Elisabeth Bendstrup

Autoimmune pulmonary alveolar proteinosis: treatment options in year 2013

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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.

Original languageEnglish
JournalRespirology
Volume18
Issue1
Pages (from-to)82-91
Number of pages10
ISSN1323-7799
DOIs
Publication statusPublished - Jan 2013

    Research areas

  • Antibodies, Monoclonal, Murine-Derived, Autoimmune Diseases, Bronchoalveolar Lavage, Granulocyte-Macrophage Colony-Stimulating Factor, Humans, Immunologic Factors, Plasmapheresis, Pulmonary Alveolar Proteinosis, Treatment Outcome

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