From the Guidelines
The primary treatment for an acute flare-up of Primary Biliary Cholangitis (PBC) involves ursodeoxycholic acid (UDCA) at a dose of 13-15 mg/kg/day, which should be continued long-term. This recommendation is based on the most recent and highest quality study available, which emphasizes the importance of UDCA in managing PBC 1. The use of UDCA has been consistently shown to improve liver function, reduce symptoms, and potentially delay disease progression 1.
Key Considerations
- For acute symptom management, cholestyramine (4 g before meals) can help control pruritus, while antihistamines like hydroxyzine (25 mg every 6-8 hours) may provide additional relief.
- If the flare is severe with significant elevation in liver enzymes, a short course of prednisolone (20-30 mg daily with gradual taper over 2-4 weeks) might be considered.
- For patients not responding to UDCA, obeticholic acid (5-10 mg daily) may be added as second-line therapy, although its use during pregnancy or lactation is not recommended due to lack of safety data 1.
- Supportive care includes maintaining hydration, monitoring for complications like portal hypertension, and addressing nutritional deficiencies with fat-soluble vitamin supplementation.
Mechanism and Rationale
UDCA works by reducing toxic bile acids, protecting hepatocytes, and improving bile flow 1. This mechanism is crucial in managing the autoimmune process underlying PBC. Regular liver function tests should be performed to monitor treatment response, with follow-up every 3-6 months during and after the flare.
Additional Recommendations
- Vitamin K deficiency related to cholestasis should be corrected 1.
- Imaging with ultrasound or magnetic resonance cholangiopancreaticography may be recommended to exclude obstruction by gallstones or progress of high-grade strictures 1.
From the Research
Treatment for Primary Biliary Cholangitis (PBC) Acute Flare-up
- The primary treatment for PBC is ursodeoxycholic acid (UDCA) 2, 3, 4, 5.
- For patients with inadequate response to UDCA, second-line treatments such as obeticholic acid (OCA) 2, 3, 4, 5, bezafibrate 2, 4, 5, and budesonide 2, 4 may be considered.
- The goal of therapy is to normalize ALP and bilirubin levels below 0.6 the upper limit of normal 2.
- Triple therapy with UDCA, OCA, and bezafibrate may be considered in patients showing inadequate response to dual therapy 2.
- Management of side effects such as pruritus and fatigue is also important, with options including cholestyramine for pruritus and lifestyle modifications for fatigue 3, 4.
Second-line Treatment Options
- OCA has been shown to improve surrogate markers of prognosis in PBC, but may cause pruritus as a side effect 2, 3, 5.
- Bezafibrate has been shown to improve surrogate endpoints, pruritus, and clinical outcome compared to UDCA monotherapy, but may cause elevated serum creatinine 2, 5.
- Budesonide may be considered in patients with marked portal inflammation 2, 4.