Update on inflammatory bowel disease in patients with primary sclerosing cholangitis

Abstract

Patients with primary sclerosing cholangitis (PSC) complicated by inflammatory bowel disease (IBD) represent a distinct subset of patients with unique characteristics, which have serious clinical implications. The aim of this literature review was to shed light to the obscure clinical and molecular aspects of the two diseases combined utilizing current data available and putting issues of diagnosis and treatment into perspective. The prevalence of IBD, mainly ulcerative colitis in PSC patients is estimated to be 21%-80%, dependent on screening programs and nationality. PSC-associated colitis is likely to be extensive, characterized by rectal sparing, backwash ileitis, and generally mild symptoms. It is also more likely to progress to colorectal malignancy, making it imperative for clinicians to maintain a high level of suspicion when tackling PSC patients. There is no optimal surveillance strategy but current guidelines advocate that colonoscopy is necessary at the time of PSC diagnosis with annual endoscopic follow-up. Random biopsies have been criticized and a shift towards targeted biopsies using chromoendoscopy, laser endomicroscopy and narrow-band imaging has been noted. Techniques directed towards genetic mutations instead of histological abnormalities hold promise for easier, more accurate diagnosis of dysplastic lesions. Chemopreventive measures against colorectal cancer have been sought in these patients. Ursodeoxycholic acid seemed promising at first but subsequent studies yielded conflicting results showing anticarcinogenic effects in low doses (8-15 mg/kg per day) and carcinogenic properties in high doses (15-30 mg/kg per day).

Keywords: Primary sclerosing cholangitis; Inflammatory bowel disease; Ulcerative colitis; Crohn’s disease

Core tip: Combination of primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD) has recently arisen as a challenging research field. Recent data highlight the specific clinical and genetic traits that differentiate PSC-IBD from the two diseases individually. We reviewed the literature on colorectal neoplastic susceptibility in this subset of patients and the underlying pathogenetic mechanisms. We also emphasize the technological advances that have provided novel diagnostic tools for more accurate detection of dysplastic lesions. Finally, we present current guidelines on follow-up as well as all evidence available as to whether ursodeoxycholic acid should be used prophylactically against colorectal cancer.