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 (S-acin) and conductive ventilation heterogeneity (S-cond). At baseline, a distinct abnormality was seen for S-acin and LCI, while S-cond was at the upper limit of normal for this subject. S-acin, in particular, was in excess of the S-acin abnormality corresponding to a 20-pack-year smoking history. Immediately after WLL, S-acin and S-cond both fell to within a normal range while LCI also decreased but remained abnormal. The S-acin decrease was much greater than the S-cond 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 S-acin and S-cond 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.
|Number of pages||5|
|Publication status||Published - 2012|
- Ventilation distribution
- Pulmonary alveolar proteinosis
- Whole-lung lavage