Abstract
Autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are chronic liver diseases of presumed autoimmune origin. Conditions exhibiting features of two different autoimmune liver diseases are commonly designated overlap syndromes, although there is no current agreement on what constitutes an overlap syndrome or specific diagnostic criteria. Identification of patients with features of overlap syndrome is relevant for management as outcomes may differ from outcomes of patients with diagnosis of classic autoimmune liver diseases and treatment may need to be adjusted. Due to their rarity, no large therapeutic trials are available and treatment of overlap conditions is empirical and based upon extrapolation of data from the primary autoimmune liver diseases. AIH-PBC overlap is the most frequently described overlap syndrome and may be associated with a poor prognosis. This may represent an important and unrecognized cause of resistance to ursodeoxycholic acid (UDCA) in patients with PBC. AIH-PSC overlap is less commonly reported. Prognosis may be better than in patients with PSC alone, however worse than in patients with AIH alone. Further studies are needed for determining diagnosis, natural history and optimal therapeutic strategies of overlap syndromes of autoimmune liver diseases.
Highlights
Autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are presumed autoimmune liver diseases
We have described that patients with PBC diagnosed with overlap syndrome based on the revised IAIHG scoring system had higher prevalence of features of portal hypertension and cirrhosis, and had a survival disadvantage compared to those with PBC alone [58]
Floreani et al [90] have shown that patients benefit from immunosuppression and ursodeoxycholic acid (UDCA) therapy, with lack of progression of Mayo score prognostic index during follow-up and survival that was apparently better than in PSC alone after a median of 207 months; no comparisons were made with survival in patients with AIH alone in this study
Summary
Autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are presumed autoimmune liver diseases. Much debate has ensured whether these represent presentations within a spectrum of autoimmune liver disease, variants of the classical autoimmune liver diseases, or distinct clinical entities These conditions exhibiting features of two different autoimmune liver diseases have commonly been designated overlap syndromes. Sudden deterioration of liver function or suboptimal response to treatment of a previously well controlled autoimmune liver disease should raise the suspicion of an overlap syndrome. The recognition of these potentially clinically distinct entities might have important implications from a classification standpoint, and for management [7,10] and for a better approach to understanding the pathophysiology of autoimmune liver diseases [7]
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