Monitoring

Ursodiol should be continued indefinitely. Both US and European guidelines recommend assessing biochemical response to treatment at 12 months to risk stratify patients. Low risk patients (ursodiol responders) should then have individualized follow up according to symptom burden and disease stage. High risk patients (those who display an inadequate response to treatment) should be considered for second line treatments.[14][15]

Response to treatment can be assessed using quantitative (GLOBE and UK-PBC risks scores) or qualitative (Paris-I, Paris-II, Rotterdam, Toronto, Rochester, Ehime criteria) scoring systems.[30]​ The chosen tool should include ALP and bilirubin measurements as these are the two strongest variables used to predict prognosis.[15]

Liver stiffness measurement (LSM) by transient elastography (TE) has recently emerged as an important technique to assess prognosis and treatment response. US guidelines mention it as an emerging technique, whereas European guidelines define a clear role for it, both in terms of risk stratification at baseline and during follow up on treatment.[14][15] There is currently a lack of evidence regarding the optimal time frame between subsequent LSMs, but the current suggestion is to repeat LSM every 2 years in patients with early disease and annually in patients with advanced disease.[15]

Regular screening for hepatocellular carcinoma with ultrasound +/- alpha-fetoprotein is recommended at 6 monthly intervals in patients with cirrhosis and male patients.[14][15]

US guidelines also recommend monitoring of liver tests every 3-6 months, bone mineral densitometry every 2 years, annual TSH, annual monitoring of fat soluble vitamins in patients with jaundice, and upper endoscopy every 1-3 years if cirrhotic, Mayo risk score >4.1, or transient elastography shows a score ≥17 kPa.[14]

Deterioration in liver synthetic function (elevation in bilirubin concentration and prothrombin time and fall in albumin concentration) is an indicator of progressive disease or cirrhosis and should prompt further investigation.[27]

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