title: Autoimmune pulmonary alveolar proteinosis: treatment options in year 2013.
abstract: Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by accumulation of a periodic acid Schiff (PAS)-positive eosinophilic material in the distal airways. For decades, the standard treatment of PAP has been whole lung lavage (WLL), where large quantities of saline are instilled into the lungs to remove the proteinaceous material. However, not all patients respond to this treatment. Thus, new treatment modalities, such as subcutaneous or inhaled granulocyte macrophage colony-stimulating factor (GM-CSF), and the CD20 antibody rituximab and plasmapheresis, have been investigated. Based on the current literature, a stepwise treatment plan is suggested starting with WLL, continuing to inhaled GM-CSF, and then to rituximab if the former treatment regimes are unsuccessful.
authors.affiliation: Department of Respiratory Diseases and Allergology, Aarhus University Hospital, Aarhus C, Denmark. stefleth@rm.dk
authors.forename: Steffen
authors.initials: S
authors.lastname: Leth
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Bendstrup
authors.forename: Hanne
authors.initials: H
authors.lastname: Vestergaard
authors.forename: Ole
authors.initials: O
authors.lastname: Hilberg
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000628:therapy*; D018893:Bronchoalveolar Lavage; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage / Q000627:therapeutic use; D010956:Plasmapheresis / Q000379:methods; D011649:Pulmonary Alveolar Proteinosis / Q000188:drug therapy / Q000628:therapy*; D000069283:Rituximab; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab; D016178:Granulocyte-Macrophage Colony-Stimulating Factor