricle ICD …   Wikipedia

  • Pulmonary consolidation — Classification and external resources Pneumonia as seen on chest x ray. A: Normal chest x ray. B: Abnormal chest x ray with consolidation from pneumonia in the right lung, middle or inferior lobe (white area, left side of image) …   Wikipedia

  • Pulmonary embolism — Classification and external resources Chest spiral CT scan with radiocontrast agent showing multiple filling defects both at the bifurcation and in the pulmonary arteries. ICD 10 …   Wikipedia

  • Pulmonary surfactant — Diagram of the alveoli with both cross section and external view Pulmonary surfactant is a surface active lipoprotein complex (phospholipoprotein) formed by type II alveolar cells. The proteins and lipids that surfactant comprises have both a… …   Wikipedia

  • proteinosis — A state characterized by disordered protein formation and distribution, particularly as manifested by the deposition of abnormal proteins in tissues. [protein + G. osis, condition] lipoid p. [MIM*247100] a disturbance of lipid metabolism …   Medical dictionary

Share the article and excerpts

Direct link
Do a right-click on the link above
and select “Copy Link”

We are using cookies for the best presentation of our site. Continuing to use this site, you agree with this.