Primary biliary cirrhosis in Brunei Darussalam

BACKGROUND:Primary biliary cirrhosis(PBC)is an uncommon 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.