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| Overlap of concurrent extrahepatic autoimmune diseases is associated with milder disease severity of newly diagnosed autoimmune hepatitis |
| Tobias Mühling a , Helmut Rohrbach b , Wolfgang Schepp c , Felix Gundling c , d , ∗ |
a Department of Gastroenterology, Internal Medicine II, University of Würzburg, Würzburg, Germany
b Department of Pathology, Academic Teaching Hospital Bogenhausen, Technical University of Munich, Munich 81925, Germany
c Department of Gastroenterology, Hepatology and Gastrointestinal Oncology, Academic Teaching Hospital Bogenhausen, Technical University of Munich, Munich 81925, Germany
d Department of Internal Medicine, Division of Gastroenterology, Gastrointestinal Oncology and Diabetology, Kemperhof Koblenz, Koblenz, Germany
∗ Corresponding author at: Department of Internal Medicine, Division of Gastroenterology, Gastrointestinal Oncology and Diabetology, Kemperhof Koblenz, Koblenz, Germany
E-mail address: felix.gundling@gmx.de (F. Gundling). |
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Abstract Background: Concurrent extrahepatic autoimmune disorders (CEHAID) are frequently observed in autoimmune 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 during an 8-year period (2009–2016). All records were manually screened for presence of associated autoimmune 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 autoimmune disease was diagnosed. Thirty-four subjects were referred to our hospital because of acute presentation 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 statistical 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.
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2021, Vol. 20 
