Department of Medicine, Teikyo University School of Medicine, Tokyo Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa Department of Gastroenterology and Hepatology, Keio University School of Medicine, Tokyo Clinical Research Center, NHO Nagasaki Medical Center, Omura Department of Gastroenterology and Rheumatology, Fukushima Medical University School of Medicine, Fukushima Department of Medicine, Division of Gastroenterology and Hepatology, Shinshu University School of Medicine, Matsumoto Department of Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Ehime Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama The 4th Department of Internal Medicine, Teikyo University, School of Medicine, Kanagawa Division of Gastroenterology and Hepatology, Department of Internal Medicine, Jikei University School of Medicine, Tokyo Department of Digestive and Life-Style Related Disease, Health Research Course, Human and Environmental Sciences, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.
Hepatol Res. 2011 Sep;41(9):877-86. doi: 10.1111/j.1872-034X.2011.00844.x. Epub 2011 Aug 24.
Primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) may simultaneously coexist in some patients, designated as PBC-AIH overlap syndrome. Previous studies suggest that combination therapy of ursodeoxycholic acid (UDCA) and corticosteroids may be effective. In the current study, we aimed to describe clinical features of these cases and to propose a rationale for combination treatment in PBC-AIH overlap.
We enrolled patients with PBC-AIH overlap from eight referral centers for liver diseases in Japan, and clinical, biochemical and immunological features were examined. Liver histology of all patients at diagnosis were analyzed altogether in detail. Eighty-nine and 44 patients with PBC and AIH alone were included and served as controls.
We identified 33 patients with PBC-AIH overlap. The mean follow-up period was 6.1 years. On liver histology, the HA (hepatitis activity) score was significantly higher than the CA (cholangitis activity) score (P < 0.001). At the end of the follow-up period, corticosteroids were used in 23 patients (72%), and neither liver-related death nor liver transplantation had been noted. The sensitivity and specificity of the simplified AIH scoring system for prediction of patients who required corticosteroids during clinical course was 92% and 75% in the training set (n = 17), and 91% and 80% in the validation set (n = 16) of overlap. Only 3% of PBC patients were diagnosed as having indication for corticosteroid use.
In PBC-AIH overlap, AIH-like features are dominant in liver histology. The simplified AIH scoring system could predict patients who needed corticosteroids with a higher specificity.
原发性胆汁性肝硬化（PBC）和自身免疫性肝炎（AIH）在一些患者中可能同时存在，被称为 PBC-AIH 重叠综合征。先前的研究表明，熊去氧胆酸（UDCA）和皮质类固醇的联合治疗可能有效。在本研究中，我们旨在描述这些病例的临床特征，并提出 PBC-AIH 重叠综合征联合治疗的原理。
我们从日本 8 个肝病转诊中心招募了 PBC-AIH 重叠患者，并检查了其临床、生化和免疫学特征。所有患者的肝组织学均在诊断时进行了详细分析。还纳入了 89 例 PBC 患者和 44 例 AIH 患者作为对照。
我们共鉴定出 33 例 PBC-AIH 重叠患者。平均随访时间为 6.1 年。在肝组织学上，HA（肝炎活动）评分明显高于 CA（胆管炎活动）评分（P<0.001）。在随访期末，23 例（72%）患者使用了皮质类固醇，未发生与肝脏相关的死亡或肝移植。简化的 AIH 评分系统预测重叠患者在临床过程中需要皮质类固醇的敏感性和特异性在训练组（n=17）分别为 92%和 75%，在验证组（n=16）分别为 91%和 80%。仅有 3%的 PBC 患者被诊断为需要皮质类固醇治疗。
在 PBC-AIH 重叠中，肝组织学上以 AIH 样特征为主。简化的 AIH 评分系统可以预测需要皮质类固醇的患者，特异性更高。
