Autoimmune Hepatitis Associated with Sjögrenʼs Syndrome: A Case Report

Abstract

Purpose: Introduction: Autoimmune hepatitis (AIH) is a chronic necroinflammatory liver disorder characterized by periportal hepatitis and the presence of serum autoantibodies. It can be associated with a number of other autoimmune diseases. Here,we report a case of AIH associated with Sjögren's syndrome, which is rare and seldom reported in literature. Case: A 45-year-old Northeast Chinese female was admitted to our hospital. The patient suffered from fatigue and anorexia, and had persistently high level of serum aminotransferase for twelve months. Over the last six months, she had dry eyes and dry mouth. She denied any family history or illicit drugs. On admission physical examination, mild percussion pain over the liver was identified, and no enlargement of the parotid and submandibular glands. Laboratory data revealed increased levels of alanine aminotransferase (391 U/L), aspartate aminotransferase (202 U/L), and γ-globulin. Serological testing for autoimmune displayed high titer of anti-nuclear antibodies (1/3200), and positive values of anti-SSA and anti-SSB. Histological examination showed a large number of IgG-positive lymphocytes in labial salivary gland biopsy specimen, as well as a lymphoplasmacytic interface hepatitis in liver biopsy specimen. Discussion: In this study, we demonstrated that AIH was concomitant with Sjögren's syndrome, which is rarely reported in literature. Therefore,we should pay attention to possible existence of Sjögren's syndrome when evaluating clinically symptomatic extrahepatic manifestations of AIH.