Gastroenterology and Hepatology Unit, Department of Medicine, Raja Isteri Pengiran Anak Saleha Hospital, Bandar Seri Begawan BA 1710, Brunei Darussalam.
Hepatobiliary Pancreat Dis Int. 2010 Dec;9(6):622-8.
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.
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).
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.
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.
原发性胆汁性肝硬化（PBC）是一种罕见的自身免疫性胆汁淤积性疾病，主要影响女性。某些人类白细胞抗原（HLA）已被报道与 PBC 的易感性有关。我们描述了文莱达鲁萨兰国的 PBC 特征。
从我们的前瞻性数据库中确定了所有 PBC 患者（n=10）。将 HLA 图谱（n=9，PBC）与对照组（n=65）和自身免疫性肝炎患者（n=13，AIH）进行比较。
所有患者均为女性，诊断时的中位年龄为 51 岁（27-83 岁）。该疾病的患病率为 25.6/百万人口，估计发病率为每年 0 至 10.3/百万人口。与马来人（22.62/百万）相比，华人（41.15/百万）和土着人（42.74/百万）的患病率更高。女性人口的患病率为 54.6/百万人口。所有患者均因异常肝脏谱而被转介。有 5 名患者在就诊时有症状：黄疸（20%）、疲劳（20%）、关节炎（30%）和瘙痒（20%）。80%的患者血清抗线粒体抗体阳性。30%的患者与 AIH 重叠。8 例患者进行了肝活检（n=8），结果显示 I 期（n=2）、II 期（n=4）和 III 期（n=2）纤维化。PBC 和 AIH 之间的 HLA 图谱没有显著差异。与对照组相比，PBC 患者 HLA Ⅰ类等位基因 B7（P=0.003）、Cw7（P=0.002）和 Cw12（P=0.007）明显增加，但 HLA Ⅱ类等位基因没有明显增加。在中位随访 23.5 个月（2 至 108 个月）期间，所有患者均存活且无疾病进展证据。
PBC 在我们当地的环境中也是一种主要的女性疾病，大多数患者的病情较轻。我们患者的 HLA 图谱与已报道的不同。
