Summary for clinicians: ERS guidelines on pulmonary alveolar proteinosis.
Pulmonary alveolar proteinosis (PAP) is a rare lung disease caused by accumulation of surfactant in the alveoli, leading to debilitating respiratory symptoms and impaired gas exchange. The recent European Respiratory Society guidelines provide evidence-based recommendations for its diagnosis and man...
| Publicado en: | Breathe Vol. 21; no. 2; pp. 1 - 13 |
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| Autores principales: | , , , , |
| Formato: | diagnostic images practice guidelines tables/charts Journal Article |
| Publicado: |
European Respiratory Society
Apr2025
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=185791015&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 185791015 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 18106838 85SQ jtl: Breathe issn: 18106838 maglogo: N pubinfo: dt: Apr2025 vid: 21 iid: 2 pid: 76609 pub: European Respiratory Society artinfo: ui: 185791015 185791015 185791015 10.1183/20734735.0224-2024 185791015 ppf: 1 ppct: 12 formats: fmt: @attributes: type: P tig: atl: Summary for clinicians: ERS guidelines on pulmonary alveolar proteinosis. aug: au: Alfaro O., Tiago McCarthy, Cormac Bonella, Francesco Bendstrup, Elisabeth O'Callaghan, Marissa affil: Pneumology Unit, Unidade Local de Saúde de Coimbra, Coimbra, Portugal sug: subj: Pulmonary Alveolar Proteinosis Diagnosis Pulmonary Alveolar Proteinosis Therapy Lung Diseases Diagnosis Lung Diseases Therapy Medical Practice, Evidence-Based Autoimmune Diseases Granulocyte-Macrophage Colony-Stimulating Factor Autoantibodies Bronchoalveolar Lavage Rituximab Therapeutic Use Plasmapheresis Lung Transplantation ab: Pulmonary alveolar proteinosis (PAP) is a rare lung disease caused by accumulation of surfactant in the alveoli, leading to debilitating respiratory symptoms and impaired gas exchange. The recent European Respiratory Society guidelines provide evidence-based recommendations for its diagnosis and management. Autoimmune PAP (aPAP) is the most common form, driven by granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibodies. Recommended diagnostic tools include bronchoalveolar lavage and quantitative GM-CSF antibody testing. Whole lung lavage and inhaled GM-CSF are first-line treatments for symptomatic or progressive aPAP. Rituximab, plasmapheresis, and lung transplantation are options for refractory disease. Referral to expert centres is advised for diagnostic and therapeutic guidance. This case-based summary for clinicians highlights the best clinical approach to patients with suspicion or confirmation of PAP. pubtype: Academic Journal doctype: diagnostic images practice guidelines tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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