MapPBC | Official Site

primary biliary cholangitis diagnosis map

SPOT the signs

PBC presentation and progression are unique to each patient

Some patients may be asymptomatic or present with one or a combination of the following manifestations throughout the course of the disease^1,2:

Up to

60% of patients

do not exhibit symptoms
at diagnosis.³

symptom infographic of fatigue, brain fog, pruritus, sicca complex, abdominal pain, bone and joint ache

If left unmanaged, PBC will progress and can lead to cirrhosis, transplant, and even death.⁵

Disease progression in PBC⁵

Inflammation—caused by damage to the small bile ducts

Cholestasis–chronic inflammation leads to impaired synthesis and transport of bile acids

Fibrosis–chronic cholestasis leads to fibrosis and cirrhosis

Cirrhosis–a permanently damaged cirrhotic liver may require transplant

Inflammation

Caused by damage to
the small bile ducts

Cholestasis

Chronic inflammation leads to impaired synthesis and transport of bile acids

Fibrosis

Chronic cholestasis leads
to fibrosis and cirrhosis

Cirrhosis

A permanently damaged
cirrhotic liver may
require a transplant

If left unmanaged, PBC will progress and can lead to cirrhosis, transplant, and even death.⁵

Disease progression in PBC⁵

Inflammation, cholestasis, fibrosis, cirrhosis

In a study of patients with PBC, the majority of whom were on UDCA, 46% of patients with biochemically early-stage PBC progressed to a moderately advanced stage within 5 years.⁶

MAP the route

Consistent monitoring is key to a proactive treatment plan

All PBC patients need frequent assessment of their treatment response and disease progression.^5,7

Biochemical response to treatment is the key prognostic measure used to assess the risk and speed of disease progression.^8-11

In order to support your patients along their treatment journey, consider the following:

EVALUATE

at 6 months

Consider evaluating response to treatment every 6 months, at which time changes can be implemented to help slow disease progression.¹²

MONITOR

ALP levels

Elevated ALP levels are associated with disease progression and can be measured to assess treatment response.^4,11,13

CONSIDER

side eﬀects

Some treatments for PBC may exacerbate existing symptoms or introduce new symptoms.⁵

Discussing the impact of disease symptoms and the impact of side effects can help in selecting a treatment that supports a patient’s treatment goals

Recognize patients with inadequate treatment response.

Inadequate biochemical response

ALP levels above 1.67 x ULN are correlated with higher risk of negative outcomes, including liver transplant and death.¹²

Rapid and sustained reduction in ALP levels and maintaining normal bilirubin levels are goals of PBC treatment⁴

Patients who respond to treatment may still be at risk of disease progression

Over time, patients who respond to first-line treatment may start to see rising ALP or bilirubin levels.⁶

Regular follow-ups and monitoring of PBC patients can help to prevent disease progression⁵

Implementing a disease management plan immediately may help to mitigate further disease progression.¹³

Inadequate treatment response can lead to cirrhosis, liver transplant, or death. A proactive treatment plan consisting of personalized, collaborative treatment and closer monitoring may be beneficial for some patients.^5,7

Up to 50% of patients treated with first-line treatment do not adequately respond, putting those patients at risk of cirrhosis and death.^9,13

Up to 50% of patients
treated with first-line treatment
do not adequately respond,
putting those patients at risk of
cirrhosis and death.^9,13

LEAD the way

Consider patients who may be at higher risk

Certain patients may be at risk of disease progression, inadequate treatment response, and poorer outcomes.⁵

Risk factors include:

Age: Patients diagnosed with PBC before age 45 are at risk of severe disease with a poorer response to treatment, putting them at higher risk of liver transplant or death.^5,13,14

Sex: Males with PBC are at a higher risk of disease progression and poorer outcomes largely due to delayed diagnosis. 47% of men with PBC are at moderate or advanced disease stage when UDCA treatment is initiated.^5,15

Ethnicity/Race: Hispanic ethnicity is a predictor of poor response to UDCA.¹⁶ Black patients have a 47% increased risk of death compared to White patients.¹⁷

High-risk patient types require a personalized treatment plan and, in some cases, earlier consideration of additional treatment.¹⁸

Effectively identify patients that need reassessment

Frequent monitoring based on evolving guidance can help identify signs of progression and is an important part of a proactive treatment plan.⁴

Routine Monitoring

A 3-month routine monitoring cadence is advised based on AASLD guidelines.⁴

An EHR or EMR database can be used to identify patients who may need evaluation or who may require adjustments to their routine monitoring schedule⁵
Contacting patients for follow-up may help to engage patients who have not been assessed in the previous 12 months

ICD-10 codes

Patients who require a PBC treatment plan may fall under a variety of ICD-10 codes.^5,19 When reviewing patient records, consider:

K74.3 (PBC)
R74.8 (elevated ALP)
K74.5 (biliary cirrhosis)
K8 (cholangitis)
L.29.9 (pruritus)

Help identify PBC patients in your practice

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AASLD=American Association for the Study of Liver Diseases; ALP=alkaline phosphatase; EHR=electronic health record; EMR=electronic medical record; PBC=primary biliary cholangitis; UDCA=ursodeoxycholic acid; ULN=upper limit of normal.

References: 1. Younossi ZM, Bernstein D, Shiﬀman ML, et al. Diagnosis and management of primary biliary cholangitis. Am J Gastroenterol. 2019;114(1):48-63. 2. Rice S, Albani V, Minos D, et al; UK-PBC Consortium. Eﬀects of primary biliary cholangitis on quality of life and health care costs in the United Kingdom. Clin Gastroenterol Hepatol. 2021;19(4):768-776.e10. 3. Prince MI, Chetwynd A, Craig WL, et al. Asymptomatic primary biliary cirrhosis: clinical features, prognosis, and symptom progression in a large population-based cohort. Gut. 2004;53(6):865-870. 4. Lindor KD, Bowlus CL, Boyer J, et al. Primary biliary cholangitis: 2018 practice guidance from the American Association for the Study of Liver Diseases. Hepatology. 2019;69(1):394-419. 5. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: the diagnosis and management of patients with primary biliary cholangitis. J Hepatol. 2017;67(1):145-172. 6. Gatselis NK, Goet JC, Zachou K, et al; Global Primary Biliary Cholangitis Study Group. Factors associated with progression and outcomes of early stage primary biliary cholangitis. Clin Gastroenterol Hepatol. 2020;18(3):684-692. 7. Hirschfield GM, Dyson JK, Alexander GJM, et al. The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines. Gut. 2018;67(9):1568-1594. 8. Corpechot C, Heurgue A, Tanne F, et al. Non-invasive diagnosis and follow-up of primary biliary cholangitis. Clin Res Hepatol Gastroenterol. 2022;46(1):101770. 9. Montano-Loza AJ, Corpechot C. Definition and management of patients with primary biliary cholangitis and an incomplete response to therapy. Clin Gastroenterol Hepatol. 2021;19(11):2241-2251.e1. 10. Samur S, Klebanoﬀ M, Banken R, et al. Long-term clinical impact and cost-eﬀectiveness of obeticholic acid for the treatment of primary biliary cholangitis. Hepatology. 2017;65(3):920-928. 11. Lammers WJ, Kowdley KV, van Buuren HR. Predicting outcome in primary biliary cirrhosis. Ann Hepatol. 2014;13(4):316-326. 12. Kowdley KV, Bowlus CL, Levy C, et al. Application of the latest advances in evidence-based medicine in primary biliary cholangitis. Am J Gastroenterol. 2023;118(2):232-242. 13. Hirschfield GM, Chazouillères O, Cortez-Pinto H, et al. A consensus integrated care pathway for patients with primary biliary cholangitis: a guideline-based approach to clinical care of patients. Expert Rev Gastroenterol Hepatol. 2021;15(8):929-939. 14. Carbone M, Mells GF, Pells G, et al. Sex and age are determinants of the clinical phenotype of primary biliary cirrhosis and response to ursodeoxycholic acid. Gastroenterology. 2013;144(3):560–569.e7. 15. Cheung AC, Lammers WJ, Murillo Perez CF, et al; Global PBC Study Group. Eﬀects of age and sex of response to ursodeoxycholic acid and transplant-free survival in patients with primary biliary cholangitis. Clin Gastroenterol Hepatol. 2019;17(10):2076-2084.e2. 16. Levy C, Naik J, Giordano C, et al. Hispanics with primary biliary cirrhosis are more likely to have features of autoimmune hepatitis and reduced response to ursodeoxycholic acid than non-Hispanics. Clin Gastroenterol Hepatol. 2014;12(8):1398-1405. 17. Adejumo AC, Akhtar DH, Dennis BB, et al. Gender and racial diﬀerences in hospitalizations for primary biliary cholangitis in the USA. Dig Dis Sci. 2021;66(5):1461-1476. 18. Levy C, Manns M, Hirschfield G. New treatment paradigms in primary biliary cholangitis. Clin Gastroenterol Hepatol. 2023;21(8):2076-2087. 19. 2024 ICD-10-CM codes. ICD10Data.com. Accessed February 27, 2024. https://www.icd10data.com/ICD10CM/Codes