Inhaled GM-CSF in a Pulmonary Alveolar Proteinosis Patient Refractory to Plasmapheresis Combined with Multiple Whole Lung Lavages

Francesca Mariani, Elena Paracchini, Davide Piloni, Zamir Kadija, Elena Salvaterra, Laura Divizia, Giuseppe Rodi, Carmine Tinelli, Federica Meloni and Ilaria Campo*

A  autoimmune  Pulmonary  Alveolar  Proteinosis  (PAP)  patient  with  persistent  disease  underwent  3 Whole Lung Lavages (WLLs), 10 plasmapheresis sessions and further 3 WLL, from October 2004 to May 2007. Nevertheless HRTC and pulmonary function test (PFT) showed a persistent residual disease of mild degree. At the beginning of 2010, the patient was admitted to inhaled rGM-CSF (Sargramostim) therapy as compassionate treatment. GM-CSF was administered by Akita 2 nebulizer (Vectura), as follow: 250 mcg/day every other week for 12 weeks, then 250 mcg/day on 2 consecutive days every 2 weeks for 6 months. Follow up visits were scheduled at 3, 10, 18, 30 months and after that once a year. Functional and HRCT data and PaO2 were collected. Since the start of the inhaled GM-CSF therapy, the patient no more required WLL. Furthermore we found a significant increase in DLCO% (p=0.013) and FVC% (p=0.023) while %FEV1 show  a  positive  trend. No  substantial  differences  in  blood  gas  analysis. The  pulmonary  involvement  at HRCT shows a significant decrease of lung infiltrates (p=0.039) in terms of pathological segments. These data underscore the utility of inhaled GM-CSF not only in case of progressing disease but also in case of refractory patients with persistent lung infiltrates, in order to increase the response rate.

Keywords: Inhaled therapy; Pulmonary alveolar proteinosis; GM-CSF; Whole lung lavage

Published on: Apr 6, 2017 Pages: 16-19

Full Text PDF Full Text HTML DOI: 10.17352/aprc.000018
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