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Background: Concurrent extrahepatic autoimmune disorders (CEHAID) are frequently observed in autoim- mune hepatitis (AIH). It is not clear whether there is any prognostic significance of CEHAID on AIH. The aim of this study was to examine the prognostic impact of CEHAID and the correlation with the disease severity of AIH.Methods: This study included 65 hospitalized subjects who fulfilled the accepted criteria for AIH dur- ing an 8-year period (2009–2016). All records were manually screened for presence of associated au- toimmune diseases. Disease severity of AIH was assessed by liver laboratory tests including the ratio of aspartate aminotransferase to alanine aminotransferase (AST/ALT) and liver histology. Results: Among the enrolled patients, 52 (80％) were female (median age 61 years, IQR 45–75). Fifty-six (86.2％) were classified as type-1 AIH. In 26 (40％) patients at least one additional extrahepatic autoim- mune disease was diagnosed. Thirty-four subjects were referred to our hospital because of acute presen- tation of AIH (supposed by an acute elevation of hepatic enzymes) for subsequent liver biopsy resulting in initial diagnosis of AIH. This group was stratified into 3 subgroups: (A) AIH alone ( n = 14); (B) overlap with primary biliary cirrhosis (PBC) / primary sclerosing cholangitis (PSC) ( n = 11); and (C) with CEHAID ( n = 9). AST/ALT ratio was the lowest in subgroup C (median 0.64, IQR 0.51–0.94; P = 0.023), compared to subgroup A (median 0.91, IQR 0.66–1.10) and subgroup B (median 1.10, IQR 0.89–1.36). Patients with AIH alone showed a trend to the highest grade of fibrosis (mean 2.3; 95％ CI: 1.5–3.0) with no statisti- cal significance compared to subjects with CEHAID (lowest grade of fibrosis; mean 1.5; 95％ CI: 0.2–2.8; P = 0.380) whereas the ongoing inflammation was comparable. Conclusions: AST/ALT ratio and extent of fibrosis were lower in subjects with AIH and CEHAID, compared to subjects with only AIH. Therefore, the occurrence of CEHAID might be a predictor for lower disease severity of newly diagnosed acute onset AIH, possibly caused by an earlier diagnosis or different modes of damage.