原发性脾脏淋巴瘤伴脾梗死1例并文献复习 - 第三军医大学学报.docVIP

原发性胆汁性肝硬化合并原发性干燥综合征1例并文献复习 杨政1， 胡勤明1 ，杨忠民1 ，袁喆2 （1.华中科技大学同济医学院附属荆州医院 感染科 湖北 荆州 434020；2.重庆医科大学附属第一医院感染科、重庆市传染病寄生虫病学重点实验室，重庆400016） [摘要] 目的 分析原发性胆汁性肝硬化合并原发性干燥综合征的临床特点，为提高临床医师对该疾病的认识。方法 报道我院1例原发性胆汁性肝硬化合并原发性干燥综合征病例，并复习相关文献。结果 通过中国知网以“原发性胆汁性肝硬化”、“原发性干燥综合征”为主题词，对1985年至2014年期间中文文献进行检索，命中181篇文献，剔除会议报道及文献综述，以2000年美国肝脏病学会制定的诊断PBC标准和2002年pSS国际诊断标准为纳入标准，纳入12篇临床研究，观察161例PBC合并pSS患者，进行临床资料分析，得出该疾病以中老年女性多见；临床表现以乏力最常见，其次为皮肤瘙痒、口干、脾脏肿大、眼干、黄疸、纳差等；实验室研究结果显示碱性磷酸酶（ALP）、谷氨酰转肽酶（GGT）、抗线粒体抗体（AMA）阳性率高达90.24%以上；免疫球蛋白IgG、IgM、IgA在诊断PBC合并pSS患者中有一定价值。结论 原发性胆汁性肝硬化合并原发性干燥综合征病例在我国并不常见，对有乏力、皮肤瘙痒、眼干、口干、黄疸、肝酶异常、AMA阳性、免疫球蛋白异常的中老年女性患者，要警惕该疾病发生可能。 关键词 胆汁性；肝硬化；干燥综合征 Primary biliary cirrhosis associated with primary Sj?grens syndrome:a case report and review of literature ,China) [Abstract] Objective To study the clinical characteristics of primary biliary cirrhosis (PBC) associated with primary Sj?grens syndrome (pSS), in order to improve the understanding of the disease for clinicians. Methods A case of primary biliary cirrhosis associated with primary Sj?grens syndrome was reported and relevant literature was also reviewed. Results Literatures with key words “primary biliary cirrhosis ”and “primary Sj?grens syndrome” were selected in China National Knowledge Infrastructure (CNKI)database from 1985 to 2014.181 results were searched, of which conference reports and literature review were excluded.Inclusion criteria of PBC was confirmed by the standard of the American Association for the Study of Liver Disease in 2000, and inclusion criteria of pSS was made by the international diagnostic criteria in 2002.Total 12 literatures including 161 patients were enrolled in this study. The study showed that the main manifestation of patients was fatigue, followed by pruritus, oral dry, splenomegaly, ocular dry, jaundice, anorexia. Laboratory results indicated that the positive rate of alkaline phosphatase (ALP), (gamma glutamyl transpeptidase) (GGT), antimitochondrial antibody (AMA) were all more than 90.24%. IgG,