| Objective To analyze clinical and pathologic characteristics of autoimmune hepatitis(AIH),and provide basis for early diagnosis and therapy of AIH.Methods A retrospective study was conducted to analyze the general information,clinical manifestations,biochemical and immunological features,therapeutic reaction of 16 patients with AIH who were admitted to the Affiliated Hospital of Qing Dao Medical School from March 2004 to December 2008.Results1.General Information There were 16 patients(13 females and 3males) with the female male ratio(F-M) of 4.3:1.The medial age of onset was 46.2 years,(24 years old-65 years old) and the medial time from onset to visiting doctors was 8 months,(20 days-3 years).2.Clinical Manifestations Among the 16 patients,the main clinical manifestations were anorexia 7(43.75%),(with nausea 2),jaundice 7(43.75%),fatigue 6(37.5%), pain in hepatic region 5(31.25%),fever 4(25%),joint pain(25%), abdominal distention 2(12.5%),dry mouth2(12.5%),(with dental ulcer1) and upper gastrointestinal hemorrhage 1(6.25%);in physical examination,abdominal tenderness 5 (31.25%),hepatomegaly 4(25%),Myphy sign(+) 2(12.5%) and splenomegaly 2 (12.5%) were found.Patients complicated with autoimmune diseases out of liver accounted for 37.5%,primary biliary cirrhosis 3(18.75%),pulmonary interstitial fibrosis 2(12.5%),Sjogren Syndrome 2(12.5%),Hashimoto's thyroiditis 1(6.25%),rheumatic fever 1(6.25%),panniculitis 1(6.25%) and dermatomyositis 1(6.25%) were found.3.Liver Function and Immunological Tests Liver function tests:all indexes of the liver function showed abnormality and the markedly elevated indexes are as follows: Alanine aminotransferase(ALT ),aspartate aminotransferase(AST ), gamma-glutamyl-transpeptidase(r-GT),alkaline phosphatase(ALP),and globulin (GLB);Immunological tests:IgG was elevated in 6 patients(37.5%),IgA 3(18.75%), IgM 2(12.5%).The positive rates of anti- nuclear antibody(ANA ),anti- smooth muscle antibody(SMA),anti- mitochondria antibody(AMA),anti- R52 antibody,anti SS-A antibody,antiSS-B antibody,PCNA,J-l,Nucleosomes and Histones were 87.5%,31.25%,18.75%,37.5%,18.75%,6.25%,6.25%,6.25%,6.25%and 6.25% respectively;In addition,Blood sedimentation rate(BSR) was elevated in 8 patients(50%) and C reactive protein(CRP) was elevated in 7 patients(43.75%). 4.Viral Markers:All patients had negative viral markers(A,B,C,D,E type) except tow cases,one with HBsAb(+),HBeAb(+),HBcAb(+) and HBV-DNA<10~3copy/ml and the other with HBsAb(+) and HBcAb(+).5.Blood,Urine and Hemagglutinin Routine Tests:roughly normal.6.Tumor Markers:8 patients were examined AFP,CEA,CA199 and CA125;CA199 was elevated in 4 cases(25%),AFP was elevated in 3(18.75%),and other datas were normal.7.Imageology Tests:Ultrasonography and CT tests revealed hepatic cirrisis with splenomegaly 4(25%),fatty liver 3(18.75%),chronic cholecystitis 5(31.25%),gall bladder stone 1(6.25%),pulmonary interstitial fibrosis with pleural effusion 1 (6.25%),ovarian cyst with renal cyst1(6.25%)。8.Treatment and Response:10 patients were treated with Corticosteroids alone,3 patients were treated with combination of Corticosteroids and azathioprine and other 3 patients were treated with liver-protecting drugs alone.There were no severe side effects during the immunosuppressive therapy.Among most of the patients,the hepatic function and cardinal symptoms obviously improved at the second week of treament.Conclusion AIH patients are mainly female,who usually present with atypical manifestations.The main type of AIH is Type I,and the main detectable antibodies are ANA and/or SMA;AIH patients usually complicate with autoimmune diseases out of liver;AIH could be diagnosed by combination of autoantibody detection and clinical manifestations;Immunosuppressive therapy is effective. |
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