Ertan Emek1, Ayfer Serin1, Tolga Sahin1, Pinar Yazici2, Yildiray Yuzer1, Yaman Tokat1, Birkan Bozkurt1. 1. Sisli Florence Nightingale Hospital, Liver Transplantation Institute, Hospital of Istanbul Bilim University, Istanbul, Turkey. 2. Sisli Florence Nightingale Hospital, Liver Transplantation Institute, Hospital of Istanbul Bilim University, Istanbul, Turkey. Electronic address: drpinaryazici@gmail.com.
Abstract
PURPOSE: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown origin. Although the course of PSC is variable, it frequently is progressive, leading to cirrhosis and requiring a liver transplantation (LT) in more than half of the patients. PSC is the fifth most common indication for LT in the United States and one of the leading indications in Scandinavian countries, whereas PSC affects fewer than 5% of patients undergoing LT in Turkey. In this study, we analyzed our results in the patients with LT owing to PSC. MATERIALS AND METHODS: Between March 2013 and August 2017, all adult patients (>18 years) with LT owing to PSC were analyzed, and clinical data were obtained via retrospective review of patient charts. Demographic features, presence of any concomitant inflammatory bowel disease (IBD), time to LT, and outcome data were recorded. RESULTS: There were 15 patients (8 men and 7 women) with a mean age of 46 ± 13 (age at diagnosis = 36 y). Median time to transplantation was 3 years (range: .5-14 yrs.). All patients had a pretransplant history of IBD. Concomitant cholangiocarcinoma was diagnosed in 1 patient (6.5%). Postoperative complications were observed in 4 patients (26%), and in 2 patients (13%) PSC recurred at a mean of 52 months postorthotopic LT. Disease-free survival and overall survival were 37.3 and 38 ± 21 months, respectively. One of the patients with recurrence and 1 with graft failure owing to rejection died in the follow-up period. CONCLUSIONS: In one single-center study of adults with PSC, we found that all patients with PSC had IBD at diagnosis. The recurrence rate (13%) was comparable to the literature (20% [5.7-59%]). Despite the low frequency of PSC in our clinic, LT in these patients resulted in favorable outcomes regarding postoperative morbidity and mortality compared with other etiologies.
PURPOSE:Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown origin. Although the course of PSC is variable, it frequently is progressive, leading to cirrhosis and requiring a liver transplantation (LT) in more than half of the patients. PSC is the fifth most common indication for LT in the United States and one of the leading indications in Scandinavian countries, whereas PSC affects fewer than 5% of patients undergoing LT in Turkey. In this study, we analyzed our results in the patients with LT owing to PSC. MATERIALS AND METHODS: Between March 2013 and August 2017, all adult patients (>18 years) with LT owing to PSC were analyzed, and clinical data were obtained via retrospective review of patient charts. Demographic features, presence of any concomitant inflammatory bowel disease (IBD), time to LT, and outcome data were recorded. RESULTS: There were 15 patients (8 men and 7 women) with a mean age of 46 ± 13 (age at diagnosis = 36 y). Median time to transplantation was 3 years (range: .5-14 yrs.). All patients had a pretransplant history of IBD. Concomitant cholangiocarcinoma was diagnosed in 1 patient (6.5%). Postoperative complications were observed in 4 patients (26%), and in 2 patients (13%) PSC recurred at a mean of 52 months postorthotopic LT. Disease-free survival and overall survival were 37.3 and 38 ± 21 months, respectively. One of the patients with recurrence and 1 with graft failure owing to rejection died in the follow-up period. CONCLUSIONS: In one single-center study of adults with PSC, we found that all patients with PSC had IBD at diagnosis. The recurrence rate (13%) was comparable to the literature (20% [5.7-59%]). Despite the low frequency of PSC in our clinic, LT in these patients resulted in favorable outcomes regarding postoperative morbidity and mortality compared with other etiologies.