1. Long-term inhaled granulocyte macrophage-colony-stimulating factor in autoimmune pulmonary alveolar proteinosis: effectiveness, safety, and lowest effective dose
- Author
-
Spyros Papiris, Panagiotis Tsirigotis, Effrosyni D. Manali, Georgia Papadaki, Christina Triantafillidou, Anastasia Papaporfyriou, Matthias Griese, Likurgos Kolilekas, Anna Karakatsani, Konstantinos Kagouridis, and Andriana I. Papaioannou
- Subjects
Adult ,Male ,medicine.medical_specialty ,CD34 ,Granulocyte ,Pulmonary Alveolar Proteinosis ,Gastroenterology ,Autoimmune Diseases ,Cohort Studies ,Young Adult ,Pharmacotherapy ,Internal medicine ,Medicine ,Humans ,Pharmacology (medical) ,Young adult ,Aged ,Retrospective Studies ,Dose-Response Relationship, Drug ,business.industry ,Granulocyte-Macrophage Colony-Stimulating Factor ,Retrospective cohort study ,General Medicine ,Middle Aged ,Effective dose (pharmacology) ,Haematopoiesis ,medicine.anatomical_structure ,Granulocyte macrophage colony-stimulating factor ,Treatment Outcome ,Immunology ,Female ,business ,medicine.drug ,Follow-Up Studies - Abstract
Granulocyte macrophage–colony-stimulating factor (GM-CSF) causes variable improvement in autoimmune pulmonary alveolar proteinosis (aPAP). Upon response to short-term treatment, patients are divided into responders and non-responders. The aim of this study was to test the hypothesis that long-term inhaled GM-CSF (iGM-CSF) is effective in all patients and that attainment of remission permits gradual de-escalation of the dose to the lowest effective safe dose. Patients were treated with iGM-CSF 250 μg once a day given 4 days on and 4 days off for as long as necessary (the “as far as it takes” protocol). Upon remission, defined as absence of symptoms, oxygen desaturation
- Published
- 2014