Whole lung lavage combined with Granulocyte-macrophage colony stimulating factor inhalation for an adult case of refractory pulmonary alveolar proteinosis.

Hong Yan Yu,Yan Xun Wang,Hui Huang,Zuo Jun Xu,Xue Feng Sun

doi:10.1186/1471-2466-14-87

Abstract

BackgroundWhole-lung lavage (WLL) is classically the first-line treatment for symptomatic pulmonary alveolar proteinosis (PAP). However, some patients require multiple WLLs because of refractory nature of their PAP. In this instance, these patients may benefit from new treatment regimens, and new therapies should be tried for these patients.Case presentationWe describe a 47-year-old Chinese woman who was confidently diagnosed with pulmonary alveolar proteinosis (PAP) after bronchoalveolar lavage and transbronchial lung biopsy. The patient received four sessions of bilateral whole lung lavage (WLL) and one session of WLL in combination with plasmapheresis, each only producing short-term symptomatic relief. The patient was given a trial of combination therapy, which consisted of WLL and Granulocyte-macrophage colony-stimulating factor (GM-CSF) inhalation. The patient showed a gradual improvement in oxygenation and her daily activity, as well as a dramatic improvement in her pulmonary CT examination.ConclusionBilateral WLL, in combination with GM-CSF inhalation, may be an effective treatment option for severe refractory PAP.

Highlights

Whole-lung lavage (WLL) is classically the first-line treatment for symptomatic pulmonary alveolar proteinosis (PAP)
Pulmonary alveolar proteinosis (PAP) is a rare but treatable disease that is characterized by the accumulation of a periodic acid Schiff (PAS) positive lipoproteinaceous substance in the alveoli
Shah et al shared their experience in treating PAP patients with Whole lung lavage (WLL) [5], concluding that in their experience more than 60% of patients had a good response within two sessions of WLL

Summary

Background

Pulmonary alveolar proteinosis (PAP) is a rare but treatable disease that is characterized by the accumulation of a periodic acid Schiff (PAS) positive lipoproteinaceous substance in the alveoli. Hematoxylin and eosin staining of the patient’s transbronchial lung biopsy (TBLB) showed multiple alveolar spaces and bronchioles filled with a granular acidophilic acellular material, which stained bright pink with PAS Her room air arterial blood gas analysis was consistent with type 1 respiratory failure, but could be corrected with oxygen via nasal cannula (NC) at 2 L/min. The patient received WLL on June 1st 2012 first, and was started on rhGM-CSF inhalation therapy at a dosage of 150 μg twice daily for 8 days, which was stopped for 6 days. This cycle was repeated every 2 weeks over the 3 months.

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Discussion

Conclusion