Abstract
BACKGROUND: Autoimmune pulmonary alveolar proteinosis is an ultra-rare pulmonary disease. Whole lung lavage (WLL) is considered the gold standard therapy. We report a protocol for a new modified lavage technique (nMLT) in which controlled repetitive manual hyperinflation (MH) and intermittent chest percussion are used to enhance WLL efficacy. METHODS: We included all subjects with autoimmune pulmonary alveolar proteinosis treated with nMLT between 2013 and 2018. nMLT consisted of repetitive MH with intermittent chest percussion every third wash. We reported: instilled volume, protein concentration, and optical density using spectrophotometry. Pulmonary function (FVC %predicted and DLCO %predicted) at start of nMLT was recorded. Data are displayed as mean (±SD), median [interquartile range], or number (%). Comparisons within individuals were made using Students t test. RESULTS: We included 11 subjects (64% male) in whom a total of 67 nMLTs were performed. One nMLT consisted of 15 [12-18] washes. Protein removal was 9.80 [7.52-12.66] g per nMLT. After the first, second, and third cycle of 3 washes, 56% [49% to 61%], 81% [77% to 84%], and 91% [88% to 94%] of the final protein yield was removed, respectively. Optical density was measured 116 times and increased from 1.13 (±0.52) to 1.31 (±0.52) after MH (P<0.001). CONCLUSION: Efficacy of WLL seems to be enhanced by applying MH every 3 washes. Our technique of WLL with nMLT could be used to increase the amount of protein recruited while instilling the lung with the smallest volume of fluid as possible.