Primary biliary cirrhosis

Hepatology

Volumn 50, Issue 1, 2009, Pages 291-308

  Lindor, Keith D ^a   Gershwin, M Eric ^b   Poupon, Raoul ^c   Kaplan, Marshall ^d   Bergasa, Nora V ^e   Heathcote, E Jenny ^f  

^a MAYO CLINIC   (United States)

^b UNIVERSITY OF CALIFORNIA   (United States)

^c HÔPITAL SAINT ANTOINE   (France)

^d TUFTS MEDICAL CENTER   (United States)

^e METROPOLITAN HOSPITAL CENTER   (United States)

^f UNIVERSITY OF TORONTO   (Canada)

Author keywords

[No Author keywords available]

Indexed keywords

AZATHIOPRINE; BUDESONIDE; CHLORAMBUCIL; COLCHICINE; COLESEVELAM; COLESTIPOL; COLESTYRAMINE; CORTICOSTEROID; CYCLOSPORIN; FIBRIC ACID DERIVATIVE; FLUOXETINE; IMMUNOGLOBULIN M; MALOTILATE; METHOTREXATE; MITOCHONDRION ANTIBODY; MODAFINIL; MYCOPHENOLIC ACID 2 MORPHOLINOETHYL ESTER; NALMEFENE; NALTREXONE; ONDANSETRON; PENICILLAMINE; PHENOBARBITAL; PLACEBO; PYRUVATE DEHYDROGENASE COMPLEX; RIFAMPICIN; SERTRALINE; SILYMARIN; THALIDOMIDE; UNINDEXED DRUG; URSODEOXYCHOLIC ACID;

ALANINE AMINOTRANSFERASE BLOOD LEVEL; ALKALINE PHOSPHATASE BLOOD LEVEL; ASPARTATE AMINOTRANSFERASE BLOOD LEVEL; AUTOIMMUNE HEPATITIS; BLOATING; CHOLANGIOGRAPHY; CLINICAL TRIAL; CONSTIPATION; DAYTIME SOMNOLENCE; DIARRHEA; DIFFERENTIAL DIAGNOSIS; DRUG DOSE COMPARISON; DRUG DOSE INCREASE; ESOPHAGUS VARICES; FATIGUE; GENETIC PREDISPOSITION; GINGIVA HYPERPLASIA; HAIR LOSS; HEMOLYSIS; HEPATITIS; HUMAN; HYPERBILIRUBINEMIA; HYPERCHOLESTEROLEMIA; HYPERIMMUNOGLOBULINEMIA; HYPERLIPIDEMIA; HYPOTHYROIDISM; INFLAMMATORY INFILTRATE; KIDNEY DYSFUNCTION; LIVER BIOPSY; LIVER CELL CARCINOMA; LIVER FAILURE; LIVER NECROSIS; LIVER TOXICITY; LIVER TRANSPLANTATION; LOOSE FECES; MORNING DOSAGE; NON INVASIVE MEASUREMENT; NONHUMAN; OSTEOPOROSIS; OVERALL SURVIVAL; PATIENT COMPLIANCE; PHYSICAL EXAMINATION; PORTAL HYPERTENSION; PRACTICE GUIDELINE; PRIMARY BILIARY CIRRHOSIS; PRIORITY JOURNAL; PROGNOSIS; PRURITUS; RECOMMENDED DRUG DOSE; REVIEW; SCORING SYSTEM; SIDE EFFECT; SJOEGREN SYNDROME; SURVIVAL RATE; SYNDROME CREST; UNITED STATES; VITAMIN DEFICIENCY; WEIGHT GAIN; WITHDRAWAL SYNDROME; ARTICLE; BILIARY CIRRHOSIS;

HUMANS; LIVER CIRRHOSIS, BILIARY;

EID: 67651160903     PISSN: 02709139     EISSN: None     Source Type: Journal    
DOI: 10.1002/hep.22906     Document Type: Review

References (222)

1. Eddy DM. A Manual for Assessing Health Practices and Designing Practice Guidelines. Philadelphia, PA: American College of Physicians; 1996:1-126.
2. Position and policy statement: American Gastroenterological Association policy statement on the use of medical practice guidelines by managed care organizations and insurance carriers. Gastroenterology 1995;108:925-926.
3. American Heart Association. Methodology Manual. http://www. heart.org/presenter.jhtml?identifier=3039683. Accessed February 2009.
4. Gershwin ME, Ansari AA, Mackay IR, Nakanuma Y, Nishio A, Rowley MJ, et al. Primary biliary cirrhosis: an orchestrated immune response against epithelial cells. Immunol Rev 2000;174:210-225. (Pubitemid 30201390)
5. Kaplan MM, Gershwin ME. Primary biliary cirrhosis. N Engl J Med 2005;353:1261-1273.
6. Selmi C, Mayo MJ, Bach N, Ishibashi H, Invernizzi P, Gish RG, et al. Primary biliary cirrhosis in monozygotic and dizygotic twins: genetics, epigenetics, and environment. Gastroenterology 2004;127:485-492. (Pubitemid 39091897)
7. Zhang L, Weetman AP, Bassendine M, Oliveira DB. Major histocompatibility complex class-II alleles in primary biliary cirrhosis. Scand J Immunol 1994;39:104-106. (Pubitemid 24232904)
8. Gershwin ME, Selmi C, Worman HJ, Gold EB, Watnik M, Utts J, et al. Risk factors and comorbidities in primary biliary cirrhosis: a controlled interview-based study of 1032 patients. HEPATOLOGY 2005;42:1194-1202. (Pubitemid 43112687)
9. Ala A, Stanca CM, Bu-Ghanim M, Ahmado I, Branch AD, Schiano TD, et al. Increased prevalence of primary biliary cirrhosis near Superfund toxic waste sites. HEPATOLOGY 2006;43:525-531.
10. Leung PS, Park O, Tsuneyama K, Kurth MJ, Lam KS, Ansari AA, et al. Induction of primary biliary cirrhosis in guinea pigs following chemical xenobiotic immunization. J Immunol 2007;179:2651-2657.
11. Migliaccio C, Nishio A, Van de Water J, Ansari AA, Leung PS, Nakanuma Y, et al. Monoclonal antibodies to mitochondrial E2 components define autoepitopes in primary biliary cirrhosis. J Immunol 1998;161:5157-5163. (Pubitemid 28519072)
12. Odin JA, Huebert RC, Casciola-Rosen L, LaRusso NF, Rosen A. Bcl-2- dependent oxidation of pyruvate dehydrogenase-E2, a primary biliary cirrhosis autoantigen, during apoptosis. J Clin Invest 2001;108:223-232. (Pubitemid 32656246)
13. Gershwin ME, Mackay IR, Sturgess A, Coppel RL. Identification and specificity of a cDNA encoding the 70 kd mitochondrial antigen recognized in primary biliary cirrhosis. J Immunol 1987;138:3525-3531. (Pubitemid 17071942)
14. Moteki S, Leung PS, Dickson ER, Van Thiel DH, Galperin C, Buch T, et al. Epitope mapping and reactivity of autoantibodies to the E2 component of 2-oxoglutarate dehydrogenase complex in primary biliary cirrhosis using recombinant 2-oxoglutarate dehydrogenase complex. HEPATOLOGY 1996;23:436-444. (Pubitemid 126664264)
15. Tanaka A, Nalbandian G, Leung PS, Benson GD, Munoz S, Findor JA, et al. Mucosal immunity and primary biliary cirrhosis: presence of antimitochondrial antibodies in urine. HEPATOLOGY 2000;32:910-915. (Pubitemid 30808413)
16. Oertelt S, Rieger R, Selmi C, Invernizzi P, Ansari AA, Coppel RL, et al. A sensitive bead assay for antimitochondrial antibodies: Chipping away at AMA-negative primary biliary cirrhosis.HEPATOLOGY 2007;45:659-665.
17. Kita H, Matsumura S, He XS, Ansari AA, Lian ZX, Van de Water J, et al. Quantitative and functional analysis of PDC-E2-specific autoreactive cytotoxic T lymphocytes in primary biliary cirrhosis. J Clin Invest 2002;109:1231-1240. (Pubitemid 34496601)
18. Shimoda S, Van de Water J, Ansari A, Nakamura M, Ishibashi H, Coppel RL, et al. Identification and precursor frequency analysis of a common T cell epitope motif in mitochondrial autoantigens in primary biliary cirrhosis. J Clin Invest 1998;102:1831-1840. (Pubitemid 28533830)
19. Corpechot C, Poupon R. Geotherapeutics of primary biliary cirrhosis: bright and sunny around the Mediterranean but still cloudy and foggy in the United Kingdom. HEPATOLOGY 2007;46:963-965. (Pubitemid 350144759)
20. Jones DEJ, Watt FE, Metcalf JV, Bassendine MF, James OF. Familiar primary biliary cirrhosis reassessed: a geographically-based population study. J Hepatol 1999;30:402-407. (Pubitemid 29113227)
21. Metcalf JV, Mitchison HC, Palmer JM, Jones DE, Bassendine MF, James OF. Natural history of early primary biliary cirrhosis. Lancet 1996;348:1399-1402. (Pubitemid 26395233)
22. Mattalia A, Quaranta S, Leung PSC, Bauducci M, Van de Water J, Calvo PL, et al. Characterization of antimitochondrial antibodies in healthy adults. HEPATOLOGY 2008;27:656-661.
23. Long RG, Scheuer PJ, Sherlock S. Presentation and course of asymptomatic primary biliary cirrhosis. Gastroenterology 1977;72:1204-1207. (Pubitemid 8116683)
24. Mahl TC, Shockcor W, Boyer JL. Primary biliary cirrhosis: survival of a large cohort of symptomatic and asymptomatic patients followed for 24 years. J Hepatol 1994;20:707-713. (Pubitemid 24216880)
25. Mitchison HC, Lucey MR, Kelly PJ, Neuberger JM, Williams R, James OF. Symptom development and prognosis in primary biliary cirrhosis: a study in two centers. Gastroenterology 1990;99:778-784. (Pubitemid 20243371)
26. Nyberg A, Loof L. Primary biliary cirrhosis: clinical features and outcome, with special reference to asymptomatic disease. Scand J Gastroenterol 1989;24:57-64. (Pubitemid 19071840)
27. Prince M, Chetwynd A, Newman W, Metcalf JV, James OF. Survival and symptom progression in a geographically based cohort of patients with primary biliary cirrhosis: follow-up for up to 28 years. Gastroenterology 2002;123:1044-1051.
28. Springer J, Cauch-Dudek K, O'Rourke K, Wanless IR, Heathcote EJ. Asymptomatic primary biliary cirrhosis: a study of its natural history and prognosis. Am J Gastroenterol 1999;94:47-53. (Pubitemid 29063443)
29. Newton JL, Jones DE. Association between fatigue and decreased survival in primary biliary cirrhosis. Gut 2007;56:1166.
30. Christensen E, Neuberger J, Crowe J, Portmann B, Williams R, Altman DG, et al. Azathioprine and prognosis in primary biliary cirrhosis. Gastroenterology 1986;90:508-509.
31. Roll J, Boyer JL, Barry D, Klatskin G. The prognostic importance of clinical and histologic features in asymptomatic and symptomatic primary biliary cirrhosis. N Engl J Med 1983;308:1-7. (Pubitemid 13211264)
32. Corpechot C, Carrat F, Bonnand AM, Poupon RE, Poupon R. The effect of ursodeoxycholic acid therapy on liver fibrosis progression in primary biliary cirrhosis. HEPATOLOGY 2000;32:1196-1199. (Pubitemid 30246580)
33. Locke GR 3rd, Therneau TM, Ludwig J, Dickson ER, Lindor KD. Time course of histological progression in primary biliary cirrhosis. HEPATOLOGY 1996;23:52-56.
34. Gores GJ, Wiesner RH, Dickson ER, Zinsmeister AR, Jorgensen RA, Langworthy A. Prospective evaluation of esophageal varices in primary biliary cirrhosis: development, natural history, and influence on survival. Gastroenterology 1989;96:1552-1559. (Pubitemid 19138700)
35. Angulo P, Batts KP, Therneau TM, Jorgensen RA, Dickson ER, Lindor KD. Long-term ursodeoxycholic acid delays histological progression in primary biliary cirrhosis. HEPATOLOGY 1999;29:644-647. (Pubitemid 29109574)
36. Combes B, Carithers RL Jr, Maddrey WC, Lin D, McDonald MF, Wheeler DE, et al. A randomized, double-blind, placebo-controlled trial of ursodeoxycholic acid in primary biliary cirrhosis. HEPATOLOGY 1995;22:759-766.
37. Heathcote EJ, Cauch-Dudek K, Walker V, Bailey RJ, Blendis LM, Ghent CN, et al. The Canadian Multicenter Double-blind Randomized Controlled Trial of ursodeoxycholic acid in primary biliary cirrhosis. HEPATOLOGY 1994;19:1149-1156. (Pubitemid 24134236)
38. Lindor KD, Therneau TM, Jorgensen RA, Malinchoc M, Dickson ER. Effects of ursodeoxycholic acid on survival in patients with primary biliary cirrhosis. Gastroenterology 1996;110:1515-1518. (Pubitemid 26134085)
39. Pares A, Caballeria L, Rodes J. Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic acid. Gastroenterology 2006;130:715-720. (Pubitemid 43374522)
40. Poupon RE, Balkau B, Eschwege E, Poupon R. A multicenter, controlled trial of ursodiol for the treatment of primary biliary cirrhosis. UDCAPBC Study Group. N Engl J Med 1991;324:1548-1554.
41. Poupon RE, Poupon R, Balkau B. Ursodiol for the long-term treatment of primary biliary cirrhosis. The UDCA-PBC Study Group. N Engl J Med 1994;330:1342-1347.
42. Poupon RE, Lindor KD, Cauch-Dudek K, Dickson ER, Poupon R, Heathcote EJ. Combined analysis of randomized controlled trials of ursodeoxycholic acid in primary biliary cirrhosis. Gastroenterology 1997; 113:884-890. (Pubitemid 27381148)
43. Poupon RE, Bonnand AM, Chretien Y, Poupon R. Ten-year survival in ursodeoxycholic acid-treated patients with primary biliary cirrhosis. The UDCA-PBC Study Group. HEPATOLOGY 1999;29:1668-1671. (Pubitemid 29247037)
44. Poupon RE, Lindor KD, Pares A, Chazouilleres O, Poupon R, Heathcote EJ. Combined analysis of the effect of treatment with ursodeoxycholic acid on histologic progression in primary biliary cirrhosis. J Hepatol 2003;39:12-16. (Pubitemid 36724102)
45. Vuoristo M, Farkkila M, Karvonen AL, Leino R, Lehtola J, Makinen J, et al. A placebo-controlled trial of primary biliary cirrhosis treatment with colchicine and ursodeoxycholic acid. Gastroenterology 1995;108:1470-1478.
46. Corpechot C, Carrat F, Bahr A, Chretien Y, Poupon RE, Poupon R. The effect of ursodeoxycholic acid therapy on the natural course of primary biliary cirrhosis. Gastroenterology 2005;128:297-1203 (Pubitemid 40431083)
47. Corpechot C, Carrat F, Poupon R, Poupon RE. Primary biliary cirrhosis: incidence and predictive factors of cirrhosis development in ursodioltreated patients. Gastroenterology 2002;122:652-658. (Pubitemid 34173817)
48. Lindor KD, Jorgensen RA, Therneau TM, Malinchoc M, Dickson ER. Ursodeoxycholic acid delays the onset of esophageal varices in primary biliary cirrhosis. Mayo Clin Proc 1997;72:1137-1140. (Pubitemid 27528324)
49. Dickson ER, Grambsch PM, Fleming TR, Fisher LD, Langworthy A. Prognosis in primary biliary cirrhosis: model for decision making. HEPATOLOGY 1989;10:1-7. (Pubitemid 19169993)
50. Grambsch PM, Dickson ER, Kaplan M, LeSage G, Fleming TR, Langworthy AL. Extramural cross-validation of the Mayo primary biliary cirrhosis survival model establishes its generalizability. HEPATOLOGY 1989;10:846-850. (Pubitemid 19270172)
51. Corpechot C, Poujol-Robert A, Wendum D, Galotte M, Chretien Y, Poupon RE, et al. Biochemical markers of liver fibrosis and lymphocytic piecemeal necrosis in UDCA-treated patients with primary biliary cirrhosis. Liver Int 2004;24:187-193. (Pubitemid 38930855)
52. Poupon R, Chazouilleres O, Balkau B, Poupon RE. Clinical and biochemical expression of the histopathological lesions of primary biliary cirrhosis. UDCA-PBC Group. J Hepatol 1999;30:408-412. (Pubitemid 29113228)
53. Sorokin A, Brown JL, Thompson PD. Primary biliary cirrhosis, hyperlipidemia, and atherosclerotic risk: a systematic review. Atherosclerosis 2007;194:293-299. (Pubitemid 47513107)
54. Nakamura M, Kondo H, Mori T, Komori A, Matsuyama M, Ito M, et al. Anti-gp210 and anti-centromere antibodies are different risk factors for the progression of primary biliary cirrhosis. HEPATOLOGY 2007;45:118-127. (Pubitemid 46144371)
55. Degott C, Zafrani ES, Callard P, Balkau B, Poupon RE, Poupon R. Histopathological study of primary biliary cirrhosis and the effect of ursodeoxycholic acid treatment on histology progression. HEPATOLOGY 1999;29:1007-1012. (Pubitemid 29168729)
56. Zein CO, Angulo P, Lindor KD. When is liver biopsy needed in the diagnosis of primary biliary cirrhosis. Clin Gastroenterol Hepatol 2003;1:89-95. (Pubitemid 37248079)
57. Corpechot C, El Naggar A, Poujol-Robert A, Ziol M, Wendum D, Chazouilleres O, et al. Assessment of biliary fibrosis by transient elastography in patients with PBC and PSC. HEPATOLOGY 2006;43:1118-1124. (Pubitemid 43724636)
58. Goldblatt J, Taylor PJ, Lipman T, Prince MI, Baragiotta A, Bassendine MF, et al. The true impact of fatigue in primary biliary cirrhosis: a population study. Gastroenterology 2002;122:1235-1241. (Pubitemid 34465165)
59. Prince MI, Chetwynd A, Craig WL, Metcalf JV, James OF. Asymptomatic primary biliary cirrhosis: clinical features, prognosis, and symptom progression in a large population based cohort. Gut 2004;53:865-870. (Pubitemid 38662138)
60. Forton DM, Patel N, Prince M, Oatridge A, Hamilton G, Goldblatt J, et al. Fatigue and primary biliary cirrhosis: association of globus pallidus magnetisation transfer ratio measurements with fatigue severity and blood manganese levels. Gut 2004;53:587-592. (Pubitemid 38372986)
61. Newton JL, Jones DE. Modafinil is effective treatment for excessive daytime somnolence and fatigue in primary biliary cirrhosis [Abstract]. HEPATOLOGY 2006;44:628A.
62. Poupon RE, Chretien Y, Chazouilleres O, Poupon R, Chwalow J. Quality of life in patients with primary biliary cirrhosis. HEPATOLOGY 2004;40:489-494. (Pubitemid 39062868)
63. Jones DE, Bala N, Burt J, Goldblatt BJ, Prince M, Newton JL. Four year follow up of fatigue in a geographically defined primary biliary cirrhosis cohort. Gut 2006;55:536-546.
64. Bergasa NV, Mason A, Floreani A, Heathcote J, Swain MG, Jones DEJ, et al. Primary biliary cirrhosis: Report of a focus group. HEPATOLOGY 2004;40:1013-1020. (Pubitemid 39288137)
65. van Os E, van den Broek WW, Mulcer PGH, ter Borg PC, Bruijn JA, van Buuren HR. Depression in patients with primary biliary cirrhosis and primary sclerosing cholangitis. J Hepatol 2007;46:1099-1103.
66. Elta GH, Sepersky RA, Goldberg MJ, Connors CM, Miller KB, Kaplan MM. Increased incidence of hypothyroidism in primary biliary cirrhosis. Dig Dis Sci 1983;28:971-975. (Pubitemid 14232080)
67. Talwalkar JA, Souto E, Jorgensen RA, Lindor KD. Natural history of pruritus in primary biliary cirrhosis. Clin Gastroenterol Hepatol 2003;1:297-302. (Pubitemid 37254457)
68. Pares A, Rodes J. Natural history of primary biliary cirrhosis. Clin Liver Dis 2003;7:779-794. (Pubitemid 37229504)
69. Ghent C, Bloomer J, Klatskin G. Elevations in skin tissue levels of bile acids in human cholestasis: relation to serum levels and to pruritus. Gastroenterology 1977;73:1125-1130. (Pubitemid 8213121)
70. Jones EA, Bergasa NV. The pruritus of cholestasis: from bile acids to opiod antagonists. HEPATOLOGY 1999;11:884-887.
71. Ng VL, Ryckman FC, Porta G, Miura IK, de Carvalho E, Servidoni MF, et al. Long-term outcome after partial external biliary diversion for intractable pruritus in patients with intrahepatic cholestasis. J Pediatr Gastroenterol Nutr 2008;30:152-156.
72. Thornton JR, Triger DR. Variceal bleeding is associated with reduced risk of severe cholestasis in primary biliary cirrhosis. Q J Med 1989;71:467-471.
73. Colina F, Pinedo F, Solis JA, Moreno D, Nevado M. Nodular regenerative hyperplasia of the liver in early histological stages of primary biliary cirrhosis. Gastroenterology 1992;102:1319-1324.
74. Boyer TD, Kokenes DD, Hertzler G, Kutner MH, Henderson JM. Effect of distal splenorenal shunt on survival of patients with primary biliary cirrhosis. HEPATOLOGY 1994;20:1482-1486. (Pubitemid 24359060)
75. Levy C, Lindor KD. Management of osteoporosis, fat-soluble vitamin deficiencies, and hyperlipidemia in primary biliary cirrhosis. Clin Liver Dis 2003;7:901-910. (Pubitemid 37229509)
76. Springer JE, Cole DE, Rubin LA, Cauch-Dudek K, Harewood L, Evrovski J, et al. Vitamin D-receptor genotypes as independent genetic predictors of decreased bone mineral density in primary biliary cirrhosis. Gastroenterology 2000;118:145-151. (Pubitemid 30020382)
77. Menon KV, Angulo P, Weston S, Dickson ER, Lindor KD. Bone disease in primary biliary cirrhosis: independent indicators and rate of progression. J Hepatol 2001;35:316-323. (Pubitemid 32844184)
78. Guanabens N, Pares A, Ros I, Caballeria L, Pons F, Vidal S, et al. Severity of cholestasis and advanced histological stage but not menopausal status are major risk factors for osteoporosis in primary biliary cirrhosis. J Hepatol 2005;42:573-577. (Pubitemid 40342938)
79. Ormarsdottir S, Ljunggren O, Mallmin H, Olsson R, Prytz H, Loof L. Longitudinal bone loss in postmenopausal women with primary biliary cirrhosis and well-preserved liver function. J Intern Med 2002;252:537-541. (Pubitemid 36001993)
80. Boulton-Jones JR, Fenn RM, West J, Logan RF, Ryder SD. Fracture risk of women with primary biliary cirrhosis: no increase compared with general population controls. Aliment Pharmacol Ther 2004;20:551-557.
81. Hodgson SF, Dickson ER, Wahner HW, Johnson KA, Mann KG, Riggs BL. Bone loss and reduced osteoblast function in primary biliary cirrhosis. Ann Intern Med 1985;103:855-860.
82. Janes CH, Dickson ER, Okazaki R, Bonde S, McDonagh AF, Riggs BL. Role of hyperbilirubinemia in the impairment of osteoblast proliferation associated with cholestatic jaundice. J Clin Invest 1995;95:2581-2586.
83. Kaplan MM, Elta GH, Furie B, Sadowski JA, Russell RM. Fat-soluble vitamin nutriture in primary biliary cirrhosis. Gastroenterology 1988;95:787-792. (Pubitemid 18207443)
84. Matloff DS, Kaplan MM, Neer RM, Goldberg MJ, Bitman W, Wolfe HJ. Osteoporosis in primary biliary cirrhosis: effects of 25-hydroxyvitamin D3 treatment. Gastroenterology 1982;83:97-102.
85. Eastell R, Dickson ER, Hodgson SF, Wiesner RH, Porayko MK, Wahner HW, et al. Rates of vertebral bone loss before and after liver transplantation in women with primary biliary cirrhosis.HEPATOLOGY 1991;14:296-300.
86. Gregory WL, Game FL, Farrer M, Idle JR, Laker MF, James OF. Reduced serum lipoprotein(a) levels in patients with primary biliary cirrhosis. Atherosclerosis 1994;105:43-50. (Pubitemid 24047746)
87. Longo M, Crosignani A, Battezzati PM, Squarcia Giussani C, Invernizzi P, Zuin M, et al. Hyperlipidaemic state and cardiovascular risk in primary biliary cirrhosis. Gut 2002;51:265-269. (Pubitemid 34791459)
88. Jahn CE, Schaefer EJ, Hoofnagle JH, Lindgren FT, Albers JJ, Jones EA, et al. Lipoprotein abnormalities in primary biliary cirrhosis. Association with hepatic lipase inhibition as well as alltered cholesterol esterification. Gastroenterology 1985;89:1266-1278.
89. Crippin JS, Lindor KD, Jorgensen R, Kottke BA, Harrison JM, Murtaugh PA, et al. Hypercholesterolemia and atherosclerosis in primary biliary cirrhosis. HEPATOLOGY 1992;15:858-862.
90. Allocca M, Crosignani A, Gritti A, Ghilardi G, Gobatti D, Caruso D, et al. Hypercholesterolaemia is not associated with early atherosclerotic lesions in primary biliary cirrhosis. Gut 2006;55:1795-1800. (Pubitemid 44893581)
91. Lanspa SJ, Chan AT, Bell JS, Go VL, Dickson ER, DiMagno EP. Pathogenesis of steatorrhea in primary biliary cirrhosis. HEPATOLOGY 1985;5:837-842. (Pubitemid 15236631)
92. Phillips JR, Angulo P, Petterson T, Lindor KD. Fat-soluble vitamin levels in patients with primary biliary cirrhosis. Am J Gastroenterol 2001;96:2745-2750. (Pubitemid 32889215)
93. Kowdley KV, Emond MJ, Sadowski JA, Kaplan MM. Plasma vitamin- K-1 level is decreased in primary biliary cirrhosis. Am J Gastroenterol 1997;92:2059-2061. (Pubitemid 27476250)
94. Kaplan MM, Goldberg MJ, Matloff DS, Neer RM, Goodman DB. Effect of 25-hydroxyvitamin D3 on vitamin D metabolites in primary biliary cirrhosis. Gastroenterology 1981;81:681-685. (Pubitemid 11014414)
95. Jeffrey GP, Muller DP, Matthews S, Kemp C, Epstein O, Metcalfe TA, et al. Vitamin E deficiency and its clinical significance in adults with. J Hepatol 1987;4:307-317.
96. Munoz SJ, Heubi JE, Balistreri WF, Maddrey WC. Vitamin E deficiency in primary biliary cirrhosis: gastrointestinal. HEPATOLOGY 1989;9:525-531.
97. Van de Water J, Gershwin ME, Leung P, Ansari A, Coppel RL. The autoepitope of the 74 kD mitochondrial autoantigen of primary biliary cirrhosis corresponds to the functional site of dihydrolipoamide acetyl transferase. J Exp Med 1988;167:1791-1799. (Pubitemid 18174270)
98. Micheletti P, Wanless IR, Katz A, Scheuer PJ, Yeaman SJ, Bassendine MF, et al. Antimitochondrial antibody negative primary biliary cirrhosis: a distinct syndrome of autoimmune cholangitis. Gut 1994;35:260-265. (Pubitemid 24092876)
99. Taylor SL, Dean PJ, Riely CA. Primary autoimmune cholangitis: an alternative to antimitochondrial antibody-negative primary biliary cirrhosis. Am J Surg Pathol 1994;18:91-99. (Pubitemid 24014498)
100. Lacerda MA, Ludwig J, Dickson ER, Jorgensen RA, Lindor KD. Antimitochondrial antibody-negative primary biliary cirrhosis. Am J Gastroenterol 1995;90:247-249.
101. Invernizzi P, Crosignani A, Battezzati PM, Covini G, de Valle G, Larghi A, et al. Comparison of the clinical features and clinical course of antimitochondrial antibody-positive and negative primary biliary cirrhosis. HEPATOLOGY 1997;25:1090-1095. (Pubitemid 27199932)
102. Miyakawa H, Tanaka A, Kikuchi K, Matsushita M, Kitazawa E, Kawaguchi N, et al. Detection of antimitochondrial auto-antibodies in immuno- fluorescence negative patients with primary biliary cirrhosis using recombinant autoantigens. HEPATOLOGY 2001;34:243-248. (Pubitemid 32702335)
103. Tsuneyama K, van De Water J, van Thiel D, Coppel R, Ruebner B, Nakanuma Y, et al. Abnormal expression of PDC-E2 on the apical surface of biliary epithelial cells in patients with antimitochondrial antibodynegative primary biliary cirrhosis. HEPATOLOGY 1995;22:1440-1446.
104. Sakauchi F, Mori M, Zeniya M, Toda G. Antimitochondrial antibody negative primary biliary cirrhosis in Japan: utilization of clinical data when patients applied to receive public financial aid. J Epidemiol 2006;16:30-34. (Pubitemid 43055685)
105. Gisbert JP, Jones EA, Pajares JM, Moreno-Otero R. Review article: is there an optimal therapeutic regimen for antimitochondrial antibodynegative primary biliary cirrhosis (autoimmune cholangitis)? Aliment Pharmacol Ther 2003;17:17-27. (Pubitemid 36140431)
106. Gossard AA, Lindor KD. Development of autoimmune hepatitis in primary biliary cirrhosis. Liver Int 2007;27:1086-1090. (Pubitemid 47399442)
107. Alvarez F, Berg PA, Bianchi FB, Bianchi L, Burroughs AK, Cancado EL, et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol 1999;31:929-938. (Pubitemid 29514229)
108. Chazouillères O, Wendum D, Serfaty L, Montembault S, Rosmorduc O, Poupon R. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome: clinical features and response to therapy. HEPATOLOGY 1998;28:296-301.
109. Lohse AW, zum Büschenfelde KH, Franz B, Kanzler S, Gerken G, Dienes HP. Characterization of the overlap syndrome of primary biliary cirrhosis (PBC) and autoimmune hepatitis: evidence for it being a hepatitic form of PBC in genetically susceptible individuals. HEPATOLOGY 1999;29:1078-1084.
110. Joshi S, Cauch-Dudek K, Wanless IR, Lindor KD, Jorgensen R, Batts K, et al. Primary biliary cirrhosis with additional features of autoimmune hepatitis: response to therapy with ursodeoxycholic acid. HEPATOLOGY 2002;35:409-413. (Pubitemid 34106382)
111. Heurgué A, Vitry F, Diebold MD, Yaziji N, Bernard-Chabert B, Pennaforte JL, et al. Overlap syndrome of primary biliary cirrhosis and autoimmune hepatitis: a retrospective study of 115 cases of autoimmune liver disease. Gastroenterol Clin Biol 2007;31:17-25. (Pubitemid 46212347)
112. Silveira MG, Talwalkar JA, Angulo P, Lindor KD. Overlap of autoimmune hepatitis and primary biliary cirrhosis: long-term outcomes. Am J Gastroenterol 2007;102:1244-1250. (Pubitemid 46802005)
113. Chazouilleres O, Wendum D, Serfaty L, Montembault S, Rosmorduc O, Poupon R. Long term outcome and response to therapy of primary biliary cirrhosis - autoimmune hepatitis overlap syndrome. J Hepatol 2006;44:400-406. (Pubitemid 43069223)
114. Colombato LA, Alvarez F, Cote J, Huet PM. Autoimmune cholangiopathy: The result of consecutive primary biliary cirrhosis and autoimmune hepatitis? Gastroenterology 1994;107:1839-1843.
115. Weyman RL,MVoigt. Consecutive occurrence of primary biliary cirrhosis and autoimmune hepatitis: a case report and review of the literature. Am J Gastroenterol 2001;96:585-587. (Pubitemid 32184957)
116. Poupon R, Chazouilleres O, Corpechot C, Chrétien Y. Development of autoimmune hepatitis in patients with typical primary biliary cirrhosis. HEPATOLOGY 2006;44:85-90. (Pubitemid 44049141)
117. Czaja AJ, Muratori P, Muratori L, Carpenter HA, Bianchi FB. Diagnostic and therapeutic implications of bile duct injury in autoimmune hepatitis. Liver Int 2004;24:322-329. (Pubitemid 39162072)
118. Farias AQ, Goncalves LL, Bittencourt PL, De Melo ES, Abrantes-Lemos CP, Porta G, et al. Applicability of the IAIHG scoring system to the diagnosis of anitmitochondrial/anti-M2 seropositive variant form of autoimmune hepatitis. J Gastroenterol Hepatol 2006;21:887-893. (Pubitemid 43793289)
119. Nezu S, Tanaka A, Yasui H, Imamura M, Nakajima H, Ishida H, et al. Presence of antimitochondrial autoantibodies in patients with autoimmune hepatitis. J Gastroenterol Hepatol 2006;21:1448-1454. (Pubitemid 44154715)
120. O'Brien C, Joshi S, Feld J, Guindi M, Dienes H, Heathcote EJ. Longterm follow-up of antimitochondrial antibody positive autoimmune hepatitis. HEPATOLOGY 2008;48:550-556.
121. Goulis J, Leandro G, Burroughs AK. Randomised controlled trials of ursodeoxycholic-acid therapy for primary biliary cirrhosis: a meta-analysis. Lancet 1999;354:1053-1060. (Pubitemid 29452816)
122. Gong Y, Huang Z, Christensen E, Gluud C. Ursodeoxycholic acid for patients with primary biliary cirrhosis: an updated systematic review and meta-analysis of randomized cinical trials using Bayesian approach as sensitivity analyses. Am J Gastroenterol 2007;102:1799-1807. (Pubitemid 47206681)
123. Lee J, Belanger A, Joucette JT, Stanca C, Friedman S, Bach N. Transplantation trends in primary bilary cirrhosis. Clin Gastroenterol Hepatol 2007;5:1313-1315. (Pubitemid 350019058)
124. Angulo P, Dickson ER, Therneau TM, Jorgensen RA, Smith C, DeSotel CK, et al. Comparison of three doses of ursodeoxycholic acid in the treatment of primary biliary cirrhosis: a randomized trial. J Hepatol 1999;30:830-835. (Pubitemid 29248983)
125. Williams CN, Al-Knawy B, Blanchard W. Bioavailability of four ursodeoxycholic acid preparations. Aliment Pharmacol Ther 2000;14:1133-1139.
126. Jorgensen RA, Dickson ER, Hofmann AF, Rossii SS, Lindor KD. Characterization of patients with a complete biochemical response to ursodeoxycholic acid. Gut 1995;36:935-938.
127. Angulo P, Lindor KD, Therneau TM, Jorgensen RA, Malinchoc M, Kamath PS, et al. Utilization of the Mayo risk score in patients with primary biliary cirrhosis receiving ursodeoxycholic acid. Liver 1999;19:115-121. (Pubitemid 29139764)
128. Balan V, Dickson ER, Jorgensen RA, Lindor KD. Effect of ursodeoxycholic acid on serum lipids of patients with primary biliary cirrhosis. Mayo Clin Proc 1994;69:923-929. (Pubitemid 24310070)
129. Lindor KD, Janes CH, Crippin JS, Jorgensen RA, Dickson ER. Bone disease in primary biliary cirrhosis: does ursodeoxyholic acid make a difference? HEPATOLOGY 1995;21:389-392.
130. Siegel JL, Jorgensen R, Angulo P, Lindor KD. Treatment with ursodeoxycholic acid is associated with weight gain in patients with primary biliary cirrhosis. J Clin Gastroenterol 2003;37:183-185. (Pubitemid 36875583)
131. Hoofnagle JH, Davis GL, Schafer DR, Peters M, Avigan MI, Pappas SC, et al. Randomized trial of chlorambucil for primary biliary cirrhosis. Gastroenterology 1986;91:1327-1334. (Pubitemid 17176843)
132. Dickson ER, Fleming TR, Wiesner RH, Baldus WP, Fleming CR, Ludwig J, et al. Trial of penicillamine in advanced primary biliary cirrhosis. N Engl J Med 1985;312:1011-1015. (Pubitemid 15114288)
133. Neuberger J, Christensen E, Portmann B, Caballeria J, Rodes J, Ranek L, et al. Double blind controlled trial of d-penicillamine in patients with primary biliary cirrhosis. Gut 1985;23:114-119. (Pubitemid 15105135)
134. Wiesner RH, Ludwig J, Lindor KD, Jorgensen RA, Baldus WP, Hornburger HA, et al. A controlled trial of cyclosporine in the treatment or primary biliary cirrhosis. N Engl J Med 1990;322:1419-1424. (Pubitemid 20152527)
135. Lombard M, Portmann B, Neuberger J, Williams R, Tygstrup N, Ranek L, et al. Cyclosporine A treatment in primary biliary cirrhosis: results of a long-term placebo controlled trial. Gastroenterology 1993;104:519-526. (Pubitemid 23033426)
136. Mitchison HC, Palmer JM, Bassendine MF, Watson AJ, Record CO, James OF, et al. A controlled trial of prenisolone treatment in primary biliary cirrhosis: Three-year results. J Hepatol 1992;15:336-344.
137. Christensen E, Neuberger J, Crowe J, Altman DG, Popper H, Portmann B, et al. Beneficial effect of azathioprine and prediction of prognosis in primary biliary cirrhosis: final results of an international trial. Gastroenterology 1985;89:1084-1091. (Pubitemid 16231574)
138. Talwalkar JA, Angulo P, Keach JC, Petz JL, Jorgensen RA, Lindor KD. Mycophenolate mofetil for the treatment of primary biliary cirrhosis in patients with an incomplete response to ursodeoxycholic acid. J Clin Gastroenterol 2005;39:168-171. (Pubitemid 40130032)
139. Hendrickse MT, Rigney E, Giaffer MH, Soomro I, Triger DR, Underwood JC, et al. Low-dose methotrexate is ineffective in primary biliary cirrhosis: long-term results of a placebo-controlled trial. Gastroenterology 1999;117:400-407. (Pubitemid 29352949)
140. Kaplan MM, Alling DW, Zimmerman HJ, Wolfe HJ, Sepersky RA, Hirsch GS, et al. A prospective trial of cochicine for primary biliary cirrhosis. N Engl J Med 1986;215:1448-1454. (Pubitemid 17051705)
141. Gong Y, Gluud C. Colchicine for primary biliary cirrhosis. Cochrane Database Syst Rev 2004;2:CD004481.
142. Battezzati PM, Zuin M, Crosignani A, Allocca M, Invernizzi P, Selmi C, et al. Ten-year combination treatment with colchicine and ursodeoxycholic acid for primary biliary cirrhosis: a double-blind, placebo-controlled trial on symptomatic patients. Aliment Pharmacol Ther 2001;15:1427-1434. (Pubitemid 32835551)
143. Combes B. Emerson SS, Flye NL. Methotrexate (MTX) plus ursodeoxycholic acid (UDCA) in the treatment of primary biliary cirrhosis. HEPATOLOGY 2005;42:1184-1193. (Pubitemid 43112686)
144. Angulo P, Jorgensen RA, Lindor KD. Incomplete response to ursodeoxycholic acid in primary biliary cirrhosis: is a double-dosage worthwhile? Am J Gastroenterol 2001;96:3152-3157.
145. Angulo P, Patel T, Jorgensen RA, Therneau TM, Lindor KD. Silymarin in the treatment of patients with primary biliary cirrhosis with a suboptimal response to ursodeoxycholic acid. HEPATOLOGY 2000;32:897-900. (Pubitemid 30808411)
146. Kaplan MM, Cheng S, Price LL, Bonis PA. A randomized controlled trial of colchicine plus ursodiol versus methotrexate plus ursodiol in primary biliary cirrhosis: ten-year results. HEPATOLOGY 2004;39:915-923. (Pubitemid 38428929)
147. Angulo P, Jorgensen RA, Keach JC, Dickson ER, Smith C, Lindor KD. Oral
148. Ohira H, Sata Y, Ueno T, Sata M. Fenofibrate treatment in patients with primary biliary cirrhosis. Am J Gastroenterol 2002;97:2147-2149. (Pubitemid 34906880)
149. Jones EA. Relief from profound fatigue associated with chronic liver disease by long-term ondansetron therapy. Lancet 1999;354:97.
150. Theal JJ, Toosi MN, Girlan L, Heslegrave RJ, Huet PM, Burak KW, et al. A randomized, controlled crossover trial of ondansetron in patients with primary biliary cirrhosis and fatigue. HEPATOLOGY 2005;41:1305-1312. (Pubitemid 40770283)
151. Talwalkar JA, Donlinger JJ, Gossard AA, Keach JC, Jorgensen RA, Petz JC, et al. Fluoxetine for the treatment of fatigue in primary biliary cirrhosis: a randomized, double-blind controlled trial. Dig Dis Sci 2006;51:1985-1991.
152. Newton JL, Gibson GJ, Tomlinson M, Wilton K, Jones D. Fatigue in primary biliary cirrhosis is associated with excessive daytime somnolence. HEPATOLOGY 2006;44:91-98. (Pubitemid 44049142)
153. Czeisler CA, Walsh JK, Roth T, Hughes RJ, Wright KP, Kingsbury L, et al. Modafinil for excessive sleepiness associated with shift-work sleep disorder. N Engl J Med 2005;353:476-486. (Pubitemid 41127292)
154. Kaplan MM, Bonis PA. Modafanil for the treatment of fatigue in PBC. Ann Intern Med 2005;143:546-547.
155. Jones DE, Newton JL. An open study of modafinil for the treatment of daytime somnolence and fatigue in primary biliary cirrhosis. Aliment Pharmacol Ther 2007;25:471-476.
156. Datta DV, Sherlock S. Cholestyramine for long term relief of the pruritus complicating intrahepatic cholestasis. Gastroenterology 1966;50:323-332.
157. Van Itallie TB, Hashim SA, Crampton RS, Tennent DM. The treatment of pruritus and hypercholesteremia of primary biliary cirrhosis with cholestyramine. N Engl J Med 1961;265:469-474.
158. Tandon P, Rowe BH, Vandermeer B, Bain VG. The efficacy and safety of bile Acid binding agents, opioid antagonists, or rifampin in the treatment of cholestasis-associated pruritus. Am J Gastroenterol 2007;102:1528-1536. (Pubitemid 46976283)
159. Pares A, Cisneros L, Salmeron JM, Caballeria L, Mas A, Torras A, et al. Extracorporeal albumin dialysis: a procedure for prolonged relief of intractable pruritus in patients with primary biliary cirrhosis. Am J Gastroenterol 2004;99:1105-1110. (Pubitemid 38917816)
160. Rifai K, Hafer C, Rosenau J, Athmann C, Haller H, Peter Manns M, et al. Treatment of severe refractory pruritus with fractionated plasma separation and adsorption (Prometheus). Scand J Gastroenterol 2006;41:1212-1217. (Pubitemid 44451686)
161. Pusl T, Denk GU, Parhofer KG, Beuers U. Plasma separation and anion adsorption transiently relieve intractable pruritus in primary biliary cirrhosis. J Hepatol 2006;45:887-891. (Pubitemid 44646686)
162. Hoensch HP, Balzer K, Dylewizc P, Kirch W, Goebell H, Ohnhaus EE. Effect of rifampicin treatment on hepatic drug metabolism and serum bile acids in patients with primary biliary cirrhosis. Eur J Clin Pharmacol 1985;28:475-477. (Pubitemid 15063822)
163. Ghent CN, Carruthers SG. Treatment of pruritus in primary biliary cirrhosis with rifampin. Results of a double-blind, crossover, randomized trial. Gastroenterology 1988;94:488-493.
164. Bachs L, Parés A, Elena M, Piera C, Rodés J. Comparison of rifampicin with phenobarbitone for treatment of pruritus in biliary cirrhosis. Lancet 1989;1:574-576. (Pubitemid 19074002)
165. Podesta A, Lopez P, Terg R, Villamil F, Flores D, Mastai R, et al. Treatment of pruritus of primary biliary cirrhosis with rifampin. Dig Dis Sci 1991;36:216-220.
166. Khurana S, Singh P. Rifampin is safe for treatment of pruritus due to chronic cholestasis: a meta-analysis of prospective randomized-controlled trials. Liver Int 2006;26:943-948. (Pubitemid 44323412)
167. Jadad AR, Moore RA, Carroll D, Jenkinson C, Reynolds DJ, Gavaghan DJ, et al. Assessing the quality of reports of randomized clinical trials: is blinding necessary? Control Clin Trials 1996;17:1-12.
168. Prince MI, Burt AD, Jones DE. Hepatitis and liver dysfunction with rifampicin therapy for pruritus in primary biliary cirrhosis. Gut 2002;50:436-439. (Pubitemid 34161065)
169. Markowitz JS, DeVane CL. Rifampin-induced slective serotonin reuptake inhibitor withdrawal syndrome in a patient treated with sertraline J Clin Psychopharmacol 2000;20:109-110.
170. Ballantyne JC, Loach AB, Carr DB. The incidence of pruritus after epidural morphine. Anaesthesia 1989;44:863.
171. Abbound TK, Lee K, Zhu J, Reyes A, Afrasiabi A, Mantilla M, et al. Prophylactic oral naltrexone with intrathecal morphine for cesarean section: effects on adverse reactions and analgesia. Anesth Analg 1990;71:367-370.
172. Bergasa NV, Jones EA. The pruritus of cholestasis: potential pathogenic and therapeutic implications of opioids. Gastroenterology 1995;108:1582-1588.
173. Bergasa NV, Alling DW, Talbot TL, Swain MG, Yurdaydin C, Turner ML, et al. Effects of naloxone infusions in patients with the pruritus of cholestasis. A double-blind, randomized, controlled trial. Ann Intern Med 1995;123:161-167.
174. Thornton JR, Losowsky MS. Opioid peptides and primary biliary cirrhosis. Br Med J 1988;297:1501-1504. (Pubitemid 19007547)
175. Wolfhagen FH, Sternieri E, Hop WC, Vitale G, Bertolotti M, Van Buuren HR. Oral naltrexone treatment for cholestatic pruritus: a doubleblind, placebo-controlled study. Gastroenterology 1997;113:1264-1269. (Pubitemid 27418980)
176. Bergasa NV, Alling DW, Talbot TL, Wells MC, Jones EA. Oral nalmefene therapy reduces scratching activity due to the pruritus of cholestasis: a controlled study. J Am Acad Dermatol 1999;41:431-434.
177. Terg R, Coronel E, Sorda J, Munoz AE, Findor J. Efficacy and safety of oral naltrexone treatment for pruritus of cholestasis, a crossover, double blind, placebo-controlled study. J Hepatol 2002;37:717-722. (Pubitemid 35404208)
178. Mansour-Ghanaei F, Taheri A, Froutan H, Ghofrani H, Nasiri-Toosi M, Bagherzadeh AH, et al. Effect of oral naltrexone on pruritus in cholestatic patients. World J Gastroenterol 2006;12:1125-1128.
179. Bergasa NV, Schmitt JM, Talbot TL, Alling DW, Swain MG, Turner ML, et al. Open-label trial of oral nalmefene therapy for the pruritus of cholestasis. HEPATOLOGY 1998;27:679-684.
180. Carson KL, Tran TT, Cotton P, Sharara AI, Hunt CM. Pilot study of the use of naltrexone to treat the severe pruritus of cholestatic liver disease. Am J Gastroenterol 1996;91:1022-1023. (Pubitemid 26143548)
181. Krystal JH, Cramer JA, Krol WF, Kirk GF, Rosenheck RA. Naltrexone in the treatment of alcohol dependence. N Engl J Med 2001;345:1734-1739.
182. Mitchell JE. Naltrexone and hepatotoxicity. Lancet 1986;i:1215.
183. Bertolotti M, Ferrari A, Vitale G, Stefani M, Trenti T, Loria P, et al. Effect of liver cirrhosis on the systemic availability of naltrexone in humans. J Hepatol 1997;27:505-511. (Pubitemid 27390898)
184. McRae CA, Prince MI, Hudson M, Day CP, James, OF, Jones DE. Pain as a complication of use of opiate antagonists for symptom control in cholestasis. Gastroenterology 2003;125:591-596. (Pubitemid 36929440)
185. Jones EA. Pruritus and fatigue associated with liver disease: is there a role for ondansetron? Expert Opin Pharmacother 2008;9:645-651.
186. Raderer M, Muller C, Scheithauer W. Ondansetron for pruritus due to cholestasis. N Engl J Med 1994;21:1540.
187. Schworer H, Ramadori G. Improvement of cholestatic pruritus by ondansetron. Lancet 1993;341:1277.
188. Jones EA, Molenaar HA, Oosting J. Ondansetron and pruritus in chronic liver disease: a controlled study. Hepatogastroenterology 2007;54:1196-1199. (Pubitemid 47013934)
189. Zylicz Z, Krajnik M, Sorge AA, Costantini M. Paroxetine in the treatment of severe non-dermatological pruritus: a randomized, controlled trial. J Pain Symptom Manage 2003;26:1105-1112. (Pubitemid 37488018)
190. Mayo MJ, Handem I, Saldana S, Jacobe H, Getachew Y, Rush AJ. Sertraline as a first-line treatment for cholestatic pruritus. HEPATOLOGY 2007;45:666-674. (Pubitemid 46450618)
191. Rishe E, Azarm A, Bergasa NV. Itch in primary biliary cirrhosis: a patient's perspective. Acta Derm Venereol 2008;88:34-37. (Pubitemid 351212133)
192. Greaves MW. Antihistamines in dermatology. Skin Pharmacol Physiol 2005;18:220-229.
193. Elias E. Liver Transplantation. J R Coll Physicians Lond 1993;27:224-232.
194. Neuberger J, Jones EA. Liver transplantation for intractable pruritus is contraindicated before an adequate trial of opiate antagonist therapy. Eur J Gastroenterol Hepatol 2001;13:1393-1394. (Pubitemid 33111990)
195. Tatlipinar S, Akpek EK. Topical ciclosporin in the treatment of ocular surface disorders. Br J Ophthalmol 2005;89:1363-1367. (Pubitemid 41638882)
196. Meadows M. Dealing with dry eye. In: FDA Consumer Magazine; May- June 2005. http://www.fda.gov/fdac/features/2005/305-eye.html
197. Richards A, Rooney J, Prime S, Scully C. Primary biliary cirrhosis. Sole presentation with rampant dental caries. Oral Surg Oral Med Oral Pathol 1994;77:16-18.
198. Mavragani CP, Moutsopoulos HM. Conventional therapy of Sjogren's Syndrome. Clin Rev Allergy Immunol 2007;32:284-291.
199. Mang FW, Michieletti P, O'Rourke K, Cauch-Dudek K, Diamant N, Bookman A, et al. Primary biliary cirrhosis, sicca complex, and dysphagia. Dysphagia 1997;12:167-170. (Pubitemid 27285481)
200. Lazaridis KN, Juran BD, Boe GM, Slusser JP, de Andrade M, Homburger HA, et al. Increased prevalence of antimitochondrial antibodies in first-degree relatives of patients with primary biliary cirrhosis. HEPATOLOGY 2007;46:785-792.
201. Jones DE, Metcalf JV, Collier JD, Bassendine MF, James OF. Hepatocellular carcinoma in primary biliary cirrhosis and its impact on outcomes. HEPATOLOGY 1997;26:1138-1142. (Pubitemid 27509294)
202. Nijhawan PK, Thernau TM, Dickson ER, Boynton J, Lindor KD. Incidence of cancer in primary biliary cirrhosis: The Mayo experience. HEPATOLOGY 1999;29:1396-1398. (Pubitemid 29211039)
203. Suzuki A, Lymp J, Donlinger J, Mendes F, Angulo P, Lindor K. Clinical predictors for hepatocellular carcinoma in patients with primary biliary cirrhosis. Clin Gastroenterol Hepatol 2007;5:259-264. (Pubitemid 46216931)
204. Bruix J, Sherman M, Practice Guideline Committee, American Association for the Study of Liver Diseases. Management of hepatocellular carcinoma. HEPATOLOGY 2005;42:1208-1236.
205. Levy C, Zein CO, Gomez J, Soldevila-Pico C, Firpi R, Morelli G, et al. Prevalence and predictors of esophageal varices in patients with primary biliary cirrhosis. Clin Gastroenterol Hepatol 2007;5:803-808. (Pubitemid 47031056)
206. Bressler B, Pinto R, El-Ashry D, Heathcote EJ. Which patients with primary biliary cirrhosis or primary sclerosing cholangitis should undergo endoscopic screening for oesophageal varices detection? Gut 2005;54:407-410.
207. Kew MC, Varma RR, Dos Santos HA, Scheuer PJ, Sherlock S. Portal hypertension in primary biliary cirrhosis. Gut 1971;12:830-834.
208. Abraham SC, Kamath PS, Eghtesad B, Demetris AJ, Krasinskas AM. Liver transplantation in precirrhotic biliary tract disease: Portal hypertension is frequently associated with nodular regenerative hyperplasia and obliterative portal venopathy. Am J Surg Pathol 2006;30:1454-1461. (Pubitemid 44655482)
209. Garcia-Tsao G, Sanyal AJ, Grace ND, Carey W. Prevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis. HEPATOLOGY 2007;46:922-938. (Pubitemid 47436138)
210. Zein CO, Jorgensen RA, Clarke B, Wenger DE, Keach JC, Angulo P, et al. Alendronate improves bone mineral density in primary biliary cirrhosis: a randomized placebo-controlled trial. HEPATOLOGY 2005;42:762-771. (Pubitemid 41401113)
211. Lindor KD, Jorgensen RA, Tiegs RD, Khosla S, Dickson ER. Etidronate for osteoporosis in primary biliary cirrhosis: a randomized trial. J Hepatol 2000;33:878-882.
212. Guanabens N, Pares A, Ros I, Alvarez L, Pons F, Caballeria L, et al. Alendronate is more effective than etidronate for increasing bone mass in osteopenic patients with primary biliary cirrhosis. Am J Gastroenterol 2003;98:2268-2271. (Pubitemid 37305369)
213. Boone RH, Cheung AM, Girlan LM, Heathcote EJ. Osteoporosis in primary biliary cirrhosis: a randomized trial of the efficacy and feasibility of estrogen/progestin. Dig Dis Sci 2006;51:1103-1112. (Pubitemid 44127061)
214. Ritzel Y, Leonhardt U, Nather M, Schafer G, ArmstrongWW,Ramadori G. Simvastatin in primary biliary cirrhosis: effects on serum lipds and distinct disease markers. J Hepatol 2002;36:454-458. (Pubitemid 34407331)
215. Sorokin A, Brown JL, Thompson PD. Primary biliary cirrhosis, hyperlipidemia, and atheroscloertic risk: a systemic review. Atherosclerosis 2007:194:293-299. (Pubitemid 47513107)
216. Chalasani N. Statins and hepatotoxicity: focus on patients with fatty liver. HEPATOLOGY 2005;41:690-695. (Pubitemid 40462927)
217. Nakamuta M, Enjoji M, Kotoh K, Shimohashi N, Tanabe Y. Long-term fibrate treatment for PBC. J Gastroenterol 2005;40:546-547.
218. Schaffner F. Paradoxical elevation of serum cholesterol by clofibrate in patients with primary biliary cirrhosis. Gastroenterology 1969;57:253-255.
219. Baldo-Enzi G, Baiocchi MR, Grotto M, Floreani AR, Zagolin M, Chiaramonte M, et al. Lipoprotein pattern and plasma lipoprotein lipase activities in patients with primary biliary cirrhosis. Dig Dis Sci 1988;33:1201-1207.
220. Sylvestre PB, Batts KP, Burgart LJ, Poterucha JJ, Wiesner RH. Recurrence of primary biliary cirrhosis after liver transplantation: histologic estimate of incidence and natural history. Liver Transpl 2003;9:1086-1093. (Pubitemid 37304465)
221. Charatcharoenwitthaya P, Pimentel S, Talwalkar JA, Enders FT, Lindor KD, Krom RA, et al. Long-term survival and impact of ursodeoxycholic acid treatment for recurrent primary biliary cirrhosis after liver transplantation. Liver Transpl 2007;13:1236-1245. (Pubitemid 47457050)
222. Neuberger J, Gunson B, Hubscher S, Nightingale P. Immunosuppression affects the rate of recurrent primary biliary cirrhosis after liver transplantation. Liver Transpl 2004;10:488-491. (Pubitemid 38443587)