| Objectives:1.We collected demographic characteristics、clinical symptoms、signs、imaging examinations and laboratory examinations of 127 patients with PBC . 2.We compared the analysis of serum biochemical index changes from PBC patients at first visit, after 3months treatment, after 6 months treatment, after 1 year treatment, after 2 years treatment and after 5 years treatment. 3.We divided the 127 PBC patients into non cirrhosis group and liver cirrhosis group, anti AMA-M2 antibody positive group and anti AMA-M2 negative group, anti GP210 antibody positive group and anti GP210 antibody negative group, anti SP100 antibody positive group and anti SP100 antibody negative group,and then the changes of serum biochemical indexes before and after treatment in each group were compared and analyzed.4.We compared the Chronic Liver Disease Questionnaire(CLDQ) of normal group with non cirrhotic group and cirrhosis group to understand the quality of lifes in patients with PBC. 5.To study the risk factors associated with the survival of PBC patients.Methods:We collected 127 cases of biochemical indexes, immunological indexes, clinical symptoms and Chronic Liver Disease Questionnaire(CLDQ) at the first visit、after 3months treatment, after 6 months treatment, after 1 year treatment, after 2 years treatment and after 5 years treatment from the PBC patients who visited the Second Affiliated Hospital of Kunming Medical University in the period of December 2006 to March 2017 . We collected 80 voluntteers from the healthy center of hospital as normal group, compared with PBC patients, in order to further understand the differences of life quality between PBC population with liver cirrhosis patients. And 42 of the PBC patients whose clinical data were adequate were selected for survival analysis.Results:①Demographic characteristics and common clinical symptoms of PBC patients.The number of male patients in PBC were 21(16.5%) and 106 were female (83.5%).The proportion of men and women was about 1:5; the age of first visit was 21-73 year,and the average age was (54.5 ± 12) year. The first symptoms of 127 PBC patients were fatigue (45/127, 34.9%), jaundice (38/127, 29.5%), pruritus (25/127, 19.4%),anorexia (21/127, 16.3%), abdominal distension (20/127, 15.5%), edema of lower limbs (6/127, 4.7%), hematemesis melena / (4/53, 3.1%), and 20 patients went to the hospital due to abnormal liver function ,without any special discomfort.The clinical signs and imaging when patients first diagnosed were followed by splenomegaly(48/127, 37.8%), renal cyst (32/127, 25.2%) and hepatic cyst (23/127, 18.1%),esophageal / stomach / abdominal varices (20/127,15.7%) and ascites (15/127,11.8%).②Serum immunological features of PBC patients. In 127 patients, 37 patients with other autoimmune diseases (29.1%):20 patients with autoimmune hepatitis(15.7%), 7 patients with Sjogren’s syndrome (5.5%), 8 patients with autoimmune thyroid disease(6.3%), 2 patients with rheumatoid arthritis (1.6%). In addition, 10 patients were associated with hypertension (18.9%), 7 with diabetes mellitus (13.2%),and 1 with coronary atherosclerotic heart disease (1.9%). In 127 patients with PBC,the immunoglobulin test was done in 66 patients at first visit. The results showed that serum IgM levels were generally higher than normal, and the mean IgA levels were within the normal range. Anti mitochondrial antibody -M2 (AMA-M2) positive in 100 patients (78.7%), anti GP210 antibody positive in 51 cases (40.2%), anti SP100 antibody positive in 20 cases (15.7%).③The serum biochemical changes before and after treatment in patients with PBC. Compared with the serum biochemical changes before and after treatment in patients with PBC, the results showed that the levels of serum ALB, ALT, AST, ALP,GGT, TBA, TBIL, DBIL and TC were decreased after 2 years treatment (P < 0.05),and the decrement was most obvious after 3 months treatment(P < 0.001), but after 5 years treatment,the results showed that the levels of serum biochemical tends to deterioration.; the other chemical index showed no significant difference before and after the treatment (P > 0.05).④The changes of serum biochemical indexes in patients with PBC cirrhosis group and non cirrhosis group, anti AMA-M2 antibody positive group and anti AMA-M2 antibody negative group,anti GP210 antibody positive group and anti GP210 antibody negative group, anti SP100 antibody positive and anti SP100 antibody negative group. In the PBC with non cirrhosis group ,the levels of serum ALB, ALT, AST, ALP, GGT, TBA, TBIL and DBIL were significantly impro,ved after the treatment, the differences were statistically significant (P < 0.05), while before and after treatment the indicators in patients with liver cirrhosis showed no significant difference (P > 0.05). The serum levels of ALB, ALT, AST, ALP, GGT, TBIL and DBIL in the anti AMA-M2 antibody positive group and the AMA-M2 negative group were improved within 1 year (P < 0.05). The levels of serum ALB, ALT, AST, ALP,GGT, TBA, TBIL and DBIL in anti GP210 antibody positive group 、anti GP210 antibody negative group、anti SP100 antibody positive group and anti SP100 antibody negative group showed a better trend after the treatment of 1 year, the differences were statistically significant (P < 0.05). The levels of serum ALT, AST, ALP, GGT,TBA, TBIL, DBIL in anti GP210 antibody positive group and anti SP100 antibody positive group were higher than that of anti GP210 antibody negative group and anti SP100 antibody negative group before and after treatment,while the serum level of ALB was lower than anti GP210 antibody negative group and anti SP100 antibody positive group before and after treatment, but the above indexes between anti GP210 antibody positive group and anti GP210 antibody negative group 、 anti SP100 antibody positive group and anti SP100 antibody negative group have no significant differences(P>0.05).⑤Chronic Liver Disease Questionnaire(CLDQ) . We gave Chronic Liver Disease Questionnaire(CLDQ) to 127 patients with PBC, there were 70 patients without cirrhosis and 57 patients with cirrhosis,compared with 80 healthy people.The results of questionaire analysis showed that the life quality of the patients with PBC cirrhosis group was worse than that of the PBC with non cirrhotic group,and the life quality of the PBC group with non cirrhosis was worse than that of the normal control group, the differences were statistically significant (P < 0.001).⑥Survival analysis. Kaplain-Meier and Log-Rank single factor analysis showed that the levels of serum ALB, ALP, TBIL and DBIL from patients with PBC were related to the survival of the them. Multivariate COX model analysis showed that the prognosis of patients with PBC was negatively correlated with level of serum ALB ,and positively correlated with ALP more than 2 times the upper limit of normal(ULN) ,the RR values were 0.238, 3.248, it means that ALB < 35g/L, ALP≥2ULN can make the risk of PBC patients with poor prognosis increase 0.238 times, 3.248 times.Conclusion:1.Fatigue, jaundice and skin itching were the main clinical symptoms of patients with PBC,and the imaging examination of elderly female patients which revealed splenomegaly、ascites、esophageal and gastric varices should routine eliminate PBC.2.The levels of serum ALP, GGT, IgM significantly increased were the serum biochemical characteristics of patients with PBC, and UDCA can enormously improve the levels of serum ALB, ALT, AST, ALP, GGT, TBA, TBIL,DBIL, TC in patients with PBC.3.The UDCA treatment can effectively improve serum biochemical indexes in patients at the stage of PBC with non cirrhosis,but no significant improvement has been observed in patients at the stage of PBC with cirrhosis .4.The positive or negative of Anti-AMA-M2 antibody has nothing to do with the patient’s response to UDCA.The respond to UDCA treatmentant in anti GP210 antibody positive and anti SP100 antibody positive PBC patients is lower than that of anti GP210 antibody negative and anti SP100 antibody negative PBC patients.5.The quality of life in PBC patients is lower than that of normal people. The quality of life in patients PBC which at the stage of cirrhosis is lower than that in non cirrhotic patients with PBC.6.The levels of serum ALB, ALP, TBIL, DBIL are associated with survival of PBC patients,and the ALB <35g/L,ALP ≥2 ULN are the independent factors affected the survival rate of PBC. |
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