Source: International Congress 2014 – ILDs 6
Disease area: Interstitial lung diseases

Abstract

Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by excessive surfactant accumulation in the lung. Whole-lung lavage (WLL) has been the most common therapeutic intervention for PAP. In severe cases WLL may be difficult to perform due to hypoxemia. Multiple segmental lavage (MSL) may be advocated then as a first-choice therapy. We performed MSL in 5 cases of severe PAP (7.5% of our PAP patients cohort). Fiberoptic bronchoscopy with local anaesthesia was performed. 0.5 - 1 liter of warm saline solution with N-acetylcysteine was given and taken back in 50-mL injectors.Each procedure was applied to lobe\segments with the most serious changes in HRCT. The procedure was repeated 2 to 5 times once in 2-3 days. All patients demonstrated marked clinical and physiologic improvement. Mean FVC changed from 58.3±7.8% pred. to 77.5±16.2%pred., DLCO - from 53.1±8.5 to 64.2±7.4%pred. In 2 cases after repeated MSL procedure WLL was performed, so MSL was estimated as “prewash” to improve physical conditions and decrease hypoxemia before long lasting general anaesthesia during WLL.On the contrary, in cases with minimal material accumulation in several segments, WLL may be too invasive procedure. We recommend MSL in these cases also. 4 these cases (6% of our PAP cohort) demonstrated positive changes on HRCT after MSL; mean FVC changed from 75.7±15.3% pred. to 91.0±2.0%pred., DLCO - from 71.1±9.5 to 84.2±8.9%pred.Conclusion: MSL is an alternative technique to WLL in PAP cases with minimal local surfactant accumulation and also in severe cases with hypoxemia. It is effective and well-tolerated. This method should be taken into consideration in the treatment of PAP.

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Citations should be made in the following way:
Y. Ilkovich, L. Novikova, A. Bazhanov (St. Petersburg, Russian Federation). Multiple segmental lavage in the treatment of pulmonary alveolar proteinosis. Eur Respir J 2014; 44: Suppl. 58, 3793

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