System Maintenance Notice

Please note that maintenance work is scheduled between 4 AM and 5 AM - CET. During this time, you may experience reduced performance. Thanks for your patience.

Publications

Journal Article Multicenter Study Epublish 1 citation

PubMed: 42425729 DOI: 10.1183/13993003.02351-2025

Lung transplantation for pulmonary alveolar proteinosis: a retrospective international multicentre analysis.

Eur Respir J 68(2)

Authors: Stefan Schwarz, Helmut Prosch, Erdal Yekeler, Piergiorgio Solli, Xavier Demant, Don Hayes, Masayuki Chida, Kieran Halloran, Mark Greer, Puneet Garcha, Laurens J Ceulemans, Sandra Lindstedt, Claire Merveilleux, Robert M Reed, Michael Musk, Osnat Shtraichman, Pablo G Sanchez, Rayid Abdulqawi, David M Sayah, Victor M Mora, Jesper M Magnusson, María Nieves Balaguer Cartagena, Ricardo A Zapata Gonzalez, Andrew J Fisher, Joel Rosado, Juan M Osses, Achim Koch, John M Reynolds, Clement Picard, Kinan El Husseini, Meghan Aversa, Vaidehi Kaza, Alberto Benazzo, Clemens Aigner, Francesco Bonella, Peter Jaksch, Konrad Hoetzenecker

Affiliations

  • Department of Thoracic Surgery, Comprehensive Center for Chest Diseases, Medical University of Vienna, Vienna, Austria.
  • Department of Biomedical Imaging and Image-guided Therapy, Medical University of Vienna, Vienna, Austria.
  • Department of Thoracic Surgery and Lung Transplantation, University of Health Sciences, Ankara City Hospital, Ankara, Turkey.
  • Department of Cardio-Thoracic Surgery and Heart and Lung Transplantation, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.
  • Service de Pneumologie et Transplantation Pulmonaire, CHU de Bordeaux, Bordeaux, France.
  • Heart Institute and Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA.
  • Department of Thoracic Surgery, Dokkyo Medical University, Tochigi, Japan.
  • Department of Medicine, Division of Pulmonary Medicine, University of Alberta, Edmonton, AB, Canada.
  • Department of Cardiothoracic, Transplant and Vascular Surgery, Hannover Medical School, Hannover, Germany.
  • Department of Medicine, Baylor College of Medicine, Houston, TX, USA.
  • Department of Thoracic Surgery, University Hospitals Leuven, Leuven, Belgium.
  • Department of Cardiothoracic Surgery and Transplantation, Skåne University Hospital, Lund, Sweden.
  • Department of Pneumology, Louis Pradel Hospital, Hospices Civils de Lyon, Lyon, France.
  • Department of Pulmonary and Critical Care Medicine, University of Maryland School of Medicine, Baltimore, MD, USA.
  • Department of Respiratory Medicine, Fiona Stanley Hospital, Perth, Australia.
  • Pulmonary Institute, Rabin Medical Center, Affiliated with Sackler School of Medicine Tel Aviv University, Petach Tikva, Israel.
  • Department of Cardiothoracic Surgery, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.
  • Lung Health Centre, Organ Transplant Centre of Excellence, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
  • Division of Pulmonary, Critical Care Medicine (D.M.S.), David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
  • Lung Transplant Unit, Department of Respiratory Medicine, H. Marqués de Valdecilla, Santander, Spain.
  • Transplant Institute, Sahlgrenska University Hospital, Gothenburg, Sweden.
  • Department of Respiratory Medicine, Institute of Medicine, Sahlgrenska University Hospital, Gothenburg, Sweden.
  • Lung Transplant Unit, University Hospital La Fe, University of Valencia, Valencia, Spain.
  • Thoracic Surgery Department, Clínica Cardio VID, Medellín, Colombia.
  • Institute of Transplantation, Freeman Hospital and Institute of Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, UK.
  • Department of Thoracic Surgery, Hospital Universitari Vall d'Hebron, Barcelona, Spain.
  • Cardiovascular and Thoracic Surgery Division, Favaloro Foundation, Buenos Aires, Argentina.
  • Department of Thoracic Transplantation and Assist Devices, Cardiothoracic Surgery, West German Heart and Vascular Center, University Hospital Essen, Essen, Germany.
  • Division of Pulmonary, Allergy, and Critical Care Medicine, Duke University School of Medicine, Durham, NC, USA.
  • Service de Pneumologie et Transplantation Pulmonaire, Hôpital Foch, Suresnes, France.
  • Pulmonology Department, Bichat University Hospital, AP-HP, Paris, France.
  • Toronto Lung Transplant Program, Ajmera Transplant Centre, University Health Network, Department of Medicine, University of Toronto, Toronto, ON, Canada.
  • Division of Pulmonary and Critical Care Medicine, UT Southwestern Medical Center, Dallas, TX, USA.
  • Department of Thoracic Surgery, Comprehensive Center for Chest Diseases, Medical University of Vienna, Vienna, Austria.
  • Centre for Interstitial and Rare Lung Disease, Department of Pneumology, Ruhrlandklinik University Hospital Essen, Essen, Germany.
  • Department of Thoracic Surgery, Vanderbilt University Medical Center, Nashville, TN, USA.

Abstract

BACKGROUND

Pulmonary alveolar proteinosis (PAP) is a rare disease. Only a small number of case reports describing lung transplantation (LTx) as a treatment option for PAP have been published. Due to this limited evidence, current guidelines on LTx for PAP remain vague. To address this gap, we collected cases of LTx for PAP worldwide.

METHODS

214 centres were approached to contribute any cases of LTx performed for PAP. Re-transplantations and multi-organ transplantations were excluded. Patients were divided into two groups based on the presence of PAP recurrence after LTx. Time-to-event analyses were performed using Kaplan-Meier curves and Cox proportional-hazards modelling was performed with PAP recurrence as a time-dependent covariate.

RESULTS

At a response rate of 64%, 31 centres contributed 63 patients. Most had primary PAP (n=20; 39.2%). Six patients with secondary PAP were identified (11.8%), while seven (13.7%) had congenital and 18 (35.3%) unclassified PAP. Aetiology was not specified in 12. Patients underwent transplantation at a median (interquartile range) 11 (3-17) years after initial diagnosis. Nine (15%) patients developed PAP recurrence after LTx. No significant difference was found regarding patient characteristics between the patients with versus without recurrence. Freedom from recurrence was 96.2% (1 year), 83.5% (5 years) and 70.1% (10 years) post-LTx. PAP recurrence as time-dependent covariate was not significantly associated with graft loss (hazard ratio 1.88, 95% CI 0.51-6.91; p=0.342) in a Cox proportional-hazards model.

CONCLUSION

LTx provides excellent peri-operative and long-term outcomes for patients with end-stage PAP and should be recommended in treatment guidelines. Although recurrence of PAP occurs in ∼15% of cases, overall survival appeared unaffected in our limited dataset.

MeSH

Pulmonary Alveolar Proteinosis, Lung, Respiration, Therapeutics, Lung Transplantation, Transplantation