TY - JOUR
T1 - Autoimmune pulmonary alveolar proteinosis in an adolescent successfully treated with inhaled rhGM-CSF (molgramostim)
AU - Gajewska, Marta E
AU - Sritharan, Sajitha S
AU - Santoni-Rugiu, Eric
AU - Bendstrup, Elisabeth M
PY - 2018
Y1 - 2018
N2 - 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).
AB - 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).
U2 - 10.1016/j.rmcr.2018.02.005
DO - 10.1016/j.rmcr.2018.02.005
M3 - Journal article
C2 - 29719809
SN - 2213-0071
VL - 23
SP - 167
EP - 169
JO - Respiratory Medicine Case Reports
JF - Respiratory Medicine Case Reports
ER -