Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: A 16-year prospective study

摘要: To investigate whether sclerosing cholangitis with an autoimmune serology characteristic of hepatitis (AIH) and AIH are distinct entities, we studied 55 consecutive children clinical and/or biochemical evidence liver disease circulating antinuclear (ANA), anti-smooth muscle (SMA), liver-kidney-microsomal type 1 (LKM1) autoantibodies. They underwent biopsy, direct cholangiography, sigmoidoscopy, rectal biopsy at presentation. Twenty-eight were diagnosed as in the absence 27 (ASC) presence radiological features cholangiopathy. Twenty-six ASC 20 had ANA SMA; 8 LKM1 autoantibody. Similarities between 2 conditions included most parameters a lower frequency HLA DR4. Inflammatory bowel histological biliary changes more common ASC; coagulopathy, hypoalbuminemia, lymphocytic periportal hepatitis, DR3 AIH. Histological observed 65% 31% patients. Eighty-nine percent responded to immunosuppression. Follow-up biopsies from 17 18 patients similarly reduced inflammatory activity no progression cirrhosis. Sixteen follow-up cholangiograms 9 unchanged, while showed progressive One child ulcerative colitis developed years after At 16 (median, 7 years) presentation, all alive, including 4 who transplantation. In conclusion, prevalent childhood; cholangiography is often needed distinguish these which likely lie within same process.