AUTOIMMUNE HEPATITIS ASSOCIATED WITH ANTIACTIN ANTIBODIES IN CHILDRENAND ADOLESCENTS

The clinical, biochemical, morphological, and evolutive features of au toimmune hepatitis associated with serum smooth muscle antibodies of a nti-actin specificity were retrospectively analyzed in 31 children and adolescents. Cirrhosis was present at diagnosis in all but six patien ts, including nine of the 12 diagnosed within 6 months from the onset. In 15 children, one or more associated diseases of an immune-mediated mechanism were present, including chronic arthritis, sclerosing chola ngitis, inflammatory bowel disease, and cutaneous vasculitis. All pati ents were treated with prednisone and azathioprine with normalization or improvement of liver function tests: 28 children are currently aliv e after a mean follow-up of 4 years, 10 months. Treatment was interrup ted in four patients only. Two patients died of liver failure in spite of immunosuppressive therapy before the era of liver transplantation. In spite of prolonged therapy, five other patients ultimately require d liver transplantation during adolescence or early adulthood. These r esults (a) further define a group of autoimmune hepatitis in children characterized by the presence of serum anti-actin antibodies; (b) indi cate that immunosuppressive therapy improves liver function, although in most cases it must be continued for a long period to maintain remis sion; and (c) suggest that progressive liver failure may occur in earl y adulthood and may require liver transplantation.