| 【Objectives】1.To analyze the clinical characteristics of AILD patients with background of PBC;2.To evaluate the efficacy of glucocorticoid combined therapy on PBC patients with features of AIH;3.To explore the influencing factors associated with the efficacy.【Methods】Through the outpatient and inpatient electronic medical record system of our hospital,461 AILD patients with background of PBC diagnosed between December 2008 and December 2019 were retrospectively enrolled in the study.According to corresponding diagnostic and classification criteria,they were divided into PBC with features of AIH group(AIH-PBC group),PBC--AIH overlap syndrome group(PBC-AIH OS group)and pure PBC group,and the clinical characteristics of AIH-PBC group were compared and analyzed.Then AIH-PBC group was divided into UDCA monotherapy group and UDCA combined glucocorticoid therapy based on different treatment methods.Clinical follow-up data were used to analyze the differences in efficacy between the two groups.Univariate analysis was used to find the factors associated with the efficacy,and multivariate Logistic regression analysis was utilized to verify.SPSS 23.0(SPSS Inc.,Chicago,IL,USA)was used for all statistical analysis,and Graphpad Prism7.0 was used to draw graphs.A two-sided P < 0.05 was considered as statistically significant between groups.【Results】1.Clinical characteristics:(1)General features: The sex ratio(male: female)of the AIH-PBC group was 1:6.4,and the mean age was 51.1±9.7 years old,showing no significant difference from PBC-AIH OS group and pure PBC group(P>0.05).The incidence of jaundice in PBC-AIH OS group was 28.0%,higher than that in AIH-PBC group(12.4%)and pure PBC group(10.7%),with statistically significant difference(P<0.05).There were no statistically significant differences in fatigue,itching,poor appetite,dry eyes and mouth,and joint pain.(2)Biochemical characteristics: The levels of ALT,AST,TBil,ALB and GLB in AIH-PBC patients were mostly between PBC-AIH OS group and pure PBC group,in which ALT and AST were lower than PBC-AIH OS group,with statistically significant differences(P<0.05).The levels of TBIL and GLB were higher than those of pure PBC group.ALB was higher than OS group and lower than PBC group,and the differences were statistically significant(P<0.05).There was no significant difference in ALP and GGT among the three groups.(3)Autoantibody and immune characteristics: The detection rates of ASMA and soluble liver antigen/liver pancrease antigen antibody(SLA/LP)in AIH-PBC group were significantly lower than that in PBC-AIH OS group.The detection rate of Ro-52 antibody was higher than that of pure PBC group(P<0.05).There was no significant difference in the detection rates of ANA,AMA,sp100 antibody and gp210 antibody in the three groups(P>0.05).IgG and Ig M levels in AIH-PBC group were higher than those in pure PBC group.(4)Ultrasonic and histological features: The incidences of cirrhosis,splenomegaly,ascites and hilar lymph node enlargement in AIH-PBC group were significantly higher than that in PBC group alone,with statistically significant differences(P<0.05).There was no significant difference in the incidence of hepatic cyst,hepatic hemangioma and gallbladder polyp(P>0.05).Hepatic histopathological examination was completed in 157 cases of AIH-PBC,50 cases of PBC-AIH OS and 184 cases of pure PBC patients at baseline time.Analysis showed that the rate of advanced grade and stage in AIH-PBC group were significantly higher than that in the group with pure PBC,with statistically significant differences(P<0.05).While there was no significant difference between AIH-PBC group and PBC-AIH OS group.2.Efficacy observation:(1)Baseline data: 145 in 178 AIH-PBC patients received UDCA monotherapy and 33 received glucocorticoid combined therapy.There was no significant difference between the two groups in gender,age,biochemical and immunological indices and pathological stage(P>0.05).(2)Treatment effect: After 1 year of treatment,biochemical data before and after treatment in UDCA monotherapy group and combined therapy group were compared,and ALT,AST,ALP,GGT and Ig M in both groups were significantly decreased(P < 0.05).There was no statistically significant difference between GLB and IgG before and after treatment(P>0.05),but GLB and IgG levels in the combined therapy group decreased after 1 year of treatment,while those in monotherapy group increased.The biochemical response of 18 patients with AIH-PBC was poor after 13.5(12.0 ～ 35.0)months of treatment with UDCA alone,while the levels of transaminases,IgG and Ig M decreased significantly after 17.0(12.0 ～ 96.0)months of combined therapy(P < 0.05).(3)Biochemical response and histological remission: The biochemical response rates of ALT,ALP and IgG in the combined therapy group were all higher than those in the UDCA monotherapy group,but the differences were not statistically significant(P > 0.05).The complete biochemical response rate of the combined therapy group was significantly higher than that of the UDCA monotherapy group(P<0.05).In terms of histology,32 patients in UDCA monotherapy group and 10 patients in combined therapy group received liver biopsy again after standard treatment for36.0(12.0 ～ 84.0)months.The histological remission rate of the combined treatment group was higher than that of the monotherapy group,but the difference was not statistically significant(P>0.05).3.Analysis of associated factors of efficacy:(1)Analysis of associated factors of biochemical response: The dependent variable was whether the complete biochemical response was achieved(0=not complete biochemical response,1=complete biochemical response),and the independent variables included age,gender,biochemical and immunological indicators,autoantibodies,treatment methods,histological grades and stages,etc.First,univariate Logistic regression was used to analyze the possible correlation factors one by one,and the test level was set as P<0.1.A total of 6 possible factors were obtained,including gender,treatment methods,ALP,IgG,Ig M and Ro-52 antibodies.Then with multivariate Logistic regression,it was found that ALP,IgG and Ro-52 antibodies were significantly associated with the complete biochemical response(P<0.05).2.Analysis of associated factors of histological remission: Whether or not histological remission was achieved was taken as the dependent variable(0=no histological remission,1=histological remission),and SSA antibody was obtained as the associated factor for histological remission(OR=0.374,95%CI: 0.194-0.719,P=0.003).【Conclusions】1.The clinical characteristics of AIH-PBC were between pure PBC and PBC-AIH OS.Its autoantibody spectrum was similar to that of pure PBC,while its biochemical profile,ultrasonic and histological manifestations were similar to those of PBC-AIH OS.2.Compared with pure PBC,AIH-PBC patients had faster disease progression and worse prognosis.3.UDCA combined with glucocorticoid could improve ALP,ALT,IgG and histological remission rates of AIH-PBC patients,but there was no statistical difference.In terms of complete biochemical response rate,combined therapy group was significantly higher than monotherapy group.4.For AIH-PBC patients with incomplete biochemical response after initial UDCA monotherapy,the combination of glucocorticoids can significantly reduce biochemical and immunological indicators.5.Baseline ALP,IgG levels and Ro-52 antibody were significantly correlated with complete biochemical response,while SSA antibody was associated with histological remission. |
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