[Autoimmune hepatitis in children].

BACKGROUND

Autoimmune mechanisms are involved in the pathogenesis of autoimmune hepatitis and primary sclerosing cholangitis. The disorders are rare but important to diagnose, as immunomodulating therapy can prevent liver failure. Cases of "overlap syndrome" between autoimmune hepatitis and primary sclerosing cholangitis are described.

MATERIAL AND METHODS

We have studied the medical records of children diagnosed with hepatitis and cholangitis treated at Rikshospitalet University Hospital from 1986 through 2004. Symptoms, findings and disease course are presented. A general overview is given of autoimmune hepatitis, primary sclerosing cholangitis and overlap syndrome in children.

RESULTS

18 children were identified; 11 with autoimmune hepatitis, six with primary sclerosing cholangitis and one with overlap syndrome. Four of the children with primary sclerosing cholangitis had inflammatory bowel disease; two of them had ulcerative colitis and two had Crohn's disease. The patient with overlap syndrome had ulcerative colitis. Pathology was found in the liver biopsies of all the children. Sixteen patients were treated with prednisolone and azathioprine. Most of the children responded to immunomodulating therapy with normalization of liver function tests. Many relapsed while tapering steroids. None of the patients have needed a liver transplant.

CONCLUSION

It is important to diagnose children with autoimmune liver disease, as children tend to respond well to immunomodulating therapy.