Indication of whole lung lavage in pulmonary alveolar proteinosis – Retrospective study

Abstract

Introduction: Pulmonary alveolar proteinosis is a rare disease consisting in accumulation of surfactant in the alveolar space (caused mostly by an autoimmune disorder), leading to alteration of gas exchange. Gold standard for treatment is whole lung lavage (WLL), an invasive procedure without standard indications. Aims and objectives: To evaluate reprospectively the main indications for WLL in 20 patients with proteinosis. Methods: All patients with proteinosis addmitted in Institute of Pneumology, Buchares during 5 years were analysed. Patients in whom WLL was performed were compared to those without WLL regarding clinical and paraclinical tests (blood samples, arterial blood gases, spirometry, pletismography, diffusing capacity, six minutes walking test). Statistical differences were assessed. Results: All 12 men and 8 women had a mean age of 43±16.59 years. The diagnosis was established using bronchoscopy and bronchoalveolar lavage in 18 patients (open lung biopsy for the rest). Respiratory noxes were noted in 4 patients, 4 current smokers were identified, and 4 former smokers. Two patients were considered too severe for WLL (and where excluded from analysis); 14 underwent WLL and in 4 patients WLL wasn9t performed (lack of indication, less severe cases). Compared to the letter, patients in whom WLL was performed had a more severe restriction estimated with total lung capacity: 2.88±0.83 versus 5±0L, (p=0.009) and residual volume: 0.89± 0.6 versus 1.75±0.5L, (p=0.030). A trend for a lower transfer factor in patients with WLL was noted (40.83±15.06 versus 70±15.55%, p=0.056). Conclusion: Most important tests in deciding the indication of WLL were pletismography and diffusing capacity.