Clinical features of autoimmune liver disease complicated by abdominal lymphadenectasis

Objective To investigate the clinical features of autoimmune liver disease (AILD) complicated by abdominal lymphadenectasis (LA) , as well as the clinical significance of LA in AILD.Methods A retrospective analysis was performed for the clinical data of 252 patients who were admitted to The First Affiliated Hospital of Dalian Medical University from January 2005 to April 2016, and among these patients, 52 had autoimmune hepatitis (AIH) , 174 had primary biliary cholangitis (PBC) , and 26 had AIH-PBC overlap syndrome (AIH-PBC OS) .A total of 78 patients underwent follow-up.According to the presence or absence of LA, these patients were divided into LA group with 70 patients and non-LA (NLA) group with 182 patients.As for general information, laboratory markers, imaging findings, and follow-up results, the normally distributed continuous data were expressed as mean±SD, and the t-test was used for comparison between groups;the non-normally distributed continuous data were expressed as median, and the Wilcoxon rank sum test was used for comparison between groups.The categorical data were expressed as rates, and the chi-square test, the corrected chi-square test, or the Fisher's exact test was used for comparison of these data between groups.Results There were no significant differences between the two groups in age, sex ratio, proportion of patients who were diagnosed due to abnormal liver function found by physical examination, and incidence rates of other autoimmune diseases.The incidence rate of abdominal LA was 22% (11/52) in the AIH group, 26.4% (46/174) in the PBC group, and50% (13/26) in the AIH-PBC OS group, and the AIH-PBC OS group had a significantly higher incidence rate than the AIH group and the PBC group (χ2=7.693, P=0.021) .The LA group had significantly higher levels of alkaline phosphatase and gamma-glutamyl transpeptidase (GGT) than the NLA group (Z=2.944 and 3.169, P=0.003 and 0.002) .For the patients with PBC, the LA group had a significantly higher level of GGT than the NLA group (Z=2.136, P=0.033) ;for the patients with AIH-PBC OS, the LA group had a significantly higher level of total bilirubin than the NLA group (Z=2.121, P=0.035) ;for the patients with AIH, there were no significant differences in these indices between the LA group and the NLA group.The LA group had a higher incidence rate of abnormal imaging findings than the NLA group (97.1% vs 81.9%, χ2=9.863, P=0.002) .A total of 78 patients were followed up with a median follow-up time of 18 months.Of all patients in the LA group, 6 (23.1%) achieved complete remission, 7 (26.9%) achieved incomplete response, 1 (3.8%) experienced recurrence, and 12 (46.2%) experienced treatment failure;of all patients in the NLA group, 21 (40.4%) achieved complete remission, 17 (32.7%) achieved incomplete response, 7 (13.5%) experienced recurrence, and 7 (13.5%) experienced treatment failure;there was a significant difference in the distribution of treatment outcomes between the LA group and the NLA group (Z=2.406, P=0.016) .Conclusion Patients with AIH-PBC OS have a higher incidence rate of abdominal LA than those with AIH and PBC, and patients with AILD complicated by LA may have marked cholestasis and liver impairment and poor treatment response, suggesting that LA might be used to determine disease severity and judge prognosis.