Whole lung lavage therapy for pulmonary alveolar proteinosis: a global survey of current practices and procedures

Abstract

BackgroundWhole lung lavage (WLL) is the current standard of care treatment for patients affected by pulmonary alveolar proteinosis (PAP). However, WLL is not standardized and international consensus documents are lacking.Our aim was to obtain a factual portrayal of WLL as currently practiced with respect to the procedure, indications for its use, evaluation of therapeutic benefit and complication rate.MethodsA clinical practice survey was conducted globally by means of a questionnaire and included 27 centers performing WLL in pediatric and/or adult PAP patients.ResultsWe collected completed questionnaires from 20 centres in 14 countries, practicing WLL in adults and 10 centers in 6 countries, practicing WLL in pediatric patients.WLL is almost universally performed under general anesthesia, with a double-lumen endobronchial tube in two consecutive sessions, with an interval of 1–2 weeks between sessions in approximately 50 % of centres. The use of saline warmed to 37 °C, drainage of lung lavage fluid by gravity and indications for WLL therapy in PAP were homogenous across centres.There was great variation in the choice of the first lung to be lavaged: 50 % of centres based the choice on imaging, whereas 50 % always started with the left lung. The choice of position was also widely discordant; the supine position was chosen by 50 % of centres. Other aspects varied significantly among centres including contraindications, methods and timing of follow up, use of chest percussion, timing of extubation following WLL and lung isolation and lavage methods for small children. The amount of fluid used to perform the WLL is a critical aspect. Whilst a general consensus exists on the single aliquot of fluid for lavage (around 800 ml of warm saline, in adults) great variability exists in the total volume instilled per lung, ranging from 5 to 40 liters, with an average of 15.4 liters/lung.ConclusionsThis international survey found that WLL is safe and effective as therapy for PAP. However these results also indicate that standardization of the procedure is required; the present survey represents the a first step toward building such a document.Electronic supplementary materialThe online version of this article (doi:10.1186/s13023-016-0497-9) contains supplementary material, which is available to authorized users.

Elevated Serum Anti-GM-CSF Antibodies before the Onset of Autoimmune Pulmonary Alveolar Proteinosis in a Patient with Sarcoidosis and Systemic Sclerosis

“…WLL is the traditional therapy for PAP (Campo et al 2016). According to the pathogenesis of autoimmune PAP, we have other therapeutic options: inhalation or subcutaneous administration of GM-CSF (Tazawa et al 2010;Khan et al 2012), Rituximab (Borie et al 2009), and plasmapheresis ).…”

Novel method of noninvasive ventilation supported therapeutic lavage in pulmonary alveolar proteinosis proves to relieve dyspnea, normalize pulmonary function test results and recover exercise capacity: a short communication

“…Prehospitalization spiral tomography suggested interstitial pneumonia, whereas bronchofiberoscopy revealed normal airway anatomy with abundant milky secretions. BAL showed a low cell count (8.7×10 6 ) with lymphocyte predominance (65%) and CD4/CD8 of 2.73. Pulmonary function tests (PFTs) revealed restriction with significant carbon monoxide diffusing capacity (DLCO) impairment ( Figure 1).…”

“…During WLL development, 3 L of saline/procedure was administered (11); which is comparable to our first attempt. Our method, due to used positive end-expiratory pressure (PEEP), stabilizes alveolar space, decreases the risk of hypoxemia, and increases the amount of recovered saline enabling selective instillation of 12.5 L of warmed saline to the most affected segments of both lungs, similar to that used in WLL (mean 15.4; range 5-40 L) (6).…”

“…WLL, although considered as reasonably safe and efficient if performed in well-experienced specialized centers, has serious limitations, which include post procedure complications, the most common being fever, hypoxemia, wheezing, pneumonia and saline leakage due to malposition of the endotracheal tube (4,6). Also pneumothorax, pleural effusion, cardiac arrest (4) and one case of WLL-associated death (7) were reported.…”

Inhaled GM-CSF in a Pulmonary Alveolar Proteinosis Patient Refractory to Plasmapheresis Combined with Multiple Whole Lung Lavages

“…The gold standard of treatment is the whole lung lavage (WLL), even if this procedure is not yet standardized [6,7] and is associated with a very variable response rate [8,9]. WLL physically removes the accumulated surfactant, however in some patients it is ineffective, despite repeated WLL treatments.…”