Asymptomatic autoimmune hepatitis type 1 with normal IgG level and IgA deficiency – case report

Abstract

Introduction. Autoimmune hepatitis (AIH) in children is a chronic inflammatory liver disease with a broad clinical spectrum, ranging from isolated elevated transaminases to acute liver failure. AIH is a rare condition, with an in­creased prevalence in the last years (2.4-9.9 per 100,000 chil­dren). Case report. We present the case of a young girl, 7 years old, who was admitted to our hospital for an in­crease of liver enzymes, discovered accidentally. She was asymptomatic, and no physical signs of chronic or acute liver disease were found at admission. The laboratory pa­ra­meters revealed increased transaminases without cho­les­tasis. The viral causes of hepatitis were excluded, along with alpha-1 antitrypsin deficiency and Wilson’s disease. She had a normal level of serum IgG and a low IgA level with positive antinuclear antibodies (1:80) and anti-smooth muscle antibodies (1:320), suggestive for the diagnosis of AIH type 1 and anti-liver kidney microsomal type 1 anti­bodies. The treatment with prednisone was started, and the evolution was favorable, with the normalization of la­bo­ra­tory parameters. Conclusions. This case highlights the importance of the early recognition of this pathology and the careful evaluation of any increased serum trans­ami­nases, even in asymptomatic children.