Table 3

Stepwise approach to management of fatigue in patients with PBC

Treat direct contributorsPruritus
Pruritus, particularly at night, is a significant factor in sleep disturbance and secondary fatigue
Associated autoimmune disease
AIH (overlap syndrome), thyroid, celiac disease, pernicious anaemia, autoimmune haemolytic anaemia and Addison’s disease are all linked to PBC, are associated with fatigue and are treatable
Age-related conditions
Diabetes, heart failure and renal failure are common in the typical PBC patient age range, are associated with fatigue and are responsive to treatment
Modify exacerbating
This is rarely a primary factor but can exacerbate and treatment may improve overall function
Autonomic dysfunction
Strongly associated with fatigue and in vasomotor forms can cause significant falls; volume repletion and assessment (through 24 hours blood pressure (BP) monitoring and, where appropriate, tilt testing) and adjustment of inappropriate antihypertensive therapy can be helpful
Sleep disturbance
Daytime somnolence can be strongly associated with fatigue; assessment and treatment for obstructive sleep apneoa can be beneficial; case series of the use of modafinil for severe daytime somnolence in PBC with improvement in linked fatigue
Assist with effecting lifestyle adjustments and developing coping mechanismsPatients need to be advised and supported to develop coping strategies while retaining ownership of the problem. Pacing strategies (using available energy to its best advantage) and timing strategies (fatigue is worse later in the day typically so arranging key tasks for earlier in the day can make them more achievable) are recommended
 SupportFatigue in PBC can reduce life quality. Awareness and understanding from carers is helpful in developing positive patient attitudes and coping strategies