Acute presentation of autoimmune hepatitis: A multicentre study with detailed histological evaluation in a large cohort of patients

Intractable Liver and Biliary Diseases Study Group of Japan

Research output: Contribution to journalArticle

22 Citations (Scopus)

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.

Original languageEnglish
JournalJournal of Clinical Pathology
DOIs
Publication statusAccepted/In press - Apr 20 2017

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Autoimmune Hepatitis
Multicenter Studies
Emperipolesis
Chronic Hepatitis
Plasma Cells
Hepatitis
Hepatocytes
Fibrosis
Necrosis
Macrophages
Liver
Inflammation
Biopsy

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Acute presentation of autoimmune hepatitis : A multicentre study with detailed histological evaluation in a large cohort of patients. / Intractable Liver and Biliary Diseases Study Group of Japan.

In: Journal of Clinical Pathology, 20.04.2017.

Research output: Contribution to journalArticle

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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.",
author = "{Intractable Liver and Biliary Diseases Study Group of Japan} and Canh, {Hiep Nguyen} and Kenichi Harada and Hirofumi Ouchi and Yasunori Sato and Koichi Tsuneyama and Masayoshi Kage and Masayuki Nakano and Kaname Yoshizawa and Atsushi Takahashi and Masanori Abe and Kang, {Jong Hon} and Kazuhiko Koike and Ayano Inui and Tomoo Fujisawa and Akinobu Takaki and Teruko Arinaga-Hino and Takuji Torimura and Yoshiyuki Suzuki and Keiichi Fujiwara and Mikio Zeniya and Hiromasa Ohira and Atsushi Tanaka and Hajime Takikawa",
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AU - Intractable Liver and Biliary Diseases Study Group of Japan

AU - Canh, Hiep Nguyen

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AU - Ouchi, Hirofumi

AU - Sato, Yasunori

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AU - Kang, Jong Hon

AU - Koike, Kazuhiko

AU - Inui, Ayano

AU - Fujisawa, Tomoo

AU - Takaki, Akinobu

AU - Arinaga-Hino, Teruko

AU - Torimura, Takuji

AU - Suzuki, Yoshiyuki

AU - Fujiwara, Keiichi

AU - Zeniya, Mikio

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N2 - 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.

AB - 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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