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

Published on Oct 1, 2019in Respiratory Medicine3.237
· DOI :10.1016/j.rmed.2019.09.001
A. Gangemi1
Estimated H-index: 1
(TU: Temple University),
Catherine N. Myers (TU: Temple University)+ 7 AuthorsA. James Mamary9
Estimated H-index: 9
(TU: Temple University)
Abstract
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.
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References22
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#1A. Gangemi (TU: Temple University)H-Index: 1
#2C.N. Myers (TU: Temple University)H-Index: 1
Last. A.J. Mamary (TU: Temple University)H-Index: 1
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#1Maryam Valapour (Cleveland Clinic)H-Index: 7
#2Carli J. Lehr (Cleveland Clinic)H-Index: 3
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#2Elyse E. Lower (UC: University of Cincinnati)H-Index: 39
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Objective The goal of this study was to assess the prognostic strength of factors in predicting respiratory death in a large cohort of patients with sarcoidosis with at least 8 years' follow-up. Methods Data were collected on age, sex, self-declared race, time of death, spirometry findings, diffusing capacity for carbon monoxide, chest radiograph stage, extent of fibrosis on high-resolution CT (HRCT) scanning, and presence of precapillary pulmonary hypertension (PH). The Gender-Age-Physiology (G...
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Clinical Characteristics and Outcome of Hepatic Sarcoidosis: A Population-Based Study 1976–2013
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Sarcoidosis is a chronic systemic inflammatory disease which is histopathologically characterized by the presence of noncaseating granulomas. When the extent of the disease is limited, without endangering the function of affected organs, clinical observation can be sufficient given that in a majority of cases, inflammation will subside with time. In more advanced sarcoidosis, especially when one or more specific organs are threatened, immunomodulatory treatment, of which steroids are the key ele...
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Chronic pulmonary aspergillosis (CPA) complicating sarcoidosis (SA) is associated with high mortality, and there is a lack of clarity regarding the relative contributions of SA or CPA. This was a retrospective single-centre study on CPA-SA. In total, 65 patients (44 men), aged 41.4±13.5 and 48.3±11.9 years at the time of SA and CPA diagnoses, respectively, were included between 1980 and 2015. Of these, 64 had fibrocystic SA, most often advanced, with composite physiological index (CPI) >40 (65% ...
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Introduction: Sarcoidosis is a multi-system, granulomatous disorder of unknown etiology that is associated with a variable prognosis and sometimes results in death. There are conflicting reports regarding the causes of death in patients with sarcoidosis. Methods: Forty-four consecutive patients with sarcoidosis who underwent an autopsy (35 patients) or died at Mayo Clinic (Rochester, MN, USA) over a 20-yr period, from January 1, 1994 to December 31, 2013 were analyzed. Results: The median age at...
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Objective Lung transplantation is the ultimate treatment for end-stage pulmonary sarcoidosis. Post-transplant survival outcomes remain unclear. Methods Survival models were used to assess survival and graft outcomes in patients with sarcoid among 20 896 lung transplants performed in the USA. Results 695 lung recipients were transplanted for pulmonary sarcoidosis. Sarcoid lung recipients had similar median survival rate (69.7 months (IQR 60.2–79.3)) compared with the non-sarcoid lung recipients (...
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Background —This study was designed to assess the epidemiology, characteristics and outcome of cardiac sarcoidosis (CS) in Finland. Methods and Results —We identified in retrospect all adult (>18 yr) patients diagnosed with histologically confirmed CS in Finland between 1988 and 2012. Totally 110 patients (71 women) aged 51 ± 9 yr (mean ± SD) were found and followed for outcome events to the end of 2013. The annual detection rate of CS increased more than 20-fold during the 25-year period reachi...
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The appropriate selection of lung transplant recipients is an important determinant of outcomes. This consensus document is an update of the recipient selection guidelines published in 2006. The Pulmonary Council of the International Society for Heart and Lung Transplantation (ISHLT) organized a Writing Committee of international experts to provide consensus opinion regarding the appropriate timing of referral and listing of candidates for lung transplantation. A comprehensive search of the medi...
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