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PBC

Podcasts

Beyond the Mic: Exploring the Journeys of Leaders.

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Cholestatic Pruritus Across the Age Spectrum: What Are You Missing?

When considering cholestatic pruritus across the age spectrum, a key point to remember is that while the underlying mechanism is similar, pediatric patients often present with unique challenges in diagnosis and management due to factors like limited communication abilities, potential side effects of medications, and the need for careful dosage adjustments; additionally, older adults might have co-morbidities that complicate treatment and require a more nuanced approach to managing pruritus

Podcasts

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Importance of Understanding Impact on Disease Outcomes: Progression to Fibrosis and Cirrhosis

Thank you Intercept for your support of this podcast episode.

Welcome to the CLDF GHAPP PBC Podcast Series. In this episode, Dr. Kimberly Brown is joined by Dr. Steven Flamm for an in-depth discussion on primary biliary cholangitis (PBC). Together, they explore the unpredictable progression of PBC to fibrosis and cirrhosis, offering real-world insights into how liver disease develops even in patients who may not exhibit symptoms early on.

The conversation emphasizes the importance of early diagnosis, close monitoring, and timely intervention to improve outcomes. Drs. Brown and Flamm dive into non-invasive methods for assessing fibrosis—including elastography and proprietary serum biomarkers—and discuss why simple tools like APRI and FIB-4 are helpful for initial risk stratification but less reliable for monitoring long-term disease progression. They also discuss the clinical significance of rising bilirubin levels in PBC, the limitations of routine imaging in identifying advancing fibrosis, and the importance of identifying secondary causes of liver disease.

This episode also touches on when to consider second-line therapies, how to identify candidates for liver cancer screening, and the role of transplant centers in managing advanced disease. If you’re a clinician treating PBC or other chronic liver conditions, this conversation provides valuable guidance on how to optimize care and improve outcomes across the entire spectrum of liver disease.
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Building a Sense of Urgency for PBC Patient Evaluation/Monitoring

Thank you Intercept for your support of this podcast episode.

In this episode of the CLDF GHAPP PBC Podcast Series, Dr. Kimberly Brown joins Dr. Steven Flamm for an important discussion on building a sense of urgency around evaluating and monitoring patients with Primary Biliary Cholangitis (PBC). Together, they explore how clinical practice has evolved to recognize the variability and unpredictability of PBC progression, emphasizing the importance of early assessment, frequent follow-up, and timely adjustments to therapy. Drawing on new guidance from the CLDF and their own experience at major transplant centers, Drs. Brown and Flamm stress the importance of tracking alkaline phosphatase and bilirubin levels as key indicators of disease activity and risk. They highlight that certain high-risk populations—such as men, patients of color, and younger individuals—require even closer monitoring due to more aggressive disease trajectories. The conversation also addresses the need to reassess patients within three to six months of initiating therapy, the clinical implications of inadequate response to first-line treatment with ursodiol (URSO), and when to consider second-line options. Additionally, the episode explores the intersection of PBC and other liver conditions like MASH, underscoring the importance of a comprehensive approach in complex patients. This discussion, rooted in real-world hepatology practice in the Midwest, offers practical, timely guidance for clinicians nationwide who are striving to deliver optimal care for patients with PBC.
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Need for Earlier Evaluation of UDCA Response, Need for Earlier Consideration of Second-Line Therapy

Thank you Intercept for your support of this podcast episode.

In this episode of the CLDF/GHAPP PBC Podcast Series, Dr. Kimberly Brown, Professor of Medicine at Henry Ford Hospital in Detroit, Michigan, and Dr. Sonal Kumar, Assistant Professor and Director of Clinical Hepatology at Weill Cornell Medicine in New York City, explore how clinical practice is evolving when it comes to earlier intervention and individualized treatment in Primary Biliary Cholangitis (PBC). With second-line therapies now available, the discussion centers on when to move beyond ursodiol (UDCA), how to evaluate response using markers like alkaline phosphatase and bilirubin, and why the traditional 12-month assessment window is shifting earlier to 3 or 6 months. Drs. Brown and Kumar share insights from their respective practices, emphasizing a more proactive and personalized approach to managing PBC patients—especially those with advanced fibrosis at baseline, men, and patients from underrepresented populations who may be at higher risk for progression. They also discuss the importance of treatment normalization, monitoring response trends over time, and using early biochemical signals to determine the need for second-line therapies. This episode provides real-world perspectives from hepatology leaders in two major academic centers, highlighting the importance of shifting away from “wait-and-see” models and toward timely, aggressive care that optimizes long-term outcomes in PBC.
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Webcasts

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Expert Perspectives on Primary Biliary Cholangitis (PBC)

Steven Flamm, MD | Ira Jacobson, MD | Andrea Gossard, APRN, CNP
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PBC New Therapeutic Options Management Strategies

Live from the GHAPP National 2024 Conference, this Primary Biliary Cholangitis (PBC) Symposium explores the latest advancements in diagnosing, treating, and managing PBC, a chronic autoimmune liver disease. Moderated by Christina Hanson, NP, and featuring expert insights from Lucy Mathew, NP (Cedars-Sinai), Lindsay Yoder, PA-C (Indiana University Health), and Anne Moore, NP (Arizona Liver Health), this session provides a comprehensive look at new therapies, disease progression, and symptom management for PBC patients.

The discussion covers early identification and staging of PBC, including the importance of anti-mitochondrial antibody (AMA) testing, alkaline phosphatase (ALP) monitoring, and non-invasive fibrosis assessment. Experts detail the latest PBC treatment guidelines, starting with ursodeoxycholic acid (UDCA) as first-line therapy and moving to second-line options such as obeticholic acid (OCA), fibrates, elafibranor, and seladelpar, which offer promising new strategies for patients with an inadequate response to UDCA.

The session also highlights the management of PBC-associated pruritus, a debilitating symptom affecting up to 80% of patients, discussing bile acid sequestrants, Rifampin, naltrexone, SSRIs, and emerging therapies that improve quality of life. Additionally, the panel addresses bone density screening, autoimmune overlap syndromes, and real-world treatment challenges, emphasizing personalized approaches to PBC care.

Watch now for expert guidance on optimizing PBC management and improving patient outcomes. For more educational resources, visit the GHAPP website or download the GHAPP ACE app.
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Health-Related Quality of Life and Emerging Therapies In PBC

with Paul Kwo, MD, and John Vierling, MD
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Publications

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Expert Perspectives on PBC: Interactive Management Algorithm and Chatbot
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An Up-To-Date Algorithm for the Treatment of PBC

The key features of the algorithm include new guidance-informed suggestions for staging PBC using noninvasive testing (NIT), earlier assessment of lower thresholds to gauge ursodeoxycholic acid (UDCA) response after initiation of therapy, possible earlier initiation of second-line therapy with obeticholic acid (OCA) at lower levels of alkaline phosphatase (ALP) or bilirubin, avoidance of OCA in patients with cirrhosis complicated by portal hypertension or liver decompensation, and the safety and durability of response with long-term OCA therapy and off-label use of fibrates.

The algorithm is intended for use patients:
With a PBC diagnosis confirmed by + antimicrobial antibodies (AMA), + antinuclear antibodies (ANA; sp100 Gp210) or biopsy.
Confirmed PBC that is staged via vibration controlled transient elastography (VCTE) or magnetic resonance elastography (MRE)

All patients that quality, per the criteria above steps, should be started on UDCA 13-15 mg/kg/day for:

12 months with lower stage of fibrosis (VCTE or TE <10 kPa, MRE <4.3 kPa)
6 months with more advanced fibrosis (VCTE or TE ≥ 10kPa, compensated liver disease and no signs of portal hypertension)

Following this treatment, patients should be monitored for response to therapy and UDCA intolerance by following the steps in the algorithm.

Patients with PBC with an inadequate response to or intolerance of UDCA should be considered for second-line therapy with OCA. Second-line therapy consists of OCA, as reviewed in detail above, or off-label bezafibrate or fenofibrate in patients with an inadequate response and if there are no signs of decompensated liver disease or clinically significant portal hypertension.

OCA
OCA therapy should not be used in patients with thrombocytopenia (i.e., in those with a platelet count < 120 x 109/L, ascites, esophageal varices, or hepatic encephalopathy), complications of cirrhosis (i.e., spontaneous bacterial peritonitis, hepatic encephalopathy.

Abstract Library

Unlocking Boundless Knowledge Through Abstracts

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Comment on "Risk of colon polyps and colorectal cancer in primary biliary cholangitis, a population-based retrospective cohort study in Taiwan".

Jin MeiLing (M)

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Reply to "Comment on 'Risk of colon polyps and colorectal cancer in primary biliary cholangitis, a population-based retrospective cohort study in Taiwan'".

Chen Pei-Tzu (PT);Chen Hsuan-Wei (HW)

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Autoimmune liver diseases in Latin America: Current landscape and challenges.

Couto Cláudia Alves (CA);Villamil Alejandra (A);Terrabuio Debora Raquel Benedita (DRB);Tejada Sofia (S);Castro-Narro Graciela (G);Ridruejo Ezequiel (E); ()