Table 5

Frequently encountered clinical questions

Frequently asked questionsCurrent opinion
Can patients with PBC take exercise?It is perfectly safe to take exercise with PBC and in fact there is some pilot trial evidence that exercise therapy is helpful for the treatment of fatigue.213 Patients with PBC frequently lack confidence to undertake exercise so support can be useful291
Do patients with PBC need to follow a specific diet?Expert opinion is that the vast majority of patients with early stage disease, and who are not overtly cholestatic, have no dietary problems and can eat a normal healthy diet. Where patients have cholestasis or one of the associated malabsorption syndromes, fat malabsorption can be an issue which can lead to nutritional problems.292 In this group, fat-soluble vitamin deficiency should be considered251 293
Should patients with PBC give up smoking?General advice for health is to stop smoking. There is, however, also specific evidence in PBC to suggest that smoking is more prevalent,294 and may be associated with more aggressive disease.122 There is therefore a specific rationale for patients with PBC to avoid smoking
Can patients with PBC drink alcohol?There is no evidence to support an association between either the development of PBC or disease severity, and expert opinion is therefore that there is no reason why patients with PBC cannot drink alcohol within accepted safe limits. Patients with advanced liver disease are however advised to abstain from alcohol
Are any drugs contraindicated in PBC?As with any liver disease, expert opinion is that caution must always be applied in therapeutics; however, there are no specific concerns regarding drug toxicity in PBC per se
Is PBC associated with cancer risk?This issue has been extensively looked at295 and the only malignancy associated with PBC is HCC in patients with advanced disease296 (with a particularly increased risk in UDCA non-responding patients and in male patients.297 Previous concerns regarding breast cancer risk have not been substantiated in well-designed studies298
Is PBC inherited?Daughters in particular of patients with PBC show a slightly increased risk of the disease, but this does not represent Mendelian inheritance.37 It is thought to represent either shared immunogenetic susceptibility or, potentially, shared exposure to environmental triggers. The lifetime risk of the daughter of a patient with PBC in the UK developing PBC is less than 1%, and on this basis screening is not routinely recommended
Is cardiac risk increased in PBC?This has been extensively explored and there is no robust evidence to suggest that cardiac atherosclerotic risk is increased in PBC,299 300 despite the elevations in cholesterol seen in the disease. Patients with PBC do, however, have a normal level of cardiac risk and appropriate cardiac preventative screening and intervention is recommended. Differential cholesterol assessment is necessary because of the HDL hypercholesterolaemia of the condition and the smoking association is key
Is PBC transmissible to others?No; although infectious agents have been postulated as triggers for disease, there is no evidence that shared exposure triggers disease and patients should be advised and reassured