What is your approach to the management of extra-hepatic manifestations/complications of PBC?

It is very important to ask and monitor extrahepatic symptoms in PBC, as this is often underappreciated and under-treated.

For pruritus, the management now also ties in closely with treatment for PBC itself. UDCA treatment does not typically improve pruritus. However, if there is an insufficient biochemical response to UDCA (i.e., if ALP is still elevated) after 6-12 months of treatment, then starting second-line therapy with a PPAR can not only treat PBC but can also improve pruritus. If ALP is normal, then it may be challenging to obtain a PPAR. Options include non-pharmacologic treatment for mild symptoms (lotions, hypoallergenic detergents, etc.), cholestyramine (or other bile-acid binding resins), or off-label use of medications (i.e.,...