UNUSUAL PRESENTATION OF PRIMARY SCLEROSING CHOLANGITIS

A 23-year-old man presenting with acute pancreatitis and autoimmune he molytic anemia was diagnosed with primary sclerosing cholangitis (PSC) without evidence of ulcerative colitis. This constellation of rare as sociations constitutes a unique mode of presentation of PSC. Within tw o years he also developed ankylosing spondylitis with sacroiliitis. Di sordered immune regulation as a major factor in the mechanism of injur y in PSC is supported by its increased association with other immunolo gically mediated disorders, most notably ulcerative colitis. Autoimmun e hemolytic anemia, however, has been reported to be associated with P SC on only two occasions, and ankylosing spondylitis in the absence of ulcerative colitis is also unusual. In addition, the presentation of PSC with acute pancreatitis has rarely been described. This patient pr esented with several unusual features of PSC.