Open AccessAutoimmune sequential overlap syndrome (autoimmune hepatitis/primary sclerosing cholangitis) and inflammatory bowel disease: three clinical cases
Author(s) -
Pauliúñez Figueroa,
Rocío Sedaño,
Rodrigo Quera,
Gonzalo Carrasco Aviño,
Andrés O’Brien,
J Brahm
Publication year - 2020
Publication title -
revista española de enfermedades digestivas/revista española de enfermedades digestivas
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.331
H-Index - 38
eISSN - 2340-4167
pISSN - 1130-0108
DOI - 10.17235/reed.2020.6938/2020
Subject(s) - medicine , autoimmune hepatitis , primary sclerosing cholangitis , inflammatory bowel disease , overlap syndrome , gastroenterology , autoimmune disease , hepatitis , disease , immunology
Inflammatory bowel disease (IBD) is related to different liver extraintestinal manifestations and occurs with or without a link to disease activity. Primary sclerosing cholangitis (PSC) is the most common hepatobiliary manifestation. Other autoimmune hepatopathies may develop during the evolution of the latter, which is known as overlap syndrome. Sequential overlap syndrome occurs when these conditions appear in subsequent stages, and it is less frequently associated with IBD. We report three cases of sequential overlap syndrome with autoimmune hepatitis as the first manifestation, followed by PSC after 7-19 years and subsequently IBD. Liver extraintestinal manifestations may precede IBD by several years. Therefore, it is crucial to keep this association in mind, thereby reducing the diagnostic delay.