From the Guidelines
The primary management of cholestasis involves treating the underlying cause and providing symptomatic relief, with bezafibrate being the recommended first-line pharmacological treatment for moderate to severe pruritus in sclerosing cholangitis and other forms of fibrosing cholangiopathy, as per the most recent guidelines 1. Here's a concise approach:
- Identify and treat the underlying cause (e.g., remove obstructing gallstones, treat viral hepatitis, discontinue offending medications).
- For symptomatic relief, especially pruritus:
- First-line: Bezafibrate, as it has shown a clear-cut benefit in alleviating moderate to severe itch in people with PSC and PBC treated with UDCA 1.
- Second-line: Rifampicin 150-300 mg daily, which may induce drug-induced hepatitis after 4-12 weeks in up to 12% of cholestatic patients 1.
- Third-line: Naltrexone, starting at very low doses (12.5 mg) to avoid early side effects resembling an opioid withdrawal syndrome 1.
- Manage complications:
- Fat-soluble vitamin supplementation (A, D, E, K)
- Calcium and vitamin D for bone health
- Medium-chain triglyceride supplements for nutrition
- For intrahepatic cholestasis of pregnancy:
- Ursodeoxycholic acid 10-15 mg/kg/day in 2-3 divided doses, as it improves bile flow and has anti-inflammatory properties 1.
- Monitor liver function tests and bilirubin levels regularly
- Consider liver transplantation for end-stage liver disease Bezafibrate works by exerting strong additive anticholestatic effects in PSC and PBC, and its antipruritic effect has been described as sustained under cholestatic conditions 1. Addressing the underlying cause is crucial for long-term management, and symptomatic treatments provide relief while the primary condition is being addressed or in cases where the cause cannot be immediately corrected.
From the FDA Drug Label
In patients with partial biliary obstruction, the reduction of serum bile acid levels by cholestyramine resin reduces excess bile acids deposited in the dermal tissue with resultant decrease in pruritus
The management of cholestasis may involve the use of cholestyramine to reduce serum bile acid levels, which can help decrease pruritus in patients with partial biliary obstruction 2.
From the Research
Management of Cholestasis
The management of cholestasis involves addressing both the cause and the consequences of retained bile acids within the liver, and diminished delivery of bile to the gastrointestinal tract 3. The treatment should focus on:
- Addressing symptoms, mostly pruritus and prevention, particularly osteoporosis and osteomalacia 3, 4
- Managing complications such as portal hypertension, which can be an early event in chronic cholestatic liver disease 3
- Using therapies such as ursodeoxycholic acid, which improves the biochemical markers of cholestasis regardless of cause and may delay liver disease progression 3, 5
- Considering liver transplantation, which is potentially curative 3, 4
Diagnostic Approach
The diagnostic approach to cholestatic liver disease involves:
- Performing liver ultrasound to ascertain if cholestasis is extrahepatic or intrahepatic 4
- Using endoscopic retrograde cholangio-pancreatography (ERCP) or trans-hepatic cholangiography (THC) if the probability of interventional treatment is high 4
- Performing cholangio-MRI if the probability of interventional therapeutics is low 4
- Considering specific clinical situations, such as drug-induced cholestatic disease in the elderly, or cholestatic viral hepatitis in young people with risk factors 4
Treatment of Complications
The treatment of complications of cholestasis includes:
- Managing pruritus, osteopenia, and correcting fat-soluble vitamin deficiencies 4
- Considering liver transplantation when hepatocellular failure or portal hypertension-related complications occur 4
- Using novel anticholestatic therapeutic options, such as obeticholic acid, for patients not adequately responding to ursodeoxycholic acid 5