From the Guidelines
The treatment for a primary biliary cholangitis (PBC) acute flare-up primarily involves optimizing ursodeoxycholic acid (UDCA) therapy, typically at a dose of 13-15 mg/kg/day, as recommended by the EASL clinical practice guidelines 1. When managing a PBC acute flare-up, it is essential to assess medication adherence and potentially adjust the UDCA dosing. The guidelines suggest that patients with PBC, including those with asymptomatic disease, should be treated with UDCA on a long-term basis 1. Key considerations in managing a flare include:
- Optimizing UDCA therapy to reduce toxic bile acids, protect hepatocytes, and improve bile flow
- Assessing and adjusting medication adherence
- Considering a short course of corticosteroids for severe symptoms
- Managing symptoms such as pruritus with cholestyramine and addressing fatigue with supportive care
- Avoiding hepatotoxic medications and alcohol during flares
- Monitoring liver function tests regularly to assess treatment response, typically every 2-4 weeks until improvement is noted 1. In cases of severe flares with signs of decompensation, hospitalization is necessary. The goal of treatment is to improve symptoms, reduce inflammation, and prevent disease progression, ultimately enhancing the patient's quality of life during the flare.
From the FDA Drug Label
OCALIVA can cause serious side effects, including: Worsening of liver problems or liver failure, in some cases leading to liver transplant or death, has happened in people with primary biliary cholangitis (PBC) with liver cirrhosis when taking OCALIVA provider right away if you have any of the following symptoms of worsening liver problems during treatment with OCALIVA: swelling of your stomach-area from a build-up of fluid yellowing of your skin or the whites of your eyes black, tarry, or bloody stools coughing up or vomiting blood, or your vomit looks like "coffee grounds" mental changes such as confusion, sleepier than usual or harder to wake up, slurred speech, mood swings, or changes in personality
The FDA drug label does not provide a specific treatment for a primary biliary cholangitis (PBC) acute flare-up. It does provide information on the management of symptoms and side effects, but it does not directly address the treatment of an acute flare-up.
From the Research
Treatment for Primary Biliary Cholangitis (PBC) Acute Flare-up
- The primary treatment for PBC is ursodeoxycholic acid (UDCA) at a dose of 13-15 mg/kg per day 2, 3.
- For patients with an inadequate response to UDCA, second-line treatments such as obeticholic acid (OCA) and fibrates may be considered 4, 5, 3.
- OCA has been shown to improve surrogate markers of prognosis in PBC, but may cause pruritus as a side effect 5.
- Bezafibrate is another option for patients with inadequate response to UDCA, and may improve surrogate endpoints, pruritus, and clinical outcome 5.
- Budesonide may be considered in patients with marked portal inflammation 5.
- Triple therapy with UDCA, OCA, and bezafibrate may be considered in patients showing inadequate response to dual therapy 5.
- Management of symptoms and complications, including pruritus, sicca syndrome, and osteoporosis, is also important 2, 3.
Treatment Targets and Therapeutic Options
- Treatment targets for PBC include modifying bile, inflammation, cell survival, and fibrosis 6.
- Potential therapeutic options include targeting cytokines, immune checkpoints, and anti-mitochondrial antibodies, as well as stem cell therapy and lymphocyte trafficking inhibition 6.
- Anti-fibrotic therapies may also be useful for preventing progression of PBC 6.
- Nuclear hormone receptors, such as farnesoid X receptor (FXR) and peroxisome proliferator-activated receptor (PPAR), may be potential therapeutic targets in PBC 6.