Table 2.
Agent | Dose | Additional notes |
Cholestyramine | 4 g/day to a maximum of 16 g/day as tolerated | Must be given 2–4 hours before or after UDCA (usually give UDCA at night) Pharmacy advice to avoid interactions with concomitant medications Suggest give at breakfast time (an hour before or after eating) if gallbladder in situ; rarely much incremental benefit beyond 8–12 g/day, or tolerance Mixing with orange squash and leaving in fridge overnight improves palatability Gastrointestinal (GI) symptoms: constipation |
Rifampicin | 300–600 mg/day | Risk of hepatotoxicity – need regular monitoring, start at 150 mg once to twice daily then titrate upwards as per symptoms and lung function test (LFT) monitoring. Maximum 600 mg daily Check LFTs in 2–4 weeks; caution in advanced liver disease; consider vitamin K supplementation if icteric |
Gabapentin* | Dose titrate as normal | Dose titrate according to side effects and efficacy |
Naltrexone* | 50 mg/day (normal maximum dose, although higher doses have been used in the specialist clinic setting) | Start at 12.5 mg/day and titrate slowly to avoid withdrawal symptoms Some patients require an intravenous induction stage |
Sertraline* | 100 mg/day | Titrate dose to symptoms and as tolerated Needs interaction at the primary/secondary care interface; change over if on alternative antidepressant |
*Beyond the routine first- and second-line use of cholestyramine and rifampicin, the choice of other agents is frequently based on an individual clinician’s experience and preference.