622 • Hepatobiliary Pancreat Dis Int ，Vol 9，No 6 • December 15 ，2010 • www.hbpdint.com Author Affiliations: Gastroenterology and Hepatology Unit, Department of Medicine (Chong VH and Jalihal A) and Department of Pathology (Telisinghe PU), Raja Isteri Pengiran Anak Saleha (RIPAS) Hospital, Bandar Seri Begawan BA 1710, Brunei Darussalam Corresponding Author: Vui Heng Chong, MRCP, FAMS, FRCP, Gastro- enterology and Hepatology Unit, Department of Medicine, Raja Isteri Pengiran Anak Saleha (RIPAS) Hospital, Bandar Seri Begawan BA 1710, Brunei Darussalam (Tel: +673 8778218; Fax: +673 2242690; Email: chongvuih@yahoo.co.uk) © 2010, Hepatobiliary Pancreat Dis Int. All rights reserved. BACKGROUND: Primary biliary cirrhosis (PBC) is an uncom- mon autoimmune cholestatic disease that predominantly affects women. Certain human leukocyte antigens (HLAs) have been reported to be associated with susceptibility for PBC. We describe the profiles of PBC in Brunei Darussalam. METHODS: All patients with PBC (n=10) were identified from our prospective databases. The HLA profiles (n=9, PBC) were compared to controls (n=65) and patients with autoimmune hepatitis (n=13, AIH). RESULTS: All patients were women with a median age of 51 years (27-83) at diagnosis. The prevalence rate of the disease was 25.6/million-population and the estimated incidence rate varied from 0 to 10.3/million-population per year. Chinese (41.15/million) and the indigenous (42.74/million) groups had higher prevalence rates compared to Malays (22.62/ million). The prevalence among female population was 54.6/ million-population. All patients were referred for abnormal liver profiles. Five patients had symptoms at presentations: jaundice (20%), fatigue (20%), arthralgia (30%) and pruritus (20%). Serum anti-mitochondrial antibody was positive in 80% of the patients. Overlap with AIH was seen in 30%. Liver biopsies (n=8) showed stage I (n=2), II (n=4) and III (n=2) fibrosis. There were no significant differences in the HLA profiles between PBC and AIH. Compared to the controls, PBC patients had significantly more HLA class I alleles specifically B7 (P=0.003), Cw7 (P=0.002) and Cw12 (P=0.007) but not the class II alleles. At a median follow-up of 23.5 months (2 to 108), all patients were alive without evidence of disease progression. CONCLUSIONS: PBC is also a predominant female disorder in our local setting and most had mild disease. The HLA profiles of our patients were different to what have been reported. (Hepatobiliary Pancreat Dis Int 2010 ; 9: 622-628) KEY WORDS: primary biliary cirrhosis; cholestasis; chronic liver disease; human leukocyte antigen; Southeast Asian Introduction P rimary biliary cirrhosis (PBC) is a chronic cholestatic autoimmune disease of the liver of unknown etiologies that affects predominantly middle aged females usually presenting between the fifth and seventh decade. [1] It is characterized by the presence of serum anti-mitochondrial antibody (AMA) against the pyruvate dehydrogenase complex, an enzyme complex that is found in the mitochondria and the slow progressive destruction of the small bile ducts within the liver. [1] In the early stages of the disease, most patients are asymptomatic and may only have mild cholestatic liver profiles. Like many other conditions, both genetic and environmental factors have important roles in the development of PBC. [2, 3] The human leukocytes antigens (HLAs) DRB1 *08 and DRB1 * 12 have been reported to predispose to the development of PBC whereas DRB1 * 11 and DRB1 * 13 are protective according to studies in the Western populations. [4] However, other studies have not found such associations. Therefore some genetic associations may be population or ethnic specific. [5] Data on PBC in the Southeast Asia region are still limited. [6-10] We present the clinical and HLA profiles of our patients with PBC in Brunei Darussalam, a Malay predominant developing nation. Methods All the patients diagnosed and followed up for PBC were identified from three prospective databases: i) the hepatology clinics register; ii) the Department of Pathology register; and ii) the central pharmacy database. They were identified to those who were prescribed ursodeoxycholic acid (UDCA). We also contacted hepatologists at the other district hospitals who were involved in looking after patients with hepatic disorders Primary biliary cirrhosis in Brunei Darussalam Vui Heng Chong, Pemasiri Upali Telisinghe and Anand Jalihal Bandar Seri Begawan, Brunei Darussalam Original Article / Biliary