Clinical characteristics and the incidence of extrahepatic autoimmune disease and malignant tumor in primary biliary cirrhosis-autoimmune hepatitis overlap syndrome / 中华消化杂志

ABSTRACT

Objective To analyze clinical pathologic characteristics of patients with primary biliary cirrhosis autoimmune hepatitis overlap syndrome (PBC AIH OS),the incidence of extrahepatic autoimmune disease,malignant tumor and the abdominal lymph node enlargement.Methods From January 2000 to January 2012,the clinical data of 49 patients with PBC AIH OS were retrospectively analyzed,which included general information,clinical manifestations,biochemical parameters,immunological parameters,liver histopathological features,the incidence of extrahepatic autoimmune disease and malignant tumor,imaging findings and the efficacy.Results Among 49 PBC-AIH OS patients,the percentage of patients between 51 and 60 years old was 59.2％ (29/49),and the mean age of onset was (57.2±8.9) years old.Female was 83.7％ (41/49).The percentage of jaundice and pruritus in the main symptoms of initial visit was 42.9％ (21/49).The serum level of alanine aminotransferase (ALT),aspartate transaminase (AST),alkaline phosphatase (ALP),γ-glutamyl transpeptadase (GGT) and total bilirubin (TBil) of 49 patients all increased.Among 31 patients who accepted IgA,IgG and IgM level test,the level of IgM increased in 58.1％ (18/31) of patients,and the level of IgG increased in 61.3％ (19/31) of patients.Ninety-eight percent of patients were antinuclear antibody (ANA) positive,6.1 ％ (3/49) were anti-smooth muscle antibody (SMA) positive,89.8％ (44/49) of patients were anti mitochondrial antibody (AMA) and or AMA-M2 positive.All patients had interface hepatitis.Forty-nine percent (24/49) of patients had both histological features of autoimmune hepatitis (AIH) and primary biliray cirrhosis (PBC).After being treated with ursodeoxycholic acid (UDCA) and immunosuppressant,the percentage of remission,incomplete response and failure was 65.3％ (32/49),26.5％ (13/49) and 8.2％ (4/49),respectively.After remission,five of six patients with medicine withdrawal relapsed.Among 49 patients,40.8％ (20/49) with extrahepatic autoimmune disease and the percentage of Sjogren's syndrome,autoimmune thyroid disease,interstitial pneumonia,systemic lupus erythematosus,psoriasis,sarcoidosis and membranous nephropathy was 16.3％ (8/49),12.2％ (6/49),4.1％ (2/49),2.0％ (1/49),2.0％ (1/49),2.0％ (1/49) and 2.0％ (1/49),respectively.Among 49 patients,14.3％ (7/49) with malignant tumor and the percentage of endometrial cancer,breast cancer,thyroid cancer and lymphoma was 4.1％ (2/49),4.1％ (2/49),4.1％ (2/49) and 2.0％ (1/ 49).Among 49 patients,abdominal ultrasound showed abnormality in 93.9％ (46/49) of patients.Abdominal lymph nodes enlargement was found in 91.8％ (45/49) of patients.Conclusions Autoimmune disease and malignant tumor should be screened when patients with PBC-AIH OS were assessed.Abdominal lymph nodes enlargement found by imaging examination may have guiding significance to the diagnosis.