抗线粒体抗体阴性原发性胆汁性肝硬化患者的临床、生化和病理学特点

Clinical,biochemical and pathological features of anti-mitochondrial antibody-negative primary biliary cirrhosis

目的分析抗线粒体抗体(AMA)阴性的原发性胆汁性肝硬化(PBC)患者的临床、生化和病理学特点。方法 76例PBC患者,将间接免疫荧光法测定AMA及酶联免疫吸附法检测AMA-M2均为阳性的患者归为AMA阳性组,均为阴性的为AMA阴性组。观察两组临床表现;检测血清学和免疫学指标,如血清总胆红素(TBil)、直接胆红素(DBil)、丙氨酸转氨酶(ALT)、天冬氨酸转氨酶(AST)、碱性磷酸酶(ALP)、γ-谷氨酰转移酶(GGT)、球蛋白(GLO)、免疫球蛋白(IgG、IgM、IgA)、抗核抗体(ANA)、抗平滑肌抗体(SMA)、抗线粒体抗体(AMA)、抗可溶性酸性磷酸化核蛋白抗体(sp100)及抗核孔膜糖蛋白抗体(gp210);行肝穿刺肝组织学检查,并进行比较。结果 76例PBC患者中,AMA阴性15例,占19.7%。AMA阴性组与AMA阳性组临床表现、肝功能各项指标、病理分期及组织学表现上比较P均>0.05。AMA阴性组、AMA阳性组IgG中位数分别为1 780、1 590 mg/L,IgM中位数分别为384.0、481.5 mg/L,GLO中位数分别为39、42 g/L,两组比较P均<0.05。AMA阴性组、AMA阳性组ANA阳性率分别为93.3%、23.0%,SMA阳性率分别为20.0%、1.6%,sp100阳性率分别为33.3%、24.6%,gp210阳性率分别为40.0%、29.5%,两组ANA、SMA阳性率比较P均<0.05,但两组sp100、gp210阳性率比较P均>0.05。结论 AMA阴性PBC的临床表现、肝功能和病理学特点与AMA阳性PBC相似,但IgG水平和SMA、ANA阳性率较高,IgM和GLO水平较低。

Objective To analyze the clinical, biochemical and pathological characteristics of anti-mitochondrial anti- body （AMA）-negative primary bihary cirrhosis （PBC）. Methods Seventy-six PBC patients were divided into two groups： AMA-positive group, in which the AMA measured by indirect immunofluorescence and AMA-M2 tested by enzyme-linked im- munosorbent assay was positive, and AMA-negative group, in which the AMA and AMA-M2 was negative. The clinical mani- festations of AMA-positive group and AMA-negative group were observed. The serological and immunological parameters such as serum total bilirubin （TBil）, direct biliruhin （ DBil）, alanine aminotransferase （ ALT）, aspartate aminotransferase （AST）, alkaline phosphatase （ALP）, ^-glutamyl transferase （GGT）, globulin （GLO）, immunoglobulins （IgG, IgM, IgA）, antinuclear antibodies （ANA）, anti-smooth muscle antibodies （SMA）, anti-mitochondrial antibody （AMA）, anti- phospho-soluble acidic nuclear protein antibody （sp100） and antinuclear antibodies pore membrane glycoprotein （gp210） were detected. All patients got liver histological examination and the results were compared. Results Among 76 PBC pa- tients, 15 cases were included in the AMA-negative group, accounting for 19.7%. There was no significant difference be- tween AMA-negative group and AMA-positive group in the clinical manifestations, liver function indicators, pathology and histology （all P 〉0.05）. Median IgG of the AMA-negative group, AMA-positive group were 1 780 and 1 590 mg/L; the me-dian IgM were 384.0 and 481.5 mg/L; and the median GLO were 39 and 42 g/L, respectively （all P 〈 0.05）. The ANA positive rates of AMA-negative group and AMA-positive group were 93.3% and 23.0%, SMA positive rates were 20.0% and 1.6%, respeetively （all P〈0. 05）. spl00 positive rates were 33.3% and 24.6%, gp210 positive rates were 40.0% and 29.5% , respectively （all P 〉 0.05 ）. Conclusions There is no significant difference between AMA-negative PBC and AMA-positive PBC in the clinical manifestations, liver function indicators, and pathology. However, the IgG, positive rates of ANA and SMA are higher, and GLO and IgA levels are lower.