Autoimmune hepatitis in childhood: A 20-year experience

These in investigators retrospectively reviewed the medical records of 52 children consecutively diagnosed with autoimmune hepatitis (AIH) presenting to King's College Hospital in London, England, over the past 20 years. Their clinical, biochemical, and histologic outcome were studied to determine the natural history of the disease. Thirty-two patients with a mean age of 10 years were antinuclear and/or smooth muscle antibody (ANA/SMA) positive, and 20 children with a mean age of 7 years were liver/kidney microsomal antibody (LKM) positive. The median follow-up period was 5 years with a range of 0.3 to 19 years. Independent variables predictive of outcome were baseline bilirubin and international normalized prothrombin ratio (INR). HLA typing was performed and demonstrated a higher frequency of haplotype A1/B8/DR3/DR52a in ANA/SMA positive patients. Other causes of acute and chronic hepatitis were excluded, and hepatitis C virus testing was also performed. All patients were treated with prednisolone (and azathioprine if the liver function tests increased when the corticosteroid was tapered), resulting in a gradual remission in approximately 20% of the ANA/SMA positive patients. Penicillamine and cyclosporin were used in a few of the more recently treated patients. However, it was not possible to wean the LKM-positive children off immunosuppression. LKM-positive children were younger at presentation (median 7.4 vs. 10.5 years), had higher bilirubin and AST values, had a higher incidence of associated IgA deficiency, and were more likely to present in acute hepatic failure. The ANA/SMA-positive children were more likely to present already with cirrhosis with abnormal liver synthetic function as evidenced by decreased albumin and elevated INR, presumably following a long period of silent disease. Seventy-five percent of both groups were female. There are three clinical patterns of presentation: the first is acute hepatitis, which occurred in 29 patients, of whom 6 developed fulminant hepatic failure and encephalopathy. Four of the latter 6 patients underwent liver transplantation, one patient died within 72 hours of diagnosis while awaiting transplantation, and the remaining patient gradually went into remission with steroids. The second type of presentation was a more insidious onset of fatigue and intermittent jaundice for months or years before diagnosis. The third subgroup presented with manifestations of chronic illness with cirrhosis and its complications, particularly bleeding from esophageal varices and easy bruisability. The authors concluded that children with AIH, whether ANA/SMA positive or LKM positive, have some clinical, histologic, and biochemical differences, but were similar in severity and long-term outcome. The two independent risk factors identified as significant for death and/or transplantation were bilirubin concentration and INR at the time of diagnosis.