title: Whole lung lavage therapy for pulmonary alveolar proteinosis: a global survey of current practices and procedures.
abstract: Whole 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.
  
  
  
  A clinical practice survey was conducted globally by means of a questionnaire and included 27 centers performing WLL in pediatric and/or adult PAP patients.
  
  
  
  We 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.
  
  
  
  This 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.
authors: Pneumology Unit, Fondazione IRCCS Policlinico San Matteo and University of Pavia, Pavia, Italy. i.campo@smatteo.pv.it.|Laboratorio di Biochimica e Genetica, S.C. Pneumologia, Fondazione IRCCS Policlinico San Matteo, via Taramelli 5, 27100, Pavia, Italy. i.campo@smatteo.pv.it.
authors: Ilaria
authors: 0000-0002-4423-2482
authors: I
authors: Campo
authors: Pneumology Unit, Fondazione IRCCS Policlinico San Matteo and University of Pavia, Pavia, Italy.
authors: Maurizio
authors: M
authors: Luisetti
authors: Kinderklinik und Kinderpoliklinik im Dr. von Haunerschen Kinderspital, University of Munich, Munich, Germany.
authors: Matthias
authors: M
authors: Griese
authors: Translational Pulmonary Science Centre, Cincinnati Children's Hospital, Cincinnati, OH, USA.
authors: Bruce C
authors: BC
authors: Trapnell
authors: Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany.
authors: Francesco
authors: F
authors: Bonella
authors: Centre of Interstitial Lung Diseases, St. Antonius Hospital Nieuwegein, Nieuwegein, The Netherlands.
authors: Jan
authors: J
authors: Grutters
authors: Niigata University Medical and Dental School, Niigata, Japan.
authors: Koh
authors: K
authors: Nakata
authors: Centre of Interstitial Lung Diseases, St. Antonius Hospital Nieuwegein, Nieuwegein, The Netherlands.
authors: Coline H M
authors: CH
authors: Van Moorsel
authors: Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany.
authors: Ulrich
authors: U
authors: Costabel
authors: National Reference Centre for Rare Pulmonary Disease, Hopital Louis Pradel, Lyon, France.
authors: Vincent
authors: V
authors: Cottin
authors: Tokyo Medical University Hachioji Medical Center, Tokyo, Japan.
authors: Toshio
authors: T
authors: Ichiwata
authors: Department of Diffuse Lung Diseases and Respiratory Failure, Clinical Research Centre, National Hospital Organization Kinki-Chuo Chest Medical Centre, Osaka, Japan.
authors: Yoshikazu
authors: Y
authors: Inoue
authors: Department of Anesthesiology and Intensive Care, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.
authors: Antonio
authors: A
authors: Braschi
authors: Pneumology Unit, Fondazione IRCCS Policlinico San Matteo and University of Pavia, Pavia, Italy.
authors: Giacomo
authors: G
authors: Bonizzoni
authors: Department of Anesthesiology and Intensive Care, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.
authors: Giorgio A
authors: GA
authors: Iotti
authors: Clinical Epidemiology and Biometric Unit, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
authors: Carmine
authors: C
authors: Tinelli
authors: Department of Anesthesiology and Intensive Care, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.
authors: Giuseppe
authors: G
authors: Rodi
mesh_terms: D018893:Bronchoalveolar Lavage / Q000379:methods*; D001992:Bronchoalveolar Lavage Fluid; D005260:Female; D006801:Humans; D008168:Lung / Q000378:metabolism / Q000473:pathology; D017563:Lung Diseases, Interstitial / Q000378:metabolism / Q000628:therapy; D008297:Male; D011649:Pulmonary Alveolar Proteinosis / Q000378:metabolism / Q000628:therapy*; D011795:Surveys and Questionnaires
keywords: Interstitial lung disease; Pulmonary alveolar proteinosis; Rare disease; Whole lung lavage