title: A plasmapheresis protocol for refractory pulmonary alveolar proteinosis.
abstract: Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of proteinaceous material within the lungs. While typically managed with whole lung lavage (WLL), more recent PAP therapies aimed at reducing granulocyte-macrophage colony stimulating factor autoantibodies (anti-GM-CSF) have reduced symptoms and improved lung function. We present a patient with PAP refractory to WLL, exogenous GM-CSF and rituximab who underwent a novel plasmapheresis protocol as a therapeutic trial. While previously reported regimens have utilized plasmapheresis sessions distributed over months, our patient underwent five consecutive days of plasmapheresis, followed by rituximab. Anti-GM-CSF levels decreased from 24.8 to 2.7 mcg/mL post-plasmapheresis. This reduction of autoantibody correlated with reduction in WLL frequency, increase in diffusing capacity for carbon monoxide, and subjective improvement in dyspnea. Our case suggests that five consecutive days of plasmapharesis results in increased clearance of anti-GM-CSF and may be potentially efficacious in cases of refractory PAP.
authors.affiliation: Department of Internal Medicine, David Geffen School of Medicine at UCLA, 757 Westwood Plaza, Suite 7236E, Los Angeles, CA, 90095, USA, bgarber@mednet.ucla.edu.
authors.forename: Bryan
authors.initials: B
authors.lastname: Garber
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Albores
authors.forename: Tisha
authors.initials: T
authors.lastname: Wang
authors.forename: Thanh H
authors.initials: TH
authors.lastname: Neville
mesh_terms: D000328:Adult; D001323:Autoantibodies / Q000097:blood; D018893:Bronchoalveolar Lavage; D002985:Clinical Protocols; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000276:immunology; D006801:Humans; D008297:Male; D010956:Plasmapheresis; D011649:Pulmonary Alveolar Proteinosis / Q000628:therapy*; D019233:Retreatment
chemical_list: D001323:Autoantibodies; D016178:Granulocyte-Macrophage Colony-Stimulating Factor