Aims Although liver biopsy is crucial to diagnose and guide treatment decisions, a detailed histological analysis of autoimmune hepatitis (AIH) with clinically acute presentations has not yet been performed. This study aimed to characterise the histological features and explore potential histological hallmarks to diagnose the acute presentation of AIH.
Methods We systematically evaluated liver specimens of 87 adult patients with acute presentation of AIH retrospectively enrolled from Japanese multicentre facilities. Each histological feature was predefined by consensus based on the diagnostic criteria.
Results Key findings were that acute presentation of AIH revealed histological features of both acute hepatitis and chronic hepatitis accompanying various degrees of fibrosis. The prominent features were lobular necrosis/inflammation (97.7%), plasma cell infiltration (96.4%), emperipolesis (89.3%), pigmented macrophages (84.5%), cobblestone appearance of hepatocytes (82.6%) and perivenular necroinflammatory activity, including centrilobular necrosis (81.4%).
Conclusions The acute presentation of AIH represents the entire histological spectrum of acute hepatitis and chronic hepatitis with various activity grades and fibrosis stages that clinically correspond to acute-onset AIH and acute exacerbation of classic AIH, respectively. Although there are no pathognomonic features for the pathological diagnosis, the prominent presence of lobular and perivenular necroinflammatory activity, pigmented macrophages and cobblestone appearance of hepatocytes in addition to the classic AIH features, such as plasma cell infiltration and emperipolesis, are useful for the pathological diagnosis of the acute presentation of AIH.
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Handling editor Cheok Soon Lee
Twitter Follow Hiep Nguyen Canh @Nguyen Canh Hiep
Contributors Each of the authors contributed to the manuscript. The concept of this study was created by the Intractable Liver and Biliary Diseases Study Group of Japan. KH, KT, MK and MN developed the protocol. KH, KT, MK, MN, HO, YS and HNC reviewed the slides, collected, analysed and interpreted the data. HNC drafted the manuscript and KH critically revised the manuscript. KY, AT, MA, J-HK, KK, AI, TF, AT, TA-H, TT, YS, KF, MZ, HO, AT and HT identified clinical cases, and collected data. All authors read and approved the final manuscript.
Funding This study was conducted and supported by the Health Labour Science Research Grants from Research on Measures for Intractable Diseases, the Intractable Hepato-Biliary Diseases Study Group in Japan and grant No. 26460416 from the Japan Society for the Promotion of Science (KH). HNC received the Japanese Government (Monbukagakusho: MEXT) Scholarship, No. 153345.
Competing interests None declared.
Patient consent Obtained.
Ethics approval Fukushima Medical University Ethics Committee (no.2099).
Provenance and peer review Not commissioned; externally peer reviewed.
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