| Objective (s):To investigate and analyze the incidence, clinical manifestations,diagnosis, treatment and prognosis of autoimmune liver disease, and to provide reference for clinical diagnosis and treatment.Methods:The general situation, laboratory examination, imaging findings,pathologic features, treatment regimen and short-term prognosis of hospitalized patients admitted to the first hospital of Kunming Medical University from January 2006 to December 2015 were analyzed retrospectively.Results:1.General situation: A total of 614 cases of AILD, the incidence was increasing year by year, including AIH 107 cases, PBC 461 cases, PSC 11 cases, AIH-PBC 33 cases, PBC-PSC 2 cases. The proportion of male and female was 1: 7.53, aged 13～82 years, mean 55.84 ± 12.41 years old. The ratio of AIH male to female was 1: 5.29,aged 19～82 years, mean52.29± 13.92 years. PBC patients with male to female ratio of 1: 9.98, aged 13～83 years, mean 56.95 ± 11.98 years; PSC male to female ratio of 2.67: 1, aged 30～70 years, mean 56.45 ± 13.44years; AIH-PBC male to female ratio of 1: 7.25, aged27～70 years, mean 52,33 ± 10.30 years; PBC-PSC male to female ratio of 1: 1,aged 48 ～69 years, an average of 58.50± 14.85years old. The age of PBC group was statistically significant (P <0.05) in AIH group and AIH-PBC group.2.Clinical manifestations:AILD patients with common symptoms of jaundice(57.49%), fatigue (47.88%), anorexia (39.25%), abdominal distension (31.92%), a small number of patients without obvious symptoms but found abnormal liver function (8.79%). The main common signs were jaundice (53.54%), ascites (15.96%),edema (15.64%), liver palm (14.82%), hepatomegaly(13.52%). There were no specific symptoms and signs between the groups. A few AILD patients have similar immune illnesses (2.61%). 94 cases (15.31%) of AILD patients with other extrahepatic autoimmune diseases, which Sjogren’s syndrome (13.19%),hyperthyroidism or depression (3.09%) is the most common. Some patients with liver cirrhosis (28.01%), hypersplenism (25.90%), hypoproteinemia (36.97%), esophageal and gastric variceal bleeding(20.03%), hyperlipidemia (21.01%) and other diseases.3.Serum biochemical markers: The levels of ALT, AST, TB, DB, IB, TBA, ALP and GGT were higher in each group than in PBC group and AIH-PBC group. (P<0.05). The total number of AST values in the PBC group was higher than that in the AIH group and the AIH-PBC group was statistically significant (P <0.05).4.Autoantibodies:In the four groups of AIH, PBC, PSC, AIH-PBC, there were significant differences between AIH group (82.29%) and PBC (68.48%) and PSC(40.00%) in the positive rate of ANA antibody in AIH, PBC, PSC and AIH-PBC (P<0.05). There was significant difference between AIH / PBC group (89.65%) and PBC group (68.48%) and PSC group (40.00%) (P <0.05).In the positive rate of AMA-M2 antibody, the difference between AIH group (18.74%) and PBC group(53.82%) , AIH/PBC group (84.85%) and other three groups was statistically significant (P <0.05). There was significant difference between AIH group (21.56%)and PBC group (8.93%) in the positive rate of SSA-RO 60KD antibody (P <0.05). In the positive rate of LKM-1 antibody, AIH / PBC group (15.24%), PBC group (1.36%)and AIH group (4.75%) were statistically significant (P <0.05).5.Imaging examination: Of the 614 patients, 435 underwent ultrasound B,127routine CT examination, 212 underwent MRI, 155 underwent MRCP examination,5 underwent ERCP examination, the negative rate were 2.30%, 1.57%, 7.08%,64.52%, 7.08%.204 cases of endoscopy, 94 cases showed varicose veins, 43 cases of portal hypertensive gastropathy / gastroduodenal disease, 29 cases of ulcers, 21 cases associated with erosion, 10 cases with bleeding.6.Pathological examination:7.00% of patients underwent liver biopsy, a total of 43 cases:(1) In 12 cases of AIH, main appearance as the liver tissue inflammatory cells infiltration,;3 cases (25.00%) did not know the pathological stage, 1 case (8.33%)were on stage G1, 2 cases (16.67%) were on stage G2,6 cases (50.00%) were on stage G3.(2) In 24 casesof PBC, more visible non-suppurative destructive cholangitis,some visible cholestasis, bile formation;and;17 cases (70.83%) had no pathological stage, 1 case (4.17%)were on stage G1, 2 cases (8.33%) were on stageG2, 4 cases(16.67%) were on stageG3; the disease stage of7 cases (29.17%) were unknown, 1(4.17%) were on stage Ⅰ,3 (12.50%) were on stage Ⅰ-Ⅱ, 6 (25.00%)were on stageⅡ, 1 (4.17%) were on stage Ⅱ-Ⅲ, 2 cases (8.33%) were on stage Ⅲ, 3 cases(12.50%) were on stage Ⅱ-Ⅳ , 1 case (4.17%) were on stage Ⅳ.7.Treatment: Medical treatment accounted for 100%. Except for patients with immunosuppressive agents and cytotoxic drugs, AIH patients were divided into steroid treatment group (16.82%) and non-hormone therapy group (76.64%)according to whether or not hormone was used.The total number of hospital stay days in the hormone treatment group was larger than that in the non-hormone therapy group,The levels of ALT, AST, TB, DB and ALP in the hormone treatment group were significantly lower than those in the non-hormone treatment group. After treatment,the levels of ALT, AST, TB, IB, ALP, GGT were significantly decreased, the difference was statistically significant (P <0.05).After removal of the patients using immunosuppressive agents and cytotoxic drugs, PBC patients were divided into ursodeoxycholic acid treatment group (79.39%) and heroin combined with ursodeoxycholic acid treatment group (11.71%) according to the use of hormone and UDCA.The number of hospitalized days in the UDCA treatment group was lower.The overall mean number of ALT, AST and ALP treatment in ursodeoxycholic acid treatment group was lower than that before treatment, and the mean number of TB,DB, IB and TBA after treatment was higher than that before treatment (P <0.05). PSC patients were given UDCA and conventional liver treatment, of which 3 patients with hormone therapy. AIH-PBC patients, mainly to UDCA and conventional liver treatment, of which 8 patients with hormone therapy. PBC-PSC patients were given UDCA and Conventional liver treatment.Surgical, endoscopic interventional therapy accounted for 5.86%.On line TIPS were the most common (47.22%), the time of endoscopic hemostatic treatment (36.11%), underwent partial splenectomy or total resection (13.8%), splenic embolization (5.56%) for less, percutaneous transhepatic biliary drainage (PTCD) (2.78%) and liver transplantation (2.78%) rarely.8.Recurrence and hospital outcome: 614 cases of AILD cases, 57.17% of patients with recurrence records. Confirmed liver cancer in 1 cases accounted for 0.16%,consider the possibility of liver cancer accounted for 0.81%. Of all patients, 90.39%were discharged, 2.44% transferred to treatment, 1.79% died in hospital.Conclusion(s):1.The incidence of AILD patients in Yunnan Province is increasing, AIH, PBC and AIH-PBC are more common in our hospital. PSC and PBC-PSC are rare, and the high incidence of AIH, PBC and OS are 50 years old perimenopausal female, and PSC patients with a higher proportion of men.2. The diagnosis of AILD is mainly based on specific blood biochemical abnormalities, immunoglobulin, autoantibodies and liver histological features,autoantibody detectioncontribute to disease diagnosis and treatment, classification, for the diagnosis of AILD preferred method, the liver biopsy rate of suspected AILD cases in Yunnan Province is relatively low.3 AILD often combined with other extrahepatic autoimmune diseases,the condition developed to end stage, it is difficulty in treatment.4.Currently, AILD treatment is mainly treated with drug therapy. UDCA (90.72%)and hormone (15.31%) have been treated in our hospital. The application of hepatoprotective drugs is more (98.37%). Immunosuppressive agents and cytotoxic drugs are rare(2.12%). In the event of serious complications, a small number of patients are also interventional therapy or endoscopic treatment.5.AILD longer duration, easy to relapse, can develop to liver cancer, cirrhosis,but the province patients with poor follow-up, should be strengthened long-term follow-up. |
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