RT Journal Article
SR Electronic
T1 Acute presentation of autoimmune hepatitis: a multicentre study with detailed histological evaluation in a large cohort of patients
JF Journal of Clinical Pathology
JO J Clin Pathol
FD BMJ Publishing Group Ltd and Association of Clinical Pathologists
SP 961
OP 969
DO 10.1136/jclinpath-2016-204271
VO 70
IS 11
A1 Nguyen Canh, Hiep
A1 Harada, Kenichi
A1 Ouchi, Hirofumi
A1 Sato, Yasunori
A1 Tsuneyama, Koichi
A1 Kage, Masayoshi
A1 Nakano, Masayuki
A1 Yoshizawa, Kaname
A1 Takahashi, Atsushi
A1 Abe, Masanori
A1 Kang, Jong-Hon
A1 Koike, Kazuhiko
A1 Inui, Ayano
A1 Fujisawa, Tomoo
A1 Takaki, Akinobu
A1 Arinaga-Hino, Teruko
A1 Torimura, Takuji
A1 Suzuki, Yoshiyuki
A1 Fujiwara, Keiichi
A1 Zeniya, Mikio
A1 Ohira, Hiromasa
A1 Tanaka, Atsushi
A1 Takikawa, Hajime
A1 
YR 2017
UL http://jcp.bmj.com/content/70/11/961.abstract
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.