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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\u2212macrophage 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. 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