Pulmonary alveolar proteinosis: diagnostic and therapeutic challenges
<p>Abstract</p> <p>Pulmonary Alveolar Proteinosis (PAP) is a rare syndrome characterized by pulmonary surfactant accumulation within the alveolar spaces. It occurs with a reported prevalence of 0.1 per 100,000 individuals and in distinct clinical forms: autoimmune (previously refer...
| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Mattioli 1885
2012-06-01
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| Series: | Multidisciplinary Respiratory Medicine |
| Subjects: | |
| Online Access: | http://www.mrmjournal.com/content/7/1/4 |
| _version_ | 1827264488842199040 |
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| author | Campo Ilaria Kadija Zamir Mariani Francesca Paracchini Elena Rodi Giuseppe Mojoli Francesco Braschi Antonio Luisetti Maurizio |
| author_facet | Campo Ilaria Kadija Zamir Mariani Francesca Paracchini Elena Rodi Giuseppe Mojoli Francesco Braschi Antonio Luisetti Maurizio |
| author_sort | Campo Ilaria |
| collection | DOAJ |
| description | <p>Abstract</p> <p>Pulmonary Alveolar Proteinosis (PAP) is a rare syndrome characterized by pulmonary surfactant accumulation within the alveolar spaces. It occurs with a reported prevalence of 0.1 per 100,000 individuals and in distinct clinical forms: autoimmune (previously referred to as the idiopathic form, represents the vast majority of PAP cases, and is associated with Granulocyte-Macrophage Colony Stimulating Factor (GM-CSF) auto-antibodies; GMAbs), secondary (is a consequence of underlying disorders), congenital (caused by mutations in the genes encoding for the GM-CSF receptor), and PAP-like syndromes (disorders associated with surfactant gene mutations). The clinical course of PAP is variable, ranging from spontaneous remission to respiratory failure. Whole lung lavage (WLL) is the current standard treatment for PAP patients and although it is effective in the majority of cases, disease persistence is not an unusual outcome, even if disease is well controlled by WLL.</p> <p>In this paper we review the therapeutic strategies which have been proposed for the treatment of PAP patients and the progress which has been made in the understanding of the disease pathogenesis.</p> |
| first_indexed | 2024-03-07T18:34:56Z |
| format | Article |
| id | doaj.art-496163df17bc4f59a86890f62b9b2535 |
| institution | Directory Open Access Journal |
| issn | 2049-6958 |
| language | English |
| last_indexed | 2025-03-22T03:46:52Z |
| publishDate | 2012-06-01 |
| publisher | Mattioli 1885 |
| record_format | Article |
| series | Multidisciplinary Respiratory Medicine |
| spelling | doaj.art-496163df17bc4f59a86890f62b9b25352024-04-28T11:27:18ZengMattioli 1885Multidisciplinary Respiratory Medicine2049-69582012-06-0171410.1186/2049-6958-7-4Pulmonary alveolar proteinosis: diagnostic and therapeutic challengesCampo IlariaKadija ZamirMariani FrancescaParacchini ElenaRodi GiuseppeMojoli FrancescoBraschi AntonioLuisetti Maurizio<p>Abstract</p> <p>Pulmonary Alveolar Proteinosis (PAP) is a rare syndrome characterized by pulmonary surfactant accumulation within the alveolar spaces. It occurs with a reported prevalence of 0.1 per 100,000 individuals and in distinct clinical forms: autoimmune (previously referred to as the idiopathic form, represents the vast majority of PAP cases, and is associated with Granulocyte-Macrophage Colony Stimulating Factor (GM-CSF) auto-antibodies; GMAbs), secondary (is a consequence of underlying disorders), congenital (caused by mutations in the genes encoding for the GM-CSF receptor), and PAP-like syndromes (disorders associated with surfactant gene mutations). The clinical course of PAP is variable, ranging from spontaneous remission to respiratory failure. Whole lung lavage (WLL) is the current standard treatment for PAP patients and although it is effective in the majority of cases, disease persistence is not an unusual outcome, even if disease is well controlled by WLL.</p> <p>In this paper we review the therapeutic strategies which have been proposed for the treatment of PAP patients and the progress which has been made in the understanding of the disease pathogenesis.</p>http://www.mrmjournal.com/content/7/1/4GM-CSFPulmonary alveolar proteinosisWLL |
| spellingShingle | Campo Ilaria Kadija Zamir Mariani Francesca Paracchini Elena Rodi Giuseppe Mojoli Francesco Braschi Antonio Luisetti Maurizio Pulmonary alveolar proteinosis: diagnostic and therapeutic challenges Multidisciplinary Respiratory Medicine GM-CSF Pulmonary alveolar proteinosis WLL |
| title | Pulmonary alveolar proteinosis: diagnostic and therapeutic challenges |
| title_full | Pulmonary alveolar proteinosis: diagnostic and therapeutic challenges |
| title_fullStr | Pulmonary alveolar proteinosis: diagnostic and therapeutic challenges |
| title_full_unstemmed | Pulmonary alveolar proteinosis: diagnostic and therapeutic challenges |
| title_short | Pulmonary alveolar proteinosis: diagnostic and therapeutic challenges |
| title_sort | pulmonary alveolar proteinosis diagnostic and therapeutic challenges |
| topic | GM-CSF Pulmonary alveolar proteinosis WLL |
| url | http://www.mrmjournal.com/content/7/1/4 |
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