Mortality for sarcoidosis patients on the transplant wait list in the Lung Allocation Score era: Experience from a high volume center

Abstract

Rationale: Sarcoidosis progresses to end stage fibrotic lung disease in 10% of patients and may necessitate lung transplantation. Organ allocation is currently determined by the Lung Allocation Score (LAS), but its performance in a sarcoidosis population has not been evaluated. Objectives: To determine sarcoidosis-specific wait list mortality and identify predictive factors of death on the transplantation wait list. Methods: This was a single-center retrospective study of all sarcoidosis patients listed for lung transplant from March 2012 to February 2019. We compared patients who were transplanted to those who died awaiting organs. We collected baseline listing characteristics, physiologic testing, and outcomes data. Statistical analysis was performed by 2-tailed Student's t-test, Mann-Whitney U test, and Chi-Square analysis (where appropriate). Receiver-operating characteristic curves were constructed for variables reaching statistical significance. Results: Twenty eight sarcoidosis patients were included in analysis. Mortality among wait listed patients was 18%, which exceeded the mortality of COPD and IPF. LAS scores did not differ at initial listing (41 vs. 46, p = 0.35) or at transplant/death (41 vs. 41, p = 0.91); wait list times also did not statistically differ (307 days vs. 177 days, p = 0.19). We identified bilirubin (AUC = 0.92), DLCO (AUC = 0.84), FEV1/FVC at transplant/death (AUC = 0.85), and composite physiologic index (AUC = 0.86) as predictors of death on the transplant list. Pulmonary hypertension was not associated with death. Conclusion: Unexpected sudden death was common in our cohort and was associated with markers of advanced fibrotic disease, not pulmonary hypertension.

Prevalence of Sarcoidosis-Associated Pulmonary Hypertension: A Systematic Review and Meta-Analysis

“…Furthermore, four other studies were conducted using large databases with only documented records for the diagnosis of sarcoidosis and PH ( 25 , 26 , 28 , 29 ). In addition, five studies included patients with advanced sarcoidosis ( 11 , 12 , 27 , 37 , 43 ); the others included the general sarcoidosis population. The basic characteristics of the included studies are shown in Table 1 .…”

“…Eleven studies ( 12 , 23 , 24 , 27 , 32 – 34 , 37 , 38 , 41 , 43 ) described the prevalence of SAPH by RHC. Of these, seven studies focused on the general sarcoidosis population, while four studies focused on the population with advanced sarcoidosis.…”

Long-Term Benefits of Tailored Exercise in Severe Sarcoidosis: A Case Report

“…Frailty is considered an important phenotype in lung transplant candidates independent of the severity of the disease [35]. On the other hand, 10% of patients with sarcoidosis progress to fibrotic lung disease and may be in need of lung transplant [18]. In this regard, although lung transplantation was originally proposed as a future option for our patient, the remission of right atrium dilatation, mild mitral insufficiency, and PAH, together with improvements in NYHA class and CRF made it possible to discard transplant by the end of first year of the intervention.…”

“…Approximately 10% of patients with sarcoidosis develop end-stage fibrotic lung disease with high indication for lung transplantation [18]. Prior to this, patients may experience long-term and progressive muscle weakness [19], also affecting the inspiratory musculature [20].…”

Advanced Pulmonary Sarcoidosis

“…78 A recent single-center study found that increased bilirubin, higher CPI, and reduced DLCO and FEV1/FVC at transplant listing (but not pulmonary hypertension) were predictors of death on the transplant list. 79 Sudden death was found to be high (18%) amongst sarcoidosis patients listed for LTx and was more common than that for COPD or IPF patients.…”