A case of primary sclerosing cholangitis associated with ulcerative colitis and abnormal immunological findings

Abstract

A 46-year-old male was admitted for evaluation of hepatic dysfunction and bloody diarrhea on January 1986. Physical examination at that time disclosed icterus of palpebral conjuctiva and increased borborygmus in the left iliac region. When admitted the following laboratory examination values were noted: total bilirubin 2.6mg/dl, GOT 392IU/l, GPT 368IU/l, LDH 635IU/l, Al-p 2, 243IU/l, γ-GTP 328IU/l, LAP 363IU/l, total protein 8.2g/dl, γ-globulin 28.5%, ESR 29mm/h, CRP 1+, and occult blood for stool 1+, Barium enema examination showed loss of haustral markings. Diffuse multiple erosions and irregular ulcers were also observed endoscopically. Endoscopic retrograde cholangiogram showed irregular narrowing and dialation of intrahepatic bile ducts and extrahepatic biliary tracts. He had histories neither of biliary calculi nor surgical intervention. From these findings above and clinical courses, primary sclerosing cholangitis associated with ulcerative colitis was diagnosed. Interestingly, following immunological tests showed abnormal values: IgG 2, 431mg/dl, IgM 429mg/dl, positive antismooth muscle antibody, decreased activity of lymphocyte blastogenesis. He responded well to the therapy with prednisolone and salazopyrin. Laboratory examination values on March 1986 improved as follows: total bilirubin 0.6mg/dl, GOT 57IU/l, GPT 132IU/l, LDH 287IU/l, Al-p 622IU/l, γ-GTP 238IU/l, LAP 171IU/l, total protein 6.6g/dl, ESR 7mm/h, CRP-and negative anti-smooth muscle antiboby. This case suggests that abnormal immunological conditions may be involved in the pathogenesis of primary sclerosing cholangitis.