Autoimmune hepatitis-PBC overlap syndrome

A simplified scoring system may assist in the diagnosis

Matthias Neuhauser, Einar Bjornsson, Sombat Treeprasertsuk, Felicity Enders, Marina Silveira, Jayant Talwalkar, Keith Lindor

Research output: Contribution to journalArticle

71 Citations (Scopus)

Abstract

Objectives: Primary biliary cirrhosis (PBC) with features consistent with autoimmune hepatitis (AIH) has been described as an overlap syndrome. Recently, a simplified AIH scoring system has been proposed by the International Autoimmune Hepatitis Group (IAIHG), which is based on only four clinical components. We aimed to evaluate the performance of the new simplified AIH scoring system as a diagnostic instrument for PBC-AIH overlap syndrome compared with the revised 1999 IAIHG criteria. Furthermore, we sought to compare the outcome in PBC patients with and without the features of AIH overlap. Methods: Retrospective analysis of PBC patients was carried out. Parameters relevant to the revised criteria were recorded, and outcomes were compared between those with and without features of overlap. Results: Of 368 patients (318 females) with a definite diagnosis of PBC, 43 (12%) were diagnosed as probable PBC-AIH overlap with the revised criteria and 23 (6%) with the simplified criteria. In both scoring systems the frequency of cirrhosis, portal hypertension, gastrointestinal (GI) bleeding, ascites, and esophageal varices was significantly higher in the overlap group at the time of follow-up. Patients with features of overlap according to the new criteria had more frequent liver-related death and liver transplantation (P0.0025, log rank test). Conclusions: The simplified AIH scoring system appears to be more specific in patients with PBC and could assist in clinical assessment. Worse outcome was observed in patients with overlap features, demonstrated as increased liver-related mortality with the new criteria. The new criteria should be able to replace the revised criteria for the diagnosis of PBC-AIH overlap syndrome.

Original languageEnglish (US)
Pages (from-to)345-353
Number of pages9
JournalAmerican Journal of Gastroenterology
Volume105
Issue number2
DOIs
StatePublished - Feb 2010
Externally publishedYes

Fingerprint

Autoimmune Hepatitis
Biliary Liver Cirrhosis
Esophageal and Gastric Varices
Liver
Portal Hypertension
Ascites
Liver Transplantation
Fibrosis
Hemorrhage

ASJC Scopus subject areas

  • Gastroenterology

Cite this

Autoimmune hepatitis-PBC overlap syndrome : A simplified scoring system may assist in the diagnosis. / Neuhauser, Matthias; Bjornsson, Einar; Treeprasertsuk, Sombat; Enders, Felicity; Silveira, Marina; Talwalkar, Jayant; Lindor, Keith.

In: American Journal of Gastroenterology, Vol. 105, No. 2, 02.2010, p. 345-353.

Research output: Contribution to journalArticle

Neuhauser, M, Bjornsson, E, Treeprasertsuk, S, Enders, F, Silveira, M, Talwalkar, J & Lindor, K 2010, 'Autoimmune hepatitis-PBC overlap syndrome: A simplified scoring system may assist in the diagnosis', American Journal of Gastroenterology, vol. 105, no. 2, pp. 345-353. https://doi.org/10.1038/ajg.2009.616
Neuhauser M, Bjornsson E, Treeprasertsuk S, Enders F, Silveira M, Talwalkar J et al. Autoimmune hepatitis-PBC overlap syndrome: A simplified scoring system may assist in the diagnosis. American Journal of Gastroenterology. 2010 Feb;105(2):345-353. https://doi.org/10.1038/ajg.2009.616
Neuhauser, Matthias ; Bjornsson, Einar ; Treeprasertsuk, Sombat ; Enders, Felicity ; Silveira, Marina ; Talwalkar, Jayant ; Lindor, Keith. / Autoimmune hepatitis-PBC overlap syndrome : A simplified scoring system may assist in the diagnosis. In: American Journal of Gastroenterology. 2010 ; Vol. 105, No. 2. pp. 345-353.
@article{ccccb2556fd44afc897b67c36776f113,
title = "Autoimmune hepatitis-PBC overlap syndrome: A simplified scoring system may assist in the diagnosis",
abstract = "Objectives: Primary biliary cirrhosis (PBC) with features consistent with autoimmune hepatitis (AIH) has been described as an overlap syndrome. Recently, a simplified AIH scoring system has been proposed by the International Autoimmune Hepatitis Group (IAIHG), which is based on only four clinical components. We aimed to evaluate the performance of the new simplified AIH scoring system as a diagnostic instrument for PBC-AIH overlap syndrome compared with the revised 1999 IAIHG criteria. Furthermore, we sought to compare the outcome in PBC patients with and without the features of AIH overlap. Methods: Retrospective analysis of PBC patients was carried out. Parameters relevant to the revised criteria were recorded, and outcomes were compared between those with and without features of overlap. Results: Of 368 patients (318 females) with a definite diagnosis of PBC, 43 (12{\%}) were diagnosed as probable PBC-AIH overlap with the revised criteria and 23 (6{\%}) with the simplified criteria. In both scoring systems the frequency of cirrhosis, portal hypertension, gastrointestinal (GI) bleeding, ascites, and esophageal varices was significantly higher in the overlap group at the time of follow-up. Patients with features of overlap according to the new criteria had more frequent liver-related death and liver transplantation (P0.0025, log rank test). Conclusions: The simplified AIH scoring system appears to be more specific in patients with PBC and could assist in clinical assessment. Worse outcome was observed in patients with overlap features, demonstrated as increased liver-related mortality with the new criteria. The new criteria should be able to replace the revised criteria for the diagnosis of PBC-AIH overlap syndrome.",
author = "Matthias Neuhauser and Einar Bjornsson and Sombat Treeprasertsuk and Felicity Enders and Marina Silveira and Jayant Talwalkar and Keith Lindor",
year = "2010",
month = "2",
doi = "10.1038/ajg.2009.616",
language = "English (US)",
volume = "105",
pages = "345--353",
journal = "American Journal of Gastroenterology",
issn = "0002-9270",
publisher = "Nature Publishing Group",
number = "2",

}

TY - JOUR

T1 - Autoimmune hepatitis-PBC overlap syndrome

T2 - A simplified scoring system may assist in the diagnosis

AU - Neuhauser, Matthias

AU - Bjornsson, Einar

AU - Treeprasertsuk, Sombat

AU - Enders, Felicity

AU - Silveira, Marina

AU - Talwalkar, Jayant

AU - Lindor, Keith

PY - 2010/2

Y1 - 2010/2

N2 - Objectives: Primary biliary cirrhosis (PBC) with features consistent with autoimmune hepatitis (AIH) has been described as an overlap syndrome. Recently, a simplified AIH scoring system has been proposed by the International Autoimmune Hepatitis Group (IAIHG), which is based on only four clinical components. We aimed to evaluate the performance of the new simplified AIH scoring system as a diagnostic instrument for PBC-AIH overlap syndrome compared with the revised 1999 IAIHG criteria. Furthermore, we sought to compare the outcome in PBC patients with and without the features of AIH overlap. Methods: Retrospective analysis of PBC patients was carried out. Parameters relevant to the revised criteria were recorded, and outcomes were compared between those with and without features of overlap. Results: Of 368 patients (318 females) with a definite diagnosis of PBC, 43 (12%) were diagnosed as probable PBC-AIH overlap with the revised criteria and 23 (6%) with the simplified criteria. In both scoring systems the frequency of cirrhosis, portal hypertension, gastrointestinal (GI) bleeding, ascites, and esophageal varices was significantly higher in the overlap group at the time of follow-up. Patients with features of overlap according to the new criteria had more frequent liver-related death and liver transplantation (P0.0025, log rank test). Conclusions: The simplified AIH scoring system appears to be more specific in patients with PBC and could assist in clinical assessment. Worse outcome was observed in patients with overlap features, demonstrated as increased liver-related mortality with the new criteria. The new criteria should be able to replace the revised criteria for the diagnosis of PBC-AIH overlap syndrome.

AB - Objectives: Primary biliary cirrhosis (PBC) with features consistent with autoimmune hepatitis (AIH) has been described as an overlap syndrome. Recently, a simplified AIH scoring system has been proposed by the International Autoimmune Hepatitis Group (IAIHG), which is based on only four clinical components. We aimed to evaluate the performance of the new simplified AIH scoring system as a diagnostic instrument for PBC-AIH overlap syndrome compared with the revised 1999 IAIHG criteria. Furthermore, we sought to compare the outcome in PBC patients with and without the features of AIH overlap. Methods: Retrospective analysis of PBC patients was carried out. Parameters relevant to the revised criteria were recorded, and outcomes were compared between those with and without features of overlap. Results: Of 368 patients (318 females) with a definite diagnosis of PBC, 43 (12%) were diagnosed as probable PBC-AIH overlap with the revised criteria and 23 (6%) with the simplified criteria. In both scoring systems the frequency of cirrhosis, portal hypertension, gastrointestinal (GI) bleeding, ascites, and esophageal varices was significantly higher in the overlap group at the time of follow-up. Patients with features of overlap according to the new criteria had more frequent liver-related death and liver transplantation (P0.0025, log rank test). Conclusions: The simplified AIH scoring system appears to be more specific in patients with PBC and could assist in clinical assessment. Worse outcome was observed in patients with overlap features, demonstrated as increased liver-related mortality with the new criteria. The new criteria should be able to replace the revised criteria for the diagnosis of PBC-AIH overlap syndrome.

UR - http://www.scopus.com/inward/record.url?scp=76349119699&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=76349119699&partnerID=8YFLogxK

U2 - 10.1038/ajg.2009.616

DO - 10.1038/ajg.2009.616

M3 - Article

VL - 105

SP - 345

EP - 353

JO - American Journal of Gastroenterology

JF - American Journal of Gastroenterology

SN - 0002-9270

IS - 2

ER -