Update in the Care and Management of Patients with Primary Sclerosing Cholangitis

Abstract

Drugs under investigation include norursodeoxycholic acid, nuclear receptor agonists, anti-fibrotics, antibiotics, and anti-inflammatory drugs. Endoscopic therapy is indicated for symptomatic dominant strictures and in the work-up of malignancies. Recently, the use of stents was associated with an increased rate of complications compared to balloon dilatation; and long-term stenting should be avoided. Malignancies currently account for most of the PSC-related mortality. Many drugs are emerging for the treatment of PSC but liver transplantation is the only treatment modality shown to prolong survival. PSC recurrence occurs in up to 35% of transplanted allografts within a median of 5 years. Surveillance for hepatobiliary and colorectal malignancies is indicated.

Stent versus Balloon Dilation for the Treatment of Dominant Strictures in Primary Sclerosing Cholangitis: A Systematic Review and Meta-Analysis

“…22,25 Despite the unclear long-term benefits of endoscopic therapy, multiple techniques for symptom relief in patients affected by PSC have been studied, particularly the endoscopic treatment of dominant strictures. 7,25 Studies comparing the endoscopic management of PSC-related dominant strictures are necessary, since such procedures carry the potential to alter the survival and prognosis of patients, although the duration of liver transplant-free survival associated with these interventions remains unclear. 23 Based on the results of this systematic review and meta-analysis of over 460 patients, stent placement for dominant strictures in PSC appeared to associate with higher complication rates without significant differences in efficacy when compared to balloon dilation.…”

“…The presentation of PSC is highly variable, ranging from asymptomatic in the early stages, to recurrent episodes of cholangitis with fever, jaundice, pruritus, abdominal pain and fatigue. However, median survival tends to be significantly decreased in patients who are symptomatic at the time of diagnosis [ 6 , 7 ]. Epidemiological studies of PSC have reported a prevalence rate of approximately 1 per 10,000, and incidence rates between 0.4 and 2.0 per 100,000 per year in Northern Europe and the US [ 8 ].…”

“…However, median survival tends to be significantly decreased in patients who are symptomatic at the time of diagnosis. 6,7 Epidemiological studies of PSC have reported a prevalence rate of approximately 1 per 10,000, and incidence rates between 0.4 and 2.0 per 100,000 per year in Northern Europe and the US. 8 PSC conforms to the definition of rare diseases, affecting less than 200,000 individuals in the…”

“…Although liver transplantation remains the treatment of choice for advanced diseases and represents the only curative therapy for PSC, patients with dominant extra-hepatic biliary strictures may potentially benefit from endoscopic management. 7,11,21 Dominant biliary strictures are functionally defined as a narrowing within the biliary tree that halts the normal flow of bile and, by most consensus guidelines, is considered a stricture < 1.5 mm in the bile duct or < 1 mm in the hepatic ducts, with distance < 2 cm from the bifurcation in the hepatic hilum. 22,23 During the natural history of the disease, many patients experience symptoms such as pruritus, right upper quadrant pain, fatigue, fever, and jaundice due to impeded biliary drainage.…”

Modelling Recurrent Primary Biliary Cholangitis and Primary Sclerosing Cholangitis as Infectious Diseases Following Liver Transplantation

“…Preliminary studies are starting to suggest the presence of a distinct intestinal microbiome in PSC patients characterized by an overabundance of potentially pathogenic bacteria [4]. Multiple studies have also demonstrated biochemical response in PSC patients treated with different antibiotics, suggesting that infection may play a role in the development of PSC [5]. All of this goes towards suggesting that an infectious etiology may underlie the pathogenesis of both PBC and PSC.…”