New data from a single-center study indicate that not only was the rate of mortality high among patients with advanced pulmonary sarcoidosis who were awaiting lung transplant, but the lung allocation score did not predict death on the waitlist in this patient population.
Among 28 patients with advanced pulmonary sarcoidosis listed for lung transplant from March 2012 to February 2019 at Temple University Hospital in Philadelphia, 18.2% of patients died while awaiting transplant — a proportion that exceeded that of patients with idiopathic pulmonary fibrosis (12.3%) and patients with COPD (8.7%) who were also on the transplant list, according to the researchers.
Of the six patients who died, one died from clear progressive respiratory failure, one died from witnessed ventricular arrhythmia, one died in a setting of respiratory distress at home but without a determined etiology and three died at home and no autopsies were performed.
Notably, lung allocation scores between those who survived to transplant and those who died on the waitlist did not differ significantly at the initial listing (41 vs. 46) or at time of death or transplant (41 vs. 41). Waitlist time was numerically longer among those who survived to transplant vs. those who died, but this finding did not reach statistical significance (307 vs. 177 days), according to the data.
However, the researchers found that physiologic markers of advanced fibrotic disease were predictive of waitlist mortality among patients with advanced pulmonary sarcoidosis. Top predictors included lower bilirubin values (area under the curve = 0.92), which had 100% sensitivity and 81% specificity for predicting death on the waitlist at a cutoff of 0.35 mg/dL or less, and diffusion capacity of the lungs for carbon monoxide (AUC = 0.84), which had 100% sensitivity and 67% specificity at a cutoff of less than 21%. The composite physiologic index also had good predictive value (AUC = 0.86), with 100% sensitivity and 77% specificity for a score of at least 67.7. An FEV1/FVC ratio of 64.5% or greater also had 100% sensitivity but only 46% specificity.
Pulmonary hypertension, which has previously been linked to worse outcomes in sarcoidosis, was not associated with waitlist mortality.
In light of these data, the researchers wrote, “Reconsideration of current referral and transplant candidacy listing recommendations for advanced pulmonary sarcoidosis may be warranted.”
Study limitations included the study’s single-center, retrospective design and its small sample size. The researchers noted, however, that the study was conducted at a high-volume center and that the proportion of patients transplanted for advanced pulmonary sarcoidosis reflects national trends. – by Melissa Foster
Disclosures: The authors report no relevant financial disclosures.