乳糜泻合并自身免疫性肝病10例临床特征

Clinical features of celiac disease in patients with autoimmune liver disease: Report of 10 cases

目的通过分析乳糜泻(celiac disease,CD)合并自身免疫性肝病(autoimmune liver disease,AILD)的临床特点及预后,提高对CD合并AILD的诊疗水平.方法回顾性分析2012-2016年在郑州大学第一附属医院确诊的10例合并AILD的CD患者和34例单纯CD患者的临床资料,以临床转归为终点事件,比较2组患者临床资料和预后.结果2组无症状、乏力、纳差、腹痛、腹泻、口干和/或眼干差异无统计学意义,白蛋白、凝血酶原时间活动度、钠离子、钾离子、钙离子及免疫球蛋白M、γ-球蛋白、血红蛋白和麦胶特异性抗体滴度差异无统计学意义,黄疸、体质量下降、谷丙转氨酶、谷草转氨酶、谷氨酰转肽酶、碱性磷酸酶、总胆红素,免疫球蛋白G等差异有统计学意义(P<0.05).合并AILD的患者中7例(7/10)经治疗后临床症状及生化检测恢复正常,3例(3/10)死亡,34例CD患者中33例(33/34)好转,1例(1/34)死亡.2组预后差异有统计学意义(P=0.032).结论CD合并AILD并非少见,以合并原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)、自身免疫性肝炎(autoimmune hepatitis,AIH)+PBC型重叠综合征居多,其临床症状不典型,部分患者无胃肠道症状.严格无麦胶饮食(gluten-free diet,GFD)联合激素和/或硫唑嘌呤、熊去氧胆酸治疗有效;CD合并AILD和CD的临床表现、IgG水平存在差异性,CD合并AILD预后更差.

AIM To analyze the clinical features and prognosis of celiac disease（CD） in patients with autoimmune liver disease（AILD） to improve the level of diagnosis and therapy of this disorder.METHODS Forty-four patients were enrolled at the First Affiliated Hospital of Zhengzhou University from 2012 to 2016, including 10 CD patients with AILD and 34 CD patients without AILD. Clinical data and survival were evaluated and compared between the two groups.RESULTS Clinical manifestations and baseline biochemical data in the two groups were comparable（P〉 0.05）, including no symptoms, fatigue, anorexia, stomachache, diarrhea, dryness of mouth or eye, albumin, prothrombin activity, K＋, Na＋, immunoglobulin M（IgM）, γ-globulin, hemoglobin（Hb） and titers of specific antibodies. There were significant differences in the two groups with regard to jaundice, weight loss, glutamic-pyruvic transaminase（ALT）, glutamic-oxaloacetic transaminase（AST）, γ-glutamyl transferase（GGT）, alkaline phosphatase（ALP）, total bilirubin（TBil）, and immunoglobulin G（IgG）（P 〈0.05）. After treatment, 7（7/10） cases with CD plus had persistently normalized clinical, biochemical parameters, although 3（3/10） cases did not respond to the treatment and died. In the CD alone group, 33（33/34） cases had improvement after treatment, and only one patient died. The prognosis in the two groupswas significantly different（P = 0.032）.CONCLUSION CD in patients with AILD is not rare. Most of these patients have primary biliary cirrhosis（PBC） or autoimmune hepatitis（AIH） ＋ PBC overlap syndrome. The clinical manifestations of CD with AILD are quite atypical and some patients have no gastrointestinal symptoms. Gluten-free diet（GFD） with glucocorticoid, azathioprine or ursodesoxycholic acid is effective. There is diversity in clinical manifestations and IgG level in CD patients with AILD, and the prognosis is much worse in CD patients with AILD.