Hiep Nguyen Canh1, Kenichi Harada1, Hirofumi Ouchi1, Yasunori Sato1, Koichi Tsuneyama2, Masayoshi Kage3, Masayuki Nakano4, Kaname Yoshizawa5, Atsushi Takahashi6, Masanori Abe7, Jong-Hon Kang8, Kazuhiko Koike9, Ayano Inui10, Tomoo Fujisawa10, Akinobu Takaki11, Teruko Arinaga-Hino12, Takuji Torimura12, Yoshiyuki Suzuki13, Keiichi Fujiwara14, Mikio Zeniya15, Hiromasa Ohira6, Atsushi Tanaka16, Hajime Takikawa16. 1. Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan. 2. Department of Pathology Lab. Med., Tokushima University, Graduate School, Tokushima, Japan. 3. Kurume University Research Center for Innovative Cancer Therapy, Kurume, Japan. 4. Division of Diagnostic Pathology, Shonan Fujisawa Tokushukai Hospital, Fujisawa, Japan. 5. Department of Gastroenterology, National Hospital Organization, Shinshu Ueda Medical Center, Ueda, Japan. 6. Department of Gastroenterology, Fukushima Medical University School of Medicine, Fukushima, Japan. 7. Department of Gastroenterology and Metabiology, Ehime University Graduate School of Medicine, Ehime, Japan. 8. Center of Gastroenterology, Teine Keijinkai Hospital, Sapporo, Japan. 9. Department of Gastroenterology and Hepatology, The Third Hospital of Jikei University School of Medicine, Tokyo, Japan. 10. Department of Pediatric Hepatology and Gastroenterology, Eastern Yokohama Hospital, Yokohama, Japan. 11. Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan. 12. Department of Medicine, Kurume University School of Medicine, Kurume, Japan. 13. Department of Hepatology, Toranomon Hospital, Tokyo, Japan. 14. Department of Gastroenterology and Nephrology, Graduate School of Medicine, Chiba University, Chiba, Japan. 15. Sanno Medical Center, International University of Health and Welfare, Tokyo, Japan. 16. Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan.
Abstract
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. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.
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. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.
Authors: Tania Franceschini; Francesco Vasuri; Paolo Muratori; Luigi Muratori; Maria Guido; Marco Lenzi; Antonia D'Errico Journal: Virchows Arch Date: 2021-06-30 Impact factor: 4.064