| Objective: To investigate and explore the clinical features of the patients with autoimmune liver disease, and therefore to provide reference data for early diagnosis and treatment. We focus on the clinical value of Ig G4 serum detection in patients with autoimmune liver disease at diagnosis, differential diagnosis and illness evaluation to provide certain reference data for clinical work of serum Ig G4 detection in the value of patients with autoimmune liver disease.Methods: Retrospective analyzing general information of 522 cases of patients with autoimmune liver disease including their clinical manifestation, autoantibodies, serum biochemistry, liver biopsy pathology analysis detection. These patients included 211 cases of autoimmune hepatitis(AIH), 148 cases of primary biliary cirrhosis(PBC), 155 cases of autoimmune hepatitis and primary biliary cirrhosis overlap syndrome(OS) and 8 cases of primary sclerosing cholangitis(PSC). Then, we choose 65 cases of patients who have been treated in our hospital more than 1 year, and have two serum specimens before and after the treatment in our laboratory specimens library. These 65 cases of patients with autoimmune liver disease included 30 cases of AIH, 25 cases of PBC and 10 cases of OS. The serum specimen of these 65 cases of patients with autoimmune liver disease were collected and detected the value of serum Ig G and IgG4 using the ELISA. Then we analyzed and compared the difference of Ig G4 and Ig G4/Ig G of three kinds of autoimmune liver disease and the correlation of clinical indicators and Ig G4 serum levels, and finally we analyzed the difference of Ig G4 and IgG4/Ig G before and after the treatment.Results:1. In the 522 patients with autoimmune liver disease 41-60 years old age group accounted for most and the ratio of male and female was about 1:6. The fatigue, anorexia and nausea of patients with AIH were far more common than the rest of the groups. Jaundice is common clinical manifestations of patients with PBC, itchy skin in patients with PSC is more common than the rest of the groups. Some patients had to begin with cirrhosis of the liver, spleen or ascites and there were more patients with PBC had liver cirrhosis when they come to our hospital for the first time than the rest of the groups.2. The serum biochemical index in the reaction of liver cell damage enzymology indexes of ALT and AST increased in AIH group more obviously, and the reaction of bile duct injury enzymology indexes of ALP and GGT index increases more obviously in PBC and PSC. The ALB in PBC group decrease more significantly than the other groups. The immunoglobulin IgG rise in AIH group was obviously and Ig M in the PBC group increased more obviously. The Ig G and Ig M of PSC group had no specificity.3. In serum autoantibodies detection, ANA positive in patients with AIH was most common followed by ASMA, p ANCA, SSA and SSB. And AMA-M2 positive was most common in patients with PBC. Some patients with PBC were only with AMA-M2 positive and without the AMA positive. The above several kinds of antibody positive can also appear in the patients with OS.4. The liver biopsy pathology detection of the group of AIH patients rarely affects bile duct and main showed as the liver tissue inflammatory cells infiltration, degeneration necrosis of liver cells and interface of hepatitis. The liver biopsy pathology detection of the patients with PBC group was obviously silting bile and small bile duct damage and hyperplasia. The histopathologic of patients with PSC main showed as bile duct surrounding edema, fibrosis around the bile duct, portal area lack of bile duct and cholestasis. The liver biopsy pathology of patients in OS group can has the characteristics of AIH and PBC or PSC at the same time.5. Autoimmune liver disease in this region offen were usually complicated with other autoimmune system diseases, including rheumatoid arthritis, thyroid disease, type 1 diabetes, systemic lupus erythematosus(SLE), Sjogren’s syndrome and ulcerative colitis. Patients with AIH were complicated with rheumatoid arthritis most commonly.There were 2 cases of the 8 patients with PSC merger in ulcerative colitis.6. In the 65 patients with autoimmune liver disease, there were 5 cases(7.7%) patients with serum Ig G4 level equal or greater than 1350 mg/l, including 1 case of patients with PBC and 4 cases were patients with AIH. In the 4 patients with AIH, 3 case was with diabetes mellitus and 1 patients with other autoimmune diseases.7. The serum Ig G4 level of three kinds of autoimmune liver disease patients was significantly and positively correlated with the serum ALP and Ig G4 / Ig G, but there was no significant correlation with other indicators.8. There was no correlation between Ig G4 serum level and different diagnosis of AILD. However the median of serum Ig G4 and Ig G4 / Ig G levels in AIH patients slightly higher than in the PBC and OS group of patients.9. The serum transaminase levels of these patients decreased significantly after treatment, but there was no statistically significant difference in the level of serum Ig G4 and Ig G4/Ig G between before and after treatment.Conclusions:1. The patients with autoimmune liver disease in southwestern China were with high-risk groups for perimenopausal women around the age of 50. AIH, PBC and OS were common and PSC was rare. Autoimmune hepatitis is given priority to with type I, in this region. The proportion of male in patients with PSC was less than other domestic reports. The illness of quite some of the autoimmune liver disease patients have been progress when they come to our hospital for the first time in this region.2. Autoimmune hepatitis in southwest in China mainly combined with rheumatoid arthritis and PSC patients ulcerative colitis ratio is less than other domestic reports. The AMA-M2 detection of the PBC patients with AMA negative is particularly important. Most of the patients with PSC were with autoantibodies pANCA positive but there was no specific serum biochemistry, antibodies and immune globulin chan ges of patients with PSC, specific imaging examination and histopathologic biopsy were very important for the diagnosis. Synthesizing the clinical manifestation, antibodies, immune globulin level, serum biochemical indexes, merging other autoimmune disease s, imaging examination and pathological biopsy was important for the diagnosis of autoimmune liver disease.3. The Ig G4 serum level of patients with AIH was a bit higher than patients with the PBC, OS. The Ig G4 serum level elevated more obviously in patients with other autoimmune diseases and metabolic disease. There was no significant correlation in Ig G4 serum levels and three different diagnosis of autoimmune liver disease, Ig G4 and Ig G4 / Ig G are not appropriate indicators of three kinds of the differential diagnosis of autoimmune liver disease temporarily. The serum Ig G4 level of three kinds of autoimmune liver disease patients was significantly related with the serum ALP suggesting that Ig G4 may participate in the bile duct injury. There was no obvious correlation between the serum Ig G4 and the improvement of disease in AILD. |
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