bs_bs_banner
doi:10.1111/jgh.12869
E D U C AT I O N A N D I M A G I N G
Hepatology: De novo primary sclerosing cholangitis post liver transplantation a
b
c
Figure 1 Liver biopsy showing (a)duct-centric mixed inflammatory infiltrate on H&E stain and (b) moderate concentric peri-ductal fibrosis on trichrome stain. ERCP showing irregular stricturing of the intra- and extrahepatic bile ducts with a “beaded” appearance (c).
Primary Sclerosing Cholangitis (PSC) is a well-established indication for orthotopic liver transplantation. Recurrence of PSC has been well documented post transplant, with a reported incidence of 5–20%, and can lead to allograft loss and the need for consideration of re-transplantation. However, the possibility of de novo PSC in patients transplanted for other indications has only been suggested in one previous report. We describe the first case of de novo PSC in a patient who had undergone liver transplantation for alcoholic cirrhosis. A 54-year-old male underwent liver transplantation in June 2010 for end-stage liver disease secondary to alcoholic cirrhosis. The patient had no history of PSC or inflammatory bowel disease prior to transplant. Pre-transplant colonoscopy revealed no evidence of mucosal inflammation. Careful examination of the explant revealed evidence of cirrhosis with no histological features suggestive of PSC. All donor variables were carefully reviewed. The donor had no history of inflammatory bowel disease or PSC and a pre-transplant donor liver biopsy was histologically unremarkable. The immediate post-transplant course was uncomplicated with no vascular or bile duct complications. He was noted to have elevation in his liver tests 2 years post transplant and underwent an extensive evaluation, including doppler ultrasound and serologic tests, all of which were negative. He was on a stable immunosuppressive regimen with adequate trough levels of tacrolimus. Liver biopsy revealed evidence of bile duct injury with a duct-centric mixed inflammatory infiltrate and moderate concentric peri-ductal fibrosis on trichrome stain (Fig. 1a,b). He subsequently developed severe diarrhea with blood and a non-healing skin ulcer on his right leg above the medial malleolus. Colonoscopy with biopsies confirmed a diagnosis of ulcerative pan colitis
1114
and dermatologic evaluation, including skin biopsy, confirmed a diagnosis of pyoderma gangrenosum. Since his liver tests continued to remain elevated in a cholestatic pattern, ERCP was performed. This revealed irregular stricturing of the intra- and extrahepatic bile ducts with a “beaded” appearance, radiographically diagnostic of PSC (Fig. 1c). Subsequent clinical course was characterized by continued, persistent elevation of liver tests in a cholestatic pattern with no biochemical improvement with Ursodeoxycholic acid. Initially reported by Lerut et al in 1988, recurrence of PSC post-liver transplantation is diagnosed based on cholangiographic features of PSC accompanied by obliterative peri-ductal fibrosis on histology, when available. This report describes the first ever case of de novo PSC post-transplant in a patient transplanted for another indication. The diagnosis was based on a compatible liver profile, typical findings on cholangiography, corroborative findings on liver histology and the development of known extra-hepatic associations of PSC (ulcerative colitis and pyoderma gangrenosum). The natural history of de novo PSC post-transplant and the impact of immunosuppression on disease progression is unclear. Re-transplantation for allograft dysfunction due to de novo PSC post transplant has never been reported before. Longer duration of follow up is necessary to better define the natural history of de novo PSC post liver transplantation and identify potential therapeutic agents. Contributed by S Kumar*,† *Center for Liver Disease & Transplantation, Aurora St. Luke’s Medical Center, Milwaukee, WI, USA and †Digestive Disease Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, UAE
Journal of Gastroenterology and Hepatology 30 (2015) 1114 © 2015 Journal of Gastroenterology and Hepatology Foundation and Wiley Publishing Asia Pty Ltd
doi:10.1111/jgh.12869
E D U C AT I O N A N D I M A G I N G
Hepatology: De novo primary sclerosing cholangitis post liver transplantation a
b
c
Figure 1 Liver biopsy showing (a)duct-centric mixed inflammatory infiltrate on H&E stain and (b) moderate concentric peri-ductal fibrosis on trichrome stain. ERCP showing irregular stricturing of the intra- and extrahepatic bile ducts with a “beaded” appearance (c).
Primary Sclerosing Cholangitis (PSC) is a well-established indication for orthotopic liver transplantation. Recurrence of PSC has been well documented post transplant, with a reported incidence of 5–20%, and can lead to allograft loss and the need for consideration of re-transplantation. However, the possibility of de novo PSC in patients transplanted for other indications has only been suggested in one previous report. We describe the first case of de novo PSC in a patient who had undergone liver transplantation for alcoholic cirrhosis. A 54-year-old male underwent liver transplantation in June 2010 for end-stage liver disease secondary to alcoholic cirrhosis. The patient had no history of PSC or inflammatory bowel disease prior to transplant. Pre-transplant colonoscopy revealed no evidence of mucosal inflammation. Careful examination of the explant revealed evidence of cirrhosis with no histological features suggestive of PSC. All donor variables were carefully reviewed. The donor had no history of inflammatory bowel disease or PSC and a pre-transplant donor liver biopsy was histologically unremarkable. The immediate post-transplant course was uncomplicated with no vascular or bile duct complications. He was noted to have elevation in his liver tests 2 years post transplant and underwent an extensive evaluation, including doppler ultrasound and serologic tests, all of which were negative. He was on a stable immunosuppressive regimen with adequate trough levels of tacrolimus. Liver biopsy revealed evidence of bile duct injury with a duct-centric mixed inflammatory infiltrate and moderate concentric peri-ductal fibrosis on trichrome stain (Fig. 1a,b). He subsequently developed severe diarrhea with blood and a non-healing skin ulcer on his right leg above the medial malleolus. Colonoscopy with biopsies confirmed a diagnosis of ulcerative pan colitis
1114
and dermatologic evaluation, including skin biopsy, confirmed a diagnosis of pyoderma gangrenosum. Since his liver tests continued to remain elevated in a cholestatic pattern, ERCP was performed. This revealed irregular stricturing of the intra- and extrahepatic bile ducts with a “beaded” appearance, radiographically diagnostic of PSC (Fig. 1c). Subsequent clinical course was characterized by continued, persistent elevation of liver tests in a cholestatic pattern with no biochemical improvement with Ursodeoxycholic acid. Initially reported by Lerut et al in 1988, recurrence of PSC post-liver transplantation is diagnosed based on cholangiographic features of PSC accompanied by obliterative peri-ductal fibrosis on histology, when available. This report describes the first ever case of de novo PSC post-transplant in a patient transplanted for another indication. The diagnosis was based on a compatible liver profile, typical findings on cholangiography, corroborative findings on liver histology and the development of known extra-hepatic associations of PSC (ulcerative colitis and pyoderma gangrenosum). The natural history of de novo PSC post-transplant and the impact of immunosuppression on disease progression is unclear. Re-transplantation for allograft dysfunction due to de novo PSC post transplant has never been reported before. Longer duration of follow up is necessary to better define the natural history of de novo PSC post liver transplantation and identify potential therapeutic agents. Contributed by S Kumar*,† *Center for Liver Disease & Transplantation, Aurora St. Luke’s Medical Center, Milwaukee, WI, USA and †Digestive Disease Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, UAE
Journal of Gastroenterology and Hepatology 30 (2015) 1114 © 2015 Journal of Gastroenterology and Hepatology Foundation and Wiley Publishing Asia Pty Ltd
