1.Selmi C，Bowlus CL，Gershwin ME，et al. Primary biliary cirrhosis. Lancet，2011，377（9777）：1600-1609.

2.Hirschfield GM，Gershwin ME. The immunobiology and pathophysiology of primary biliary cirrhosis. Annu Rev Pathol，2013，8：303-330.

3.Shimoda S，Nakamura M，Ishibashi H，et al. Molecular mimicry of mitochondrial and nuclear autoantigens in primary biliary cirrhosis. Gastroenterology，2003，124（7）：1915-1925.

4.Mao TK，Davis PA，Odin JA，et al. Sidechain biology and the immunogenicity of PDC-E2，the major autoantigen of primary biliary cirrhosis. Hepatology，2004，40（6）：1241-1248.

5.Miozzo，M，Selmi C，Gentilin B，et al. Preferential X chromosome loss but random inactivation characterize primary biliary cirrhosis. Hepatology，2007，46（2）：456-462.

6.Lleo A，Bowlus CL，Yang GX，et al. Biliary Apotopes and Anti-Mitochondrial Antibodies Activate Innate Immune Responses in Primary Biliary Cirrhosis. Hepatology，2010，52（3）：987-998.

7.Rong G，Zhong R，Lleo A，et al. Epithelial Cell Specificity and Apotope Recognition by Serum Autoantibodies in Primary Biliary Cirrhosis. Hepatology，2011，54（1）：196-203.

8.Lleo A，Invernizzi P，Selmi C，et al. Autophagy：Highlighting a novel player in the autoimmunity scenario. Journal of Autoimmunity，2007，29（2-3）：61-68.

9.Mells GF，Floyd JA，Morley KI，et al. Genome-wide association study identifies 12 new susceptibility loci for primary biliary cirrhosis. Nat Genet，2011，43（4）：329-332.

10.Salas JT，Banales JM，Sarvide S，et al. Ae2a，b-deficient mice develop antimitochondrial antibodies and other features resembling primary biliary cirrhosis. Gastroenterology，2008，134（5）：1482-1493.

11.Lazaridis KN，Juran BD，Boe GM，et al. Increased prevalence of antimitochondrial antibodies in first-degree relatives of patients with primary biliary cirrhosis. Hepatology，2007，46（3）：785-92.

12.Selmi C，Mayo MJ，Bach N，et al. Primary biliary cirrhosis in monozygotic and dizygotic twins：genetics，epigenetics，and environment. Gastroenterology，2004，127（2）：485-492.

13.Invernizzi P，Selmi C，Poli F，et al. Human leukocyte antigen polymorphisms in Italian primary biliary cirrhosis：a multicenter study of 664 patients and 1992 healthy controls. Hepatology，2008，48（6）：1906-1912.

14.Hirschfield GM，Liu X，Han Y，et al. Variants at IRF5-TNPO3，17q12-21 and MMEL1 are associated with primary biliary cirrhosis. Nat Genet，2010，42（8）：655-657.

15.Nakamura M，Yasunami M，Kondo H，et al. Analysis of HLA-DRB1 polymorphisms in Japanese patients with primary biliary cirrhosis（PBC）：The HLA-DRB1polymorphism determines the relative risk of antinuclear antibodies for disease progression in PBC. Hepatol Res，2010，40（5）：494-504.

16.Walker EJ，Hirschfield GM，Xu C，et al. CTLA4/ICOS gene variants and haplotypes are associated with rheumatoid arthritis and primary biliary cirrhosis in the Canadian population. Arthritis Rheum，2009，60（4）：931-937.

17.Liu X，Invernizzi P，Lu Y，et al. Genome-wide meta-analyses identify three loci associated with primary biliary cirrhosis. Nat Genet，2010，42（8）：658-660.

18.Hirschfield GM，Xie G，Lu E，et al. Association of primary biliary cirrhosis with variants in the CLEC16A，SOCS1，SPIB and SIAE immunomodulatory genes. Genes Immun，2012，13（4）：328-335.

19.Kikuchi K，Lian ZX，Yang GX，et al. Bacterial CpG induces hyper-IgMproduction in CD27（+） memory B cells in primary biliary cirrhosis. Gastroenterology，2005，128（2）：304-312.

20.Shimoda S，Harada K，Niiro H，et al. Biliary epithelial cells and primary biliary cirrhosis：the role of liver-infiltrating mononuclear cells. Hepatology，2008，47（3）：958-965.

21.Shimoda S，IshibashiH，Harada M. Autoreactive T-cellresponsesin primarybiliary cirrhosisare proinflammatory whereas those of controls are regulatory. Gastroenterology，2006，131（2）：606-618.

22.Kita H，Naidenko OV，Kronenberg M，et al. Quantitation and phenotypic analysis of natural killer T cells in primary biliary cirrhosis using a human CD1d tetramer. Gastroenterology，2002，123（4）：1031-1043.

23.Jin Q，Moritoki Y，Lleo A，et al. Comparative analysis of portal cell infiltrates in anti-mitochondrial autoantibodypositive versus antimitochondrial autoantibody-negative primary biliary cirrhosis. Hepatology，2012，55（5）：1495-1506.

24.Yang GX，Lian ZX，Chuang YH，et al. Adoptive transfer of CD8（+） T cells from transforming growth factor beta receptor type Ⅱ（dominant negative form） induces autoimmune cholangitis in mice. Hepatology，2008，47（6）：1974-1982.

25.Hsu W，Zhang W，Tsuneyama K，et al. Differential mechanisms in the pathogenesis of autoimmune cholangitis versus inflammatory bowel disease in interleukin-2Ralpha（-/-） mice. Hepatology，2009，49（1）：133-140.

26.Lan RY，Cheng C，Lian ZX，et al. Liver-targeted and peripheral blood alterations of regulatory T cells in primary biliary cirrhosis. Hepatology，2006，43（4）：729-737.

27.Huang W，Kachapati K，Adams D，et al. Murine autoimmune cholangitis requires two hits：cytotoxic KLRG1（+）CD8 effector cells and defective T regulatory cells. J Autoimmun，2014，50：123-134.

28.Bernuzzi F，Fenoglio D，Battaglia F，et al. Phenotypical and functional alterations of CD8 regulatory T cells in primary biliary cirrhosis. Journal of Autoimmunity，2010，35（3）：176-180.

29.Lan RY，Salunga TL，Tsuneyama K，et al. Hepatic IL-17 responses in human and murine primary biliary cirrhosis. J Autoimmun，2009，32（1）：43-51.

30.Neuberger J. Liver transplantation for primary biliary cirrhosis. Autoimmun Rev，2003，2（1）：1-7.

31.Myers RP，Swain MG，Lee SS，et al. B-cell depletion with rituximab in patients with primary biliary cirrhosis refractory to ursodeoxycholic acid. Am J Gastroenterol，2013，108（6）：933-41.

32.Dhirapong A，Lleo A，Yang GX，et al. B cell depletion therapy exacerbates murine primary biliary cirrhosis.Hepatology，2011，53（2）：527-535.

33.Moritoki Y，Lian ZX，Lindor K，et al. B-cell depletion with anti-CD20 ameliorates autoimmune cholangitis but exacerbates colitis in transforming growth factor-beta receptor Ⅱ dominant negative mice. Hepatology，2009，50（6）：1893-1903.

34.Moritoki Y，Zhang W，Tsuneyama K，et al. B cells suppress the inflammatory response in a mouse model of primary biliary cirrhosis. Gastroenterology，2009，136（3）：1037-1047.

35.Lleo A，Gershwin ME，Mantovani A，et al. Towards common denominators in primary biliary cirrhosis：the role of IL-12.J Hepatol，2012，56（3）：731-733.

36.Vignali DA，Kuchroo VK. IL-12 family cytokines：immunological playmakers. Nat Immunol，2012，13（8）：722-728.

37.Chuang YH，Lian ZX，Tsuneyama K，et al. Increased killing activity and decreased cytokine production in NK cells in patients with primary biliary cirrhosis. J Autoimmun，2006，26（4）：232-240.

38.Wang YD，Chen WD，Wang M，et al. Farnesoid X Receptor Antagonizes Nuclear Factor kappa B in Hepatic Inflammatory Response. Hepatology，2008，48（5）：1632-1643.

39.Mao TK，Lian ZX，Selmi C，et al. Altered monocyte responses to defined TLR ligands in patients with primary biliary cirrhosis. Hepatology，2005，42（4）：467a-467a.

40.Lleo A，Liao J，Invernizzi P，et al. Immunoglobulin Mlevels inversely correlate with CD40 ligand promoter methylation in patients with primary biliary cirrhosis. Hepatology，2012，55（1）：153-160.

41.Gershwin ME，Selmi C，Worman HJ，et al. Risk factors and comorbidities in primary biliary cirrhosis：a controlled interview-based study of 1032 patients. Hepatology，2005，42（5）：1194-1202.

42.Corpechot C，Chrétien Y，Chazouillères O，et al. Demographic，lifestyle，medical and familial factors associated with primary biliary cirrhosis. Journal of Hepatology，2010，53（1）：162-169.

43.Prince MI，Ducker SJ，James OFW. Case-control studies of risk factors for primary biliary cirrhosis in two United Kingdom populations. Gut，2010，59（4）：508-512.

44.McNally RJ，James PW，Ducker S，et al. Seasonal variation in the patient diagnosis of primary biliary cirrhosis：further evidence for an environmental component to etiology. Hepatology，2011，54（6）：2099-2103.

45.Rieger R，Gershwin ME. The X and why of xenobiotics in primary biliary cirrhosis. Journal of Autoimmunity，2007，28（2-3）：76-84.

46.Long SA，Van de Water J，Gershwin ME. Antimitochondrial antibodies in primary biliary cirrhosis：the role of xenobiotics. Autoimmunity reviews，2002，1（1-2）：37-42.

47.Smyk DS，Bogdanos DP，Kriese S，et al. Urinary tract infection as a risk factor for autoimmune liver disease：from bench to bedside. Clin Res Hepatol Gastroenterol，2012，36（2）：110-121.

48.Bogdanos DP，Baum H，Grasso A，et al. Microbial mimics are major targets of crossreactivity with human pyruvate dehydrogenase in primary biliary cirrhosis. Journal of Hepatology，2004，40（1）：31-39.

49.Bogdanos DP，Baum H，Okamoto M，et al. Primary biliary cirrhosis is characterized by IgG3 antibodies cross-reactive with the major mitochondrial autoepitope and its lactobacillus mimic. Hepatology，2005，42（2）：458-465.

50.Mattner J，Savage PB，Leung P，et al. Liver autoimmunity triggered by microbial activation of natural killer T cells. Cell Host&Microbe，2008，3（5）：304-315.

51.Wakabayashi K，Lian ZX，Leung PS，et al. Loss of tolerance in C57BL/6 mice to the autoantigen E2 subunit of pyruvate dehydrogenase by a xenobiotic with ensuing biliary ductular disease. Hepatology，2008，48（2）：531-540.

52.Selmi C，The evidence does not support a viral etiology for primary biliary cirrhosis. Journal of Hepatology，2011，54（6）：1315-1316.