| Objective: A retrospective analysis was conducted to analyse the clinical manifestations,laboratory findings and treatment options and prognosis of patients with primary biliary cholangitis combined with extrahepatic autoimmune diseases,and to compare them with patients with primary biliary cholangitis alone,in order to better understand this disease and to study in depth the various factors that affect its prognostic development,so as to provide early diagnosis and The aim is to better understand this disease and to study in depth the various factors affecting its prognosis,so as to provide clinical guidance recommendations for early diagnosis and treatment options for patients with primary biliary cholangitis and to improve quality of life and prognosis.Methods: From January 2012 to June 2022,406 patients diagnosed with PBC at the the First Hospital of the University of Science and Technology of China were collected.187 patients with PBC who met the criteria were included,including 81 patients with combined extrahepatic autoimmune diseases and 106 patients with PBC alone,and the patients’ consultation process was followed up and recorded.This included an analysis of the general condition of the two groups at the time of the initial consultation,the clinical symptoms and other extra-hepatic autoimmune diseases,the level of laboratory tests,the incidence of cirrhotic decompensation and the difference in the occurrence of subsequent decompensation complications,liver failure,liver cancer and other adverse liver disease events.Results:1.Of the 187 patients with primary biliary cholangitis included in this study,106 patients had primary cholangitis alone and a total of 81 patients had combined extrahepatic autoimmune diseases.The distribution of the combination was 41 cases(50.6%)of dry syndrome,26 cases(32.1%)of systemic lupus erythematosus,5 cases(5.2%)of rheumatoid arthritis,3 cases(3.7%)of Hashimoto’s thyroiditis,1 case(2.5%)of adult STILL disease,and 1 case each(1.2% each)of leukoaraiosis,ANCA-associated vasculitis,systemic sclerosis and Raynaud’s syndrome.The general condition of patients in the combined disease group was compared with that of patients in the simple group.At the first visit,patients in both groups had complaints of poor appetite,fatigue,dry eyes and mouth,gastrointestinal bleeding,jaundice,liver damage and abdominal distension,with more patients in the combined group having dry mouth and eyes as the main complaint(P < 0.05).2.When comparing the first laboratory test data between the two groups,the liver function indexes were higher in the combined disease group than in the simple group in terms of ALT and AST,and higher in the simple group than in the combined disease group in terms of GGT,ALP and bilirubin(P < 0.05).3.At the end of the study,63 patients had developed cirrhosis,of whom 20(24.7%)were in the combined disease group and 39(39.6%)were in the simple group.The probability of liver function failure in the combined disease group was lower than that in the simple group,and the difference was statistically significant(P < 0.05).4.In the distribution of autoantibodies between the combined disease group and the simple group,a total of 172 patients tested positive for AMA antibodies,of which 95(89.7%)were in the simple group and 77(95.1%)were in the combined disease group,with no difference in antibody distribution between the two groups.There was no difference in the distribution of antibodies between the two groups,and the detection rate of specific ANA antibodies in the two groups was above 50%,with a higher proportion of patients in the combined group being double positive for gp210+sp100,and the results were different(P<0.05).The incidence of cirrhosis loss was much higher in patients presenting with positive autoantibodies to gp210 than in those with negative gp210,with a positive correlation between the two.The difference was statistically significant(p<0.05).5.Through follow-up records and telephone follow-up,in the long-term survival of patients with primary biliary cholangitis included in the study was recorded,and relevant indicators were statistically analysed in relation to patient prognosis.In a oneway Cox analysis,it was found that RBC,PLT,ALP,TBIL levels,ALB levels,PT,Child-Pugh classification,and the presence of combined EHAIDs all had a predictive effect on patient survival time,(p<0.05).where the combination of EHAIDs may be a protective factor for the occurrence of adverse prognostic events in the liver.6.The above factors,included in the COX regression model again for multi-factor analysis,the results of the analysis showed that prolonged PT time and liver Child-Pugh classification score had a significant effect on the prognostic survival time of patients,the difference was statistically significant.Conclusion:Primary biliary cholangitis predominantly affects middle-aged and elderly women,and the combination of extrahepatic autoimmune diseases can reach more than 40% of patients,with dry syndrome being the most common.Patients with combined EHAIDs have a more pronounced increase in abnormal liver transaminase markers,while patients in the simple group have a more pronounced increase in abnormal cholestasis enzymatic markers.In a multifactorial Cox analysis,prolonged PT time and elevated liver Child-pugh score were independent risk factors for the development of adverse events.In the long-term treatment of patients with PBC,more attention needs to be paid to the development of complications and monitoring of changes in relevant indicators.Patients with EHAIDs should be treated with attention to changes in liver function and the dosage of hormones and immunosuppressive drugs should be adjusted.Individualised treatment protocols are advocated to reduce the probability of adverse prognostic events and to improve the quality of life of patients. |
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