Primary biliary cirrhosis - Autoimmune hepatitis overlap syndrome

A rationale for corticosteroids use based on a nation-wide retrospective study in Japan

Atsushi Tanaka, Kenichi Harada, Hirotoshi Ebinuma, Atsumasa Komori, Junko Yokokawa, Kaname Yoshizawa, Masanori Abe, Yasuhiro Miyake, Kentaro Kikuchi, Hiromasa Ohira, Mikio Zeniya, Kazuhide Yamamoto, Hiromi Ishibashi, Morikazu Onji, Yasuni Nakanuma, Hirohito Tsubouchi, Hajime Takikawa

Research output: Contribution to journalArticle

33 Citations (Scopus)

Abstract

Aims: Primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) may simultaneously coexist in some patients, designated as PBC-AIH overlap syndrome. Previous studies suggest that combination therapy of ursodeoxycholic acid (UDCA) and corticosteroids may be effective. In the current study, we aimed to describe clinical features of these cases and to propose a rationale for combination treatment in PBC-AIH overlap. Methods: We enrolled patients with PBC-AIH overlap from eight referral centers for liver diseases in Japan, and clinical, biochemical and immunological features were examined. Liver histology of all patients at diagnosis were analyzed altogether in detail. Eighty-nine and 44 patients with PBC and AIH alone were included and served as controls. Results: We identified 33 patients with PBC-AIH overlap. The mean follow-up period was 6.1years. On liver histology, the HA (hepatitis activity) score was significantly higher than the CA (cholangitis activity) score (P<0.001). At the end of the follow-up period, corticosteroids were used in 23 patients (72%), and neither liver-related death nor liver transplantation had been noted. The sensitivity and specificity of the simplified AIH scoring system for prediction of patients who required corticosteroids during clinical course was 92% and 75% in the training set (n=17), and 91% and 80% in the validation set (n=16) of overlap. Only 3% of PBC patients were diagnosed as having indication for corticosteroid use. Conclusion: In PBC-AIH overlap, AIH-like features are dominant in liver histology. The simplified AIH scoring system could predict patients who needed corticosteroids with a higher specificity.

Original languageEnglish
Pages (from-to)877-886
Number of pages10
JournalHepatology Research
Volume41
Issue number9
DOIs
Publication statusPublished - 2011 Sep

Fingerprint

Autoimmune Hepatitis
Biliary Liver Cirrhosis
Japan
Adrenal Cortex Hormones
Retrospective Studies
Histology
Liver
Ursodeoxycholic Acid
Cholangitis
Liver Transplantation
Hepatitis
Liver Diseases
Referral and Consultation
Sensitivity and Specificity

Keywords

  • Corticosteroids
  • The revised International Autoimmune Hepatitis Group scoring system
  • The simplified scoring system

ASJC Scopus subject areas

  • Hepatology
  • Infectious Diseases

Cite this

Primary biliary cirrhosis - Autoimmune hepatitis overlap syndrome : A rationale for corticosteroids use based on a nation-wide retrospective study in Japan. / Tanaka, Atsushi; Harada, Kenichi; Ebinuma, Hirotoshi; Komori, Atsumasa; Yokokawa, Junko; Yoshizawa, Kaname; Abe, Masanori; Miyake, Yasuhiro; Kikuchi, Kentaro; Ohira, Hiromasa; Zeniya, Mikio; Yamamoto, Kazuhide; Ishibashi, Hiromi; Onji, Morikazu; Nakanuma, Yasuni; Tsubouchi, Hirohito; Takikawa, Hajime.

In: Hepatology Research, Vol. 41, No. 9, 09.2011, p. 877-886.

Research output: Contribution to journalArticle

Tanaka, A, Harada, K, Ebinuma, H, Komori, A, Yokokawa, J, Yoshizawa, K, Abe, M, Miyake, Y, Kikuchi, K, Ohira, H, Zeniya, M, Yamamoto, K, Ishibashi, H, Onji, M, Nakanuma, Y, Tsubouchi, H & Takikawa, H 2011, 'Primary biliary cirrhosis - Autoimmune hepatitis overlap syndrome: A rationale for corticosteroids use based on a nation-wide retrospective study in Japan', Hepatology Research, vol. 41, no. 9, pp. 877-886. https://doi.org/10.1111/j.1872-034X.2011.00844.x
Tanaka, Atsushi ; Harada, Kenichi ; Ebinuma, Hirotoshi ; Komori, Atsumasa ; Yokokawa, Junko ; Yoshizawa, Kaname ; Abe, Masanori ; Miyake, Yasuhiro ; Kikuchi, Kentaro ; Ohira, Hiromasa ; Zeniya, Mikio ; Yamamoto, Kazuhide ; Ishibashi, Hiromi ; Onji, Morikazu ; Nakanuma, Yasuni ; Tsubouchi, Hirohito ; Takikawa, Hajime. / Primary biliary cirrhosis - Autoimmune hepatitis overlap syndrome : A rationale for corticosteroids use based on a nation-wide retrospective study in Japan. In: Hepatology Research. 2011 ; Vol. 41, No. 9. pp. 877-886.
@article{5bb61019a293472599f3097070be537d,
title = "Primary biliary cirrhosis - Autoimmune hepatitis overlap syndrome: A rationale for corticosteroids use based on a nation-wide retrospective study in Japan",
abstract = "Aims: Primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) may simultaneously coexist in some patients, designated as PBC-AIH overlap syndrome. Previous studies suggest that combination therapy of ursodeoxycholic acid (UDCA) and corticosteroids may be effective. In the current study, we aimed to describe clinical features of these cases and to propose a rationale for combination treatment in PBC-AIH overlap. Methods: We enrolled patients with PBC-AIH overlap from eight referral centers for liver diseases in Japan, and clinical, biochemical and immunological features were examined. Liver histology of all patients at diagnosis were analyzed altogether in detail. Eighty-nine and 44 patients with PBC and AIH alone were included and served as controls. Results: We identified 33 patients with PBC-AIH overlap. The mean follow-up period was 6.1years. On liver histology, the HA (hepatitis activity) score was significantly higher than the CA (cholangitis activity) score (P<0.001). At the end of the follow-up period, corticosteroids were used in 23 patients (72{\%}), and neither liver-related death nor liver transplantation had been noted. The sensitivity and specificity of the simplified AIH scoring system for prediction of patients who required corticosteroids during clinical course was 92{\%} and 75{\%} in the training set (n=17), and 91{\%} and 80{\%} in the validation set (n=16) of overlap. Only 3{\%} of PBC patients were diagnosed as having indication for corticosteroid use. Conclusion: In PBC-AIH overlap, AIH-like features are dominant in liver histology. The simplified AIH scoring system could predict patients who needed corticosteroids with a higher specificity.",
keywords = "Corticosteroids, The revised International Autoimmune Hepatitis Group scoring system, The simplified scoring system",
author = "Atsushi Tanaka and Kenichi Harada and Hirotoshi Ebinuma and Atsumasa Komori and Junko Yokokawa and Kaname Yoshizawa and Masanori Abe and Yasuhiro Miyake and Kentaro Kikuchi and Hiromasa Ohira and Mikio Zeniya and Kazuhide Yamamoto and Hiromi Ishibashi and Morikazu Onji and Yasuni Nakanuma and Hirohito Tsubouchi and Hajime Takikawa",
year = "2011",
month = "9",
doi = "10.1111/j.1872-034X.2011.00844.x",
language = "English",
volume = "41",
pages = "877--886",
journal = "Hepatology Research",
issn = "1386-6346",
publisher = "Wiley-Blackwell Publishing Ltd",
number = "9",

}

TY - JOUR

T1 - Primary biliary cirrhosis - Autoimmune hepatitis overlap syndrome

T2 - A rationale for corticosteroids use based on a nation-wide retrospective study in Japan

AU - Tanaka, Atsushi

AU - Harada, Kenichi

AU - Ebinuma, Hirotoshi

AU - Komori, Atsumasa

AU - Yokokawa, Junko

AU - Yoshizawa, Kaname

AU - Abe, Masanori

AU - Miyake, Yasuhiro

AU - Kikuchi, Kentaro

AU - Ohira, Hiromasa

AU - Zeniya, Mikio

AU - Yamamoto, Kazuhide

AU - Ishibashi, Hiromi

AU - Onji, Morikazu

AU - Nakanuma, Yasuni

AU - Tsubouchi, Hirohito

AU - Takikawa, Hajime

PY - 2011/9

Y1 - 2011/9

N2 - Aims: Primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) may simultaneously coexist in some patients, designated as PBC-AIH overlap syndrome. Previous studies suggest that combination therapy of ursodeoxycholic acid (UDCA) and corticosteroids may be effective. In the current study, we aimed to describe clinical features of these cases and to propose a rationale for combination treatment in PBC-AIH overlap. Methods: We enrolled patients with PBC-AIH overlap from eight referral centers for liver diseases in Japan, and clinical, biochemical and immunological features were examined. Liver histology of all patients at diagnosis were analyzed altogether in detail. Eighty-nine and 44 patients with PBC and AIH alone were included and served as controls. Results: We identified 33 patients with PBC-AIH overlap. The mean follow-up period was 6.1years. On liver histology, the HA (hepatitis activity) score was significantly higher than the CA (cholangitis activity) score (P<0.001). At the end of the follow-up period, corticosteroids were used in 23 patients (72%), and neither liver-related death nor liver transplantation had been noted. The sensitivity and specificity of the simplified AIH scoring system for prediction of patients who required corticosteroids during clinical course was 92% and 75% in the training set (n=17), and 91% and 80% in the validation set (n=16) of overlap. Only 3% of PBC patients were diagnosed as having indication for corticosteroid use. Conclusion: In PBC-AIH overlap, AIH-like features are dominant in liver histology. The simplified AIH scoring system could predict patients who needed corticosteroids with a higher specificity.

AB - Aims: Primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) may simultaneously coexist in some patients, designated as PBC-AIH overlap syndrome. Previous studies suggest that combination therapy of ursodeoxycholic acid (UDCA) and corticosteroids may be effective. In the current study, we aimed to describe clinical features of these cases and to propose a rationale for combination treatment in PBC-AIH overlap. Methods: We enrolled patients with PBC-AIH overlap from eight referral centers for liver diseases in Japan, and clinical, biochemical and immunological features were examined. Liver histology of all patients at diagnosis were analyzed altogether in detail. Eighty-nine and 44 patients with PBC and AIH alone were included and served as controls. Results: We identified 33 patients with PBC-AIH overlap. The mean follow-up period was 6.1years. On liver histology, the HA (hepatitis activity) score was significantly higher than the CA (cholangitis activity) score (P<0.001). At the end of the follow-up period, corticosteroids were used in 23 patients (72%), and neither liver-related death nor liver transplantation had been noted. The sensitivity and specificity of the simplified AIH scoring system for prediction of patients who required corticosteroids during clinical course was 92% and 75% in the training set (n=17), and 91% and 80% in the validation set (n=16) of overlap. Only 3% of PBC patients were diagnosed as having indication for corticosteroid use. Conclusion: In PBC-AIH overlap, AIH-like features are dominant in liver histology. The simplified AIH scoring system could predict patients who needed corticosteroids with a higher specificity.

KW - Corticosteroids

KW - The revised International Autoimmune Hepatitis Group scoring system

KW - The simplified scoring system

UR - http://www.scopus.com/inward/record.url?scp=80052245126&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=80052245126&partnerID=8YFLogxK

U2 - 10.1111/j.1872-034X.2011.00844.x

DO - 10.1111/j.1872-034X.2011.00844.x

M3 - Article

VL - 41

SP - 877

EP - 886

JO - Hepatology Research

JF - Hepatology Research

SN - 1386-6346

IS - 9

ER -