原发性胆汁性肝硬化114例

Clinical features of primary biliary cirrhosis:An analysis of 114 cases

目的:总结原发性胆汁性肝硬化(primary bili-ary cirrhosis,PBC)的特征,探讨血清抗线粒体抗体(antimitochondrial antibody,AMA)及分型对疾病的诊断意义及病情评价的临床意义.方法:对114例PBC患者的临床表现、血清生化指标、血清抗体及病理学特征等进行回顾性的分析.通过在M2抗体阳性者中,根据M4及M9是否阳性进行分组,即分为单纯M2阳性者、M2-M9阳性者、M2-M4阳性者及M2-M4-M9阳性者4组,并比较各组间的血清学指标及其并发症.结果:PBC临床表现主要为乏力、黄疸、皮肤瘙痒等;生化学主要以碱性磷酸酶(alkalinity phosphatase,ALP)、-谷氨酰转肽酶(-glutamyl transpeptadase,GGT)升高为主,伴有谷丙转氨酶(alanine transaminase,ALT)、谷草转氨酶(automatic spanning tree,AST)及总胆红素(totalbilirubin,TBIL)不同程度升高;血清免疫球蛋白以IgM升高为主;血清抗体中AMA及M2型抗体对该病的诊断具有较高的特异性及敏感性.通过分组比较发现M2-M4阳性者ALP及IgM高于单纯M2阳性者,P值分别为0.010、0.014,M2-M4-M9阳性者AST、TBIL及IgG高于M2-M9阳性者,P值分别为0.039、0.016、0.039;M2-M4-M9阳性者TBIL明显高于单纯M2阳性者,P=0.023,单纯M2阳性者IgG高于M2-M9阳性者,P=0.031,上述差异均有统计学意义(P<0.05).故血清M4、M9抗体对该病的病情判断具有一定的临床意义.结论:掌握PBC的特征,做到早诊断早干预,不仅延缓病程的进展,还能改善预后.

AIM： To summarize the clinical characteristics of primary biliary cirrhosis （PBC） and to ex- plore the clinical significance of serum anti-mi- tochondrial antibodies （AMAs） in the diagnosis and assessment of severity of PBC. METHODS： A retrospective analysis of clinical manifestations, serum biochemical parameters, serum antibodies and pathological features was performed in 114 patients with PBC. M2 anti- body-positive patients （105 cases） were divided into four groups： those positive for only M2 （61 cases）, for both M2 and M9 （9 cases）, for both M2and M4 （25 cases）, and for M2, M4, and M9 （10 cases）. Serological markers and complications were compared among the four groups. RESULTS： Main clinical manifestations of PBC include fatigue, jaundice, and skin itch- ing. The levels of alkalinity phosphatase （ALP）, y-glutamyl transpeptidase （GGT）, alanine ami- notransferase （ALT）, aspartate aminotransferase （AST）, and total bilirubin （TBIL） increased to varying degrees. Serum AMA and M2 antibod- ies had a very high specificity and sensitivity in diagnosing PBC. Compared to the M2-positive group, the levels of ALP and IgM in M2-M4- positive group were higher （P = 0.010, 0.014, P 〈 0.05）. Compared to the M2-M9-positive group, the levels of AST, TBIL, and IgG in the M2- M4-M9 positive group were higher （P = 0.039, 0.016, 0.039, P 〈 0.05）. Compared to the M2-pos- itive group, the level of TBIL in the M2-M4-M9 positive group was higher （P = 0.023, P 〈 0.05）. Compared to the M2-M9 positive group, the lev- el of IgG in the M2-positive group was higher （P = 0.031, P 〈 0.05）. Thus, serum M4 and M9 levels have clinical significance in the diagnosis of PBC. CONCLUSION： Understanding the clinical char- acteristics of PBC can help improve the diagno- sis, treatment, and prognosis of this disease.