The Survival Paradox in Sarcoidosis Patients Undergoing Lung transplantation: Overview of the Lung Allocation Score Era

T. Lin¹, A. Rybachok¹, M. Shacker², L. Chang¹, S. Biswas Roy³, A. Arjuna⁴, R. Walia⁴, R. Bremner⁴, J. Huang⁴, M. Smith^4
1Creighton University School of Medicine, Phoenix, Arizona ²Creighton University School of Medicine, San Jose, California ³Norton Thoracic Institute, Phoenix, Arizona ⁴Norton Thoracic Institute, St. Joseph's Hospital and Medical Center, Phoenix, Arizona

Purpose: Lung transplantation is reserved for a subset of sarcoidosis patients with advanced fibrosis or pulmonary hypertension. We investigated the short- and long-term outcomes following lung transplantation for sarcoidosis patients as compared to those with chronic obstructive pulmonary disease (COPD) or idiopathic pulmonary fibrosis (IPF) in the Lung Allocation Score era.

Methods: The United Network for Organ Sharing/Organ Procurement and Transplantation Network STAR file was retrospectively reviewed for single or bilateral lung transplant recipients aged 18 to 79 from 5/4/2005 to 3/8/2023 with a transplant diagnosis of sarcoidosis, COPD, or IPF. Recipient demographics, perioperative characteristics, and postoperative outcomes were compared across groups. Continuous variables were reported using medians.

Results: A total of 21,144 patients were identified; 7,706 (36.4%) had COPD, 12,477 (59.0%) had IPF, and 961 (4.5%) had sarcoidosis as the listing diagnosis. The sarcoidosis group was younger (COPD: 62, IPF: 64, Sarcoidosis: 55, p < 0.001) and more likely to undergo bilateral lung transplantation (COPD: 67%, IPF: 60%, Sarcoidosis: 86%, p < 0.001). Mean pulmonary artery pressures (mmHg) at time of transplantation were higher in the sarcoidosis group (COPD: 25, IPF: 24, Sarcoidosis: 34, p < 0.001). Post-transplant, the sarcoidosis group had higher rates of post-transplant ventilator support (COPD: 13%, IPF: 20%, Sarcoidosis: 28%, p < 0.001), ECMO at 72 hrs (COPD: 3.9%, IPF: 7.3%, Sarcoidosis: 16%, p < 0.001), intubation at 72 hours (COPD: 20%, IPF: 29%, Sarcoidosis: 44%, p < 0.001), and inhaled nitric oxide use at 72 hours (COPD: 3.7%, IPF: 8.9%, Sarcoidosis: 14%, p < 0.001). Sarcoidosis patients had worse 1-year survival (COPD: 88.8%, IPF: 86.9%, Sarcoidosis: 82.3%, p < 0.001), but improved 10-year survival (COPD: 27.5%, IPF: 26.3%, Sarcoidosis: 38.6%, p < 0.001). A multivariate analysis demonstrated that prior cardiac surgery was a significant risk factor for death in sarcoidosis patients undergoing lung transplant (HR: 2.05, 95% CI: 1.25-3.4 p = 0.005).

Conclusion: Patients with sarcoidosis who undergo lung transplantation are more likely to have severe pulmonary hypertension. Short-term post-transplant survival in pulmonary sarcoidosis patients is lower compared to COPD or IPF, but long-term survival is paradoxically better, likely due to younger age at transplant. Lung transplantation is an efficacious salvage therapy for end-stage pulmonary sarcoidosis.

Identify the source of the funding for this research project: This research project did not require any funding.