Emergency Department, Beijing Ditan Hospital, Capital Medical University, Beijing 100015, China.
Institute of Infectious Diseases, Beijing Ditan Hospital, Capital Medical University, Beijing 100015, China.
Can J Gastroenterol Hepatol. 2021 Mar 17;2021:5557814. doi: 10.1155/2021/5557814. eCollection 2021.
Diagnosis and treatment of primary biliary cholangitis (PBC) are often complicated by hepatic and/or extrahepatic manifestations, which in turn affect the natural course and prognosis of PBC. This study evaluated the clinical characteristics and prognosis of PBC co-occurring with intrahepatic and extrahepatic autoimmune disease (AID).
Clinical data of patients with PBC who were admitted to the Beijing Ditan Hospital from September 2008 to December 2014 were retrospectively reviewed, assessed for other autoimmune diseases, and analyzed statistically. All patients received ursodeoxycholic acid (UDCA) treatment.
Data from 505 patients were evaluated. Approximately 35.0% of patients had at least one additional AID. AIDs included Sjögren's syndrome (SS; 26.3%), autoimmune hepatitis (AIH; 7.1%), rheumatoid arthritis (RA; 1.4%), hypothyroidism (0.8%), Graves's thyroiditis (0.6%), systemic lupus erythematosus (SLE; 0.4%), and Hashimoto's thyroiditis (0.2%). No differences in response rates of UDCA were found between the PBC group and the PBC-SS group or PBC complicated with AID group (both > 0.05). White blood cell (WBC, RR = 1.072, 95% CI: 1.016-1.130, =0.011), platelet counts (PLT, RR = 0.995, 95% CI: 0.992-0.998, =0.003), and prothrombin time and international normalized ratio (PT/INR, RR = 1.799, 95% CI: 1.010-3.206, =0.046) were independent prognostic factors in patients with PBC. The overall survival time of patients in PBC-AIH and PBC-SS groups was shorter than that of those with PBC ( < 0.001).
AIH was the most common in hepatic comorbidity. SS was the most frequent extrahepatic comorbidity. WBC, PLT, and PT/INR were independent prognostic factors in patients with PBC. AID coexisted with PBC impaired patients' survival.
原发性胆汁性胆管炎（PBC）的诊断和治疗常因肝内和/或肝外表现而变得复杂，这些表现反过来又影响 PBC 的自然病程和预后。本研究评估了 PBC 合并肝内和肝外自身免疫性疾病（AID）的临床特征和预后。
回顾性分析 2008 年 9 月至 2014 年 12 月北京地坛医院收治的 PBC 患者的临床资料，评估其他自身免疫性疾病，并进行统计学分析。所有患者均接受熊去氧胆酸（UDCA）治疗。
共评估了 505 例患者的数据。约 35.0%的患者至少合并一种其他 AID。AIDs 包括干燥综合征（SS；26.3%）、自身免疫性肝炎（AIH；7.1%）、类风湿关节炎（RA；1.4%）、甲状腺功能减退症（0.8%）、格雷夫斯病甲状腺炎（0.6%）、系统性红斑狼疮（SLE；0.4%）和桥本甲状腺炎（0.2%）。PBC 组与 PBC-SS 组或 PBC 合并 AID 组之间 UDCA 反应率无差异（均>0.05）。白细胞（WBC，RR=1.072，95%CI：1.016-1.130，=0.011）、血小板计数（PLT，RR=0.995，95%CI：0.992-0.998，=0.003）和凝血酶原时间及国际标准化比值（PT/INR，RR=1.799，95%CI：1.010-3.206，=0.046）是 PBC 患者的独立预后因素。PBC-AIH 和 PBC-SS 组患者的总生存时间短于 PBC 组（<0.001）。
AIH 是最常见的肝合并症，SS 是最常见的肝外合并症。WBC、PLT 和 PT/INR 是 PBC 患者的独立预后因素。AID 合并 PBC 会影响患者的生存。
