Autoimmune hepatitis in Japan: trends in a nationwide survey

Takahashi, Atsushi; Arinaga‐Hino, Teruko; Ohira, Hiromasa; Torimura, Takuji; Zeniya, Mikio; Abe, Masanori; Yoshizawa, Kaname; Takaki, Akinobu; Suzuki, Yoshiyuki; Kang, Jong Hon; Nakamoto, Nobuhiro; Fujisawa, Tomoo; Yonemoto, Koji; Tanaka, Atsushi; Takikawa, Hajime

doi:10.1007/s00535-016-1267-0

Cited by 52 publications

(86 citation statements)

References 20 publications

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“…The presence of emperipolesis indicates a close immune interaction between lymphocytes and hepatocytes, which may subsequently induce hepatocyte apoptosis and has been proposed as an additional mechanism of autoimmune-mediated liver injury in AIH. The frequency of emperipolesis found in the present study was far higher than that of the 2015 Japanese nationwide survey,22 which was also higher than the rate reported in classic AIH 68–70. Interestingly, emperipolesis was found to be more frequent in AIH patients with acute hepatitis than in those with chronic hepatitis (21.7% vs 12.2%, p=0.02) 22.…”

Section: Discussioncontrasting

confidence: 79%

“…The frequency of emperipolesis found in the present study was far higher than that of the 2015 Japanese nationwide survey,22 which was also higher than the rate reported in classic AIH 68–70. Interestingly, emperipolesis was found to be more frequent in AIH patients with acute hepatitis than in those with chronic hepatitis (21.7% vs 12.2%, p=0.02) 22. This may be a justification for our higher frequency than that of classic AIH, and emperipolesis may have been underestimated in the 2015 Japanese nationwide survey.…”

Section: Discussioncontrasting

confidence: 79%

“…In earlier reports,16 22 34–36 52 67 CN frequency varied from 21.8% to 100% of AIH patients with acute presentations compared with 57% in the present study. The reason for this divergence is unclear, but several feasible explanations can be proposed, such as patient populations with different diagnostic criteria, differences in disease severities, differences in the histological definition of CN among reports and/or natural heterogeneity of the disease.…”

Section: Discussioncontrasting

confidence: 56%

“…These cases represented a wide histological spectrum of early-stage AIH with variously combining lesions in lobular, centrilobular and portal areas, including features of the convalescent stage of acute hepatitis. Furthermore, CN was more often observed in AIH with an acute presentation than in that with a chronic presentation (53% vs 17.5–30.7%) 22 34 51 53 68. Hofer et al 51 reported that patients with AIH and CN more frequently have an acute presentation of disease than those without CN.…”

Section: Discussionmentioning

confidence: 99%

“…Although defined as a chronic disease,4 AIH can have an acute presentation at its onset that mimics acute hepatitis of other causes (8.7–75%) 5–20. In Japanese nationwide surveys conducted in 2009 and 2015, 10.9% and 11.7%, respectively, of patients with AIH had a histological diagnosis of acute hepatitis 21 22. AIH may also present as acute severe hepatitis or even acute liver failure 16 23–30.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2017

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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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Section: Discussioncontrasting

confidence: 79%

Section: Discussioncontrasting

confidence: 79%

Section: Discussioncontrasting

confidence: 56%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

et al. 2017

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Interleukin‐33/ST2‐Mediated Inflammation Plays a Critical Role in the Pathogenesis and Severity of Type I Autoimmune Hepatitis

Abe¹,

Takahashi²,

Fujita³

et al. 2019

Hepatol Commun

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Interleukin (IL)‐33 was recently described as a new member of the IL‐1 family; members of this family have proinflammatory activity. IL‐33 and its soluble receptor ST2 (sST2) have been implicated in the pathogenesis of autoimmune diseases. This study investigated serum IL‐33 and sST2 in type I autoimmune hepatitis (AIH) and the relationship of these molecules with clinical and pathologic parameters. Subjects included 65 patients with AIH who were diagnosed in our hospital. The control population included 17 healthy individuals and 36 patients with primary biliary cholangitis (PBC). Mean age at AIH diagnosis was 55.5 years, and the male‐to‐female ratio was 6:59. Serum IL‐33 and sST2 levels were significantly higher in patients with AIH than in those with PBC or controls. Importantly, immunohistochemistry revealed high IL‐33 expression in liver sections from patients with AIH. In particular, serum IL‐33 and sST2 levels were significantly higher in acute‐onset AIH than in chronic‐onset AIH. Serum IL‐33 levels were positively correlated with serum total bilirubin (TB), alanine aminotransferase (ALT), and necroinflammatory activity in AIH. We performed multivariate logistic regression analysis and found serum IL‐33 levels to be independent factors for severe activity. Serum sST2 levels were positively correlated with serum TB and ALT and negatively correlated with serum albumin and prothrombin time in AIH. In particular, serum sST2 levels were significantly higher in severe symptoms of AIH. Serum IL‐33 and sST2 levels in patients with AIH responsive to treatment with prednisolone were significantly decreased after treatment. Interestingly, serum IL‐33 level was associated with a significantly increased risk of relapse. Conclusion: IL‐33/ST2 may play an important role in the pathogenesis and severity of AIH and may be a promising target for AIH therapy.

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Non‐alcoholic fatty liver disease in patients with autoimmune hepatitis

et al. 2018

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Background and AimThe incidence of non‐alcoholic fatty liver disease (NAFLD) is increasing all over the world. NAFLD develops in patients with liver disease, including patients with autoimmune hepatitis (AIH). NAFLD and AIH have some similar laboratory and histological findings. The aim of this study was to elucidate the characteristics of AIH patients with NAFLD.MethodsWe re‐evaluated the nationwide survey performed in Japan in 2015 of AIH patients diagnosed between 2009 and 2013.ResultsA total of 1151 subjects (144 men and 1007 women) were enrolled in the present study. The overall prevalence of NAFLD was 17.0%. Compared to AIH without NAFLD, AIH patients with NAFLD had the following characteristics: (i) low female‐to‐male ratio, (ii) older age, (iii) mild elevation in hepatobiliary enzymes, (iv) histologically progressive fibrosis and mild plasma cell infiltration or mild lobular hepatitis, (v) lower prevalence of prednisolone administration and higher prevalence of ursodeoxycholic acid administration, (vi) higher levels of hepatic enzymes and immunoglobulin G after treatment, and (vii) similar prevalence of autoimmune and malignant complications.ConclusionAIH patients with NAFLD have many features that are different from AIH patients without NAFLD. Understanding these differences is essential for the proper diagnosis and treatment of AIH patients with NAFLD.

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Autoimmune hepatitis in Japan: trends in a nationwide survey

Abstract: In the present nationwide survey of AIH patients in Japan, patients with acute hepatitis had clinical features different from those of patients with chronic hepatitis.

Cited by 52 publications

References 20 publications

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

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

Interleukin‐33/ST2‐Mediated Inflammation Plays a Critical Role in the Pathogenesis and Severity of Type I Autoimmune Hepatitis

Non‐alcoholic fatty liver disease in patients with autoimmune hepatitis

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