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Updated: Jul 12, 2026
Left Lung Orthotopic Transplantation in a Juvenile Porcine Model for ESLP
Published on: February 14, 2022
Lung Transplantation for Pulmonary Alveolar Proteinosis - a Retrospective International Multi-Center Analysis
Stefan Schwarz1, Helmut Prosch2, Erdal Yekeler3
1Department of Thoracic Surgery, Comprehensive Center for Chest Diseases, Medical University of Vienna, Vienna, Austria stefan.a.schwarz@meduniwien.ac.at.
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 centers 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 by 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 centers contributed 63 patients. Most had primary PAP (n=20; 39.2%). 6 patients with secondary PAP were identified (11.8%), while 7 (13.7%) had congenital and 18 (35.3%) unclassified PAP. Etiology was not specified in 12. Patients underwent transplantation at a median 11 years (IQR 3-17) after initial diagnosis. Nine patients (15%) 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% (1y), 83.5% (5y) and 70.1% (10y) post-LTx. PAP recurrence as time-dependent covariate was not significantly associated with graft loss (HR=1.88, 95%CI:0.51-6.91, p=0.342) in a cox-proportional-hazards-model.
Conclusion:
LTx provides excellent perioperative and long-term outcomes for patients with end-stage PAP and should be recommended in treatment guidelines. Although recurrence of PAP occurs in approximately 15% of cases, overall survival appeared unaffected in our limited dataset.
