Sarcoidosis lung transplantation waitlist mortality, a national registry database study

Abstract

The Lung Allocation Score prioritizes lung transplantation candidates balancing waitlist-mortality and post-transplant survival. The score groups sarcoidosis candidates based on mean pulmonary artery pressure: ≤30 mmHg (Sarcoidosis A) are grouped with COPD and >30 mmHg (Sarcoidosis D) with idiopathic pulmonary fibrosis. We hypothesize that sarcoidosis candidates have a higher waitlist-mortality than other candidates within their LAS grouping. This is a retrospective cohort study of consecutive lung transplantation candidates from the Scientific Registry of Transplant Recipients database from May 2005 to May 2019. We included candidates ≥18 y/o diagnosed with sarcoidosis, COPD or IPF. Univariate, multivariate and survival estimate analysis were performed. We identified 385 sarcoidosis A, 642 sarcoidosis D, 7081 COPD, 10 639 IPF lung transplantation candidates. 17.3% of sarcoidosis D, 14.8% of IPF, 14.3% of sarcoidosis A, and 9.8% of COPD candidates died awaiting transplant. Sarcoidosis A was an independent risk factor for waitlist-mortality. Sarcoidosis A had a lower waitlist survival probability compared to COPD. Sarcoidosis D had the highest waitlist-mortality. IPF candidates had lower waitlist survival probability than sarcoidosis D in the first 60 days after listing. Based on our results the grouping of candidates with sarcoidosis in the allocation systems should be revised to mitigate the waitlist-mortality disparity.

High-Risk Sarcoidosis: A Focus on Pulmonary, Cardiac, Hepatic and Renal Advanced Diseases, as Well as on Calcium Metabolism Abnormalities

“…Indeed, sarcoidosis is grouped within this system with other end-stage lung diseases according to the mean pulmonary artery pressure value: when <30 mmHg (group A sarcoidosis), patients are grouped with COPD, whereas when >30 mmHg (sarcoidosis group D), they are grouped with IPF. This study showed that group D had the highest mortality on the waitlist, and the authors suggested a revision of sarcoidosis LT candidates grouping in allocations systems [112] to mitigate waitlist mortality disparity. (7) Holistic management of APS In all cases, the treatment of APS requires a holistic and comprehensive management of all dimensions of the disease (symptoms, psychological distress, fatigue).…”

“…Survival after LT in sarcoidosis is similar to that of other ILDs, but two factors are predictive of worse survival: older age and the extent of preoperative lung fibrosis [111], suggesting again to take them into account when considering referral to a LT expert center. Interestingly, the LAS has been recently challenged [112]. Indeed, sarcoidosis is grouped within this system with other end-stage lung diseases according to the mean pulmonary artery pressure value: when <30 mmHg (group A sarcoidosis), patients are grouped with COPD, whereas when >30 mmHg (sarcoidosis group D), they are grouped with IPF.…”

Clinical Manifestations and Management of Fibrotic Pulmonary Sarcoidosis

“…Following the implementation of the lung allocation score (LAS) in 2005, a greater percentage of sarcoidosis patients received lung allografts, leading to reduced waitlist mortality [ 119 ]. However, recent studies have found that, compared with patients with COPD and IPF, individuals with sarcoidosis continue to face disproportionately higher waitlist mortalities [ 120 ]. The several factors contributing to waitlist mortality were identified as pulmonary hypertension, poorer functional status, oxygen dependence, lower reduced output, and female sex [ 121 , 122 ].…”