Long-term durable benefit after whole lung lavage in pulmonary alveolar proteinosis

Abstract

Long-term durable benefit after whole lung lavage in pulmonary alveolar proteinosis. M. Beccaria, M. Luisetti, G. Rodi, A. Corsico, M.C. Zoia, S. Colato, P. Pochetti, A. Braschi, E. Pozzi, I. Cerveri. #ERS Journals Ltd 2004. ABSTRACT: Whole lung lavage (WLL) is still the gold-standard therapy for pulmonary alveolar proteinosis (PAP). The few studies on the duration of the effect of WLL, belonging to a rather remote period, show significant but transient benefits.In 21 patients with idiopathic PAP, the duration of any benefit and, in 16 of them, the time course of lung function improvement (at baseline, 1 week, 6 months, 1 yr and then every 2 yrs after WLL) were evaluated. The present WLL technique takes longer, is invasively monitored and partially modified with respect to past techniques.More than 70% of patients remained free from recurrent PAP at 7 yrs. The bulk of the improvement in spirometric results was almost completely gained in the immediate post-WLL period due to the efficient clearance of the alveoli. At a median of 5 yrs, recovery of diffusing capacity of the lung for carbon monoxide was incomplete (75¡19% of the predicted value) and there were residual gas exchange abnormalities (alveolar to arterial oxygen tension difference 3.6¡1.5 kPa (27¡11 mmHg)) and exercise limitation, probably explained by engorgement of lymphatic vessels.In conclusion, whole lung lavage for idiopathic pulmonary alveolar proteinosis is currently a safe procedure in an experienced setting, and provides long-lasting benefits in the majority of patients.

Plasmapheresis for treatment of pulmonary alveolar proteinosis

“…Lung function improved, as evidenced by a change in the gas exchange after the WLL therapy. The therapeutic effects of WLL were transient and lasted only a few months, in contrast to most PAP patients treated by WLL at our centre using the same technique [4]. minimal and transient improvement.…”

Successful pregnancy in a severely hypoxemic patient with pulmonary alveolar proteinosis

“…It can be performed one lung at a time or in both lungs sequentially if patient is considered to tolerate one-lung ventilation of the lung that has just been treated. 10,11 Improvement in arterial oxygen tension can be observed after one week. In this case, dyspnea, impact on daily activities and development of respiratory failure were indications to BPL 10,12 and actual improvement was noted after the procedures, allowing signifi cant decrease in oxygen needs.…”

Anestesia para lavagem pulmonar em paciente pediátrico portador de proteinose alveolar pulmonar

“…WLL remains the treatment of choice for PAP and is a safe procedure that provides lasting benefits with more than 70% of patients free of recurrence for seven years [13][14][15][16][17][18] .…”

“…Currently the WLL technique with a double-lumen tube and lung isolation is well established for adult patients 3,4,[13][14][15][16][17][18][19] . However, pediatric patients present a technical challenge for WLL due to the absence of DLET compatible with the airway caliber of these patients [20][21][22][23] .…”