| Pulmonary alveolar proteinosis. | |
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MedLine Citation:
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PMID: 21632797 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease characterised by alveolar accumulation of surfactant. It may result from mutations in surfactant proteins or granulocyte macrophage-colony stimulating factor (GM-CSF) receptor genes, it may be secondary to toxic inhalation or haematological disorders, or it may be auto-immune, with anti-GM-CSF antibodies blocking activation of alveolar macrophages. Auto-immune alveolar proteinosis is the most frequent form of PAP, representing 90% of cases. Although not specific, high-resolution computed tomography shows a characteristic "crazy paving" pattern. In most cases, bronchoalveolar lavage findings establish the diagnosis. Whole lung lavage is the most effective therapy, especially for auto-immune disease. Novel therapies targeting alveolar macrophages (recombinant GM-CSF therapy) or anti-GM-CSF antibodies (rituximab and plasmapheresis) are being investigated. Our knowledge of the pathophysiology of PAP has improved in the past 20 yrs, but therapy for PAP still needs improvement. |
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Authors:
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R Borie; C Danel; M-P Debray; C Taille; M-C Dombret; M Aubier; R Epaud; B Crestani |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: European respiratory review : an official journal of the European Respiratory Society Volume: 20 ISSN: 1600-0617 ISO Abbreviation: Eur Respir Rev Publication Date: 2011 Jun |
Date Detail:
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Created Date: 2011-06-02 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9111391 Medline TA: Eur Respir Rev Country: Denmark |
Other Details:
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Languages: eng Pagination: 98-107 Citation Subset: IM |
Affiliation:
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Service de Pneumologie A, Centre de Compétences des Maladies Pulmonaires Rares, ¶ Laboratoire d'Anatomie Pathologique, + Service de Radiologie, Assistance Publique Hôpitaux de Paris, Hôpital Bichat, # Inserm, Unité 700, Faculté Bichat, Université Paris 7, § Université Paris Diderot, Faculté de Médecine site Bichat, Paris, f Inserm, Unité 955, Université Paris-Est Créteil Val de Marne, and ** Centre intercommunal de Créteil, Créteil, France. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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