Clinical Features Of Primary Biliary Cirrhosis With Autoimmune Disease Of46Patients

Objective:To retrospectively analysis the clinical features of primary biliary cirrhosis (PBC)with autoimmune disease, and compared with those merely have PBC, in order toimprove the understanding of disease and prognosis.Methods:231cases of patients with a clinical diagnosis of PBC during January2000andJanuary2014in the Gastroenterology Department of the First Affiliated Hospital ofDalian Medical University were collected. All the patients were diagnosed according tothe guidelines of PBC issued by American Association for the Study of Liver Disease,and according to the diagnostic criteria of autoimmune hepatitis(AIH), Sj gren’ssyndrome (SS) and thyroid disease in the textbook of Internal Medicine (8th edition).The complete data of60cases with PBC were available in PBC group.46cases of PBCwith autoimmune disease were available in PBC with autoimmune disease group.AIHwas diagnosed in25cases, SS in13cases, and thyroiditis in8cases. Analyze age,gender, indicators of liver function in patients with PBC.Results:1. The comparison of general informationIn this study,46cases of patients (20%) were diagnosed with autoimmune diease(PBC/AIH10.8%, PBC/SS5.6%and PBC/thyroiditis3.4%). The mean age of patientswith PBC was55.3±5.5years, and the mean age of those with autoimmune diease was56.4±3.2years. The difference in mean age was not significant (P>0.05). The mean ages of patients with PBC/AIH, PBC/SS, PBC/thyroiditis were56.1±4.2years,59.9±5.8years,55.9±4.3years, respectively. The mean age of patients with PBC/SS wassignificantly greater than PBC group (P<0.05). The mean ages of patients withPBC/AIH and PBC/thyroiditis were similar to that of PBC group (P>0.05).2. The comparison of liver functionAST and ALT levels were significantly higher in patients in PBC with autoimmunediease group than patients in PBC group (P<0.05). In addition, AST and ALT levelswere significantly higher in patients with PBC/AIH, PBC/thyroiditis than patients inPBC group(P<0.05). However, the difference in AST and ALT levels was notstatistically significant between patients with PBC/SS and those in PBC group(P>0.05).The difference in ALP, GGT and ALB, was not statistically significant between patientsin both groups (P>0.05).3. The comparison of immunoglobulin levelsThe IgG levels in PBC with autoimmune disease group and PBC group were1580±277.47mg/dL and1815±422.7mg/dL, respectively (P<0.05). In addition, the IgGlevels were1961±287.41mg/dL,1820±247.217mg/dL,1630±2432.191mg/dL inpatients with PBC/AIH, PBC/SS and PBC/thyroiditis. IgG levels were significantlyhigher in patients with PBC/AIH and PBC/SS than PBC group (P<0.05). However, thedifference in IgG level was not statistically significant between patients in PBC groupand those with PBC/thyroiditis. The difference in IgA and IgM levels seemed higherwith patients in PBC with autoimmune diease group than patients in PBC group,thedifference was not statistically significant (P>0.05).4. The distribution of immunofluorescent pattern of anti-nuclear antibody (ANA)There were18(39%) cases of patients with single pattern of ANA in PBC withautoimmune diease group,24(52.2%) cases of patients were double kinds of hybridpattern,and4(8.7%) cases of patients were three kinds of hybrid pattern. And there were27(45%)cases of patients with single pattern of ANA in PBC group,26(43.3%) cases ofpatients were double kinds of hybrid pattern,and7(11.7%) cases of patients were threekinds of hybrid pattern. Cytoplasmic granular and nuclear granular karyotypes were themain ANA patterns in PBC group and PBC with autoimmune diease group, but there was no significant difference between this two groups (P>0.05). There were9(15%)cases of patients in PBC group with cytoplasmicgranular/centromere pattern,andsignificantly more than this in PBC with autoimmune diease group (P＜0.05)Conclusion:1. The elderly were prone to PBC/SS.2.AST,ALT levels increased significantly in patients with PBC/AIH，PBC/SS.3. IgG level increased significantly in patients with PBC/AIH，PBC/thyroiditis.4. Cytoplasmic granular and nuclear granular karyotypes were the main ANApatterns in patients with PBC, cytoplasmicgranular/centromere pattern in patients ofPBC with autoimmune diease was rare.