Abstract
Despite the presumed immunological pathogenesis of primary biliary cirrhosis, no clear
or even harmful consequences resulted from some specific treatments addressed to modify
the immunological condition. However, ursodeoxycholic acid (UDCA; 13–16 mg/kg/d) has
clear favorable effects not only by improving biochemical cholestasis, but also by
delaying the histological progression. Long -term treatment with UDCA is associated
with excellent survival, free of transplantation in cases showing biochemical response
at one year. In the remaining patients, data on the effect of fibrates, budesonide,
or obeticholic acid are encouraging. Pruritus is usually managed using resins; further
steps are needed in resistant cases with the use of rifampicin, naltrexone, sertraline,
or invasive procedures such as albumin dialysis. Osteoporosis, which is highly prevalent
in patients with deep and prolonged cholestasis, improves with bisphosphonates; current
data indicate that both weekly alendronate and monthly ibandronate increase bone mass
in patients with osteoporosis. Nutritional and fat-vitamin supplementation is also
mandatory in patients with severe cholestasis.
Keywords
ursodeoxycholic acid - fibrates - budesonide - obeticholic acid - bisphosphonates
- pruritus - osteoporosis
