Author: David W. Blevins, Assistant professor of Chemistry, Molecular Imaging and Translational Research Program, University of Tennessee Graduate School of Medicine
This is a summary of the main recommendations of the European Society of Gastrointestinal Endoscopy (ESGE) and of the European Association for the study of the Liver (EASL) on the role of endoscopy in primary sclerosing cholangitis (PSC).[i] Each recommendation given was assessed by a “GRADE” system to define the strength of the recommendation and the quality of the evidence that supports it.
- Strong Recommendation=SR
- Weak Recommendation=WR
- Very Low Quality Evidence=VLQE
- Low Quality Evidence=LQE
- Moderate Quality Evidence=MQE
- High Quality Evidence=HQE
Main Recommendations for the diagnosis and surveillance of Primary Sclerosing Cholangitis:
- The primary diagnostic method for PSC should be magnetic resonance cholangiography (MRC) instead of the more invasive endoscopic retrograde cholangiopancreatography (ERCP). MQE/SR
- ERCP can be considered if MRC and liver biopsy results are inconclusive, or if for other medical reasons the MRC is not advisable. The risks of an ERCP have to be weighed against the potential benefits with regard to surveillance and treatment recommendations. LQE/WR
- For the diagnosis of PSC, ERCP is the only endoscopic technique recommended. Other endoscopic techniques not recommended include endoscopic ultrasound, intraductal ultrasound, cholangioscopy, and confocal endomicroscopy.
ERCP in established PSC patients.
- A dominant stricture at ERCP should be defined as a narrowing of the common bile duct to 1.5 millimeter (mm) or less, and 1.0 mm or less for a hepatic duct within 2 centimeters of the main hepatic duct branching point. WR/LQE
- ERCP and ductal sampling (brush cytology and endobiliary biopsies) should be considered in established PSC patients in the case of: a) clinically relevant or worsening symptoms such as jaundice, cholangitis, or pruritus. b) A rapid increase in cholestatic enzyme levels. c) New dominant strictures identified at MRC along with the appropriate clinical findings. WR/LQE
- For established PSC patients, MRC results should be considered before therapeutic ERCP. WR/LQE
- Any ERCP treatment should include ductal sampling (brush cytology and endobiliary biopsies) of suspected significant strictures identified during MRC. SR/LQE
Balloon dilation versus stent therapy.
- The choice of using stents or balloon dilation should the endoscopist’s decision. WR/LQE
Role of Spinchterotomy.
- Biliary papillotomy/spinchterotomy should only be carried out after weighing the potential benefits against the risks. This should be done on a case-by-case basis. SR/MQE
- For a balloon dilation to open a stricture, the balloon size should be limited to the duct diameter. WR/LQE
- In the case of a relapsing dominant stricture, the dilation should be repeated if: a) the dominant stricture is the cause of recurrent symptoms (cholangitis, pruritus, or significantly increased cholestasis). b) the patient’s response to previous dilations has been satisfactory. WR/LQE
- For a dominant stricture in the bile ducts outside of the liver, a single 10-Fr size stent should be used. In the case of strictures in the hilar ductal region extending into the left or right hepatic ducts, two 7-Fr size stents should be used (final stent diameters in the case of stepwise stenting). WR/LQE
Duration of Stenting.
- Stents used for treating a dominant stricture should be removed within 1-2 weeks after insertion. WR/LQE
Complications of Endoscopic Therapy.
- Only an experienced pancreatobiliary endoscopist should carry out ERCP in PSC patients. SR/LQE
Post ERCP pancreatitis.
- All ERCP patients should receive 100 milligrams of Diclofenac or Indomethacin immediately before or after the procedure unless for other medical reasons this is not advisable. In cases with a high risk for post ERCP pancreatitis, the placement of precautionary, protective 5-Fr size pancreatic stents should be considered. SR/HQE
- Protective, preventative antibiotics should be administered before an ERCP in patients with PSC. SR/LQE
PSC and Cholangiocarcinoma.
- Cholangiocarcinoma (CCA) should be suspected in any patient with worsening cholestasis, weight loss, increased CA19-9 (cancer marker) in blood tests, and/or a new or progressive dominant stricture, particularly with an associated growing mass lesion.
- Elevated CA19-9 levels in blood tests supports the diagnosis of CCA, but it is not always specific. WR/LQE
ERCP results that suggest cholangiocarcinoma.
- The initial investigation for CCA should include brush cytology and ductal tissue sampling. SR/HQE
- If brush cytology results of a suspected CCA are inconclusive, then fluorescent in-situ hybridization or an equivalent chromosomal assessment test should be used. WR/LQE
- Other investigative techniques such as cholangioscopy, endoscopic ultrasound, and probe-based confocal endomicroscopy may be useful in some cases. WR/LQE
Endoscopic monitoring of PSC-associated inflammatory bowel disease.
- PSC patients should receive ileocolonoscopy at the time of PSC diagnosis. SR/HQE
If inflammatory bowel disease is present then yearly surveillance colonoscopies are needed. SR/LQE
- In the case of no inflammatory bowel disease, the next ileocolonoscopy should be considered at 5 years or whenever inflammatory bowel disease symptoms appear. WR/LQE
Endoscopic method and technique.
- For screening for inflammatory bowel disease in PSC patients, the ileocolonoscopy should include four-quadrant biopsies from all colonic segments including the terminal ileum. SR/LQE
- In the case of PSC associated inflammatory bowel disease, ileocolonoscopy monitoring of the disease should be performed using a tissue staining dye (chromoendoscopy) with targeted biopsies. SR/LQE
Polyps and colorectal tissue changes (dysplasia).
- Endoscopic removal of any visible abnormal/damaged tissue (lesions) and assessment of the surrounding area (surrounding mucosal tissue) is recommended.
Removal of the rectum and colon (proctocolectomy) is recommended in the case of abnormalities in the surrounding mucosal tissue, or if the lesion cannot be completely removed.
- In the case of invisible lesions with precancerous cells (high-grade dysplasia) that is confirmed by two expert pathologists proctocolectomy is advised. SR/LQE
- In the case of invisible lesions with abnormal cells (low-grade dysplasia) confirmed by two expert pathologists, a repeat colonoscopy using a tissue staining dye (chromoendoscopy) is recommended. SR/LQE
[i] Aabakken, L., Karlsen, T. H., Albert, J., Arvanitakis, M., Chazouilleres, O., Dumonceau, J. M., … & Marzioni, M. (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, 49(06), 588-608.