INTRODUCTION:Wegener’s Granulomatosis is an uncommon disorder characterized by sometimes severe vasculitis of small and medium sized vessels. Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by an excess accumulation of lipoproteinacious-rich material within the alveoli with both idiopathic and acquired forms. A finding of both diagnoses in the same patient has not been previously reported. A trial of inhaled GM-CSF was initiated with very favorable result.
CASE PRESENTATION:Our patient is a 32 year-old white female with a diagnosis of Wegener’s Granulomatosis made by kidney biopsy and elevated ANCA three years prior to moving to the Albany area. At the time of presentation to our clinic she was significantly hypoxic and dyspneic without cough or fevers. She was on prednisone and cytoxan at the time of evaluation. A CT scan revealed bilateral diffuse ground glass opacities, but a subsequent bronchoscopy would be negative for infection or progressive bloody return. In addition her ANCA profile, ESR and urine sediment analysis were normal. Despite aggressive therapy for presumptive vasculitis flair, her condition did not improve. An open lung biopsy was performed which revealed prominent alveolar accumulation of proteinacious material and focal accumulations of hemosiderin-laden macrophages without active vasculitis. Lung function at the time revealed a FVC of 2.3L (73% predicted), a TLC of 3.6L (73% predicted), and a 6 minute walk of 60 feet with desaturation to 80% on 3LNC. A whole lung lavage was attempted, but significant post-operative hypoxia only allowed for a unilateral lavage. The dyspnea and hypoxia progressed and a CT scan two months after the initial procedure revealed persistent and diffuse ground glass. Serum antibody to GM-CSF was negative. A trial of inhaled leukine 250mcg (GM-CSF) was initiated twice daily- one week on and on week off for 3 months. A CT scan within one month of initiating therapy showed resolution of the ground glass. Lung function revealed a FVC of 3.64L (100% predicted), a 6 minute walk of 900 feet with a saturation of 97% on room air.
DISCUSSIONS:To our knowledge, this is the first reported case of Wegener’s Granulomatosis and pulmonary alveolar proteinosis occurring simultaneously in the same patient. The possibility of a neutralizing autoantibody from the wegener’s disease against GM-CSF protein is very feasible, yet the test for antibody to GM-CSF in this patient was negative. To date, whole lung lavage has been the standard therapy for PAP. Numerous case reports and a retrospective case series suggest significant efficacy with aerosolized GM-CSF. A therapeutic trial of inhaled GM-CSF in our patient has shown tremendous results thus far in symptoms, lung function and imaging after just one month of therapy.
CONCLUSION:In conclusion, we present out experience with aerosolized GM-CSF in our patient with PAP and wegener’s granulomatosis. This therapy appears to be safe and effective in treating PAP providing a viable alternative to whole lung lavage.
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