The European Association for the Study of the Liver (EASL) has issued clinical practice guidelines on the diagnosis and management of patients with primary biliary cholangitis. The new guidelines have been published online in the Journal of Hepatology and will be presented at the International Liver Congress 2017 in Amsterdam, The Netherlands.
“The EASL Primary Biliary Cholangitis guidelines are meant to support clinicians in establishing a long-term commitment with patients and their disease, providing indications on how to stratify the risk from diagnosis to disease progression. The guidelines will also help identify which patients require second line treatment, in which advances are fortunately being made,” said Prof Marco Marzioni, Professor of Gastroenterology, Università Politecnica delle Marche – “Ospedali Riuniti” University Hospital of Ancona, Italy and one of the authors of the guidelines.
Primary biliary cholangitis, an uncommon disease which often affects women, is regarded as a chronic inflammatory autoimmune cholestatic liver disease. The goal of treatment for these patients is to reduce disease-related symptoms which may affect a patient’s quality of life, as the disease often progresses to the point of end-stage liver disease.
To slow disease progression, pharmacological agents such as ursodeoxycholic acid and obeticholic acid may be used. EASL recommends the use of oralursodeoxycholic acid at 13—15mg/kg/day as first-line treatment for patients with primary biliary cholangitis; treatment is usually continued for life (Strong recommendation based on randomized controlled trials). With regards to obeticholic acid, a farnesoid X receptor agonist, EASL suggests it be used in patients who have had an inadequate response to ursodeoxycholic acid, or as monotherapy in patients intolerant to ursodeoxycholic cid. (Weaker recommendation based on randomized controlled trials). In addition, fibric acid derivatives and budesonide are considered potential, off-label therapies. Currently, data regarding their use have not yet been published, therefore a recommendation by EASL cannot be made.
Pruritus, sicca complex, and fatigue are classic symptoms associated with primary biliary cholangitis. In addition, symptoms such as bone and joint pain, abdominal pain, and restless leg syndrome have also been reported by patients. The new guidelines provide information on the management of these symptoms as they can significantly impair a patient’s quality of life.
The entire clinical practice guideline can be found here.
For more information visit journal-of-hepatology.eu.