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.
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McDonnell, M.J., Reynolds, C., Tormey, V. et al. Pulmonary alveolar proteinosis: report of two cases in the West of Ireland with review of current literature. Ir J Med Sci 183, 123–127 (2014). https://doi.org/10.1007/s11845-013-0976-0
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DOI: https://doi.org/10.1007/s11845-013-0976-0