Recurrent Severe Hepatitis of Autoimmune Origin

The autoimmune liver disease constituent conditions include autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis and IgG-4 associated cholangitis. They remain a diagnostic challenge to the practicing physician due to their close resemblance in clinical course, and laboratory and imaging findings to the vast array of other etiologies of liver injury. We report a case of recurrent severe hepatitis of autoimmune origin in a female patient. The disease course was marked by initial onset at age 39, followed by nearly four years of remission, and a second flare with a more exaggerated severity. Systemic lupus erythematosus was initially deemed as the culprit, however formal diagnostic criteria were not fulfilled and the serological findings were not reproduced at a later date. With the aim of ascertaining the underlying process, the patient underwent an extensive array of testing with regards to infectious, genetic, systemic and autoimmune disease. Positive anti-dsDNA (double stranded DNA) and an antinuclear antibody titer of 1:160 provided the strongest support for an autoimmune etiology, specifically autoimmune hepatitis or possibly an overlap syndrome. An excellent outcome was achieved via treatment with corticosteroids, ursodeoxycholic acid and plasmapheresis.