Study objectives: To describe the population of patients with sarcoidosis listed for orthotopic lung transplantation (OLT) in the United States, and to determine outcomes for these subjects relative to persons awaiting OLT for idiopathic pulmonary fibrosis (IPF).
Design: Retrospective analysis of the United Network for Organ Sharing transplant database over the period between January 1995 and December 2000.
Patients: All patients listed for OLT with an underlying diagnosis of either sarcoidosis or IPF.
Measurements and results: During the study period, 427 patients with sarcoidosis and 2,115 patients with IPF were registered on the list for OLT. Demographically, the patients with sarcoidosis were younger and more likely to be female African Americans than were patients with IPF. Pulmonary function was worse in patients with sarcoidosis. The mean FVC was 42.6% of predicted, as compared to 45.0% of predicted in patients with IPF (p = 0.0044). The FEV1 also differed between the populations (36.0% vs 46.0% of predicted for patients with sarcoidosis and IPF, respectively; p < 0.0001). Only 30.1% of patients with sarcoidosis and 32.4% of patients with IPF lacked functional limitations. For the subset of patients with hemodynamic data available, the mean pulmonary artery pressure was significantly higher in the sarcoidosis population (34.4 mm Hg vs 25.6 mm Hg, respectively; p < 0.0001). Neither the pulmonary capillary wedge pressure nor the cardiac index differed between the groups. Patients with sarcoidosis were less likely to receive a transplant. Approximately 30% of patients with sarcoidosis underwent OLT, compared to 37.3% of IPF patients (p = 0.0102). For those who did undergo transplantation, the median wait until OLT was 803 days for patients with sarcoidosis compared to 555 days for patients with IPF (p < 0.0001). Mortality rates were similar in both groups. In the sarcoidosis group, 28.1% of patients died, compared to 31.1% of patients with IPF (p = not significant).
Conclusions: Patients with sarcoidosis are at as high a risk for mortality as patients with IPF while awaiting transplantation. Nonetheless, patients with sarcoidosis are less likely to undergo OLT. Pulmonary hypertension is a major concern in patients with advanced sarcoidosis awaiting transplantation.