Long-term observation of acute-onset autoimmune hepatitis presenting clinically and radiologically as acute hepatitis

Fujiwara, Keiichi; Fukuda, Yoshihiro; Seza, Katsushi; Saito, Masaya; Yasui, Shin; Nakano, Masayuki; Yokosuka, Osamu; Kato, Naoya

doi:10.1007/s12072-018-9848-z

Cited by 12 publications

(14 citation statements)

References 30 publications

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“…Similar good treatment response and long‐term prognosis have been recently reported in a retrospective analysis of 30 Japanese patients with an acute but not severe form of the disease . Absence of ANA reactivity almost in half of our AS‐AIH patients is not a surprise as previous studies have shown that ANA and/or SMA could be negative in patients with acute AIH, a finding that might lead to delay of diagnosis .…”

Section: Discussionsupporting

confidence: 87%

Prompt initiation of high‐dose i.v. corticosteroids seems to prevent progression to liver failure in patients with original acute severe autoimmune hepatitis

Zachou

Arvaniti

Lygoura

et al. 2018

Hepatology Research

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Aims: The definition of original acute severe autoimmune hepatitis (AS-AIH) is unclear. However, its rapid recognition and early treatment is potentially life-saving. Therefore, we present herein an open, real-world observational study for the assessment of the efficacy and safety of early high-dose i.v. corticosteroids in original AS-AIH patients.Methods: Prospectively collected data from 184 AIH patients were analyzed retrospectively. Original AS-AIH defined as an acute symptomatic presentation of newly diagnosed AIH (transaminases >10× upper limit of normal, bilirubin >4 mg/dL, and international normalized ratio [INR] ≥1.5) without histological lesions of chronic disease.Results: Thirty-four of 184 (18.5%) patients had original AS-AIH.These patients were promptly treated with i.v. corticosteroids (either 1 g methylprednisolone for 3 consecutive days followed by i.v. 1 mg/kg/day prednisolone or i.v. 1.5 mg/kg/day prednisolone from the beginning). Only 1/34 (2.9%) died due to sepsis; none required liver transplantation during follow-up (65 [1-175] months). No significant differences were detected in baseline characteristics between original AS-AIH patients and those with insidious presentation (not-AS-AIH; n = 117) apart from antinuclear antibodies negativity (P = 0.038), and higher immunoglobulin G, transaminases, INR, and bilirubin in original AS-AIH patients (P = 0.001 for all). Complete response and corticosteroids withdrawal (for patients treated >12 months) were significantly more frequent in original AS-AIH (n = 28) than in not-AS-AIH (n = 79; P = 0.026 and P = 0.016, respectively). Presence of original AS-AIH was the only independent predictor for achieving complete response.Conclusions: Prompt initiation of high-dose i.v. corticosteroids in original AS-AIH seems safe and efficient as it prevents disease deterioration and the need for liver transplantation. The long-term overall survival of these patients was high (97% for 5.3 years), and the long-term treatment response and corticosteroids withdrawal rates were higher compared to not-AS-AIH patients.

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Section: Discussionsupporting

confidence: 87%

Prompt initiation of high‐dose i.v. corticosteroids seems to prevent progression to liver failure in patients with original acute severe autoimmune hepatitis

Zachou

Arvaniti

Lygoura

et al. 2018

Hepatology Research

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“…The accumulation of findings of AIH with an acute presentation [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18] and its novel diagnostic guidelines 22 may have affected the actual characteristics of AIH in Japan after the previous 2015 survey. The aim of this study was to elucidate the recent trends in AIH in Japan by comparing the results of the 2015 survey to a 2018 survey.…”

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confidence: 99%

Increasing incidence of acute autoimmune hepatitis: a nationwide survey in Japan

Takahashi

Ohira

Abe

et al. 2020

Sci Rep

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The Japanese diagnostic guidelines for autoimmune hepatitis (AIH) were proposed in 2014. This study aimed to determine the trends and characteristics of AIH based on a Japanese nationwide survey. Data for 796 patients who were newly diagnosed with AIH from 2014 to 2017 were collected from January to March, 2019 from 54 hospitals throughout Japan. Clinical characteristics, including treatment, were compared with those reported in a prior 2015 survey. The population had a median age of 63 years at diagnosis, and the male to female ratio was 1:5.3. The numbers of women was significantly lower in this survey than in the 2015 survey. Moreover, the incidence of AIH with histological acute hepatitis increased significantly from 11.0 to 21.7%. The changes in the laboratory findings, such as in transaminase and immunoglobulin G levels and antinuclear antibody titers, as well as in prednisolone treatment, reflected an increasing incidence of acute AIH. The clinical characteristics of AIH changed rapidly, in parallel with the increasing incidence of acute AIH. The elucidation and diagnosis of AIH with acute hepatitis are important in the management of AIH. Although autoimmune hepatitis (AIH) with an acute presentation is difficult to diagnose based on a scoring system because of low immunoglobulin G (IgG) levels and antinuclear antibody (ANA) titers, its clinical and histological characteristics have been gradually elucidated by several reports 1-18. AIH with an acute presentation includes two types: (1) the acute exacerbation phase, in which patients present with the clinical features of acute hepatitis, with histological evidence of chronic hepatitis; and (2) the acute hepatitis phase, in which patients present with histological features of acute hepatitis 11. Regular nationwide surveys of AIH have been conducted since 1975 in Japan 1,2,19-21. The 2015 survey reported the trends in 1682 patients who were diagnosed with AIH from 2009 to 2013 2. The proportion of AIH cases with acute hepatitis (11.

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“…Autoimmune hepatitis (AIH) is a poorly known immune-mediated autodestructive acute or chronic liver disease with female predominance and potential for the development of liver fibrosis [1][2][3][4][5][6][7][8]. AIH is characterized by elevated serum aminotransferase levels, the presence of hypergammaglobulinemia, circulating autoantibodies, and interface hepatitis with portal plasma cell lymphocytic necroinfiltration and rosetting of hepatocytes in histological examination [3][4][5][6][7][8][9][10][11]. Interface hepatitis in this pathology is closely related to the process of liver fibrosis [1,4,5,[10][11][12][13].…”

Section: Introductionmentioning

confidence: 99%

“…AIH is characterized by elevated serum aminotransferase levels, the presence of hypergammaglobulinemia, circulating autoantibodies, and interface hepatitis with portal plasma cell lymphocytic necroinfiltration and rosetting of hepatocytes in histological examination [3][4][5][6][7][8][9][10][11]. Interface hepatitis in this pathology is closely related to the process of liver fibrosis [1,4,5,[10][11][12][13]. It is a relatively rare but devastating disease, particularly aggressive in children/adolescents, leading in some cases to cirrhosis, liver failure, and death [5,7,10,[12][13][14][15].…”

Section: Introductionmentioning

confidence: 99%

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Ultrastructural Profile Combined with Immunohistochemistry of a Hepatic Progenitor Cell Line in Pediatric Autoimmune Hepatitis: New Insights into the Morphological Pattern of the Disease

Łotowska

Sobaniec-Łotowska

Sobaniec

2021

Cells

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Considering that the heterogenic population of a hepatic progenitor cell line (HPCL) can play a vital role in autoimmune hepatitis (AIH), we decided to conduct pioneering retrospective evaluation of these cells in pediatric AIH by means of transmission electron microscopy (TEM). The aim of the study was to assess the ultrastructure of the HPCL in children with untreated AIH. Ultrastructural analysis of the HPCL population, preceded by immunohistochemical staining for cytokeratin 7 (CK7), was performed using pretreatment liver biopsies from 23 children with clinicopathologically diagnosed AIH. Immunohistochemical assessment for CK7 allowed detection of proliferating immature epithelial cells differentiating towards periportal and intralobular intermediate hepatocytes without marked formation of ductular reactions in AIH children. Using TEM, we distinguished three morphological types of HPCs: I—the most undifferentiated progenitor cells; III—intermediate hepatocyte-like cells; II—intermediate bile duct cells. Most frequent were the cells differentiating towards hepatocytes, most rare—those differentiating towards cholangiocytes. The results indicate that an HPCL may be an important source of hepatocyte regeneration. Ultrastructural analyses of the HPCL population, combined with immunohistochemistry for CK7, might be a useful tool to evaluate liver cell regeneration, including fibrogenesis, and may help better understand the morphological pattern of the disease, in pediatric AIH. Frequent appearance of an HPCL in the vicinity of fibrotic foci, often accompanied by hyperactive Kupffer cells and transitional hepatic stellate cells, may indicate their significant involvement in liver fibrogenesis.

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Long-term observation of acute-onset autoimmune hepatitis presenting clinically and radiologically as acute hepatitis

Abstract: Rapid progression of fibrosis could occur in A-AIH. Treatment response and long-term prognosis were good, and not different between patients with histologically acute and chronic hepatitis.

Cited by 12 publications

References 30 publications

Prompt initiation of high‐dose i.v. corticosteroids seems to prevent progression to liver failure in patients with original acute severe autoimmune hepatitis

Prompt initiation of high‐dose i.v. corticosteroids seems to prevent progression to liver failure in patients with original acute severe autoimmune hepatitis

Increasing incidence of acute autoimmune hepatitis: a nationwide survey in Japan

Ultrastructural Profile Combined with Immunohistochemistry of a Hepatic Progenitor Cell Line in Pediatric Autoimmune Hepatitis: New Insights into the Morphological Pattern of the Disease

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