Terry E Robinson1, Bruce C Trapnell2, Michael L Goris3, Lynne M Quittell4, David N Cornfield5. 1. Center of Excellence in Pulmonary Biology, Stanford University Medical Center, Stanford, CA. Electronic address: ter@stanford.edu. 2. Division of Pulmonary Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH. 3. Department of Radiology, Stanford University Medical Center, Stanford, CA. 4. Division of Pulmonary Medicine, New York-Presbyterian Hospital/Columbia University Medical Center, New York, NY. 5. Center of Excellence in Pulmonary Biology, Stanford University Medical Center, Stanford, CA.
Abstract
BACKGROUND: Autoimmune pulmonary alveolar proteinosis (APAP) is characterized by autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF) in blood and tissues, resulting in alveolar surfactant protein accumulation. Patients with APAP present with ground-glass opacities (GGOs) and interlobular septal thickening on thin-slice chest CT scans. Aerosolized GM-CSF therapy (aeroGM-SCF) has qualitatively improved the clinical condition of patients with APAP. This report details quantitative chest CT responses to aeroGM-CSF. METHODS: Two adolescent patients (aged 16 and 19 years) with APAP were treated with aeroGM-CSF. Clinical parameters, including pulmonary function tests and chest CT scans, were obtained before and after aeroGM-CSF therapy. To evaluate the effect of the therapy, serial chest CT scans were analyzed using a novel approach permitting quantitative assessment of improvement in GGOs, lung weight, and gas volume. RESULTS: In association with GM-CSF treatment, nutritional status and pulmonary function improved. Quantitative analysis of the CT scans demonstrated reduction in GGOs and lung weight, concomitant with an increase in airspace volume and lung inflation. The findings were consistent with a qualitative reduction in GGOs on chest CT imaging. CONCLUSIONS: Quantitative analysis of CT holds promise as a sensitive diagnostic tool permitting longitudinal and objective analysis of the therapeutic response to aeroGM-CSF in patients with APAP.
BACKGROUND:Autoimmune pulmonary alveolar proteinosis (APAP) is characterized by autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF) in blood and tissues, resulting in alveolar surfactant protein accumulation. Patients with APAP present with ground-glass opacities (GGOs) and interlobular septal thickening on thin-slice chest CT scans. Aerosolized GM-CSF therapy (aeroGM-SCF) has qualitatively improved the clinical condition of patients with APAP. This report details quantitative chest CT responses to aeroGM-CSF. METHODS: Two adolescent patients (aged 16 and 19 years) with APAP were treated with aeroGM-CSF. Clinical parameters, including pulmonary function tests and chest CT scans, were obtained before and after aeroGM-CSF therapy. To evaluate the effect of the therapy, serial chest CT scans were analyzed using a novel approach permitting quantitative assessment of improvement in GGOs, lung weight, and gas volume. RESULTS: In association with GM-CSF treatment, nutritional status and pulmonary function improved. Quantitative analysis of the CT scans demonstrated reduction in GGOs and lung weight, concomitant with an increase in airspace volume and lung inflation. The findings were consistent with a qualitative reduction in GGOs on chest CT imaging. CONCLUSIONS: Quantitative analysis of CT holds promise as a sensitive diagnostic tool permitting longitudinal and objective analysis of the therapeutic response to aeroGM-CSF in patients with APAP.
Authors: Takuji Suzuki; Takuro Sakagami; Lisa R Young; Brenna C Carey; Robert E Wood; Maurizio Luisetti; Susan E Wert; Bruce K Rubin; Katharine Kevill; Claudia Chalk; Jeffrey A Whitsett; Carrie Stevens; Lawrence M Nogee; Ilaria Campo; Bruce C Trapnell Journal: Am J Respir Crit Care Med Date: 2010-07-09 Impact factor: 21.405