Autoimmune hepatitis as the first manifestation of systemic lupus erythematosus (SLE)

摘要

In about 20% of patients with SLE, the liver is affected but autoimmune hepatitis as the initial main manifestation of SLE is rare. We report on two girls presenting with acute hepatitis with jaundice, elevated transaminases and impaired liver function.Patient 1, a 144-year-old girl, became jaundiced after two weeks of fever, abdominal pain, vomitting and malaise. At admission she had hepatomegaly, AST 432U/L, ALT 221U/L, bilirubin 8.1 mg/dl, TPZ 78%, protein 6.2g/dl, IgG normal but ANA 1:65536 (< 1:256). Virology and metabolic screening was negative. There was suspicion of Wilson's disease due to 10-fold elevated urinary copper excretion and 5-fold elevated copper concentration in liver tissue. After a few days, there was development of neurological and psychiatric symptoms as well as haemolytic anaemia, malar rush, photosensitivity and renal impairment in the course of the disease. Liver histology included: cholestasis, hepatocellular single-cell necrosis and signs of hepatocellular regeneration, no inflammatory infiltrations. Clinical symptoms and anti-ds-DNA 64 U/L (0. led to the diagnosis of SLE.