Sequential occurrence of primary sclerosing cholangitis and autoimmune hepatitis type III in a patient with ulcerative colitis: a follow up study over 14 years

In 1983, a female patient born in 1963 presented with symptoms of ulcerativ e colitis and typical clinical and histological signs of primary sclerosing cholangitis (PSC). At this time only pANCA were positive while other marke r antibodies for autoimmune liver disorders could not be detected. In summe r 1987 the clinical picture changed and was replaced by laboratory and hist ological signs typical of autoimmune hepatitis (BIH). Thus, IgG levels incr eased considerably and cholestatic enzymes became normal. For the first tim e, anti-liver-pancreas antibodies (LP), a diagnostic marker for AIH type II I could be detected. In the following years several relapses occurred also induced by repeated discontinuation of immunosuppressive therapy. Symptoms of colitis persisted but signs of cholestasis remained absent for the follo wing ten years. In 1997, colitis exacerbated again and colectomy had to be performed together with liver transplantation. Surprisingly, histology of t he explanted liver now showed the typical features of PSC stage III/IV whil e the significant criteria for AIH were now lacking. Thus, progression to c irrhosis was, probably, mainly induced by the biliary destructive and fibro tic process although biochemical and serological data were clearly indicati ve of an autoimmune, i.e. AIH-related manifestation.