Inclusion Criteria:  All PBC patients living in Italy and aged at least 18 years can be included in the database. According to well-established criteria, PBC is diagnosed in subjects who fulfill two of the three of following criteria:  elevated alkaline phosphatase and /or GGT; positive anti-mitochondrial autoantibodies (titer ≥ 1:40) or PBC-specific antinuclear antibodies (gp-210 and sp100); characteristic histological features of florid bile ducts lesions and granulomatous lesion.  Exclusion Criteria:  The patient has explicitly declared his/her unwillingness to participate to the study