The Impact of Ulcerative Colitis on Patients With PSC
The Impact of Ulcerative Colitis on Patients With PSC
Background The impact of ulcerative colitis (UC) on the outcome of primary sclerosing cholangitis (PSC) outcome remains unclear.
Aim To investigate whether the presence of UC is associated with a worse clinical of associated PSC.
Methods A total of 222 patients with PSC (167 with UC and 55 without UC) seen and followed at a single centre from 1985 to 2011 were included. Clinical and demographic variables were obtained and patients were followed until the date of their last clinic visit.
Results The median age at presentation of PSC with associated UC was 38 vs. 47 years without UC (P < 0.001). At presentation, median serum bilirubin (2.1 vs. 4.5, P < 0.001) and the Mayo PSC Risk Score (0.95 vs. 1.69, P < 0.001) were lower in those with UC vs. those without UC. A total of 55 of 167 (32.9%) patients with PSC-UC developed colon neoplasia in contrast to 1 of the 55 (1.8%) patients with PSC. (P < 0.001) On proportional hazards analysis, UC (hazard ratio (HR) = 0.90 [95% confidence interval (CI): 0.60–1.34, P = 0.60] was not associated with death or orthotopic liver transplantation (OLT), when adjusting for gender, Mayo risk score and year of PSC diagnosis; whereas the revised Mayo risk score [HR = 5.08, 95% CI: (2.62–9.86), P < 0.001] was associated with a greater risk of OLT or death.
Conclusions Primary sclerosing cholangitis often is recognised at an early stage in patients with concurrent ulcerative colitis; ulcerative colitis has no impact on long-term prognosis in terms of liver-related outcomes when adjusted for the severity of liver disease.
Primary sclerosing cholangitis (PSC) is a chronic, liver disease commonly seen in association with underlying inflammatory bowel disease (IBD), most commonly ulcerative colitis (UC). Approximately 70–80% of patients with PSC have underlying IBD. UC in the presence of concomitant PSC presents with a distinct clinical phenotype; high prevalence of backwash ileitis, pancolitis, colorectal cancer and overall a poorer survival than patients without concomitant PSC.
The presence of IBD may affect the natural course and disease behaviour of PSC. However, differences in the natural history and clinical features of PSC in the presence or absence of IBD have not been well studied. Some studies have shown no difference in liver histology, clinical and radiographical features between PSC patients with and without IBD, whereas another study suggested that patients with PSC-IBD more often had combined intra- and extra-hepatic bile duct strictures than those without IBD (82% vs. 46%). Until recently, there was no conclusive evidence that the natural history of PSC differs between patients with and without IBD. Genetic analysis in patients with PSC has identified that patients with associated UC are at increased risk for progression to orthotopic liver transplantation (OLT) or death. Also, the presence of IBD increased the risk of development of cancer, mortality and requirement for OLT. However, this study was limited because of small number of non-IBD PSC patients (n = 19) who were included.
In an earlier study from our group, we investigated the impact of severity of PSC (requirement for liver transplantation or not) on the outcome of UC in terms of disease flares, activity and colectomy. We observed that progressive PSC requiring OLT (severe PSC) was less likely to be associated with colectomy (severe UC). However, the impact of UC on the outcome of PSC has not been investigated from North America. The aim of our study was to evaluate and compare the phenotype and prognosis of PSC in patient cohorts with and without concurrent UC. We also wanted to specifically study whether the presence of UC affects the outcome of PSC defined by development of cholangiocarcinoma, requirement for OLT and mortality.
Abstract and Introduction
Abstract
Background The impact of ulcerative colitis (UC) on the outcome of primary sclerosing cholangitis (PSC) outcome remains unclear.
Aim To investigate whether the presence of UC is associated with a worse clinical of associated PSC.
Methods A total of 222 patients with PSC (167 with UC and 55 without UC) seen and followed at a single centre from 1985 to 2011 were included. Clinical and demographic variables were obtained and patients were followed until the date of their last clinic visit.
Results The median age at presentation of PSC with associated UC was 38 vs. 47 years without UC (P < 0.001). At presentation, median serum bilirubin (2.1 vs. 4.5, P < 0.001) and the Mayo PSC Risk Score (0.95 vs. 1.69, P < 0.001) were lower in those with UC vs. those without UC. A total of 55 of 167 (32.9%) patients with PSC-UC developed colon neoplasia in contrast to 1 of the 55 (1.8%) patients with PSC. (P < 0.001) On proportional hazards analysis, UC (hazard ratio (HR) = 0.90 [95% confidence interval (CI): 0.60–1.34, P = 0.60] was not associated with death or orthotopic liver transplantation (OLT), when adjusting for gender, Mayo risk score and year of PSC diagnosis; whereas the revised Mayo risk score [HR = 5.08, 95% CI: (2.62–9.86), P < 0.001] was associated with a greater risk of OLT or death.
Conclusions Primary sclerosing cholangitis often is recognised at an early stage in patients with concurrent ulcerative colitis; ulcerative colitis has no impact on long-term prognosis in terms of liver-related outcomes when adjusted for the severity of liver disease.
Introduction
Primary sclerosing cholangitis (PSC) is a chronic, liver disease commonly seen in association with underlying inflammatory bowel disease (IBD), most commonly ulcerative colitis (UC). Approximately 70–80% of patients with PSC have underlying IBD. UC in the presence of concomitant PSC presents with a distinct clinical phenotype; high prevalence of backwash ileitis, pancolitis, colorectal cancer and overall a poorer survival than patients without concomitant PSC.
The presence of IBD may affect the natural course and disease behaviour of PSC. However, differences in the natural history and clinical features of PSC in the presence or absence of IBD have not been well studied. Some studies have shown no difference in liver histology, clinical and radiographical features between PSC patients with and without IBD, whereas another study suggested that patients with PSC-IBD more often had combined intra- and extra-hepatic bile duct strictures than those without IBD (82% vs. 46%). Until recently, there was no conclusive evidence that the natural history of PSC differs between patients with and without IBD. Genetic analysis in patients with PSC has identified that patients with associated UC are at increased risk for progression to orthotopic liver transplantation (OLT) or death. Also, the presence of IBD increased the risk of development of cancer, mortality and requirement for OLT. However, this study was limited because of small number of non-IBD PSC patients (n = 19) who were included.
In an earlier study from our group, we investigated the impact of severity of PSC (requirement for liver transplantation or not) on the outcome of UC in terms of disease flares, activity and colectomy. We observed that progressive PSC requiring OLT (severe PSC) was less likely to be associated with colectomy (severe UC). However, the impact of UC on the outcome of PSC has not been investigated from North America. The aim of our study was to evaluate and compare the phenotype and prognosis of PSC in patient cohorts with and without concurrent UC. We also wanted to specifically study whether the presence of UC affects the outcome of PSC defined by development of cholangiocarcinoma, requirement for OLT and mortality.
