title: Pulmonary alveolar proteinosis: report of two cases in the West of Ireland with review of current literature.
abstract: BACKGROUND
  Pulmonary alveolar proteinosis (PAP) is a rare lung condition characterised by the accumulation of lipoproteinaceous surfactant material within alveolar airspaces resulting in clinical manifestations ranging from asymptomatic to severe respiratory failure. Three disease subtypes are recognised: autoimmune, secondary and congenital.
  
  
  METHODS
  We describe two presentations of PAP in the West of Ireland with a review of the current literature.
  
  
  RESULTS
  Autoimmune PAP, associated with the presence of granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibodies, accounts for >90 % of cases. Treatment with whole lung lavage is the current standard of care. Novel therapies targeting alveolar macrophages (recombinant GM-CSF therapy) and anti-GM-CSF antibodies (rituximab, plasmapharesis) are under investigation.
  
  
  CONCLUSIONS
  This is a summary of available literature outlining current clinical practice in the diagnosis, management, and treatment of PAP. PAP should be considered in the differential diagnosis of any patient with a restrictive pulmonary defect. Without high clinical suspicion, this diagnosis can easily be missed.
authors.affiliation: Galway University Hospitals, Galway, Ireland, melissajanefriel@gmail.com.
authors.forename: M J
authors.initials: MJ
authors.lastname: McDonnell
authors.forename: C
authors.initials: C
authors.lastname: Reynolds
authors.forename: V
authors.initials: V
authors.lastname: Tormey
authors.forename: J J
authors.initials: JJ
authors.lastname: Gilmartin
authors.forename: R M
authors.initials: RM
authors.lastname: Rutherford
mesh_terms: D000328:Adult; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000097:blood / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D015415:Biomarkers / Q000097:blood; D018893:Bronchoalveolar Lavage; D005260:Female; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000276:immunology; D006801:Humans; D007494:Ireland; D008297:Male; D008875:Middle Aged; D011649:Pulmonary Alveolar Proteinosis / Q000097:blood / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D013256:Steroids / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; D013256:Steroids; D016178:Granulocyte-Macrophage Colony-Stimulating Factor