Clinical Characteristics Of Patients With Autoimmune Liver Disease Complicated With Sj(?)gren’s Syndrome

Objectives To analyze the clinical,biochemical,immunological and histological characteristics of patients with autoimmune liver disease(AILD)complicated with Sj(?)gren’s syndrome(SS),so as to provide reference for the diagnosis and treatment of AILD complicated with SS.Methods 91 patients with AILD hospitalized in Hebei General Hospital from August2016 to August 2021 were analyzed retrospectively.According to whether they were combined with SS,the patients were divided into AILD group(n=41)and AILD+SS group(n=49);The AILD group was further divided into AIH group(n=23)and PBC group(n=18),and the AILD+SS group was further divided into AIH+SS group(n=15)and PBC+SS group(n=34).Since there was only one patient with primary sclerosing cholangitis(PSC),it was not included in the statistical analysis.Collect the general information(age,gender,course of disease,combined with other extrahepatic diseases,liver cirrhosis,liver cirrhosis complications),clinical symptoms(fatigue,dry mouth/eyes,gastrointestinal symptoms,jaundice,skin pruritus,fever),serum biochemistry(blood routine,coagulation function,liver function),serum Immunology(autoantibody,immunoglobulin),liver pathology and other data were analyzed.Statistically,the comparison between the two groups with normal distribution of measurement data adopts independent sample t-test,and the comparison between the two groups with skew distribution adopts Mann Whitney U test.Counting data were used between the two groupsχ~2 test or Fisher exact probability method(when the theoretical frequency is less than 5).Results 1 Basic data:41 patients with AILD(23 cases of AIH and 18 cases of PBC)and 49 patients with AILD combined with SS(15 cases of AIH+SS and 34 cases of PBC+SS)were collected.Both of them were mainly female[AILD group(male:female=1:4.86),AILD+SS group(male:female=1:48)],and the proportion of female patients in AILD+SS group was higher than that in AILD group(98%vs.82.9%).The difference was statistically significant(P<0.05),The age of the two groups was 40-60years old(the average age of the AILD group was 58.4±17.6 years old,and the average age of the AILD+SS group was 58.1±12.0 years old).2 Distribution of AILD combined with extrahepatic diseases:compared with the AILD group,the AILD+SS group was more likely to be complicated with AITD(20.4%vs.4.9%),and the difference was statistically significant(P<0.05).3 Complications and complications of liver cirrhosis:compared with the AILD group,the incidence of ascites(6.1%vs.22%)and esophagogastric varices(10.2%vs.26.8%)in liver cirrhosis complications in the AILD+SS group was lower,and the difference was statistically significant(P<0.05),but there was no significant difference in the incidence of liver cirrhosis between the two groups(P>0.05).4 Clinical symptoms:the proportion of gastrointestinal symptoms(63.4%vs.24.5%)and jaundice(36.6%vs.12.2%)in AILD group was significantly higher than that in AILD+SS group.Compared with the AILD group,the symptoms of dry mouth/dry eye in the AILD+SS group were more prominent(59.2%vs.4.9%),and the difference was statistically significant(P<0.05).5 Serum biochemistry:albumin(ALB)in AILD+SS group was significantly higher than that in AILD group,while leukocyte count(WBC),prothrombin time(PT),activated partial thromboplastin time(APTT)and international standardized ratio(INR)in AILD+SS group were significantly lower than those in AILD group(P<0.05);Alanine aminotransferase(ALT)and aspartate aminotransferase(AST)in AIH group were significantly higher than those in PBC group,while alkaline phosphatase(ALP)andγ-Glutamyl transpeptidase(GGT)in PBC group was significantly higher than that in AIH group(P<0.05);Compared with PBC+SS group,the levels of ALT,PT and APTT in AIH+SS group were significantly higher,while GGT in AIH+SS group was lower(P<0.05).6 Immunological indexes:the positive rates of anti-mitochondrial antibody(AMA)(67.3%vs.36.6%),anti-centromeric B protein antibody(CENP-B)(34.7%vs.9.8%),SSA(32.7%vs.7.3%),SSB(22.4%vs.2.4%)and immunoglobulin M(Ig M)in AILD+SS group were significantly higher than those in AILD group(P<0.05);In addition,the positive rates of AMA(77.8%vs.4.3%)and anti-mitochondrial antibody M2 subtype(AMA-M2)(61.1%vs.4.3%)in PBC group were significantly higher than those in AIH group(P<0.05);The positive rate of AMA(94.1%vs.6.7%),AMA-M2(67.6%vs.0%)and Ig M level in PBC+SS group were significantly higher than those in AIH+SS group(P<0.05).7 Liver histological manifestations:the pathological manifestations of 6 patients with AIH were interfacial hepatitis,lymphocyte and plasma cell infiltration,central venous necrosis,etc.,which were in line with the typical pathological characteristics of AIH.Conclusions 1 AILD and AILD combined with SS are common in middle-aged women.AILD combined with SS is not uncommon.Patients can have the clinical characteristics of both AILD and SS,and PBC combined with SS is more common than AIH.2 Patients with AILD are easy to be complicated with autoimmune thyroid diseases,so they should carry out relevant laboratory examination and early intervention treatment.3 The degree of liver injury in AILD patients with SS is milder than those without SS.4 The increase of AST,ALT and Ig G,the presence of interfacial hepatitis,Lymphocyte,plasma cell infiltration and other liver pathological manifestations are helpful to the diagnosis of AIH.The positive of AMA/AMA-M2,the increase of ALP and GGT are helpful to the diagnosis of PBC.Compared with AILD,the emergence of CENP-B,SSA,SSB are helpful to the diagnosis of AILD combined SS.Figure 4;Table 10;Reference 252...