Primary Biliary Cholangitis: 2018 Practice Guidance From the American Association for the Study of Liver Diseases

http://aasldpubs.onlinelibrary.wiley.com/hub/journal/10.1002/(ISSN)2046-2484/video/15-1-reading-lindor a video presentation of this article

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Abbreviations

AASLD

American Association for the Study of Liver Diseases

PBC

primary biliary cholangitis

In November 2018, the American Association for the Study of Liver Diseases (AASLD) published an update to the 2009 practice guideline on the Diagnosis, Staging, and Management of Primary Biliary Cholangitis (PBC).1 The 2018 updated guidance on PBC includes updates on etiology and diagnosis, the role of imaging, clinical manifestations, and treatment of PBC since 2009. The AASLD 2018 PBC Guidance provides a data‐supported approach to screening, diagnosis, and clinical management of patients with PBC. It differs from more recent AASLD practice guidelines, which are supported by systematic reviews and a multidisciplinary panel of experts that rates the quality (level) of the evidence and the strength of each recommendation using the Grading of Recommendations Assessment, Development, and Evaluation system. In contrast, this guidance was developed by consensus of an expert panel and provides guidance statements based on formal review and analysis of published literature on the topics. The quality (level) of the evidence and the strength of each guidance statement are not rated.

Intended for use by health care providers, this guidance identifies preferred approaches to the diagnostic and therapeutic aspects of care for patients with PBC. As with clinical practice guidelines, it provides general guidance to optimize the care of the majority of patients and should not replace clinical judgment for a unique patient. The Guidance Statements are included in Table 1.

Table 1.

Guidance Statements Primary Biliary Cholangitis: 2018 Practice Guidance From the AASLD

Guidance Statements: The diagnosis of PBC can be established when two of the following three criteria are met: Biochemical evidence of cholestasis based on ALP elevation. Presence of AMA, or other PBC‐specific auto‐antibodies, including sp100 or gp210, if AMA is negative. Histologic evidence of nonsuppurative destructive cholangitis and destruction of interlobular bile ducts. The diagnosis of AMA‐negative PBC does not require a liver biopsy if other criteria are met, including cholestatic liver tests and PBC‐specific autoantibodies such as sp100 or gp210. Liver biopsy to rule out concomitant AIH or other liver disease should be considered in PBC patients when the alanine aminotransferase activity is more than 5 times the upper limit of normal. In cases of suspected PBC/AIH overlap, treatment should be targeted at the predominant histological pattern of injury. UDCA in a dose of 13 to 15 mg/kg/day orally is recommended for patients with PBC who have abnormal liver enzyme values regardless of histologic stage. For patients requiring bile acid sequestrants, UDCA should be given at least 1 hour before or 4 hours after the bile acid sequestrant. Biochemical response to UDCA should be evaluated at 12 months after treatment initiation to determine whether patients should be considered for second‐line therapy. Patients who are inadequate responders to UDCA (Table 1) should be considered for treatment with OCA, starting at 5 mg/day. Fibrates can be considered as off‐label alternatives for patients with PBC and inadequate response to UDCA. Use of OCA and fibrates is discouraged in patients with decompensated liver disease (Child‐ Pugh‐Turcotte B or C). Anion‐exchange resins should be used as initial therapy for patients with PBC who have pruritus. The following agents can be used for pruritus refractory to anion‐exchange resins: Rifampicin 150 to 300 mg twice daily. Oral opiate antagonists such as naltrexone titrated to a dose of 50 mg daily. Sertraline 75 to 100 mg daily. Management of dry eyes can include the following: Artificial tears should be used initially. Pilocarpine or cevimeline can be used in patients for whom symptoms are refractory to artificial tears. Cyclosporine or lifitegrast ophthalmic emulsion can be used in those whose disease is refractory to other agents, preferably under the supervision of an ophthalmologist. The following therapies should be used for xerostomia and dysphagia: Over‐the‐counter saliva substitutes can be tried. Pilocarpine or cevimeline can be used if patients remain symptomatic despite saliva substitutes. Patients with suspected cirrhosis should undergo endoscopic screening for varices at the time of diagnosis. Regular screening for hepatocellular carcinoma with cross‐sectional imaging at 6‐month intervals is currently advised for men and patients with cirrhosis. Patients with PBC should be provided 1,000 to 1,500 mg of calcium and 1,000 International Units of vitamin D daily in the diet and as supplements if needed. Oral alendronate (70 mg weekly) or other effective bisphosphonates should be considered if patients are osteoporotic. Oral bisphosphonates should be avoided if patients have acid reflux or known varices. Patients with elevated lipid levels and at risk for cardiovascular disease can be considered for lipid‐lowering therapy. Fat‐soluble vitamin deficiencies should be treated with parenteral or water‐soluble supplements. Patients with manifestations of end‐stage PBC should be referred for liver transplantation when their Model for End‐Stage Liver Disease score exceeds 14.

The major changes from the last guideline to this guidance include information about obeticholic acid and the adaptation of the guidance format.

Please listen to Dr. Keith D. Lindor discuss the important updates and impact on patient management from this publication.