Whole-Lung Lavage: A Successful Treatment for Restoring Acinar Ventilation Distribution in Primary Acquired Pulmonary Alveolar Proteinosis.

A 51-year-old active smoker with primary acquired pulmonary alveolar proteinosis (PAP) diagnosed by biopsy and anti-GM-CSF antibodies was treated safely with whole-lung lavage (WLL). This resulted in a rapid improvement of symptoms and arterial blood oxygenation, but not of standard lung function parameters. However, we also performed the multiple-breath nitrogen washout (MBW) test to determine the lung clearance index (LCI) as well as indices of acinar ventilation heterogeneity (Sacin) and conductive ventilation heterogeneity (Scond). At baseline, a distinct abnormality was seen for Sacin and LCI, while Scond was at the upper limit of normal for this subject. Sacin, in particular, was in excess of the Sacin abnormality corresponding to a 20-pack-year smoking history. Immediately after WLL, Sacin and Scond both fell to within a normal range while LCI also decreased but remained abnormal. The Sacin decrease was much greater than the Scond decrease, which was to be expected after 1 week of smoking cessation at the hospital (smoking was resumed after release from hospital). A follow-up visit 7 weeks after WLL revealed a spectacular improvement on CT scan and improvements in standard lung function. Another follow-up visit 14 weeks after WLL showed further improvements in standard lung function, and both Sacin and Scond remained well within the normal range, and LCI was above the upper limit of normal. We conclude that in this patient, removal of excess surfactant by WLL resulted in a restored ventilation distribution in most of the distal air spaces. Copyright © 2012 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]