The PBC Foundation is the only UK organisation exclusively dedicated to providing support and information to those affected by PBC
Specific symptoms of PBC
Cholestatic pruritus occurs in up to 70% of PBC patients. It also occurs in other cholestatic disorders, such as primary sclerosing cholangitis (PSC), obstetric cholestasis and drug-induced cholestasis. Cholestatic pruritus may be severe, causing significant reduction in quality of life. However, pruritus does not occur in all patients with cholestasis and its severity is unrelated to the severity of the liver disease.
Cholestatic pruritus may be exacerbated by dryness of the skin and patients may benefit from frequent and liberal use of a moisturizing lotion.
In general, the first-line of treatment is cholestyramine, a bile acid sequestrant that binds to bile acids and prevents their re-absorption from the small intestine. Cholestyramine is started at a dose of 4g once daily and may be increased as required to a maximum of 16g per day. Cholestyramine may bind to ursodeoxycholic acid and other medications, such as thyroxine, digoxin and oral contraceptive hormones. For this reason, it should be taken 2 – 4 hours separate from other medications. It is generally well-tolerated but many patients find it unpalatable. Some patients experience gastrointestinal side-effects, such as anorexia, discomfort or constipation.
Patients with cholestatic pruritus who do not respond to cholestyramine may require specialist care. Second-line treatments for cholestatic pruritus include rifampicin (more commonly used as an antibiotic), naltrexone (an opioid antagonist) and sertraline (a selective serotonin re-uptake inhibitor [SSRI]). For patients with severe pruritus refractory to medical treatments, non-pharmacological therapies include nasobiliary drainage, phototherapy, plasmapheresis or extracorporeal albumin dialysis. Liver transplantation may be considered when standard medical therapies have proven ineffective.
PBC patients frequently suffer from fatigue which is unrelated to the severity of the underlying liver disease. Fatigue in PBC is associated with autonomic dysfunction and sleep disturbance. There are no specific medical treatments for PBC-related fatigue, although patients with prominent daytime somnolence may benefit from modafinil. In all patients with PBC-related fatigue, supportive and understanding clinical care will improve patients’ capacity to cope. Fatigue may not improve significantly following liver transplantation and is not therefore a primary indication.