Lung Transplantation in Pulmonary Fibrosis: Challenging Early Outcomes Counterbalanced by Surprisingly Good Outcomes Beyond 15 Years

Interstitial lung disease (ILD) has been reported to have a poor outcome following lung transplantation due to difficulties getting ill recipients to transplantation and challenging early postoperative outcomes. To assess long-term outcomes for this cohort, we performed a retrospective 18-year chart review of all ILD lung transplant recipients. ILD single (SLT) and bilateral sequential lung transplantations (BSLT) were compared with all other lung transplant patients and International Society for Heart and Lung Transplantation (ISHLT) Registry data over the same time period. Of 585 lung transplantations, 90 (15%) were ILD (53 SLT, 37 BSLT); 67 (74%) were idiopathic pulmonary fibrosis (IPF), 9 (10%) were sarcoidosis, 9 (10%) were lymphangioleiomyomatosis, and 5 (6%) had other indications. Mean age was 52 years (range, 34-69 years). Actuarial survival at 1, 5, 10, 15, and 18 years compared favorably to all other lung transplantations performed (77% vs 83%, 51% vs 50%, 42% vs 26%, 28% vs 17%, and 28% vs 8%, respectively). IPF actuarial survival at 1, 5, and 10 years appeared superior to ISHLT Registry data (76% vs 72%, 50% vs 44%, and 34% vs 20%, respectively). There was equivocal survival between SLT and BSLT at 1, 5, and 10 years (78% vs 68%, 49% vs 50%, and 29% vs 50%, respectively). Our ILD figures compared favorably to lung transplantation for other diseases and international standards, while survival from SLT was as successful as BSLT both in the short and the longer term. Consideration should be given to utilizing SLT to maximize the allocation of donor lungs and to decrease waiting list mortality associated with IPF.