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Pulmonary alveolar proteinosis
Classification and external resources
ICD-9 516.0
OMIM 265120 610913 610921 610910
DiseasesDB 29642
MedlinePlus 000114
eMedicine med/1927
MeSH D011649

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.

Contents

History and pathology

PAP was first described in 1958[1] by the physicians Samuel Rosen, Benjamin Castleman, and Averill Liebow.[2] In their case series published in the New England Journal of Medicine on June 7 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.

Pathophysiology

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.[3]

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. [4]

Epidemiology

The disease is more common in males and in tobacco smokers.

In a recent epidemiologic study from Japan [5], Autoimmune PAP has an incidence and prevalence higher than previously reported and is not strongly linked to smoking, occupational exposure, or other illnesses.

Symptoms

The symptoms of PAP include:

Chest 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. On histopathological examination distal air spaces are filled with a granular, eosinophillic material that stain positively with PAS reagent and is diastase resistant.

Treatment

The standard treatment for PAP is whole-lung lavage,[7][8] 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.

Links

-Pap Foundation/ The american foundation for patients affected by PAP

-PAP Europe/ The european consortium for PAP

-ORPHANET/ The portal for rare diseases and orphan drugs

References

  1. ^ Seymour JF, Presneill JJ (July 2002). "Pulmonary alveolar proteinosis: progress in the first 44 years". Am. J. Respir. Crit. Care Med. 166 (2): 215–35. doi:10.1164/rccm.2109105. PMID 12119235. http://ajrccm.atsjournals.org/cgi/pmidlookup?view=long&pmid=12119235.  
  2. ^ Rosen SH, Castleman B, and Liebow AA. Pulmonary alveolar proteinosis. New England Journal of Medicine 1958; 258: 1123-1142.
  3. ^ Stanley E, Lieschke GJ, Grail D, et al. (June 1994). "Granulocyte/macrophage colony-stimulating factor-deficient mice show no major perturbation of hematopoiesis but develop a characteristic pulmonary pathology". Proc. Natl. Acad. Sci. U.S.A. 91 (12): 5592–6. doi:10.1073/pnas.91.12.5592. PMID 8202532. PMC 44042. http://www.pnas.org/cgi/pmidlookup?view=long&pmid=8202532.  
  4. ^ 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 (2007). "GM-CSF autoantibodies and neutrophil dysfunction in pulmonary alveolar proteinosis". N Engl J Med 356 (6): 567–79. doi:10.1056/NEJMoa062505. PMID 17287477.  
  5. ^ Inoue Y, Trapnell BC, Tazawa R,et (April 2008). "Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in". Am. J. Respir. Crit. Care Med. 177 (7): 752–62. http://ajrccm.atsjournals.org/cgi/content/full/177/7/752.  
  6. ^ Shah PL, Hansell D, Lawson PR, Reid KB, Morgan C (January 2000). "Pulmonary alveolar proteinosis: clinical aspects and current concepts on pathogenesis". Thorax 55 (1): 67–77. doi:10.1136/thorax.55.1.67. PMID 10607805. PMC 1745595. http://thorax.bmj.com/cgi/pmidlookup?view=long&pmid=10607805.  
  7. ^ Ceruti M, Rodi G, Stella GM, et al. (2007). "Successful whole lung lavage in pulmonary alveolar proteinosis secondary to lysinuric protein intolerance: a case report". Orphanet J Rare Dis 2: 14. doi:10.1186/1750-1172-2-14. PMID 17386098. PMC 1845139. http://www.ojrd.com/content/2//14.  
  8. ^ Menard KJ (April 2005). "Whole lung lavage in the treatment of pulmonary alveolar proteinosis". J. Perianesth. Nurs. 20 (2): 114–26. doi:10.1016/j.jopan.2005.01.005. PMID 15806528. http://linkinghub.elsevier.com/retrieve/pii/S1089947205000067.  
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