Background/Aims
There are no pathognomonic features of autoimmune hepatitis (AIH). Its diagnosis requires the exclusion of various other conditions. The aim of this study was to validate indirectly the International Autoimmune Hepatitis Group (IAHG) scoring system in diagnosing AIH. Methods: Twenty-six patients with Type 1 AIH and female patients with chronic hepatitis B (n=34), chronic hepatitis C (n=25), or toxic hepatitis (n=13) were evaluated according to 9 categories of pretreatment minimum required parameters proposed by IAHG. Aggregate scores of AIH to those of non-AIH groups, which were assessed before and after extracting the proportions of etiologic factors, were also compared and evaluated. Results: While aggregate scores of non-AIH groups, before extracting the proportions of etiologic factors, were 5.2±1.8, 5.6±1.1, and 7.4±1.2 in that order, those of AIH groups were 12.8±1.7. These were significantly higher than those of non-AIH groups (p<0.01). All patients in AIH groups and only 1 patient in a non-AIH group showed aggregate scores of more than 10. Aggregate scores after extracting the proportions of etiologic factors were more than 4 in all, except 2, patients. These should have been consistent with 10 if there were no etiologic factors in non-AIH groups. Conclusion: The IAHG scoring system might have a relatively excessive importance to the scores of categories excluding distinct etiologies from AIH. It might be difficult to differentiate AIH from chronic liver diseases of indistinct cause based on the IAHG scoring system.(Korean J Hepatol 2002;8:35-43)

Auto-immune hepatitis is a chronic necroinflammatory liver disorder that is characterized by hypergammaglobulinemia, auto-antibodies in serum, and, on histological examination, the presence of periportal hepatitis. Although it can be associated with a number of other auto-immune diseases, Sj?gren’s syndrome is rarely associated with auto-immune hepatitis. We herein report an unusual case of auto-immune hepatitis associated with primary Sj?gren’s syndrome. A 39-year-old woman was admitted to our hospital due to jaundice. Laboratory data showed negative viral hepatitis marker, increased serum IgG level, positive anti- nuclear antibody, and an increased rheumatoid factor titer. The patient had no history of taking medications and alcohol. Based on characteristic clinical features, liver biopsy findings, positive Schirmer’s test, and salivary scintigraph, she was diagnosed as having auto-immune hepatitis and Sj?gren’s syndrome. The patient achieved complete remission with steroid monotherapy.(Korean J Hepatol 2003;9:25-30)

Autoimmune hepatitis (AIH) is a chronic necroinflammatory liver disease of unknown cause associated with circulating autoantibodies and high serum globulin level. Systemic lupus erythematosus (SLE) is a disease of unknown etiology in which tissues and cells are damaged by pathogenic autoantibodies and immune complex, of SLE and autoimmune hepatitis has not been clearly defined in the past due to similarities in clinical and biochemical features. A scoring system for the diagnosis of AIH has been established, and AIH and SLE-associated hepatitis have been defined as two different entities, although both have the same autoimmune features such as polyarthralgia, hypergammaglobulinemia and circulating autoantibodies. AIH has been considered to occur infrequently in SLE. We report two cases of AIH which simultaneously satisfied the criteria of SLE.(Korean J Hepatol 2003;9:231-235)