Wang Qi-xia, Shen Lei, Chen Xiao-yu, Qiu De-kai, Ma Xiong
Department of Gastroenterology, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai Institute of Digestive Disease, Shanghai 200001, China.
Zhonghua Gan Zang Bing Za Zhi. 2011 May;19(5):340-4. doi: 10.3760/cma.j.issn.1007-3418.2011.05.007.
To explore the clinical and pathological features of primary biliary cirrhosis (PBC) patients with negative anti-mitochondria antibody (AMA).
Two hundreds and eight PBC patients were enrolled. The clinical and histological data of the negative AMA cases were compared with the AMA/AMA-M2 positive cases.
30 out of the 208 cases (14.4%) were AMA negative patients in our study. The general status, biochemical tests and histological findings between the two groups had no significant difference (P > 0.05). The Gamma-globulin, IgG, IgM and IgA levels of AMA/AMA-M2 positive PBC patients were higher than that of the AMA negative cases (P < 0.05). The abnormal rate of cholesterol in AMA negative PBC patients was 65.4% as compared to 50.4% in AMA/AMA-M2 positive cases, no significant difference existed between (P > 0.05). Anti-nuclear antibody (ANA) was observed in 29 (96.7%) AMA negative PBC patients, including 14 (48.3%) with granular pattern, 8 (27.6%) with nuclear membrane pattern, 6 (20.7%) with kinetochore pattern and 1 (3.4%) with homogeneous pattern. AMA negative PBC patients had elevated serum ALP, GGT, IgM and cholesterol levels, and decreased serum AST, IgG and IgA levels as compared with that of autoimmune hepatitis patients (P < 0.05, respectively).
In cholestatic patients with elevated IgM and cholesterol levels, ANA positive with non-homogeneous pattern, the diagnosis of PBC should be suspected, albeit AMA negative. The clinical, biochemical and histological features of the AMA negative PBC patients were similar to classic PBC patients, but quite different from autoimmune hepatitis.
探讨抗线粒体抗体（AMA）阴性的原发性胆汁性肝硬化（PBC）患者的临床及病理特征。
纳入208例PBC患者。将AMA阴性患者的临床和组织学数据与AMA/AMA-M2阳性患者进行比较。
本研究中208例患者中有30例（14.4%）为AMA阴性患者。两组患者的一般情况、生化检查及组织学表现无显著差异（P>0.05）。AMA/AMA-M2阳性PBC患者的γ-球蛋白、IgG、IgM和IgA水平高于AMA阴性患者（P<0.05）。AMA阴性PBC患者的胆固醇异常率为65.4%，而AMA/AMA-M2阳性患者为50.4%，两者之间无显著差异（P>0.05）。29例（96.7%）AMA阴性PBC患者检测到抗核抗体（ANA），其中14例（48.3%）为颗粒型，8例（27.6%）为核膜型，6例（20.7%）为着丝点型，1例（3.4%）为均质型。与自身免疫性肝炎患者相比，AMA阴性PBC患者的血清碱性磷酸酶（ALP）、γ-谷氨酰转肽酶（GGT）、IgM和胆固醇水平升高，血清谷草转氨酶（AST）、IgG和IgA水平降低（P均<0.05）。
在IgM和胆固醇水平升高、ANA阳性且为非均质型的胆汁淤积患者中，尽管AMA阴性，也应怀疑PBC的诊断。AMA阴性PBC患者的临床、生化和组织学特征与经典PBC患者相似，但与自身免疫性肝炎有很大不同。
