Elsevier

Journal of Hepatology

Volume 54, Issue 2, February 2011, Pages 374-385
Journal of Hepatology

Position Paper
Overlap syndromes: The International Autoimmune Hepatitis Group (IAIHG) position statement on a controversial issue

https://doi.org/10.1016/j.jhep.2010.09.002Get rights and content
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Some patients present with overlapping features between disorders within the spectrum of autoimmune liver diseases (i.e. autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC)) and are commonly classified as having an “overlap syndrome”. Standardized definitions of “overlap syndromes” are lacking.

The aim of this report by the International Autoimmune Hepatitis Group (IAIHG) is to evaluate if there are important reasons to classify conditions with overlapping features between autoimmune liver diseases as separate diagnostic entities.

Definition of diagnostic criteria for overlap conditions can only be arbitrary. The IAIHG scoring system for diagnosis of AIH has been widely used to diagnose “overlap syndromes”, but was not intended for such use and has not proven to be an efficient tool for this purpose. Some patients with overlapping features between a cholestatic and hepatitic disorder appear to benefit from treatment with a combination of ursodeoxycholic acid and immunosuppressants, but this strategy is not evidence-based, and it seems unjustified to define new diagnostic groups in this regard.

The IAIHG suggests that patients with autoimmune liver disease should be categorized according to the predominating feature(s) as AIH, PBC, and PSC/small duct PSC, respectively, and that those with overlapping features are not considered as being distinct diagnostic entities. The IAIHG scoring system should not be used to establish subgroups of patients.

Patients with PBC and PSC with features of AIH should be considered for immunosuppressive treatment. Due to the low prevalence of such “overlap syndromes”, prospective interventional therapeutic trials cannot be expected in the foreseeable future.

Keywords

Autoimmune hepatitis
Autoimmune liver disease
Overlap syndromes
Primary biliary cirrhosis
Primary sclerosing cholangitis
Ursodeoxycholic acid

Abbreviations

AIH
autoimmune hepatitis
PBC
primary biliary cirrhosis
PSC
primary sclerosing cholangitis
IAIHG
International Autoimmune Hepatitis Group
ULN
upper limit of normal
ALP
alkaline phosphatase
ANA
antinuclear antibody
SMA
smooth muscle antibody
anti-LKM-1
antibodies to liver kidney microsome type 1
anti-SLA/LP
antibodies to soluble liver antigen/liver pancreas
anti-LC1
antibodies to liver cytosol antigen type 1
pANCA
perinuclear anti-neutrophil cytoplasmic antibodies
pANNA
perinuclear anti-neutrophil antibody
AMA
anti-mitochondrial antibody
PDC-E2
pyruvate dehydrogenase complex-E2
anti-gp210
antibody against nuclear pore membrane glycoprotein
anti-Sp100
antibody against nuclear protein Sp100
IBD
inflammatory bowel disease
MRC
magnetic resonance cholangiography
γGT
γ-glutamyl transpeptidase
ALT
alanine transaminase
EASL
European Association for the Study of the Liver
UDCA
ursodeoxycholic acid
ERC
endoscopic retrograde cholangiography
ASC
autoimmune sclerosing cholangitis
HLA
human leukocyte antigen
AST
aspartate transaminase
AASLD
American Association for the Study of Liver Diseases

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