Cholestasis
Pruritus management, PBC treatment, vitamin supplementation and NICE NG133 guidance
Cholestasis — impaired bile flow — presents as jaundice, pruritus, pale stools, and dark urine. It may be intrahepatic (hepatocellular or cholestatic liver disease such as PBC or PSC) or extrahepatic (biliary obstruction from gallstones or malignancy). Management targets both the underlying cause and the distressing symptom of pruritus.
Causes and Investigation
A cholestatic LFT pattern (predominantly raised ALP and GGT with relatively preserved ALT) points towards biliary rather than hepatocellular disease. Ultrasound is the first investigation — a dilated common bile duct (>8 mm) indicates extrahepatic obstruction. Anti-mitochondrial antibody (AMA) is positive in 95% of primary biliary cholangitis (PBC). Drug-induced cholestasis is common — co-amoxiclav and flucloxacillin are frequent culprits. Painless progressive jaundice with weight loss in an older patient must raise immediate suspicion of pancreatic or biliary malignancy. Intrahepatic cholestasis of pregnancy presents with pruritus in the third trimester and carries a risk of fetal compromise.
Managing Pruritus
Cholestatic pruritus can be severely debilitating. Colestyramine 4 g once to four times daily is first-line — a bile acid sequestrant that must be taken separately from other medications as it reduces their absorption. If ineffective, rifampicin 150–300 mg twice daily is second-line (monitor LFTs monthly). Naltrexone 50 mg once daily acts as an opioid antagonist to reduce pruritus — start at 12.5 mg to avoid an opioid withdrawal-like reaction. Sertraline 75–100 mg has evidence specifically in PBC. Antihistamines have limited efficacy for cholestatic pruritus but may help with sleep.
Primary Biliary Cholangitis (PBC) and Vitamin Supplementation
UDCA (ursodeoxycholic acid) 13–15 mg/kg/day is the first-line disease-modifying treatment for PBC (NICE NG133) — it slows fibrosis progression and improves transplant-free survival. Obeticholic acid (Ocaliva) 5–10 mg once daily is available for patients with inadequate response to UDCA (NICE TA443). Chronic cholestasis causes fat malabsorption and fat-soluble vitamin deficiency — supplement vitamin K (phytomenadione 10 mg orally or IM for coagulopathy), vitamin D, vitamin A, and calcium. DXA scanning for osteoporosis is recommended in all patients with chronic cholestasis.