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.
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