Rituximab therapy in pulmonary alveolar proteinosis improves alveolar macrophage lipid homeostasis
نویسندگان
چکیده
منابع مشابه
Rituximab therapy in pulmonary alveolar proteinosis improves alveolar macrophage lipid homeostasis
RATIONALE Pulmonary Alveolar Proteinosis (PAP) patients exhibit an acquired deficiency of biologically active granulocyte-macrophage colony stimulating factor (GM-CSF) attributable to GM-CSF specific autoantibodies. PAP alveolar macrophages are foamy, lipid-filled cells with impaired surfactant clearance and markedly reduced expression of the transcription factor peroxisome proliferator-activat...
متن کاملRituximab therapy in autoimmune pulmonary alveolar proteinosis.
Idiopathic pulmonary alveolar proteinosis is presumed to be an autoimmune disorder that may lead to pulmonary insufficiency. However, steroids do not appear to be effective and the standard of therapy is whole-lung lavage. We report the first case of successful therapy with rituximab, which addresses the pathogenic mechanism of pulmonary alveolar proteinosis.
متن کاملGranulocyte macrophage colony stimulating factor therapy for pulmonary alveolar proteinosis.
We report a case of 58 year old female diagnosed with Pulmonary Alveolar Proteinosis (PAP) with recurrence of PAP after 5 repeated whole lung lavage, responding to subcutaneous injections of Granulocyte Macrophage Colony Stimulating Factor therapy (GM-CSF). Thus indicating that GM-CSF therapy is a promising alternative in those requiring repeated whole lung lavage
متن کاملPulmonary Alveolar Proteinosis
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متن کاملPulmonary alveolar proteinosis.
Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease characterised by alveolar accumulation of surfactant. It may result from mutations in surfactant proteins or granulocyte macrophage-colony stimulating factor (GM-CSF) receptor genes, it may be secondary to toxic inhalation or haematological disorders, or it may be auto-immune, with anti-GM-CSF antibodies blocking activation of alv...
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ژورنال
عنوان ژورنال: Respiratory Research
سال: 2012
ISSN: 1465-9921
DOI: 10.1186/1465-9921-13-46