Affiliations: Department of Internal Medicine, IRCCS-Istituto
Clinico Humanitas, University of Milan, Italy | Division of Rheumatology, Allergy, and Clinical
Immunology, University of California at Davis, Davis, CA, USA
Note: [] Corresponding author: Carlo Selmi MD PhD, Department of Internal
Medicine, IRCCS Istituto Clinico Humanitas, via A. Manzoni 56, 20089 Rozzano,
Milan, Italy. Tel.: +39 02 8224 5129; Fax: +39 02 8224 4590, E-mail:
carlo.selmi@unimi.it
Abstract: Primary biliary cirrhosis (PBC)is a chronic autoimmune cholestatic
liver disease that manifests a latitudinal gradient in prevalence and
incidence. The mechanisms leading to the initiation and perpetuation of PBC
remain largely enigmatic, although it is established that a combination of
genetic predisposition and environmental stimulation is required. PBC is also
characterized by a high concordance rate in monozygotic twins and is considered
a model autoimmune disease because of several features common to other
conditions and the relatively homogeneous serological and biochemical features.
From a diagnostic standpoint, PBC is characterized by the highest specificity
of serum autoantibodies directed at mitochondrial proteins. Several risk
factors have been suggested to be associated with PBC, including exposure to
infectious agents and chemical xenobiotics that will be critically discussed in
the present review article.
