Bilal Muhammad, Satapathy Sanjaya K, Ismail Mohammad K, Vanatta Jason M
Department of Gastroenterology and Hepatology, University of Tennessee Health Sciences Center, Memphis, TN, United States.
Methodist University Hospital Transplant Institute, University of Tennessee Health Sciences Center, Memphis, TN, United States.
J Clin Exp Hepatol. 2016 Jun;6(2):94-9. doi: 10.1016/j.jceh.2016.02.005. Epub 2016 Mar 3.
Hepatic sarcoidosis is a rare indication for orthotopic liver transplantation (OLT). Hence, studies evaluating these patients are scarce. We present a single center experience with OLT for hepatic sarcoidosis in a case-control study.
A retrospective chart review was performed on 970 patients with OLT at our center, and 13 patients (1.3%) were identified who underwent 14 OLTs for hepatic sarcoidosis. For each case, two controls matched for etiology of liver disease, recipient age (±5 years), and duration since transplant (within 5 years) were selected.
For the 13 patients transplanted for sarcoidosis, the median age was 46 years. The majority were women (62%) and African-American (85%). Cholestatic liver disease was the primary manifestation. Portal hypertensive complications were present in 11 patients (84%). The median MELD score at transplantation was 19. Extra-hepatic manifestations were present in ten patients (77%). All patients received whole deceased 14 donor allografts. Six patients remain alive with a median post-OLT follow-up of 8.4 years. The 1-, 3-, 5-, and 10-year patient survival rates were 84.6%, 76.9%, 61.1%, and 51.3%, respectively for the sarcoidosis group and 82.1%, 78.6%, 78.6%, and 61.9%, respectively for the matched PSC/PBC group (P = 0.739). Re-graft free survival for sarcoidosis patients was 84.6%, 76.9%, 61.5%, and 51.3% for 1-, 3-, 5-, and 10-years and for the matched control group re-graft free survival was 78.6% at 1-, 3-, 5-years, and 64.8% at 10-years (P = 0.661). Recurrence of hepatic sarcoidosis was found in 4 patients at 11 days, 112 days, 222 days, and 6.6 years.
Our study depicts the long-term benefit of liver transplantation in patients with end stage liver disease secondary to sarcoidosis. It shows statistically comparable graft and patient survival for such patients when compared to other cholestatic diseases. Disease recurrence, although possible, has not been shown to cause allograft dysfunction.
肝结节病是原位肝移植(OLT)的罕见适应证。因此,评估这些患者的研究很少。我们在一项病例对照研究中介绍了单中心肝结节病OLT的经验。
对我们中心970例行OLT的患者进行回顾性病历审查,确定13例(1.3%)因肝结节病接受了14次OLT。对于每例患者,选择两名在肝病病因、受者年龄(±5岁)和移植后时间(5年内)相匹配的对照。
13例因结节病接受移植的患者,中位年龄为46岁。大多数为女性(62%)和非裔美国人(85%)。胆汁淤积性肝病是主要表现。11例患者(84%)出现门静脉高压并发症。移植时的中位终末期肝病模型(MELD)评分是19分。10例患者(77%)有肝外表现。所有患者均接受了全尸供者的同种异体移植物。6例患者存活,OLT后的中位随访时间为8.4年。结节病组1年、3年、5年和10年的患者生存率分别为84.6%、76.9%、61.1%和51.3%,匹配的原发性硬化性胆管炎/原发性胆汁性胆管炎(PSC/PBC)组分别为82.1%、78.6%、78.6%和61.9%(P = 0.739)。结节病患者1年、3年、5年和10年的无再次移植生存率分别为84.6%、76.9%、61.5%和51.3%,匹配对照组1年、3年和5年的无再次移植生存率为78.6%,10年为64.8%(P = 0.661)。4例患者分别在术后11天、112天、222天和6.6年出现肝结节病复发。
我们的研究描述了结节病继发终末期肝病患者肝移植的长期益处。与其他胆汁淤积性疾病相比,此类患者的移植物和患者生存率在统计学上具有可比性。疾病复发虽然有可能,但尚未显示会导致同种异体移植物功能障碍。