Fig. 1.

Summary of standard therapy and promising future therapeutic options in PBC with respect to their action in the pathophysiologic chain of PBC. UDCA in a dose of 13–15 mg per kg body weight per day, administered in either one dose or divided into two administrations per day, is the only FDA-approved drug and the cornerstone in PBC therapy. Liver transplantation (LTx) is performed for liver failure in end-stage disease. The most advanced database for future therapeutic consideration is available for budesonide. Though speculative, current preliminary data suggest a future role for agonists to certain nuclear receptors: peroxisome proliferator activated receptor alpha (PPARα), farnesoid x receptor (FXR), vitamin D receptor (VDR), pregnane x receptor (PXR), and constitutive androstane receptor (CAR)