AUTOIMMUNE HEPATITIS IN CHILDREN WITH PREEXISTING LIVER DISEASE

Abstract

88 Autoimmune hepatitis (AIH) is characterized histologically by a dense mononuclear infiltrate in the portal tracts and sinusoids and serologically by the presence of non-liver specific antibodies and increased levels of immunoglobulin G (IgG) in the absence of a known etiology (Semin Liver Dis 1998; 18(3): 271-279 ). We present three cases of new onset AIH in children with preexisting liver disease. All patients had negative serology for hepatitis A, B, C, cytomegalovirus and Ebstein-Barr virus. Patient 1 is a 17 year old female with Alagille's who at age 15 years developed fever, abdominal pain and progressive jaundice with total/direct bilirubin of 7.8/6.0 mg/dl, ALT 524 IU/L, AST 357IU/L, alkaline phosphatase (AP) of 385 IU/L. Erythrocyte sedimentation rate (ESR) was 70 mm/hr and anti-nuclear antibody (ANA) titer was 1:640, antimitochondrial antibody (AMA) and anti-smooth muscle antibody (ASMA) titers were negative. Liver biopsy showed ductopenia and chronic active hepatitis with lymphoplasmacytic infiltration. Treatment with azathioprine and prednisone achieved remission. Patient 2 is a 19 year old female with cystic fibrosis liver disease, who at 17 years developed an ALT 359 IU/L, AST 409 IU/L, AP 391 IU/L and total bilirubin 2.6 mg/dl; serum IgG was 1950 mg/dl. Liver biopsy showed moderate panlobular hepatitis with numerous plasma cells and portal fibrosis. ASMA titer was 1:40, ANA 1:640, anti-DNA 1:640; AMA and anti-liver kidney microsomal antibody (anti-LKM) were negative. Prednisone and azathioprine treatment resulted in remission followed by relapse with reduction of prednisone. Patient 3 is a 25 year old female with sickle cell disease and neurological impairment who at 24 years was noted to have hepatomegaly, elevated transaminases, direct bilirubin, chronic cholecystitis and elevated IgG. She underwent open cholecystectomy and concurrent liver biopsy showed sinusoidal hemophagocytosis of sickled red blood cells, portal fibrosis and increased lobular T-lymphocytes. ESR was >130 mm/hr, ASMA titer 1:320, ANA 1:320; anti-LKM and AMA were negative. The patient expired from sepsis complicating a jejunostomy procedure and therapy for AIH was never initiated.