Premium
Autoimmune pulmonary alveolar proteinosis: Treatment options in year 2013
Author(s) -
LETH STEFFEN,
BENDSTRUP ELISABETH,
VESTERGAARD HANNE,
HILBERG OLE
Publication year - 2013
Publication title -
respirology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.857
H-Index - 85
eISSN - 1440-1843
pISSN - 1323-7799
DOI - 10.1111/j.1440-1843.2012.02274.x
Subject(s) - medicine , pulmonary alveolar proteinosis , rituximab , plasmapheresis , inhalation , alveolar macrophage , treatment modality , lung , immunology , antibody , macrophage , anesthesia , biochemistry , chemistry , in vitro
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.