Autoimmune pulmonary alveolar proteinosis in an adolescent successfully treated with inhaled rhGM-CSF (molgramostim). (2018)

• Record Type: Journal Article
• Authors: Gajewska, Marta E.; Sritharan, Sajitha S.; Santoni-Rugiu, Eric; Bendstrup, Elisabeth M.
• Is Part Of: Respiratory medicine case reports. Volume 23(2018)
• Page Start: 167
• Page End: 169
• Publication Date: 2018
• Subjects: Pulmonary alveolar proteinosis -- Granulocyte-macrophage colony-stimulating factor -- GM-SCF -- Molgramostim -- Inhalation therapy; Respiratory organs -- Diseases -- Periodicals; Chest -- Diseases -- Periodicals; Respiratory Tract Diseases -- Periodicals; Respiratory System -- Periodicals; Chest -- Diseases; Respiratory organs -- Diseases; Electronic journals; Periodicals; 616.2
• DOI: 10.1016/j.rmcr.2018.02.005
• Languages: English
• ISSNs: 2213-0071

Abstract

Abstract: Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare parenchymal lung disease characterized by accumulation of surfactant in the airways with high levels of granulocyte-macrophage colony stimulating factor (GM-CSF) antibodies in blood. Disease leads to hypoxemic respiratory failure. Whole lung lavage (WLL) is considered the first line therapy, but procedure can be quite demanding, specifically for children. Recently alternative treatment options with inhaled GM-CSF have been described but no consensus about the standard treatment exists. We here describe a unique case of a 14-year-old patient who was successfully treated with WLL and subsequent inhalations with molgramostim – new recombinant human GM-CSF (rhGM-CSF).