Autoimmune pulmonary alveolar proteinosis: Treatment options in year 2013
Article first published online: 26 DEC 2012
© 2012 The Authors. Respirology © 2012 Asian Pacific Society of Respirology
Volume 18, Issue 1, pages 82–91, January 2013
How to Cite
LETH, S., BENDSTRUP, E., VESTERGAARD, H. and HILBERG, O. (2013), Autoimmune pulmonary alveolar proteinosis: Treatment options in year 2013. Respirology, 18: 82–91. doi: 10.1111/j.1440-1843.2012.02274.x
- Issue published online: 26 DEC 2012
- Article first published online: 26 DEC 2012
- Accepted manuscript online: 4 OCT 2012 09:01AM EST
- Received 1 March 2012; invited to revise 13 April 2012, 17 June 2012; revised 10 May 2012, 19 June 2012; accepted 8 July 2012 (Associate Editor: Yuben Moodley).
- bronchoalveolar lavage;
- granulocyte macrophage colony-stimulating factor;
- pulmonary alveolar proteinosis;
- whole lung lavage
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.