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Practice guidelines released for treating primary sclerosing cholangitis

Primary sclerosing cholangitis is a chronic cholestatic liver and biliary tract disease that can shorten life and may require liver transplantation, according to study data. The prevalence in the United States is estimated to be in the range of one to more than 16 per 100,000 and varies across different regions of the world. There is no approved therapy for the disease, which can progress over time and lead to recurrent biliary tract obstruction and recurrent episodes of cholangitis to end-stage liver disease.

Researchers, including Kevin D. Lindor, MD, FACG, of the College of Health Solutions at Arizona State University in Phoenix, were invited by the Board of Trustees of the American Journal of Gastroenterology and the Practice Parameters Committee to release the 2015 practice guideline for clinicians to consider when diagnosing and evaluating a patient with primary sclerosing cholangitis (PSC).

Key points of the 24 suggested recommendations include: how to diagnose a patient; should ursodeoxycholic acid be used as a treatment; endoscopic management and how to treat patients who need a liver transplant or have inflammatory bowel disease (IBD); and general management.

Diagnosis

Endoscopic retrograde cholangiopancreatography (ERCP) has been the most common procedure in diagnosing PBS. However, magnetic resonance cholangiopancreatography (MRCP) is rapidly becoming the preferred method because it is noninvasive, cheaper and has no associated risk of pancreatitis. Using MRCP over ERCP is a strong recommendation and liver biopsy is not needed unless a patient is suspected of having small duct PSC. Other tests that should be performed are antimitochondrial testing and testing to measure for elevated serum immunogloblulin G4 levels.

Therapy

There is currently no established treatment for PSC. However, many clinicians are using doses of ursodeoxycholic acid (UDCA) at 20 mg/kg per day for treating PSC. There is a lack of data from clinical trials of UDCA, according to the researchers, so the researchers do not recommend using more than 28 mg/kg per day of UDCA for managing PSC. 

Endoscopic Management

Endoscopic treatment of dominant strictures may improve prognosis, help to relieve the complications of pruritus and cholangitis, allow for early diagnosis of cholangiocarcinoma and may lead to improved survival. ERCP with balloon dilatation is recommended for relieving symptoms of dominant strictures of PSC patients. If a PSC patient undergoes ERCP, antibiotic prophylaxis should be taken to prevent post-ERCP cholangitis. Routine stenting after dilation is not recommended. 

Liver Transplantation

Since there has not been an effective medical therapy for treating PSC, the median time for progression of the disease from diagnosis until death or liver transplantation (LT) is 10 to 12 years. A deceased donor organ for liver transplant due to PSC offers a 5-year survival rate between 80% and 85%. Therefore, it is recommended that a patient with PSC undergo liver transplant over medical therapy or surgical drainage to prolong survival. Patients with a MELD score over 14 should be referred for LT. 

IBD and PSC

In some studies, it has been said that up to 80% of patients with PSC have IBD, according to the research. Undergoing annual colon surveillance with chromoendoscopy is recommended in PSC patients with colitis at the time of PCS diagnosis, and a colonoscopy with biopsies is recommended in PSC patients whether or not there are symptoms at PSC diagnosis. An exam every 3-5 years is also recommended for patients without prior evidence of colitis.

Hepatobiliary Malignancies and Gallbladder Disease

PSC patients are at risk for developing hepatobiliary malignancies. The risk for cholangiocarcinoma is several hundred times higher in patients with PSC vs. patients without PSC. Screening for cholangiocarcinoma with regular cross-sectional imaging with ultrasound or magnetic resonance and serial CA 19-9 is recommended every 6 to 12 months. Patients with PSC with gallbladder polyps more than 8 mm should also undergo cholecystectomy.

Special Situations

Every PSC patient is unique and some patients may develop autoimmune hepatitis and other infections. Testing for autoimmune hepatitis is recommended for patients with PSC under 25 years of age and for those patients with higher than expected aminotransferases. MRCP is recommended for PSC patients under 25 years of age with autoimmune hepatitis who have elevated alkaline phosphatase serum levels.

General Management

Patients with PSC should undergo bone mineral density screening at diagnosis using duel energy X-ray absorption repeated at 2- to 4-year intervals; patients with advanced liver disease should be checked and monitored for fat-soluble vitamin deficiencies; and patients with PSC and mild pruritus should undergo local skin treatment with antihistamines or emollients to reduce symptoms.  – by Melinda Stevens

For More Information:

View the guidelines at http://www.nature.com/ajg/journal/vaop/ncurrent/full/ajg2015112a.html.

Disclosure: Lindor reports serving as a consultant in clinical trials of experimental drugs for Cholestatic liver disease and is an unpaid advisor for Lumena Pharmaceuticals and Intercept Pharmaceuticals. Please see the study for a full list of authors’ relevant financial disclosures.