Clinical characteristics and outcomes of primary sclerosing cholangitis and ulcerative colitis in Japanese patients

Abstract

BackgroundIn Western countries, most patients with primary sclerosing cholangitis (PSC) have concurrent ulcerative colitis (UC). The number of patients with UC in East Asia has increased markedly over the past two decades. However, current clinical features of PSC and of PSC associated with UC (PSC-UC) have not yet been clarified in East Asia, particularly in Japan. We aimed to reveal the clinical courses and associations with UC in Japanese patients with PSC from the mutual viewpoint of PSC and UC.MethodsWe retrospectively retrieved medical records of patients with PSC (69) and UC (1242) who were diagnosed at Chiba University Hospital between June 1991 and August 2017.ResultsIn the present cohort, 37 patients had PSC-UC; the cumulative risks of PSC in patients with UC and of UC in patients with PSC were 3.0% and 53.6%, respectively. We confirmed similar distinctive results by a Japanese nationwide survey, noting that younger patients with PSC had a notably high possibility of association with UC. From the viewpoint of the UC cohort, the occurrence of right-sided disease was significantly higher in patients with PSC-UC than in those with UC (16.2% vs. 4.2%, P = 0.003). Pancolitis was more commonly observed in PSC-UC, and proctits/left-sided colitis was less commonly found in patients with UC. The number of patients with young-onset PSC-UC may be increasing similar to an increase in patients with UC in Japan.ConclusionsIn our cohort, the comorbidity rate of PSC-UC was higher than that obtained in previous reports. The incidence of PSC-UC and UC may increase in the future in East Asia, particularly in Japan.

Clinical Characterization of Ulcerative Colitis in Patients with Primary Sclerosing Cholangitis

“…Part of the significance of diagnosing and treating IBD associated with PSC comes from the fact that patients with IBD are at an increased risk of developing colorectal cancer (CRC). CRC has been identified as a significant prognostic factor for PSC [ 13 ], although it remains to be determined whether the coexistence of IBD has an impact on the prognosis of PSC [ 14 , 15 ].…”

Elective staged proctocolectomy and living donor liver transplantation for colon cancer with sclerosing cholangitis-related ulcerative colitis: a case report

“…UC is a well-recognized complication of PSC. The current comorbidity rate of UC for patients with PSC in Japan ranges from approximately 20% to 55%, which is lower than the range of 30% to 80% for Western cohorts [ 1 – 4 ]. Although UC is also recognized as a risk factor for the development of dysplasia or cancer [ 5 – 8 ], we rarely encounter a patient with PSC-related UC and concurrent colon cancer requiring both LT and colectomy, as in the current case.…”

“…Primary sclerosing cholangitis (PSC) is one of the most common indications for liver transplantation (LT) and is a well-known complication of ulcerative colitis (UC) because of the associated autoimmune characteristics [ 1 – 4 ]. UC is a well-recognized risk factor for colon cancer [ 5 – 8 ]; therefore, we occasionally encounter a complicated situation in which a patient with PSC develops concurrent UC-related colon cancer.…”

Antigen-Specific Immunotherapy for Treatment of Autoimmune Liver Diseases

“…PSC is usually diagnosed by MRI imaging of the biliary tree to identify cholestasis and/or strictures ( 69 , 91 , 92 ). Up to 80% of PSC patients also present with inflammatory bowel disease (IBD), indicating a general gastrointestinal inflammatory phenotype ( 93 ). Taken alongside the fact that PSC is more common in men and has less strong HLA associations, the lack of known autoantibodies calls into question whether the disease is strictly autoimmune, or whether it is autoinflammatory in nature ( 94 ).…”