XV Konferencja Naukowa Polskiego Towarzystwa Hepatologicznego
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Overlap syndromes and outlier syndromes – atypical forms of autoimmune liver diseases in the material of single tertiary referral centre in Poland
Anna Pietrzak, Bożena Walewska-Zielecka, Włodzimierz Zych, Andrzej Habior
Medical Science Review - Hepatologia 2011; 11 25-30
aaICID: 970904
Article type: Review article
IC™ Value: 3.40
Abstract provided by Publisher
In adults, chronic autoimmune liver diseases consist of three main entities: primary
biliary cirrhosis (PBC), autoimmune hepatitis (AIH) and primary sclerosing cholangitis
(PSC). However, there are patients fulfilling all required criteria for two out of three diseases.
Such coincidence is classified as “overlap syndrome”. There are several different overlap syndromes,
e.g. PBC/AIH, PSC/AIH and the former is most frequent in the Western countries. The
other type of atypical autoimmune liver disease is called “outlier syndrome”. It means that there
is a lack of one important sign required for proper diagnosis. Autoimmune cholangitis (AIC)
also termed as PBC AMA (–) is the most common example of outlier syndrome. In Poland the
overlap syndromes has not been widely studied. We analysed 737 patients with autoimmune
liver disease observed in Department of Gastroenterology and Hepatology, Medical Centre
for Postgraduate Education in Warsaw between 1995 and 2010. In this group, there were
657 patients diagnosed with only one disease (397 – PBC, 164 – PSC and 96 – AIH). Using
recently recommended criteria we found 80 (10.8%) patients with atypical form of autoimmune
liver disease. In this group there were 64 (8.7%) patients with overlap syndrome (35 –
PBC/AIH, 17 – PSC/AIH, 9 – AIC/AIH, 2 – PSC/AIC and 1 – PSC/PBC) and 16 patients (2.1%)
with AIC. In analysed material of patients with autoimmune diseases the occurrence and profile
of atypical forms is similar to the results reported from Western countries.
Our analysis of a large group of patients indicates that in autoimmune liver diseases overlap
and outlier syndromes are not so rare and their diagnosis is difficult and troublesome. Patients
suspected to have such atypical disease should be referred to well equipped centre collaborating
with pathologist experienced in hepatopathology.

ICID 970904

DOI 10.5604/17305039.970904

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