TY - JOUR
T1 - Inflammatory bowel disease and primary sclerosing cholangitis
T2 - a review of the phenotype and associated specific features
AU - Palmela, Carolina
AU - Peerani, Farhad
AU - Castaneda, Daniel
AU - Torres, Joana
AU - Itzkowitz, Steven H.
N1 - Funding Information:
S.H.I. was funded in part by a grant from The Chemotherapy Foundation. F.P. would like to acknowledge the Canadian Institutes of Health Research and the Canadian Association of Gastroenterology for supporting his advanced inflammatory bowel disease fellowship and research.
PY - 2018/1
Y1 - 2018/1
N2 - Primary sclerosing cholangitis (PSC) is a chronic, progressive cholestatic disease that is associated with inflammatory bowel disease (IBD) in approximately 70% of cases. Although the pathogenesis is still unknown for both diseases, there is increasing evidence to indicate that they share a common underlying predisposition. Herein, we review the epidemiology, diagnosis, disease pathogenesis, and specific clinical features of the PSC-IBD phenotype. Patients with PSC-IBD have a distinct IBD phenotype with an increased incidence of pancolitis, backwash ileitis, and rectal sparing. Despite often having extensive colonic involvement, these patients present with mild intestinal symptoms or are even asymptomatic, which can delay the diagnosis of IBD. Although the IBD phenotype has been well characterized in PSC patients, the natural history and disease behavior of PSC in PSC-IBD patients is less well defined. There is conflicting evidence regarding the course of IBD in PSC-IBD patients who receive liver transplantation and their risk of recurrent PSC. IBD may also be associated with an increased risk of cholangiocarcinoma in PSC patients. Overall, the PSC-IBD population has an increased risk of developing colorectal neoplasia compared to the conventional IBD population. Lifelong annual surveillance colonoscopy is currently recommended.
AB - Primary sclerosing cholangitis (PSC) is a chronic, progressive cholestatic disease that is associated with inflammatory bowel disease (IBD) in approximately 70% of cases. Although the pathogenesis is still unknown for both diseases, there is increasing evidence to indicate that they share a common underlying predisposition. Herein, we review the epidemiology, diagnosis, disease pathogenesis, and specific clinical features of the PSC-IBD phenotype. Patients with PSC-IBD have a distinct IBD phenotype with an increased incidence of pancolitis, backwash ileitis, and rectal sparing. Despite often having extensive colonic involvement, these patients present with mild intestinal symptoms or are even asymptomatic, which can delay the diagnosis of IBD. Although the IBD phenotype has been well characterized in PSC patients, the natural history and disease behavior of PSC in PSC-IBD patients is less well defined. There is conflicting evidence regarding the course of IBD in PSC-IBD patients who receive liver transplantation and their risk of recurrent PSC. IBD may also be associated with an increased risk of cholangiocarcinoma in PSC patients. Overall, the PSC-IBD population has an increased risk of developing colorectal neoplasia compared to the conventional IBD population. Lifelong annual surveillance colonoscopy is currently recommended.
KW - Cholangitis
KW - Colorectal neoplasms
KW - Diagnosis
KW - Inflammatory bowel disease
KW - Liver transplantation
KW - Sclerosing
UR - http://www.scopus.com/inward/record.url?scp=85040526464&partnerID=8YFLogxK
U2 - 10.5009/gnl16510
DO - 10.5009/gnl16510
M3 - Review article
C2 - 28376583
AN - SCOPUS:85040526464
SN - 1976-2283
VL - 12
SP - 17
EP - 19
JO - Gut and Liver
JF - Gut and Liver
IS - 1
ER -