Overlap syndromes

© Springer Science+Business Media New York 2014. A proportion of patients within the spectrum of autoimmune liver diseases may present with overlapping features of two classical disorders such as autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC). These patients are often designated with the term “overlap syndromes” [1-4]. They usually represent a diagnostic challenge due to the lack of standardized diagnostic criteria. Nevertheless, recognizing this group of patients is important clinically since there may be associated therapeutic and prognostic implications. The etiopathogenesis of these “syndromes” remains illusive and whether they represent separate entities or variants of the classical disorders remains controversial. However, there is no evidence currently to support “overlap syndromes” as separate entities [5], and the International Autoimmune Hepatitis Group (IAIHG) suggests that these patients should be classified under a primary disorder, according to the predominating feature(s) [5].