Primary biliary cirrhosis

The Lancet

Volumn 386, Issue 10003, 2015, Pages 1565-1575

  Carey, Elizabeth J ^a   Ali, Ahmad H ^a   Lindor, Keith D ^a,b  

^a MAYO CLINIC   (United States)

^b ARIZONA STATE UNIVERSITY   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ANTIHISTAMINIC AGENT; BEZAFIBRATE; BUDESONIDE; COLESTYRAMINE; LIVER PROTECTIVE AGENT; MITOCHONDRION ANTIBODY; NGM 282; OBETICHOLIC ACID; ONDANSETRON; OPIATE ANTAGONIST; RECOMBINANT FIBROBLAST GROWTH FACTOR 19; RIFAMPICIN; SERTRALINE; UNCLASSIFIED DRUG; URSODEOXYCHOLIC ACID;

ARTICLE; AUTOIMMUNE HEPATITIS; CHOLESTASIS; CLINICAL RESEARCH; CORTICOSTEROID THERAPY; DISEASE ASSOCIATION; FATIGUE; HORMONE SUBSTITUTION; HUMAN; HYPERLIPIDEMIA; LIVER CELL CARCINOMA; LIVER CIRRHOSIS; LIVER TRANSPLANTATION; MEDICAL HISTORY; META ANALYSIS (TOPIC); MOLECULAR PATHOLOGY; MULTICENTER STUDY (TOPIC); OSTEOPENIA; OSTEOPOROSIS; PHASE 2 CLINICAL TRIAL (TOPIC); PORTAL HYPERTENSION; PRACTICE GUIDELINE; PREGNANCY; PRIMARY BILIARY CIRRHOSIS; PRIORITY JOURNAL; PROGNOSIS; PRURITUS; RANDOMIZED CONTROLLED TRIAL (TOPIC); SJOEGREN SYNDROME; SURVIVAL RATE; SYMPOSIUM; SYSTEMATIC REVIEW (TOPIC); VITAMIN DEFICIENCY; COMPLICATION; LIVER CIRRHOSIS, BILIARY; LIVER TUMOR; METABOLIC BONE DISEASE;

AVITAMINOSIS; BONE DISEASES, METABOLIC; CARCINOMA, HEPATOCELLULAR; FATIGUE; HUMANS; HYPERLIPIDEMIAS; HYPERTENSION, PORTAL; LIVER CIRRHOSIS, BILIARY; LIVER NEOPLASMS; PROGNOSIS; PRURITUS; SJOGREN'S SYNDROME;

EID: 84947340401     PISSN: 01406736     EISSN: 1474547X     Source Type: Journal    
DOI: 10.1016/S0140-6736(15)00154-3     Document Type: Review

References (140)

1. EH Ahrens Jr, MA Payne, HG Kunkel, WJ Eisenmenger, SH Blondheim Primary biliary cirrhosis Medicine (Baltimore) 29 1950 299 364
2. T Addison, W Gull On a certain affection of the skin - vitiligoidea-alpha plana, beta tuberosa Guys Hosp Rep 7 1851 265 276
3. S Sherlock Primary billiary cirrhosis (chronic intrahepatic obstructive jaundice) Gastroenterology 37 1959 574 586
4. KD Lindor, ME Gershwin, R Poupon, M Kaplan, NV Bergasa, EJ Heathcote the American Association for Study of Liver Diseases Primary biliary cirrhosis Hepatology 50 2009 291 308
5. A Mattalia, S Quaranta, PS Leung et al. Characterization of antimitochondrial antibodies in health adults Hepatology 27 1998 656 661
6. M Shibata, Y Onozuka, T Morizane et al. Prevalence of antimitochondrial antibody in Japanese corporate workers in Kanagawa prefecture J Gastroenterol 39 2004 255 259
7. IH Frazer, IR Mackay, TW Jordan, S Whittingham, S Marzuki Reactivity of anti-mitochondrial autoantibodies in primary biliary cirrhosis: definition of two novel mitochondrial polypeptide autoantigens J Immunol 135 1985 1739 1745
8. JA Talwalkar, KD Lindor Primary biliary cirrhosis Lancet 362 2003 53 61
9. K Boonstra, U Beuers, CY Ponsioen Epidemiology of primary sclerosing cholangitis and primary biliary cirrhosis: a systematic review J Hepatol 56 2012 1181 1188
10. AN Hamlyn, AF Macklon, O James Primary biliary cirrhosis: geographical clustering and symptomatic onset seasonality Gut 24 1983 940 945
11. JV Metcalf, RS Bhopal, J Gray, D Howel, OF James Incidence and prevalence of primary biliary cirrhosis in the city of Newcastle upon Tyne, England Int J Epidemiol 26 1997 830 836
12. K Boonstra, AE Kunst, PH Stadhouders the Epi PSC PBC study group Rising incidence and prevalence of primary biliary cirrhosis: a large population-based study Liver Int 34 2014 e31 e38
13. H Rautiainen, V Salomaa, S Niemelå et al. Prevalence and incidence of primary biliary cirrhosis are increasing in Finland Scand J Gastroenterol 42 2007 1347 1353
14. RJ McNally, PW James, S Ducker, PD Norman, OF James No rise in incidence but geographical heterogeneity in the occurrence of primary biliary cirrhosis in North East England Am J Epidemiol 179 2014 492 498
15. TR Baldursdottir, OM Bergmann, JG Jonasson, BR Ludviksson, TA Axelsson, ES Björnsson The epidemiology and natural history of primary biliary cirrhosis: a nationwide population-based study Eur J Gastroenterol Hepatol 24 2012 824 830
16. A Ala, CM Stanca, M Bu-Ghanim et al. Increased prevalence of primary biliary cirrhosis near Superfund toxic waste sites Hepatology 43 2006 525 531
17. ME Gershwin, C Selmi, HJ Worman the USA PBC Epidemiology Group Risk factors and comorbidities in primary biliary cirrhosis: a controlled interview-based study of 1032 patients Hepatology 42 2005 1194 1202
18. P Invernizzi, PM Battezzati, A Crosignani et al. Peculiar HLA polymorphisms in Italian patients with primary biliary cirrhosis J Hepatol 38 2003 401 406
19. ME Mullarkey, AM Stevens, WM McDonnell et al. Human leukocyte antigen class II alleles in Caucasian women with primary biliary cirrhosis Tissue Antigens 65 2005 199 205
20. S Onishi, T Sakamaki, T Maeda et al. DNA typing of HLA class II genes; DRB1∗0803 increases the susceptibility of Japanese to primary biliary cirrhosis J Hepatol 21 1994 1053 1060
21. P Invernizzi, C Selmi, F Poli the Italian PBC Genetic Study Group Human leukocyte antigen polymorphisms in Italian primary biliary cirrhosis: a multicenter study of 664 patients and 1992 healthy controls Hepatology 48 2008 1906 1912
22. M Carbone, A Lleo, RN Sandford, P Invernizzi Implications of genome-wide association studies in novel therapeutics in primary biliary cirrhosis Eur J Immunol 44 2014 945 954
23. C Selmi, A Lleo, S Pasini, M Zuin, ME Gershwin Innate immunity and primary biliary cirrhosis Curr Mol Med 9 2009 45 51
24. U Beuers, M Trauner, P Jansen, R Poupon New paradigms in the treatment of hepatic cholestasis: from UDCA to FXR, PXR and beyond J Hepatol 62 suppl 2015 S25 S37
25. European Association for the Study of the Liver EASL Clinical Practice Guidelines: management of cholestatic liver diseases J Hepatol 51 2009 237 267
26. H Baum, C Palmer The PBC-specific antigen Mol Aspects Med 8 1985 201 234
27. M Nakamura Clinical significance of autoantibodies in primary biliary cirrhosis Semin Liver Dis 34 2014 334 340
28. L Dubel, O Farges, H Bismuth, M Sebagh, JC Homberg, C Johanet Kinetics of anti-M2 antibodies after liver transplantation for primary biliary cirrhosis J Hepatol 23 1995 674 680
29. M Nakamura, H Kondo, T Mori et al. Anti-gp210 and anti-centromere antibodies are different risk factors for the progression of primary biliary cirrhosis Hepatology 45 2007 118 127
30. J Ludwig, ER Dickson, GS McDonald Staging of chronic nonsuppurative destructive cholangitis (syndrome of primary biliary cirrhosis) Virchows Arch A Pathol Anat Histol 379 1978 103 112
31. F Colina, F Pinedo, JA Solís, D Moreno, M Nevado Nodular regenerative hyperplasia of the liver in early histological stages of primary biliary cirrhosis Gastroenterology 102 1992 1319 1324
32. C Corpechot, F Carrat, A Poujol-Robert et al. Noninvasive elastography-based assessment of liver fibrosis progression and prognosis in primary biliary cirrhosis Hepatology 56 2012 198 208
33. M Friedrich-Rust, C Müller, A Winckler et al. Assessment of liver fibrosis and steatosis in PBC with FibroScan, MRI, MR-spectroscopy, and serum markers J Clin Gastroenterol 44 2010 58 65
34. P Angulo, KP Batts, TM Therneau, RA Jorgensen, ER Dickson, KD Lindor Long-term ursodeoxycholic acid delays histological progression in primary biliary cirrhosis Hepatology 29 1999 644 647
35. KD Lindor, RA Jorgensen, TM Therneau, M Malinchoc, ER Dickson Ursodeoxycholic acid delays the onset of esophageal varices in primary biliary cirrhosis Mayo Clin Proc 72 1997 1137 1140
36. B Combes, RL Carithers Jr, WC Maddrey et al. A randomized, double-blind, placebo-controlled trial of ursodeoxycholic acid in primary biliary cirrhosis Hepatology 22 1995 759 766
37. C Corpechot, F Carrat, AM Bonnand, RE Poupon, R Poupon The effect of ursodeoxycholic acid therapy on liver fibrosis progression in primary biliary cirrhosis Hepatology 32 2000 1196 1199
38. EJ Heathcote, K Cauch-Dudek, V Walker et al. The Canadian Multicenter Double-blind Randomized Controlled Trial of ursodeoxycholic acid in primary biliary cirrhosis Hepatology 19 1994 1149 1156
39. KD Lindor, TM Therneau, RA Jorgensen, M Malinchoc, ER Dickson Effects of ursodeoxycholic acid on survival in patients with primary biliary cirrhosis Gastroenterology 110 1996 1515 1518
40. A Parés, L Caballería, J Rodés Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic Acid Gastroenterology 130 2006 715 720
41. RE Poupon, R Poupon, B Balkau the The UDCA-PBC Study Group Ursodiol for the long-term treatment of primary biliary cirrhosis N Engl J Med 330 1994 1342 1347
42. RE Poupon, B Balkau, E Eschwège, R Poupon the UDCA-PBC Study Group A multicenter, controlled trial of ursodiol for the treatment of primary biliary cirrhosis N Engl J Med 324 1991 1548 1554
43. KD Lindor, ER Dickson, WP Baldus et al. Ursodeoxycholic acid in the treatment of primary biliary cirrhosis Gastroenterology 106 1994 1284 1290
44. RE Poupon, KD Lindor, K Cauch-Dudek, ER Dickson, R Poupon, EJ Heathcote Combined analysis of randomized controlled trials of ursodeoxycholic acid in primary biliary cirrhosis Gastroenterology 113 1997 884 890
45. C Corpechot, F Carrat, A Bahr, Y Chrétien, RE Poupon, R Poupon The effect of ursodeoxycholic acid therapy on the natural course of primary biliary cirrhosis Gastroenterology 128 2005 297 303
46. RE Poupon, KD Lindor, A Parés, O Chazouillères, R Poupon, EJ Heathcote Combined analysis of the effect of treatment with ursodeoxycholic acid on histologic progression in primary biliary cirrhosis J Hepatol 39 2003 12 16
47. R Poupon Ursodeoxycholic acid and bile-acid mimetics as therapeutic agents for cholestatic liver diseases: an overview of their mechanisms of action Clin Res Hepatol Gastroenterol 36 suppl 1 2012 S3 12
48. GQ Zhu, KQ Shi, S Huang et al. Network meta-analysis of randomized controlled trials: efficacy and safety of UDCA-based therapies in primary biliary cirrhosis Medicine (Baltimore) 94 2015 e609
49. S Lens, M Leoz, L Nazal, M Bruguera, A Parés Bezafibrate normalizes alkaline phosphatase in primary biliary cirrhosis patients with incomplete response to ursodeoxycholic acid Liver Int 34 2014 197 203
50. K Hosonuma, K Sato, Y Yamazaki et al. A prospective randomized controlled study of long-term combination therapy using ursodeoxycholic acid and bezafibrate in patients with primary biliary cirrhosis and dyslipidemia Am J Gastroenterol 110 2015 423 431
51. GM Hirschfield, A Mason, V Luketic et al. Efficacy of obeticholic acid in patients with primary biliary cirrhosis and inadequate response to ursodeoxycholic acid Gastroenterology 148 2015 751 761 e8
52. E Christensen, J Crowe, D Doniach et al. Clinical pattern and course of disease in primary biliary cirrhosis based on an analysis of 236 patients Gastroenterology 78 1980 236 246
53. GR Locke 3rd, TM Therneau, J Ludwig, ER Dickson, KD Lindor Time course of histological progression in primary biliary cirrhosis Hepatology 23 1996 52 56
54. TC Mahl, W Shockcor, JL Boyer Primary biliary cirrhosis: survival of a large cohort of symptomatic and asymptomatic patients followed for 24 years J Hepatol 20 1994 707 713
55. J Springer, K Cauch-Dudek, K O'Rourke, IR Wanless, EJ Heathcote Asymptomatic primary biliary cirrhosis: a study of its natural history and prognosis Am J Gastroenterol 94 1999 47 53
56. MH Imam, KD Lindor The natural history of primary biliary cirrhosis Semin Liver Dis 34 2014 329 333
57. KP Batts, RA Jorgensen, ER Dickson, KD Lindor Effects of ursodeoxycholic acid on hepatic inflammation and histological stage in patients with primary biliary cirrhosis Am J Gastroenterol 91 1996 2314 2317
58. WJ Lammers, HR van Buuren, GM Hirschfield the Global PBC Study Group Levels of alkaline phosphatase and bilirubin are surrogate end points of outcomes of patients with primary biliary cirrhosis: an international follow-up study Gastroenterology 147 2014 1338 1349 e5, quiz e15
59. P Angulo, KD Lindor, TM Therneau et al. Utilization of the Mayo risk score in patients with primary biliary cirrhosis receiving ursodeoxycholic acid Liver 19 1999 115 121
60. N Momah, MG Silveira, R Jorgensen, E Sinakos, KD Lindor Optimizing biochemical markers as endpoints for clinical trials in primary biliary cirrhosis Liver Int 32 2012 790 795
61. C Corpechot, L Abenavoli, N Rabahi et al. Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis Hepatology 48 2008 871 877
62. EM Kuiper, BE Hansen, RA de Vries the Dutch PBC Study Group Improved prognosis of patients with primary biliary cirrhosis that have a biochemical response to ursodeoxycholic acid Gastroenterology 136 2009 1281 1287
63. T Kumagi, M Guindi, SE Fischer et al. Baseline ductopenia and treatment response predict long-term histological progression in primary biliary cirrhosis Am J Gastroenterol 105 2010 2186 2194
64. C Corpechot, O Chazouillères, R Poupon Early primary biliary cirrhosis: biochemical response to treatment and prediction of long-term outcome J Hepatol 55 2011 1361 1367
65. M Carbone, GF Mells, G Pells the UK PBC Consortium Sex and age are determinants of the clinical phenotype of primary biliary cirrhosis and response to ursodeoxycholic acid Gastroenterology 144 2013 560 569 e7, quiz e13-14
66. GF Mells, G Pells, JL Newton the UK-PBC Consortium Impact of primary biliary cirrhosis on perceived quality of life: the UK-PBC national study Hepatology 58 2013 273 283
67. N Al-Harthy, T Kumagi, C Coltescu, GM Hirschfield The specificity of fatigue in primary biliary cirrhosis: evaluation of a large clinic practice Hepatology 52 2010 562 570
68. DE Jones, N Bhala, J Burt, J Goldblatt, M Prince, JL Newton Four year follow up of fatigue in a geographically defined primary biliary cirrhosis patient cohort Gut 55 2006 536 541
69. DE Jones, N Bhala, JL Newton Reflections on therapeutic trials in primary biliary cirrhosis: a quality of life oriented counter-view Hepatology 43 2006 633 author reply 634.
70. JL Newton, GJ Gibson, M Tomlinson, K Wilton, D Jones Fatigue in primary biliary cirrhosis is associated with excessive daytime somnolence Hepatology 44 2006 91 98
71. CM Stanca, N Bach, C Krause et al. Evaluation of fatigue in U.S. patients with primary biliary cirrhosis Am J Gastroenterol 100 2005 1104 1109
72. E Björnsson, E Kalaitzakis, M Neuhauser et al. Fatigue measurements in patients with primary biliary cirrhosis and the risk of mortality during follow-up Liver Int 30 2010 251 258
73. DE Jones, A Al-Rifai, J Frith, I Patanwala, JL Newton The independent effects of fatigue and UDCA therapy on mortality in primary biliary cirrhosis: results of a 9 year follow-up J Hepatol 53 2010 911 917
74. CO Zein, AJ McCullough Association between fatigue and decreased survival in primary biliary cirrhosis Gut 56 2007 1165 1166
75. G Abbas, RA Jorgensen, KD Lindor Fatigue in primary biliary cirrhosis Nat Rev Gastroenterol Hepatol 7 2010 313 319
76. KG Hollingsworth, JL Newton, R Taylor et al. Pilot study of peripheral muscle function in primary biliary cirrhosis: potential implications for fatigue pathogenesis Clin Gastroenterol Hepatol 6 2008 1041 1048
77. C McDonald, J Newton, HM Lai, SN Baker, DE Jones Central nervous system dysfunction in primary biliary cirrhosis and its relationship to symptoms J Hepatol 53 2010 1095 1100
78. JL Newton, KG Hollingsworth, R Taylor et al. Cognitive impairment in primary biliary cirrhosis: symptom impact and potential etiology Hepatology 48 2008 541 549
79. JL Newton, C Elliott, J Frith, C Ghazala, J Pairman, DE Jones Functional capacity is significantly impaired in primary biliary cirrhosis and is related to orthostatic symptoms Eur J Gastroenterol Hepatol 23 2011 566 572
80. JJ Theal, MN Toosi, L Girlan et al. A randomized, controlled crossover trial of ondansetron in patients with primary biliary cirrhosis and fatigue Hepatology 41 2005 1305 1312
81. JA Talwalkar, JJ Donlinger, AA Gossard et al. Fluoxetine for the treatment of fatigue in primary biliary cirrhosis: a randomized, double-blind controlled trial Dig Dis Sci 51 2006 1985 1991
82. MM Kaplan, PA Bonis Modafinil for the treatment of fatigue in primary biliary cirrhosis Ann Intern Med 143 2005 546 547
83. DE Jones, JL Newton An open study of modafinil for the treatment of daytime somnolence and fatigue in primary biliary cirrhosis Aliment Pharmacol Ther 25 2007 471 476
84. S Ian Gan, M de Jongh, MM Kaplan Modafinil in the treatment of debilitating fatigue in primary biliary cirrhosis: a clinical experience Dig Dis Sci 54 2009 2242 2246
85. JA Talwalkar, E Souto, RA Jorgensen, KD Lindor Natural history of pruritus in primary biliary cirrhosis Clin Gastroenterol Hepatol 1 2003 297 302
86. U Beuers, AE Kremer, R Bolier, RP Elferink Pruritus in cholestasis: facts and fiction Hepatology 60 2014 399 407
87. C Quarneti, P Muratori, C Lalanne et al. Fatigue and pruritus at onset identify a more aggressive subset of primary biliary cirrhosis Liver Int 35 2015 636 641
88. AE Kremer, JJ Martens, W Kulik et al. Lysophosphatidic acid is a potential mediator of cholestatic pruritus Gastroenterology 139 2010 1008 1018 e1
89. EA Jones, NV Bergasa The pruritus of cholestasis: from bile acids to opiate agonists Hepatology 11 1990 884 887
90. M Trivedi, NV Bergasa Serum concentrations of substance P in cholestasis Ann Hepatol 9 2010 177 180
91. P Tandon, BH Rowe, B Vandermeer, VG Bain The efficacy and safety of bile acid binding agents, opioid antagonists, or rifampin in the treatment of cholestasis-associated pruritus Am J Gastroenterol 102 2007 1528 1536
92. A Podesta, P Lopez, R Terg et al. Treatment of pruritus of primary biliary cirrhosis with rifampin Dig Dis Sci 36 1991 216 220
93. S Khurana, P Singh Rifampin is safe for treatment of pruritus due to chronic cholestasis: a meta-analysis of prospective randomized-controlled trials Liver Int 26 2006 943 948
94. MJ Mayo, I Handem, S Saldana, H Jacobe, Y Getachew, AJ Rush Sertraline as a first-line treatment for cholestatic pruritus Hepatology 45 2007 666 674
95. FJC Cuperus, E Halilbasic, M Trauner Fibrate treatment for primary biliary cirrhosis Curr Opin Gastroenterol 30 2014 279 286
96. JW O'Donohue, SP Pereira, AC Ashdown, CG Haigh, JR Wilkinson, R Williams A controlled trial of ondansetron in the pruritus of cholestasis Aliment Pharmacol Ther 21 2005 1041 1045
97. EA Jones, HA Molenaar, J Oosting Ondansetron and pruritus in chronic liver disease: a controlled study Hepatogastroenterology 54 2007 1196 1199
98. T Pusl, GU Denk, KG Parhofer, U Beuers Plasma separation and anion adsorption transiently relieve intractable pruritus in primary biliary cirrhosis J Hepatol 45 2006 887 891
99. C Corpechot, Y Chrétien, O Chazouillères, R Poupon Demographic, lifestyle, medical and familial factors associated with primary biliary cirrhosis J Hepatol 53 2010 162 169
100. C Lammert, DL Nguyen, BD Juran et al. Questionnaire based assessment of risk factors for primary biliary cirrhosis Dig Liver Dis 45 2013 589 594
101. A Parikh-Patel, EB Gold, H Worman, KE Krivy, ME Gershwin Risk factors for primary biliary cirrhosis in a cohort of patients from the united states Hepatology 33 2001 16 21
102. A Floreani, I Franceschet, N Cazzagon et al. Extrahepatic autoimmune conditions associated with primary biliary cirrhosis Clin Rev Allergy Immunol 48 2015 192 197
103. J Raszeja-Wyszomirska, T Miazgowski Osteoporosis in primary biliary cirrhosis of the liver Prz Gastroenterol 9 2014 82 87
104. N Guañabens, A Parés, I Ros et al. Severity of cholestasis and advanced histological stage but not menopausal status are the major risk factors for osteoporosis in primary biliary cirrhosis J Hepatol 42 2005 573 577
105. N Guañabens, A Monegal, D Cerdá et al. Randomized trial comparing monthly ibandronate and weekly alendronate for osteoporosis in patients with primary biliary cirrhosis Hepatology 58 2013 2070 2078
106. N Guañabens, A Parés, I Ros et al. Alendronate is more effective than etidronate for increasing bone mass in osteopenic patients with primary biliary cirrhosis Am J Gastroenterol 98 2003 2268 2274
107. CO Zein, RA Jorgensen, B Clarke et al. Alendronate improves bone mineral density in primary biliary cirrhosis: a randomized placebo-controlled trial Hepatology 42 2005 762 771
108. JS Rudic, G Poropat, MN Krstic, G Bjelakovic, C Gluud Hormone replacement for osteoporosis in women with primary biliary cirrhosis Cochrane Database Syst Rev 12 2011 CD009146.
109. A Sorokin, JL Brown, PD Thompson Primary biliary cirrhosis, hyperlipidemia, and atherosclerotic risk: a systematic review Atherosclerosis 194 2007 293 299
110. M Solaymani-Dodaran, GP Aithal, T Card, J West Risk of cardiovascular and cerebrovascular events in primary biliary cirrhosis: a population-based cohort study Am J Gastroenterol 103 2008 2784 2788
111. A Floreani, N Cazzagon, I Franceschet, F Canesso, L Salmaso, V Baldo Metabolic syndrome associated with primary biliary cirrhosis J Clin Gastroenterol 49 2015 57 60
112. WJ Cash, S O'Neill, ME O'Donnell et al. Randomized controlled trial assessing the effect of simvastatin in primary biliary cirrhosis Liver Int 33 2013 1166 1174
113. V Balan, ER Dickson, RA Jorgensen, KD Lindor Effect of ursodeoxycholic acid on serum lipids of patients with primary biliary cirrhosis Mayo Clin Proc 69 1994 923 929
114. N Agmon-Levin, R Kopilov, C Selmi et al. Vitamin D in primary biliary cirrhosis, a plausible marker of advanced disease Immunol Res 61 2015 141 146
115. JR Phillips, P Angulo, T Petterson, KD Lindor Fat-soluble vitamin levels in patients with primary biliary cirrhosis Am J Gastroenterol 96 2001 2745 2750
116. AH Ali, E Sinakos, MG Silveira, RA Jorgensen, P Angulo, KD Lindor Varices in early histological stage primary biliary cirrhosis J Clin Gastroenterol 45 2011 e66 e71
117. F Ikeda, R Okamoto, N Baba et al. Prevalence and associated factors with esophageal varices in early primary biliary cirrhosis J Gastroenterol Hepatol 27 2012 1320 1328
118. MH Imam, MG Silveira, E Sinakos et al. Long-term outcomes of patients with primary biliary cirrhosis and hepatocellular carcinoma Clin Gastroenterol Hepatol 10 2012 182 185
119. M Deutsch, GV Papatheodoridis, A Tzakou, SJ Hadziyannis Risk of hepatocellular carcinoma and extrahepatic malignancies in primary biliary cirrhosis Eur J Gastroenterol Hepatol 20 2008 5 9
120. Y Tomiyama, K Takenaka, T Kodama et al. Risk factors for survival and the development of hepatocellular carcinoma in patients with primary biliary cirrhosis Intern Med 52 2013 1553 1559
121. MG Silveira, A Suzuki, KD Lindor Surveillance for hepatocellular carcinoma in patients with primary biliary cirrhosis Hepatology 48 2008 1149 1156
122. PJ Trivedi, WJ Lammers, HR van Buuren on behalf of the Global PBC Study Group Stratification of hepatocellular carcinoma risk in primary biliary cirrhosis: a multicentre international study Gut 2015 10.1136/gutjnl-2014-308351 published online Jan 7.
123. JM Llovet, S Ricci, V Mazzaferro the SHARP Investigators Study Group Sorafenib in advanced hepatocellular carcinoma N Engl J Med 359 2008 378 390
124. WR Kim, JR Lake, JM Smith et al. OPTN/SRTR 2013 Annual Data Report: liver Am J Transplant 15 suppl 2 2015 1 28
125. M Carbone, S Bufton, A Monaco, L Griffiths, DE Jones, JM Neuberger The effect of liver transplantation on fatigue in patients with primary biliary cirrhosis: a prospective study J Hepatol 59 2013 490 494
126. A Bosch, J Dumortier, D Maucor Boulch et al. P1148: Long-term administration of ursodeoxycholic acid prevents recurrence of primary biliary cirrhosis after liver transplantation J Hepatol 62 2015 S783
127. MA Lacerda, J Ludwig, ER Dickson, RA Jorgensen, KD Lindor Antimitochondrial antibody-negative primary biliary cirrhosis Am J Gastroenterol 90 1995 247 249
128. F Mendes, KD Lindor Antimitochondrial antibody-negative primary biliary cirrhosis Gastroenterol Clin North Am 37 2008 479 484 viii
129. C Levy, J Naik, C Giordano et al. Hispanics with primary biliary cirrhosis are more likely to have features of autoimmune hepatitis and reduced response to ursodeoxycholic acid than non-Hispanics Clin Gastroenterol Hepatol 12 2014 1398 1405
130. O Chazouillères, D Wendum, L Serfaty, S Montembault, O Rosmorduc, R Poupon Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome: clinical features and response to therapy Hepatology 28 1998 296 301
131. MG Silveira, JA Talwalkar, P Angulo, KD Lindor Overlap of autoimmune hepatitis and primary biliary cirrhosis: long-term outcomes Am J Gastroenterol 102 2007 1244 1250
132. AA Gossard, KD Lindor Development of autoimmune hepatitis in primary biliary cirrhosis Liver Int 27 2007 1086 1090
133. R Poupon, O Chazouilleres, C Corpechot, Y Chrétien Development of autoimmune hepatitis in patients with typical primary biliary cirrhosis Hepatology 44 2006 85 90
134. G Dahlqvist, F Gaouar, F Carrat et al. Characterization and outcome of antimitochondrial type 2-positive patients without a diagnosis of primary biliary cirrhosis: a French nationwide prospective study J Hepatol 60 2014 S188
135. C Corpechot, F Gaouar, Y Chrétien, C Johanet, O Chazouillères, R Poupon Smoking as an independent risk factor of liver fibrosis in primary biliary cirrhosis J Hepatol 56 2012 218 224
136. CO Zein, K Beatty, AB Post, L Logan, S Debanne, AJ McCullough Smoking and increased severity of hepatic fibrosis in primary biliary cirrhosis: A cross validated retrospective assessment Hepatology 44 2006 1564 1571
137. A Tanaka, AT Borchers, H Ishibashi, AA Ansari, CL Keen, ME Gershwin Genetic and familial considerations of primary biliary cirrhosis Am J Gastroenterol 96 2001 8 15
138. C Efe, E Kahramanoʇlu-Aksoy, B Yilmaz et al. Pregnancy in women with primary biliary cirrhosis Autoimmun Rev 13 2014 931 935
139. PJ Trivedi, T Kumagi, N Al-Harthy et al. Good maternal and fetal outcomes for pregnant women with primary biliary cirrhosis Clin Gastroenterol Hepatol 12 2014 1179 1185 e1
140. EJ Carey, P White Ursodeoxycholic acid for intrahepatic cholestasis of pregnancy: good for the mother, not bad for the baby Evid Based Med 18 2013 e55