Primary biliary cirrhosis (PBC) is a rare autoimmune liver disease, not infrequent in the West, but hardly reported from the East. We report a case of PBC for the first time from Bangladesh. The patient was an elderly woman presenting with vague complaints. The diagnosis was confirmed by antibody testing and histopathology, whereas other possible causes of cirrhosis were also excluded.
[1]. T. Addison W. Gull 1851 On a certain affection of the skin — vitiligoidea — α plana, β tuberose Guy's Hosp. Rep. 7 265.
[2]. D. E. J. Jones F. E. Watt J. V. Metcalf et al.1999 Familial primary biliary cirrhosis revisited: a geographically based population study J. Hepatol. 30 402.
[3]. P. Mistry C. A. Seymour 1992 Primary biliary cirrhosis — from Thomas Addison to the 1990s Q. J. Med. 82 185.
[4]. M. E. Gershwin I. R. Mackay 1991 Primary biliary cirrhosis: paradigm or paradox for autoimmunity Gastroenterology 100 822.
[5]. G. Yamada I. Hodo K. Tobe et al.1990 Ultrastructural immunocytochemical analysis of lymphocytes infiltrating bile duct epithelia in primary biliary cirrhosis Hepatology 12 98.
[6]. O. M. Martinez J. C. Villanueva E. Gershwin et al.1995 Cytokine patterns and cytotoxic mediators in primary biliary cirrhosis Hepatology 31 113.
[7]. G. Ballardini R. Mirakian F. B. Bianchi et al.1984 Aberrant expression of HLA-DR antigens on bile duct epithelium in primary biliary cirrhosis: relevance to pathogenesis Lancet ii 1009.
[8]. R. G. Lee O. Epstein H. Jauregui et al.1981 Granulomas in primary biliary cirrhosis: a prognostic feature Gastroenterology 81 983.
[9]. G. H. Haydon J. Neuberger 2000 PBC: an infectious disease? Gut 47 586.
[10]. P. Buttler F. Valle J. M. T. Hamilton-Miller et al.1993 M2 mitochondrial antibodies and urinary rough mutant bacteria in patients with primary biliary cirrhosis and in patients with recurrent bacteriuria J. Hepatol. 17 408.
[11]. I. Vialgut J. Villa A. Pares et al.1994 Mycobacterium gordonae DNA in liver tissue in patients with primary biliary cirrhosis J. Hepatol. 21 87.
[12]. A. L. Mason X. Lizhe L. Guo et al.1998 Detection of retroviral antibodies in primary biliary cirrhosis and other idiopathic biliary disorders Lancet 351 1620.
[13]. M. Bruguera J. Llach J. Rodes 1992 Nonsyndromic paucity of intrahepatic bile ducts in infancy and idiopathic ductopenia in adulthood: the same syndrome? Hepatology 15 830.
[14]. C. D. Surh T. E. Roche D. J. Danner et al.1898 Antimitochondrial antibodies in primary biliary cirrhosis recognize cross-reactive epitope(s) on protein X and dihydrolipoamide acetyltransferase of pyruvate dehydrogenase complex Hepatology 10 127.
[15]. K.-L. Teoh M. J. Rowley H. Zafirakis et al.1984 Enzyme inhibitory autoantibodies to pyruvate dehydrogenase complex in primary biliary cirrhosis: applications of a semiautomated assay Hepatology 20 1220.
[16]. M. P. Manns A. Bremm P. M. Schneider et al.1991 HLA DRw8 and complement C4 deficiency as risk factors in primary biliary cirrhosis Gastroenterology 101 1367.
[17]. A. W. Lohse G. V. M. Meyer K.-H. Buschenfelde et al.1999 Characterization of the overlap syndrome of primary biliary cirrhosis (PBC) and autoimmune hepatitis: evidence for it being a hepatic form of PBC in genetically susceptible individuals Hepatology 29 1078.
[18]. S. Hall P. H. Axelsen D. E. Larson et al.1984 Systemic lupus erythromatosus developing in patients with primary biliary cirrhosis Ann. Intern. Med. 100 308.
[19]. A. Bush H. Mitchison R. Walt et al.1987 Primary biliary cirrhosis and ulcerative colitis Gastroenterology 92 2009.
[20]. C.-Y. Chen C.-L. Lu C.-F. Chiu et al.1997 Primary biliary cirrhosis associated with mixed type autoimmune haemolytic anaemia and sicca syndrome: a case report and review of the literature Am. J. Gastroenterol. 92 1547.
[21]. E. Weissman N. H. Becker 1983 Interstitial lung disease in primary biliary cirrhosis Am. J. Med. Sci. 285 21.
[22]. A. K. Burroughs I. J. Rosentein O. Epstein et al.1984 Bacteriuria and primary biliary cirrhosis Gut 25 133.
[23]. D. Howel J. V. Metcalf J. Gray et al.1999 Cancer risk in primary biliary cirrhosis: a study in northern England Gut 45 756.
[24]. P. J. Scheuer 1967 Primary biliary cirrhosis Proc. R. Soc. Med. 60 1257.
[25]. R. E. Poupon R. Poupon B. Balkau et al.1994 Ursodiol for the long-term treatment of primary biliary cirrhosis N. Engl. J. Med. 330 1342.
[26]. R. E. Poupon K. D. Lindor K. Cauch-Dudek et al.1997 Combined analysis of randomized control trials of ursodeoxycholic acid in primary biliary cirrhosis Gastroenterology 113 884.
[27]. Kumagi, T., Alswat, K., Hirschfield, G. M. et al.: New insights into autoimmune liver disease. Hepatol. Res., 2008, 1–17.
[28]. A. Pares L. Caballeria J. Rodes 2006 Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic acid Gastroenterology 130 715–720.
[29]. H. Rautianen P. Karkkainen A. Karvonen et al.2005 Budesonide combined with UDCA to improve liver histology in primary biliary cirrhosis: a three-year randomized trial Hepatology 41 747–752.
[30]. K. Ohmoto N. Yoshioka S. Yamamoto 2006 Long-term effect of bezafybrateon parameters of hepatic fibrosis in primary biliary cirrhosis J. Gastroenterol. 41 502–503.
[31]. S. Fiorucci G. Rizzo A. Donini et al.2007 Targeting farnesoid X receptor for liver and metabolic disorders Trends Mol. Med. 13 298–309.
[32]. A. G. Tzakis C. Carcassonne S. Todo et al.1989 Liver transplantation for primary biliary cirrhosis Semin. Liver Dis. 9 144.
[33]. N. Krasner P. J. Johnson B. Portman et al.1979 Hepatocellular carcinoma in primary biliary cirrhosis: report of 4 cases Gut 20 255–258.