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Correspondence |

A Global Survey on Whole Lung Lavage in Pulmonary Alveolar Proteinosis FREE TO VIEW

Ilaria Campo, PhD; Maurizio Luisetti, MD, PhD; Matthias Griese, MD; Bruce C. Trapnell, MD; Francesco Bonella, MD, PhD; Jan C. Grutters, MD, PhD; Koh Nakata, MD, PhD; Coline H.M. Van Moorsel, PhD; Ulrich Costabel, MD, PhD; Vincent Cottin, MD, PhD; Toshio Ichiwata, MD, PhD; Yoshikazu Inoue, MD, PhD; Antonio Braschi, MD, PhD; Giacomo Bonizzoni, MD; Giorgio A. Iotti, MD; Carmine Tinelli, MD; Giuseppe Rodi, MD
Author and Funding Information

Drs Campo and Luisetti contributed equally to this manuscript.

FINANCIAL/NONFINANCIAL DISCLOSURES: None disclosed.

FUNDING/SUPPORT: This study was supported by a grant from European Union E-Rare JTC 2009: EuPAPnet.

aPneumology Unit, IRCCS San Matteo Hospital Foundation and University of Pavia, Pavia, Italy

bKinderklinik und Kinderpoliklinik im Dr. von Haunerschen Kinderspital, University of Munich, Munich, Germany

cTranslational Pulmonary Science Center, Cincinnati Children’s Hospital, Cincinnati, Ohio

dInterstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany

eCentre of Interstitial Lung Diseases, St. Antonius Hospital Nieuwegein, Nieuwegein, The Netherlands

fNiigata University Medical and Dental School, Niigata, Japan

gNational Reference Centre for Rare Pulmonary Disease, Hopital Louis Pradel, Lyon, France

hTokyo Medical University Hachioji Medical Center, Tokyo, Japan

iDepartment of Diffuse Lung Diseases and Respiratory Failure, Clinical Research Centre, National Hospital Organization Kinki-Chuo Chest Medical Centre, Osaka, Japan

jDepartment of Anesthesiology and Intensive Care, IRCCS San Matteo Hospital Foundation, University of Pavia, Pavia, Italy

kClinical Epidemiology and Biometric Unit, IRCCS San Matteo Hospital Foundation, Pavia, Italy

CORRESPONDENCE TO: Ilaria Campo, PhD, Laboratorio di Biochimica e Genetica S.C. Pneumologia, Fondazione IRCCS Policlinico San Matteo, via Taramelli 5, 27100-Pavia, Italy


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2016;150(1):251-253. doi:10.1016/j.chest.2016.04.030
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Published online

Whole lung lavage (WLL) is the current standard of care to treat pulmonary alveolar proteinosis (PAP), a rare respiratory syndrome characterized by the accumulation of lipoproteinaceous material in alveoli, which impairs oxygen uptake and causes respiratory failure.,

We present a clinical practice survey on WLL, which was conducted by means of a questionnaire. Two major considerations prompted us to perform the present survey: (1) even if WLL for treatment of PAP was described more than 50 years ago, it is still an elitist procedure with poor accessibility and (2) the WLL procedure is, in most cases, the result of a long-term process of self-education, with progressive improvement of the lavage technique.

An international committee of physicians performing WLL or caring for patients with PAP, or both, developed the survey questionnaire, which comprises sections for physicians performing only WLL and physicians performing only lobar/segmental bronchoscopic lavage (LSBL), or physicians performing both WLL and LSBL.

Respondents were from 20 centers in 14 countries performing WLL in adults and 10 centers in 6 countries performing WLL in pediatric patients (Table 1). The mean (± SD) duration of experience was 18 ± 11 years (Fig 1).

Table Graphic Jump Location
Table 1 Centers Participating in the Survey

LSBL = lobar/segmental bronchoscopic lavage; WLL = whole lung lavage.

Figure Jump LinkFigure 1 Years of experience at each center in performing whole lung lavage (WLL) in adult patients with pulmonary alveolar proteinosis (PAP).Grahic Jump Location

The results show that WLL in adults is performed almost universally using general anesthesia with a double-lumen tracheal tube in two consecutive sessions (one lung per session), with an interval of 1 to 2 weeks between WLL procedures observed by 50% of centers. Other common aspects are the use of saline warmed to 37°C and drainage of instilled fluid by gravity. The amount of fluid used to perform the WLL is a critical aspect. The volume of saline aliquots infused varies greatly (800 mL of warm saline on average), and a great variability exists for the total volume used per lung, with a range from 5 to 40 L.

Most (14 of 20) centers use chest percussion to emulsify the PAP sediment to improve therapeutic efficiency. However, the method and timing vary greatly. Ten centers (50%) use manual chest percussion and four (20%) centers use mechanical percussion.

Indications for WLL vary among centers (Fig 2). There is also a great discrepancy in the choice of position: supine positioning was chosen by 50% of centers and rotation and change of positions was performed by 7 centers.

Figure Jump LinkFigure 2 Observed frequency (%) of indication for WLL among the centers. More than one answer was possible; Δ = decline from baseline.Grahic Jump Location

This international survey found that WLL is safe and effective as therapy for PAP and represents a first step in developing an evidence-based, best-practice approach to standardizing WLL therapy for PAP.

Trapnell B.C. .Whitsett J.A. .Nakata K. . Pulmonary alveolar proteinosis. N Engl J Med. 2003;349:2527-2539 [PubMed]journal. [CrossRef] [PubMed]
 
Luisetti M. .Kadija Z. .Mariani F. .Rodi G. .Campo I. .Trapnell B.C. . Therapy options in pulmonary alveolar proteinosis. Ther Adv Respir Dis. 2010;4:239-248 [PubMed]journal. [CrossRef] [PubMed]
 
Luisetti M. . Call for an international survey on therapeutic lavage for pulmonary alveolar proteinosis. Eur Respir J. 2012;39:1049- [PubMed]journal. [CrossRef] [PubMed]
 
Ramirez J. .Schultz R.B. .Dutton R.E. . Pulmonary alveolar proteinosis: a new technique and rationale for treatment. Arch Intern Med. 1963;112:419-431 [PubMed]journal. [CrossRef] [PubMed]
 
Seymour J.F. .Presneill J.J. . Pulmonary alveolar proteinosis: progress in the first 44 years. Am J Respir Crit Care Med. 2002;166:215-235 [PubMed]journal. [CrossRef] [PubMed]
 

Figures

Figure Jump LinkFigure 1 Years of experience at each center in performing whole lung lavage (WLL) in adult patients with pulmonary alveolar proteinosis (PAP).Grahic Jump Location
Figure Jump LinkFigure 2 Observed frequency (%) of indication for WLL among the centers. More than one answer was possible; Δ = decline from baseline.Grahic Jump Location

Tables

Table Graphic Jump Location
Table 1 Centers Participating in the Survey

LSBL = lobar/segmental bronchoscopic lavage; WLL = whole lung lavage.

References

Trapnell B.C. .Whitsett J.A. .Nakata K. . Pulmonary alveolar proteinosis. N Engl J Med. 2003;349:2527-2539 [PubMed]journal. [CrossRef] [PubMed]
 
Luisetti M. .Kadija Z. .Mariani F. .Rodi G. .Campo I. .Trapnell B.C. . Therapy options in pulmonary alveolar proteinosis. Ther Adv Respir Dis. 2010;4:239-248 [PubMed]journal. [CrossRef] [PubMed]
 
Luisetti M. . Call for an international survey on therapeutic lavage for pulmonary alveolar proteinosis. Eur Respir J. 2012;39:1049- [PubMed]journal. [CrossRef] [PubMed]
 
Ramirez J. .Schultz R.B. .Dutton R.E. . Pulmonary alveolar proteinosis: a new technique and rationale for treatment. Arch Intern Med. 1963;112:419-431 [PubMed]journal. [CrossRef] [PubMed]
 
Seymour J.F. .Presneill J.J. . Pulmonary alveolar proteinosis: progress in the first 44 years. Am J Respir Crit Care Med. 2002;166:215-235 [PubMed]journal. [CrossRef] [PubMed]
 
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