原发性胆汁性胆管炎合并干燥综合征患者的临床特征

Clinical Characteristics of Patients with Primary Biliary Cholangitis Complicated with Sjogren’s Syndrome

摘要: 目的：探讨原发性胆汁性胆管炎(PBC)合并干燥综合征(SS)的临床特点。方法：对334例原发性胆汁性胆管炎(PBC)患者进行了筛查，并评估了患者的自身免疫性疾病(AIDs)总体受累程度。然后比较有无SS的PBC病例的实验室和临床结果的差异，讨论了PBC与自身免疫性疾病发病率之间的不同关联。结果：女性PBC患者合并SS的比例高于单纯PBC患者的比例。与单纯PBC患者相比，合并SS的PBC患者的抗Ro-52、抗SSA、抗SSB、抗ds-DNA、ACA、ANuA、AHA抗体阳性率更高(p < 0.05)，出现恶心、下肢水肿、脾大、食管胃静脉曲张的可能性更大(p < 0.05)。伴有SS的PBC患者ALT、AST、ALB、ALP、γ-GT、TBIL、DBIL、IBIL、IgG、IgA水平高于单纯PBC患者，而IgM水平低于单纯PBC患者(p < 0.05)。SLE、RA和HT在合并SS的PBC患者中比单纯PBC患者更常见(p < 0.05)。结论：所取得的研究结果表明，合并SS的PBC患者的预后可能优于没有SS的PBC患者，尽管前者更有可能更易合并其他自身免疫性疾病。

Abstract: Objective: To investigate the clinical characteristics of primary biliary cholangitis (PBC) complicated with Sjogren’s syndrome. Methods: In total, 334 Chinese Primary biliary cholangitis (PBC) cases were screened for autoimmune diseases (AIDs), and overall involvement was evaluated. Discrepan-cies in the laboratory and clinical achievements were then compared between PBC cases without and with SS, and the diverse associations between PBC and the incidence of AIDs were then dis-cussed. Result: The proportion of women with PBC and SS was higher than that among PBC patients alone. Relative to PBC cases, PBC cases with SS were also more possible to be positive for anti-Ro-52, anti-SSA, anti-SSB, ACA, anti-dsDNA, ANuA, and AHA antibodies (p < 0.05), and were more possible to undergo nausea, lower limb edema, splenomegaly, and esophagogastric varices (p < 0.05). The levels of ALT, AST, ALB, ALP, γ-GT, TBIL, DBIL, IBIL, IgG, and IgA were greater in PBC cases suffering from SS relative to those in patients with PBC alone, whereas the levels of IgM were lesser in the former patients (p < 0.05). The presentation of SLE, RA, and HT was more common in PBC patients with SS relative to patients with PBC alone (p < 0.05). Conclusion: The achieved findings demon-strate that the prognosis of PBC cases suffering from SS tends to be better than that of PBC patients without SS, although the former patient population is more likely to exhibit other comorbid AIDs.