From the Research
Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by the presence of various autoantibodies, including anti-centromere protein B (CENP-B) antibodies, which are found in approximately 10-30% of PBC patients and are associated with a distinct clinical subtype of PBC. The relationship between PBC and Centromere Protein B (CENP-B) is complex, with anti-CENP-B antibodies being associated with a slower progression of liver disease but more pronounced features of limited systemic sclerosis (scleroderma) in some patients 1.
Key Points
- The standard treatment for PBC remains ursodeoxycholic acid (UDCA) at a dose of 13-15 mg/kg/day, regardless of CENP-B status.
- For patients who have an inadequate response to UDCA after 12 months, obeticholic acid (5-10 mg daily) can be added, as shown in recent studies 2, 3.
- Regular monitoring of liver function tests every 3-6 months is recommended, along with annual assessment for portal hypertension and osteoporosis.
- The presence of anti-CENP-B antibodies should prompt clinicians to evaluate for features of scleroderma and consider appropriate management of these extrahepatic manifestations.
Treatment Considerations
- The use of UDCA in PBC delays histological progression and extends the transplant-free survival, but 40% of PBC patients do not respond adequately to UDCA and are at high risk for serious complications 4, 5.
- Obeticholic acid (OCA) has shown promising results in PBC patients with inadequate response to UDCA, with significant reduction of serum alkaline phosphatase (ALP) and improvement of cholestasis 3.
- Other alternative therapies, such as bezafibrate and budesonide, are currently being examined in PBC patients, but more research is needed to determine their efficacy and safety 1.