Whole lung lavage in idiopathic pulmonary alveolar proteinosis: Results from a 6-year program

Purpose: In 1990 we started a program of whole lung lavage (WLL) in patients suffering from idiopathic pulmonary alveolar proteinosis (PAP). Aim of our work was to review the clinical records of patients enrolled in the program and to evaluate the long-term efficacy of the WLL. Methods: Diagnosis of idiopathic PAP was achieved by lung biopsy or pathologic level of SP-A (measured by antigen inhibition ELISA) and the exclusion of underlying conditions associated with secondary PAP. Based on marked, stable or worsening hypoxemia, eligible patients were submitted to WLL, performed by a left bilumen endotracheal tube, lavage and chest wall percussion of non-dependent lung and simultaneous single-lung ventilation of dependent lung. Results: Eleven patients (7 males/4 females, mean age 40 ± 9.6 yrs, range 26-50) with idiopathic PAP were eveluated. Seven pts received a total of 11 WLL (1 pt 4 WLL, 1 pt 2 WLL, and the 5 remaining 1 WLL each). At the end of the WLL, in 3 pts the last effluent showed SP-A levels close to normal (1±1 μg/ml). All pts were successfully weaned and extubated within 24 hrs after completion of WLL. No complications were observed and oxygenation and respiratory tests improved markedly. Conclusions: In 5 out of 7 pts a single WLL was sufficient to restore a long-term improvement of respiratory function level. In 2 pts this was achieved with multiple WLL. Clinical implications: When needed, WLL is a safe procedure that may be repeated until the natural history of idiopathic PAP is modified.