P418 Ulcerative colitis and end-stage liver disease in children with primary sclerosing cholangitis
P. Czubkowski*, M. Wozniak, J. Pawlowska, I. Jankowska, J. Kierkus
The Children’s Memorial Health Institute, Department of Gastroenterology, Hepatology, Feeding Disorders and Paediatrics, Warsaw, Poland
Primary sclerosing cholangitis (PSC) is a progressive, cholestatic disorder characterised by chronic inflammation, biliary strictures, and frequent co-morbidity with ulcerative colitis (UC). Patients are at an increased risk of malignancy arising from the colon, bile ducts, and liver. The aim of the study was to analyse the influence of end-stage liver disease (ESLD) and liver transplantation (LTx) on the course of UC in children.
We retrospectively reviewed children diagnosed with UC and PSC treated in our institution between 2000 and 2015 who developed ESDL. UC diagnosis was based on clinical presentation, endoscopy, and histopathology. PSC was confirmed by endoscopic retrograde cholangiopancreatography, magnetic resonance cholangiopancreatography, and liver biopsy.
Out of 12 patients with PSC and UC who developed ESLD, 10 underwent LTx at the median age of 16.2 years (11.5–17.5), and 2 are currently on the waiting list. In 11 patients, UC was diagnosed before ESLD at the median age of 11 years (4–17), and one patient developed UC 6 months after transplantation. Further, 10 patients (83%) presented with pancolitis, and 2 with proctosigmoiditis. Severe, moderate, and mild endoscopic changes were present in 7 (58%), 3 (25%), and 2 (16%) patients, respectively. Initial treatment consisted of mesalazyne or sulfasalazine in combination with steroids and azathioprine (AZA) with clinical remission in 75%. Three non-responding patients required cyclosporine with good outcome. None of the patients received anti-TNF treatment. At the moment of LTx or listing (at median 5 years [range 1–12] after onset of UC) clinical activity according to PUCAI was remission in 8, moderate in 1, and mild in 2 patients. PSC was diagnosed at the median age of 11 years (4–16), and all patients received UDCA, which was continued after LTx. Ten patients received deceased donor, AB0 compatible, whole liver grafts. Biliary anastomosis was hepaticojejunostomy in 9 and duct-to-duct anastomosis in 1 patient. Post-transplant immunosuppressive regiment consisted of tacrolimus (TAC), n = 7; cyclosporine (CS), n = 3; mycofenolate mofetil, n = 7; AZA, n = 1; and steroids, n = 8. Neither of the patients developed colorectal or liver cancer nor underwent colectomy within median follow-up after UC onset of 13.1(2–19) years (post-LTx follow-up, 6.8 years). Up-to-date overall patient survival is 91%. One patient died because of non-adherence. Two patients lost liver grafts because of thrombosis and chronic rejection. Currently, UC remission is maintained in 9 of 11(81%) patients, and 2 have moderate disease activity.
The clinical outcome of UC is not worsened by end-stage liver disease or liver transplantation because of PSC.