Primary biliary cirrhosis autoimmune hepatitis overlap syndrome:: Clinical features and response to therapy
Primary biliary cirrhosis autoimmune hepatitis overlap syndrome:: Clinical features and response to therapy
复制标题
DOI：
10.1002/hep.510280203
复制
发表时间：
1998-08-01
期刊：
影响因子：
13.5
通讯作者：
Poupon, R
中科院分区：
文献类型：
--
作者：
Chazouillères, O;Wendum, D;Poupon, R
原发性胆汁性肝硬化（PBC）和自身免疫性肝炎（AIH）的关联被认为是罕见的，其最佳治疗方法是未知的。在130例确诊为PBC的连续患者中，我们确定了12例（9.2%）重叠综合征（10例女性，2例男性;中位年龄50岁），其严格定义为每种疾病的三种公认的生化、血清学和组织学标准中至少有两种存在。1例患者最初为单纯PBC，在熊去氧胆酸（UDCA）治疗20个月后发展为AIH，其特征为丙氨酸转氨酶（ALT）（1，330 IU/L; N < 35）、免疫球蛋白G（IgG）（42 g/L; N < 14.0）升高和抗平滑肌抗体（ASMA）。糖皮质激素和熊去氧胆酸联合治疗后，临床和生化症状完全缓解，11名患者在就诊时同时具有这两种疾病的特征：血清碱性磷酸酶（AP）水平高（中位数; 250 IU/L; N < 100），ALT（140 IU/L）和IgG（30.8 g/L）、存在线粒体抗体（n = 9）或ASMA（n = 9;胆管病变（n = 8），中度或重度门静脉周围或间隔周围淋巴细胞片状坏死（n = 11），5例患者单独给予UDCA（13-15 mg/kg/d）可诱导生化胆汁淤积显著降低，但ALT水平未降低，3例患者肝纤维化进展。在6例患者中单独给予皮质类固醇可诱导ALT、IgG和AP水平显著降低，但均未实现生化正常化。在UDCA或皮质类固醇治疗下，pe持续异常肝功能检查的患者接受UDCA和皮质类固醇治疗。观察到进一步明显的生化改善，所有患者均无症状。我们得出结论，在PBC患者中：1）AIH重叠综合征并不罕见; 2）AIH发作可能自发发生或在UDCA下发生; 3）大多数患者需要UDCA和皮质类固醇联合治疗才能获得完全的生化反应。重叠综合征可能是PBC患者对UDCA耐药的一个重要且未被认识的原因。
The association of primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) is thought to be rare, and its optimal treatment is unknown. Of 130 consecutive patients with a diagnosis of PBC, we identified 12 cases (9.2%) of overlap syndrome (10 females, 2 males; median age, 50 years) strictly defined by the presence of at least two of the three recognized biochemical, serological, and histological criteria of each disease. One patient had initially pure PBC and developed AIH characterized by a flare of alanine transaminase (ALT) (1,330 IU/L; N < 35), elevated immunoglobulin G (IgG) (42 g/L; N < 14.0), and presence of anti-smooth muscle antibodies (ASMA) after 20 months of ursodeoxycholic acid (UDCA) therapy. A complete clinical and biochemical remission was achieved under combination of corticosteroids and UDCA, Eleven patients had features of both diseases at presentation: high serum levels of alkaline phosphatase (AP) (median; 250 IU/L; N < 100), ALT (140 IU/L), and IgG (30.8 g/L), presence of mitochondrial antibodies (n = 9) or ASMA (n = 9;), florid bile duct lesions (n = 8), and moderate or severe periportal or periseptal lymphocytic piecemeal necrosis (n = 11), UDCA (13-15 mg/kg/d) given alone in 5 patients induced a significant decrease in biochemical cholestasis but not in ALT levels, and liver fibrosis progressed in 3 patient's. Corticosteroids given alone in 6 patients induced a significant decrease in ALT, IgG, and AP levels, but none had a biochemical normalization, The patients with pe persistently abnormal liver tests under either UDCA or corticosteroids received both UDCA and corticosteroids. A further marked biochemical improvement was observed, and all patients became asymptomatic. We conclude that, in patients with PBC: 1) overlap syndrome with AIH is not rare; 2) flares of AIH may occur either spontaneously or under UDCA; and 3) combination of UDCA and corticosteroids is required in most patients to obtain a complete biochemical response. Overlap syndrome may represent an important and unrecognized cause of resistance to UDCA in patients with PBC.