Department of Medicine, King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia.
Department of Medicine, King Faisal Specialist Hospital & Research Centre; Organ Transplant Center, King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia.
Saudi J Gastroenterol. 2023 Mar-Apr;29(2):135-140. doi: 10.4103/sjg.sjg_445_21.
Primary biliary cholangitis (PBC) is a progressive autoimmune cholestatic liver disease that primarily affects females more than males and may lead to end-stage liver disease. We studied baseline characteristics, and the biochemical and clinical response to ursodeoxycholic acid (UDCA), a first-line treatment option for patients with PBC, in a cohort of patients diagnosed with PBC from Saudi Arabia.
A total of 30 patients meeting the diagnostic criteria of PBC who were being followed in the King Faisal Specialist Hospital and Research Centre in Riyadh, Saudi Arabia, between January 1, 2008, and December 31, 2017, were enrolled in the study. The patients with autoimmune hepatitis, primary sclerosing cholangitis, and overlap syndromes were excluded. The baseline characteristics of the patients were recorded. The response to UDCA treatment was assessed according to the Barcelona, Paris I, Paris II, and Toronto criteria, and clinical outcomes, and biochemical changes were ascertained.
The mean age was 46 years ± 11.7 with a female gender predominance of 93% (n = 28). Pruritus was the predominant symptom reported by 90% (n = 27) of the patients. A total of 23 (77.7%) patients underwent liver biopsy and all of them showed histological features suggestive of PBC. Cirrhosis was documented in 30% (n = 9) of the patients at baseline. Overall, 86.7% (n = 26) of the patients were positive for antimitochondrial antibodies. The biochemical response rates to UDCA based on Paris I, Paris II, Barcelona, and Toronto criteria were 73.3, 40, 56.7, and 53.3%, respectively.
Over 10 years, 30 patients with PBC were diagnosed at a large referral center in Saudi Arabia. The response to UDCA treatment was inadequate.
原发性胆汁性胆管炎（PBC）是一种进行性自身免疫性胆汁淤积性肝病，主要影响女性多于男性，并可能导致终末期肝病。我们研究了基线特征，以及熊去氧胆酸（UDCA）的生化和临床反应，UDCA 是 PBC 患者的一线治疗选择，该研究纳入了来自沙特阿拉伯的 PBC 患者队列。
2008 年 1 月 1 日至 2017 年 12 月 31 日期间，在沙特阿拉伯利雅得的法赫德国王专科医院和研究中心随访的 30 名符合 PBC 诊断标准的患者入组本研究。排除自身免疫性肝炎、原发性硬化性胆管炎和重叠综合征患者。记录患者的基线特征。根据巴塞罗那、巴黎 I、巴黎 II 和多伦多标准评估 UDCA 治疗的反应，并确定临床结果和生化变化。
平均年龄为 46 岁±11.7 岁，女性占 93%（n=28）。瘙痒是 90%（n=27）患者报告的主要症状。共有 23 名（77.7%）患者进行了肝活检，所有患者均显示出提示 PBC 的组织学特征。基线时 30%（n=9）的患者有肝硬化。总的来说，86.7%（n=26）的患者抗线粒体抗体阳性。根据巴黎 I、巴黎 II、巴塞罗那和多伦多标准，UDCA 的生化反应率分别为 73.3%、40%、56.7%和 53.3%。
在过去 10 年中，沙特阿拉伯的一家大型转诊中心诊断出 30 名 PBC 患者。UDCA 治疗的反应不足。
