Guidelines on sclerosing cholangitis in adults and children were initially released in June 2022 and published in September 2022 by the European Association for the Study of the Liver (EASL) in the Journal of Hepatology. They provide practical guidance regarding diagnostic methods, prognostic assessment, timely identification of complications, optimal care pathways, and therapeutic options, among other clinical issues. Select strong recommendations are outlined below.
In adults with elevated serum markers of cholestasis, make the diagnosis of large duct primary sclerosing cholangitis (PSC) when typical findings of sclerosing cholangitis are present on high-quality cholangiography, as well as after secondary causes have been ruled out. Magnetic resonance cholangiopancreaticography (MRCP) is the preferred study.
Do not use autoantibodies for diagnosis or risk stratification of people with PSC.
Consider liver biopsy in those with PSC and coexisting features of autoimmune hepatitis (AIH) (eg, markedly elevated transaminases, high immunoglobulin G [IgG] levels, positive autoantibodies compatible with AIH).
The EASL recommends assessing risk at the time of diagnosis and sequentially on the basis of phenotypic factors and noninvasive tests that include standard biochemistry (eg, serum bilirubin, albumin, alkaline phosphatase [ALP], alanine aminotransferase [ALT], platelets, prothrombin time [PT]), MR imaging [MRI] of the liver with MRCP, and liver elastography or serum fibrosis tests.
The EASL recommends clinical noninvasive routine liver surveillance on the following basis:
Review and standard serum liver tests (eg, bilirubin, albumin, ALP, aspartate aminotransferase [AST], platelets, PT) every 6-12 months depending on risk stratification
Liver elastography and/or serum fibrosis tests at least every 2-3 years
Assess bone mineral density with dual energy X-ray absorptiometry (DEXA) in all those with PSC at the time of diagnosis. Use current practice guidelines for follow-up and treatment of osteopenia and osteoporosis.
At diagnosis of PSC, ileocolonoscopy with biopsies from all colonic segments (eg, terminal ileum) is recommended, regardless of the presence of lesions.
In all adult patients with PSC and inflammatory bowel disease (IBD), yearly surveillance (or every 1-2 years in individualized patients without inflammatory activity), the EASL recommends colonoscopy with biopsies, regardless of the IBD duration or liver transplant status.
Do not give ursodeoxycholic acid (UDCA) at doses of 28-30 mg/kg/day.
In the absence of recurrent bacterial cholangitis, the EASL does not recommend long-term use of antibiotics.
Rule out relevant bile duct strictures in large duct sclerosing cholangitis as the cause of progressive pruritus. If present and reachable, treat relevant strictures by endoscopic balloon dilatation (or stenting, if balloon dilatation alone is insufficient) after brushing.
Treat acute bacterial cholangitis with antibiotics followed by biliary decompression in the presence of an underlying relevant stricture.
In general, manage complications of portal hypertension in PSC based on Baveno/EASL guidelines for advanced chronic liver diseases.
Ideally, a multidisciplinary team of hepatologists, biliary endoscopists, and abdominal radiologists discusses the indication for endoscopic intervention, with the procedure performed by experienced endoscopists.
Predniso(lo)ne 0.5-0.6 mg/kg/day is first-line therapy for untreated active IgG4-related cholangitis (IRC). Before tapering predniso(lo)ne, assess treatment response after (2 to) 4 weeks using clinical, biochemical, and/or radiologic criteria.
In the setting of confirmed cholangiocarcinoma (CCA) or high-grade dysplasia, refer patients to a specialized center. Consider multidisciplinary, therapeutic options such as liver transplantation, liver resection, brachy- or systemic therapy, or combinations.
At the time of PSC diagnosis, perform ileocolonoscopy with biopsies from all colonic segments including the terminal ileum, whether lesions are present or not.
All adult patients with PSC-related IBD should undergo yearly surveillance colonoscopy (or every 1-2 years in individualized patients absent inflammatory activity) with biopsies, regardless of the IBD duration or liver transplant status.
Follow current practice guidelines for treating PSC-IBD; the goal is to achieve mucosal healing.
Provide all patients with information regarding existing patient support groups.
Consider liver transplantation for people with PSC and the following:
Decompensated cirrhosis or hepatocellular carcinoma based on standard guidelines
Recurrent bacterial cholangitis and/or severe pruritus or jaundice despite endoscopic and pharmacologic treatment
Perform liver transplantation in PSC with duct-to-duct anastomosis, unless an anatomic disease site or technical surgical factors warrant Roux-en-Y hepaticojejunostomy.
Diagnostic practice in pediatric patients should be similar to that of adults, with MRCP as the diagnostic modality of choice.
The EASL recommends therapeutic endoscopic intervention in children with high-grade strictures on imaging and signs/symptoms of obstructive cholestasis and/or bacterial cholangitis. Referral to a center with expertise in pediatric interventional endoscopic retrograde cholangiopancreaticography (ERCP) is highly recommended.
The EASL recommends a coordinated multidisciplinary transition process from pediatric services to adult care, with sharing of information and focused attention on psychosocial issues and treatment adherence.
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Cite this: Sclerosing Cholangitis Clinical Practice Guidelines (EASL, 2022) - Medscape - Oct 04, 2022.