Considerations for
This website is for educational purposes only and provides a non-exhaustive description of potential care options in PBC based on peer-reviewed literature. HCPs should exercise their independent clinical judgment when selecting care options for a particular patient. This educational material does not promote the use of any particular care option.
Get startedThis educational resource was developed and is provided by Intercept Pharmaceuticals, Inc.
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Considerations for
This website is interactive. For information tailored to your practice, please select relevant criteria on as many or as few pages as you would like. The content provided is based on information from approved product prescribing information, published guidelines, and peer-reviewed literature.
If requested, this material is available through a Medical Information Request Form.
This educational resource was developed and is provided by Intercept Pharmaceuticals, Inc.
us-PB-MED-00921 08/2023
Pathway overview
Suspected PBC
Elevated ALP AND
AMA Positive
No
Additional Testing Differential DiagnosisContinue
Treatment
Yes
Adequate Response?
No
Continue
Treatment
Yes
Adequate Response?
No
This educational resource was developed and is provided by Intercept Pharmaceuticals, Inc.
us-PB-MED-00921 08/2023
For patients with high risk,
refer to a hepatologist or
transplant center.
Adequate
Response?
No
Adequate
Response?
Continue Treatment
Yes
Continue Treatment
Yes
No
Pre-diagnosis
diagnosis
1ST-LINE TREATMENT
2nd-line treatment
Patient with suspected PBC:
Select all that apply.
Laboratory testing for consideration
The following testing is not all required routinely (or in all patients) for diagnosing PBC, but may be used in AMA-negative cases and at initial assessment to rule out other causes for symptoms and/or achieve baseline measurements.
Select all that apply.
*Key considerations for PBC diagnosis and management, not comprehensive of all measures included in laboratory testing.
Liver biopsies are rarely needed to diagnose PBC but may be recommended in AMA-negative patients with the absence of PBC-specific autoantibodies, or to rule out concomitant AIH, NASH or other liver diseases.1-3
Evidence of portal hypertension include ascites, gastroesophageal varices, or persistent thrombocytopenia.9
Noninvasive imaging tests (TE, MRE) can identify patients with advanced fibrosis and at risk of hepatic decompensation.10
LSM by VCTE, in combination with established biochemical criteria of therapeutic response or prognostic score, improves outcome prediction in PBC.11
Transient elastography
Transient elastography is not necessarily needed for diagnosis of PBC but can serve as a baseline to assess the degree of fibrosis, even in the absence of cirrhosis, in patients with PBC.2,3
The FIB-4 and APRI
FIB-4 and APRI are not directly linked to diagnosing a patient, but more so to establish the fibrosis assessment and prediction of prognosis in PBC once the diagnosis of PBC has been established.9
ULN = Upper limit of normal.
*Reference intervals may vary based on lab/institution. Values provided are per LabCorp. LabCorp does not separate reference intervals by age group and sex for most adult tests.**Albumin reference ranges vary by age and sex. Reported values are for ages 31 to 50 years.
The diagnosis of PBC should be suspected in adult patients with chronic liver test abnormalities, after exclusion of other causes of liver disease,1,3 including, but not limited to:
Select all that apply.
Note: AMA positivity alone is not sufficient to make the diagnosis of PBC.1-3 Guidelines recommend following up with these patients every 6-12 months with repeat liver enzyme panels.2
The diagnosis of PBC should be suspected in adult patients with chronic cholestasis after exclusion of other causes of liver disease.1,3 While the available guidelines differ slightly in recommendations for patients with suspected PBC who are AMA-negative, the guidelines listed below agree that a PBC diagnosis can be established in patients with1,3:
*AMA positive = immunofluorescent assay titer of >1:40 or EIA >25 units.2,3
Note: AMA positivity alone is not sufficient to make the diagnosis of PBC.1-3 Guidelines recommend following up with these patients every 6-12 months with repeat liver enzyme panels.2
Additional scenarios and considerations for diagnosing PBC based on available guidelines:
Select all that apply.
The treatment goals of PBC are aimed to prevent progression to advanced liver disease and to alleviate symptoms.1-4,12
Select all that apply.
Assessing baseline risk ensures patients receive timely follow-up and allows for a personalized treatment approach.
Pre-treatment risk assessment:
Select all that apply.
LOW RISK
EMERGING DATA
Intermediate-high risk
Consider referral to a hepatologist or transplant center for further assessment
Note: Emerging data from peer-reviewed, primary research literature; not incorporated in guidelines.
Initiate 1st-line treatment
Select all that apply.
Per discretion of provider:
See warnings and precautions for UDCA. →
Continue to monitoring
Routinely monitor for biochemical response, tolerability, and progression of PBC14
Select all that apply.
Per discretion of provider:
Binary criteria and definition of response1,2
Risk scoring systems1-3
Select all that apply.
See continuous scoring calculators. →
Routine follow-up assessments should look for1-3,14
Select all that apply.
Per discretion of provider:
Discontinuing treatment should be considered in patients with increased GGT, ALP, AST, ALT, bilirubin.14
Note: The content of this page is based on information from UDCA PI14 and published guidelines.1-3
Fibrosis stage (as assessed by VCTE or MRE) can guide the decision of whether response to UDCA and subsequent second-line treatment need should be assessed at 6 months or 12 months.10
Contraindicated in patients with14:
Patients with the following should receive appropriate specific treatment14:
Combining LSM by VCTE with prognostic scores, such as GLOBE score, provides more accurate risk assessment than prognostic scores alone.11
If there is intolerance or no response, evaluate for or initiate 2nd-line treatment.
To report suspected adverse reactions, contact Intercept Pharmaceuticals, Inc. or the FDA:
Routine follow-up assessments should look for1-3,14
Select all that apply.
All chronic liver disease patients2
Select all that apply.
Laboratory testing for consideration when conducting ongoing monitoring and assessments1-6
Select all that apply.
*Key considerations for PBC diagnosis and management, not comprehensive of all measures included in laboratory testing.
If advanced liver disease is present, evaluate for liver transplantation.2
Evidence of portal hypertension includes ascites, gastroesophageal varices or persistent thrombocytopenia.9
UK-PBC Score 1-3,30
The UK-PBC Risk Score is used for patients with PBC at any stage to estimate the risk, as a percentage, that the patient will experience liver transplantation and liver-related death. Calculation is based on patient’s total bilirubin, ALT or AST, and ALP after 12 months of therapy; albumin and platelets.1-3,30
Assess your patient’s UK-PBC Score →
GLOBE Score 1-3,31
Calculation is based on patient’s age, total bilirubin, ALP, albumin, platelets.1-3,31
Assess your patient’s GLOBE Score →
MAYO Score2,3,32
Calculation is based on patient’s age, bilirubin, albumin, prothrombin time, peripheral edema status, diuretic therapy usage.2,3,32
Combining LSM by VCTE with prognostic scores, such as GLOBE score, provides more accurate risk assessment than prognostic scores alone.11
ACG/CLDF guidelines recommend calculating the Mayo score for all PBC patients.2
Despite treatment with UDCA, PBC can remain a progressive disease and has a risk of liver-related complications and death. The risk of developing end-stage complications and potential need for additional treatments should be assessed in all patients.3
Assess risk of progression based on response to treatment1,2,4,10,13
Select all that apply.
LOW RISK4/ADEQUATE RESPONSE TO UDCA
EMERGING DATA
INTERMEDIATE-HIGH RISK4/INTOLERANCE OR INADEQUATE RESPONSE TO UDCA1 (based on established criteria)
OR ALP:
OR BILIRUBIN:
OR ALBUMIN
EMERGING DATA
CONSIDER REFERRAL FOR FURTHER ASSESSMENT4
Note: Emerging data from peer-reviewed, primary research literature; not incorporated in guidelines.
For low-risk patients, continue 1st-line treatment and assess response every 3-6 months.1
Combining LSM by VCTE with prognostic scores, such as GLOBE score, provides more accurate risk assessment than prognostic scores alone.11
For intermediate- to high-risk patients, evaluate risk-benefit of continuing to 2nd-line treatment or referring to a hepatologist or transplant center for further assessment.4
Options for 2nd-line treatment10
Select all that apply.
Initiate 2nd-line treatment1-3
Select all that apply.
See boxed warning for obeticholic acid. →
Continue to monitoring
Routinely monitor for biochemical response, tolerability, and progression of PBC16
Select all that apply.
Per discretion of provider:
Closely monitor patients with16:
Select all that apply.
For:
If intolerance occurs
If there is intolerance due to pruritus,16 consider:
Select all that apply.
Discontinue treatment in patients who16:
Refer these patients to a hepatologist or transplant center.
Note: The content of this page is based on information from obeticholic acid PI16 and published guidelines.1-3
Obeticholic acid is contraindicated in patients with16:
Fibrates are discouraged in patients with decompensated liver disease.17
Fenofibrate is contraindicated in patients with17:
If fibrates are used, the following monitoring should be considered17:
To report suspected adverse reactions, contact Intercept Pharmaceuticals, Inc. or the FDA:
Strategies to manage pruritus in obeticholic acid-treated patients
Select all that apply.
Topical treatments and lifestyle interventions
Prescription medications
Other therapies*
*These physical interventions are available as salvage therapies.
If steps to manage pruritus are not successful16:
Select all that apply.
WARNING: HEPATIC DECOMPENSATION AND FAILURE IN PRIMARY BILIARY CHOLANGITIS PATIENTS WITH CIRRHOSIS16
See full prescribing information → for complete boxed warning.
Indication
Contraindications
OCALIVA is contraindicated in patients with:
Warnings and Precautions
Hepatic Decompensation and Failure in PBC Patients with Cirrhosis
Severe Pruritus
Reduction in HDL-C
Adverse Reactions
Drug Interactions
Bile Acid Binding Resins
Warfarin
CYP1A2 Substrates with Narrow Therapeutic Index
Inhibitors of Bile Salt Efflux Pump
View full prescribing information, including Boxed Warning. →
To report suspected adverse reactions, contact Intercept Pharmaceuticals, Inc. or the FDA:
Patients with cirrhosis (even if not advanced) on obeticholic acid should be carefully monitored for evidence of liver decompensation or portal hypertension.9
Routine follow-up assessments should look for1-3,6
Select all that apply.
All chronic liver disease patients2
Select all that apply.
Laboratory testing for consideration when conducting ongoing monitoring and assessments1-6
Select all that apply.
*Key considerations for PBC diagnosis and management, not comprehensive of all measures included in laboratory testing.
If advanced liver disease is present, evaluate for liver transplantation.2
Evidence of portal hypertension includes ascites, gastroesophageal varices or persistent thrombocytopenia.9
For patients who do not respond to obeticholic acid after 1 year at the highest recommended dosage that can be tolerated (maximum of 10 mg once daily), and who experience a reduction in HDL-C, weigh the potential risks against the benefits of continuing treatment.16
This website is for educational purposes only and provides a non-exhaustive description of potential care options in PBC based on peer-reviewed literature. HCPs should exercise their independent clinical judgment when selecting care options for a particular patient. This educational material does not promote the use of any particular care option.