TY - JOUR
T1 - Cutting-Edge issues in primary biliary cirrhosis
AU - Folci, Marco
AU - Meda, Francesca
AU - Gershwin, M. Eric
AU - Selmi, Carlo
PY - 2012/6
Y1 - 2012/6
N2 - Several crucial issues remain open in our understanding of primary biliary cirrhosis (PBC), an autoimmune liver disease targeting the small- and medium-sized intra-hepatic bile ducts. These issues include the high tissue specificity ofthe autoimmune injury despite the nontraditional autoantigens found in all mitochondria recognized by PBC-associated autoantibodies, the causes of the commonly observed pruritus, and the disease etiology per se. In all these fields, there has been recent interest secondary to the use of large-scale efforts (such as genome-wide association studies) that were previously considered poorly feasible in a rare disease such as PBC as well as other intuitions. Accordingly, there are now fascinating theories to explain the onset and severity of pruritus due to elevated autotaxin levels, the peculiar apoptotic features of bile duct cells to explain the tissue specificity, and genomic and epigenetic associations contributing to disease susceptibility. We have arbitrarily chosen these four aspects as the most promising in the PBC recent literature and will provide herein a discussion of the recent data and their potential implications.
AB - Several crucial issues remain open in our understanding of primary biliary cirrhosis (PBC), an autoimmune liver disease targeting the small- and medium-sized intra-hepatic bile ducts. These issues include the high tissue specificity ofthe autoimmune injury despite the nontraditional autoantigens found in all mitochondria recognized by PBC-associated autoantibodies, the causes of the commonly observed pruritus, and the disease etiology per se. In all these fields, there has been recent interest secondary to the use of large-scale efforts (such as genome-wide association studies) that were previously considered poorly feasible in a rare disease such as PBC as well as other intuitions. Accordingly, there are now fascinating theories to explain the onset and severity of pruritus due to elevated autotaxin levels, the peculiar apoptotic features of bile duct cells to explain the tissue specificity, and genomic and epigenetic associations contributing to disease susceptibility. We have arbitrarily chosen these four aspects as the most promising in the PBC recent literature and will provide herein a discussion of the recent data and their potential implications.
KW - Apoptosis
KW - Autoimmune cholangitis
KW - Autotaxin
KW - Epigenetics
KW - GWAS
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U2 - 10.1007/s12016-011-8253-3
DO - 10.1007/s12016-011-8253-3
M3 - Article
C2 - 21243445
AN - SCOPUS:84865271115
VL - 42
SP - 342
EP - 354
JO - Clinical Reviews in Allergy and Immunology
JF - Clinical Reviews in Allergy and Immunology
SN - 1080-0549
IS - 3
ER -