Clinical features of autoimmune hepatitis with acute presentation: a Japanese nationwide survey

The Japan Autoimmune Hepatitis Study Group (JAIHSG)

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

Background: Autoimmune hepatitis (AIH) is characterized by progressive inflammation and necrosis of hepatocytes and eventually leads to a variety of phenotypes, including acute liver dysfunction, chronic progressive liver disease, and fulminant hepatic failure. Although the precise mechanisms of AIH are unknown, environmental factors may trigger disease onset in genetically predisposed individuals. Patients with the recently established entity of AIH with acute presentation often display atypical clinical features that mimic those of acute hepatitis forms even though AIH is categorized as a chronic liver disease. The aim of this study was to identify the precise clinical features of AIH with acute presentation. Methods: Eighty-six AIH patients with acute presentation were retrospectively enrolled from facilities across Japan and analyzed for clinical features, histopathological findings, and disease outcomes. Results: Seventy-five patients were female and 11 were male. Patient age ranged from adolescent to over 80 years old, with a median age of 55 years. Median alanine transaminase (ALT) was 776 U/L and median immunoglobulin G (IgG) was 1671 mg/dL. There were no significant differences between genders in terms of ALT (P = 0.27) or IgG (P = 0.51). The number of patients without and with histopathological fibrosis was 29 and 57, respectively. The patients with fibrosis were significantly older than those without (P = 0.015), but no other differences in clinical or histopathological findings were observed. Moreover, antinuclear antibody (ANA)-positive (defined as × 40, N = 63) and -negative (N = 23) patients showed no significant differences in clinical or histopathological findings or disease outcomes. Twenty-five patients experienced disease relapse and two patients died during the study period. ALP ≥ 500 U/L [odds ratio (OR) 3.20; 95% confidence interval (CI) 1.12–9.10; P < 0.030] and GGT ≥ 200 U/L (OR 2.98; 95% CI 1.01–8.77; P = 0.047) were identified as independent risk factors of disease relapse. Conclusions: AIH with acute presentation is a newly recognized disease entity for which diagnostic hallmarks, such as ALT, fibrosis, and ANA, are needed. Further investigation is also required on the mechanisms of this disorder. Clinicians should be mindful of disease relapse during patient care.

Original languageEnglish
Pages (from-to)1-10
Number of pages10
JournalJournal of Gastroenterology
DOIs
Publication statusAccepted/In press - Feb 23 2018

Fingerprint

Autoimmune Hepatitis
Alanine Transaminase
Liver Diseases
Fibrosis
Antinuclear Antibodies
Recurrence
Immunoglobulin G
Odds Ratio
Confidence Intervals
Surveys and Questionnaires
Acute Liver Failure
Hepatitis
Hepatocytes
Patient Care
Japan
Chronic Disease
Necrosis
Inflammation
Phenotype

Keywords

  • Antinuclear antibody
  • Autoimmune hepatitis
  • Fibrosis
  • Histopathology
  • Immunoglobulin G

ASJC Scopus subject areas

  • Gastroenterology

Cite this

Clinical features of autoimmune hepatitis with acute presentation : a Japanese nationwide survey. / The Japan Autoimmune Hepatitis Study Group (JAIHSG).

In: Journal of Gastroenterology, 23.02.2018, p. 1-10.

Research output: Contribution to journalArticle

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title = "Clinical features of autoimmune hepatitis with acute presentation: a Japanese nationwide survey",
abstract = "Background: Autoimmune hepatitis (AIH) is characterized by progressive inflammation and necrosis of hepatocytes and eventually leads to a variety of phenotypes, including acute liver dysfunction, chronic progressive liver disease, and fulminant hepatic failure. Although the precise mechanisms of AIH are unknown, environmental factors may trigger disease onset in genetically predisposed individuals. Patients with the recently established entity of AIH with acute presentation often display atypical clinical features that mimic those of acute hepatitis forms even though AIH is categorized as a chronic liver disease. The aim of this study was to identify the precise clinical features of AIH with acute presentation. Methods: Eighty-six AIH patients with acute presentation were retrospectively enrolled from facilities across Japan and analyzed for clinical features, histopathological findings, and disease outcomes. Results: Seventy-five patients were female and 11 were male. Patient age ranged from adolescent to over 80 years old, with a median age of 55 years. Median alanine transaminase (ALT) was 776 U/L and median immunoglobulin G (IgG) was 1671 mg/dL. There were no significant differences between genders in terms of ALT (P = 0.27) or IgG (P = 0.51). The number of patients without and with histopathological fibrosis was 29 and 57, respectively. The patients with fibrosis were significantly older than those without (P = 0.015), but no other differences in clinical or histopathological findings were observed. Moreover, antinuclear antibody (ANA)-positive (defined as × 40, N = 63) and -negative (N = 23) patients showed no significant differences in clinical or histopathological findings or disease outcomes. Twenty-five patients experienced disease relapse and two patients died during the study period. ALP ≥ 500 U/L [odds ratio (OR) 3.20; 95{\%} confidence interval (CI) 1.12–9.10; P < 0.030] and GGT ≥ 200 U/L (OR 2.98; 95{\%} CI 1.01–8.77; P = 0.047) were identified as independent risk factors of disease relapse. Conclusions: AIH with acute presentation is a newly recognized disease entity for which diagnostic hallmarks, such as ALT, fibrosis, and ANA, are needed. Further investigation is also required on the mechanisms of this disorder. Clinicians should be mindful of disease relapse during patient care.",
keywords = "Antinuclear antibody, Autoimmune hepatitis, Fibrosis, Histopathology, Immunoglobulin G",
author = "{The Japan Autoimmune Hepatitis Study Group (JAIHSG)} and Satoru Joshita and Kaname Yoshizawa and Takeji Umemura and Hiromasa Ohira and Atsushi Takahashi and Kenichi Harada and Hiep, {Nguyen Canh} and Koichi Tsuneyama and Masayoshi Kage and Masayuki Nakano and Kang, {Jong Hon} and Kazuhiko Koike and Mikio Zeniya and Tetsuya Yasunaka and Akinobu Takaki and Takuji Torimura and Masanori Abe and Osamu Yokosuka and Atsushi Tanaka and Hajime Takikawa",
year = "2018",
month = "2",
day = "23",
doi = "10.1007/s00535-018-1444-4",
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T1 - Clinical features of autoimmune hepatitis with acute presentation

T2 - a Japanese nationwide survey

AU - The Japan Autoimmune Hepatitis Study Group (JAIHSG)

AU - Joshita, Satoru

AU - Yoshizawa, Kaname

AU - Umemura, Takeji

AU - Ohira, Hiromasa

AU - Takahashi, Atsushi

AU - Harada, Kenichi

AU - Hiep, Nguyen Canh

AU - Tsuneyama, Koichi

AU - Kage, Masayoshi

AU - Nakano, Masayuki

AU - Kang, Jong Hon

AU - Koike, Kazuhiko

AU - Zeniya, Mikio

AU - Yasunaka, Tetsuya

AU - Takaki, Akinobu

AU - Torimura, Takuji

AU - Abe, Masanori

AU - Yokosuka, Osamu

AU - Tanaka, Atsushi

AU - Takikawa, Hajime

PY - 2018/2/23

Y1 - 2018/2/23

N2 - Background: Autoimmune hepatitis (AIH) is characterized by progressive inflammation and necrosis of hepatocytes and eventually leads to a variety of phenotypes, including acute liver dysfunction, chronic progressive liver disease, and fulminant hepatic failure. Although the precise mechanisms of AIH are unknown, environmental factors may trigger disease onset in genetically predisposed individuals. Patients with the recently established entity of AIH with acute presentation often display atypical clinical features that mimic those of acute hepatitis forms even though AIH is categorized as a chronic liver disease. The aim of this study was to identify the precise clinical features of AIH with acute presentation. Methods: Eighty-six AIH patients with acute presentation were retrospectively enrolled from facilities across Japan and analyzed for clinical features, histopathological findings, and disease outcomes. Results: Seventy-five patients were female and 11 were male. Patient age ranged from adolescent to over 80 years old, with a median age of 55 years. Median alanine transaminase (ALT) was 776 U/L and median immunoglobulin G (IgG) was 1671 mg/dL. There were no significant differences between genders in terms of ALT (P = 0.27) or IgG (P = 0.51). The number of patients without and with histopathological fibrosis was 29 and 57, respectively. The patients with fibrosis were significantly older than those without (P = 0.015), but no other differences in clinical or histopathological findings were observed. Moreover, antinuclear antibody (ANA)-positive (defined as × 40, N = 63) and -negative (N = 23) patients showed no significant differences in clinical or histopathological findings or disease outcomes. Twenty-five patients experienced disease relapse and two patients died during the study period. ALP ≥ 500 U/L [odds ratio (OR) 3.20; 95% confidence interval (CI) 1.12–9.10; P < 0.030] and GGT ≥ 200 U/L (OR 2.98; 95% CI 1.01–8.77; P = 0.047) were identified as independent risk factors of disease relapse. Conclusions: AIH with acute presentation is a newly recognized disease entity for which diagnostic hallmarks, such as ALT, fibrosis, and ANA, are needed. Further investigation is also required on the mechanisms of this disorder. Clinicians should be mindful of disease relapse during patient care.

AB - Background: Autoimmune hepatitis (AIH) is characterized by progressive inflammation and necrosis of hepatocytes and eventually leads to a variety of phenotypes, including acute liver dysfunction, chronic progressive liver disease, and fulminant hepatic failure. Although the precise mechanisms of AIH are unknown, environmental factors may trigger disease onset in genetically predisposed individuals. Patients with the recently established entity of AIH with acute presentation often display atypical clinical features that mimic those of acute hepatitis forms even though AIH is categorized as a chronic liver disease. The aim of this study was to identify the precise clinical features of AIH with acute presentation. Methods: Eighty-six AIH patients with acute presentation were retrospectively enrolled from facilities across Japan and analyzed for clinical features, histopathological findings, and disease outcomes. Results: Seventy-five patients were female and 11 were male. Patient age ranged from adolescent to over 80 years old, with a median age of 55 years. Median alanine transaminase (ALT) was 776 U/L and median immunoglobulin G (IgG) was 1671 mg/dL. There were no significant differences between genders in terms of ALT (P = 0.27) or IgG (P = 0.51). The number of patients without and with histopathological fibrosis was 29 and 57, respectively. The patients with fibrosis were significantly older than those without (P = 0.015), but no other differences in clinical or histopathological findings were observed. Moreover, antinuclear antibody (ANA)-positive (defined as × 40, N = 63) and -negative (N = 23) patients showed no significant differences in clinical or histopathological findings or disease outcomes. Twenty-five patients experienced disease relapse and two patients died during the study period. ALP ≥ 500 U/L [odds ratio (OR) 3.20; 95% confidence interval (CI) 1.12–9.10; P < 0.030] and GGT ≥ 200 U/L (OR 2.98; 95% CI 1.01–8.77; P = 0.047) were identified as independent risk factors of disease relapse. Conclusions: AIH with acute presentation is a newly recognized disease entity for which diagnostic hallmarks, such as ALT, fibrosis, and ANA, are needed. Further investigation is also required on the mechanisms of this disorder. Clinicians should be mindful of disease relapse during patient care.

KW - Antinuclear antibody

KW - Autoimmune hepatitis

KW - Fibrosis

KW - Histopathology

KW - Immunoglobulin G

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