Primary sclerosing cholangitis (PSC) is a chronic bile duct disease with an estimated prevalence in the range of 1 to 16 per 100 000 with significant regional differences across Europe. The prevalence of PSC is increased in patients with ulcerative colitis and estimated to be in the range 1 % – 5 % [1]. Magnetic resonance imaging (MRI) studies have shown that the prevalence of imaging changes compatible with PSC in ulcerative colitis is almost fourfold higher than that detected based on clinical assessments [2]. PSC is more common in men (comprising 60 % – 70 % of patients) and most patients present with pancolitis, often with a right-sided predominance [3] [4] [5]. A major challenge in the clinical management of patients is a highly increased and unpredictable risk of biliary and colonic malignancies.

The diagnosis of PSC is based on the combination of clinical, laboratory, imaging, and histological findings. Briefly, a diagnostic work-up for PSC should be performed in all patients with inflammatory bowel disease (IBD) and abnormal liver biochemistry test findings, especially elevated alkaline phosphatase (ALP) and gamma glutamyl transferase (GGT) values, as well as in non-IBD patients with elevated cholestatic liver enzymes not otherwise explained. A proposed algorithm for PSC diagnosis has already been presented by earlier European Association for the Study of the Liver (EASL) guidelines [6], and comprehensive discussion of issues unrelated to the use of endoscopy in PSC will not be addressed in the present Guideline.

Endoscopic retrograde cholangiopancreatography (ERCP) plays a significant role in the handling of PSC because of its high accuracy and prognostic value as well as its sampling and therapeutic possibilities. However, ERCP must be integrated within well-defined clinical algorithms together with less invasive or noninvasive imaging and biochemical tests. In particular, the widespread implementation of magnetic resonance cholangiography (MRC) has led to increasing restriction of the use of ERCP to cases where the diagnosis is equivocal or when sampling or endoscopic treatment are required.

The aim of this evidence- and consensus-based Guideline, commissioned by the European Society of Gastrointestinal Endoscopy (ESGE) and the EASL, is to provide practical advice on how to utilize ERCP and colonoscopy in PSC patients, in order to maximize their benefit and minimize their burden and adverse events.

Main recommendations:

1 ESGE/EASL recommend that, as the primary diagnostic modality for PSC, magnetic resonance cholangiography (MRC) should be preferred over endoscopic retrograde cholangiopancreatography (ERCP).Moderate quality evidence, strong recommendation. 2 ESGE/EASL suggest that ERCP can be considered if MRC plus liver biopsy is equivocal or contraindicated in patients with persisting clinical suspicion of PSC. The risks of ERCP have to be weighed against the potential benefit with regard to surveillance and treatment recommendations.Low quality evidence, weak recommendation. 6 ESGE/EASL suggest that, in patients with an established diagnosis of PSC, MRC should be considered before therapeutic ERCP.Weak recommendation, low quality evidence. 7 ESGE/EASL suggest performing endoscopic treatment with concomitant ductal sampling (brush cytology, endobiliary biopsies) of suspected significant strictures identified at MRC in PSC patients who present with symptoms likely to improve following endoscopic treatment.Strong recommendation, low quality evidence. 9 ESGE/EASL recommend weighing the anticipated benefits of biliary papillotomy/sphincterotomy against its risks on a case-by-case basis.Strong recommendation, moderate quality evidence.Biliary papillotomy/sphincterotomy should be considered especially after difficult cannulation.Strong recommendation, low quality evidence. 16 ESGE/EASL suggest routine administration of prophylactic antibiotics before ERCP in patients with PSC.Strong recommendation, low quality evidence. 17 EASL/ESGE recommend that cholangiocarcinoma (CCA) should be suspected in any patient with worsening cholestasis, weight loss, raised serum CA19-9, and/or new or progressive dominant stricture, particularly with an associated enhancing mass lesion.Strong recommendation, moderate quality evidence. 19 ESGE/EASL recommend ductal sampling (brush cytology, endobiliary biopsies) as part of the initial investigation for the diagnosis and staging of suspected CCA in patients with PSC.Strong recommendation, high quality evidence.

Additional Metadata
Persistent URL dx.doi.org/10.1055/s-0043-107029, hdl.handle.net/1765/99352
Journal Endoscopy
Aabakken, L, Karlsen, T.H, Albert, J. (Jörg), Arvanitakis, M. (Marianna), Chazouilleres, O. (Olivier), Dumonceau, J.M, … Hassan, C. (2017). Role of endoscopy in primary sclerosing cholangitis: European Society of Gastrointestinal Endoscopy (ESGE) and European Association for the Study of the Liver (EASL) Clinical Guideline. Endoscopy. doi:10.1055/s-0043-107029