Primary sclerosing cholangitis associated with ulcerative colitis - a case report

Abstract

Background: Primary sclerosing cholangitis (PSC) is a rare disorder with progressive destruction of the bile ducts due to recurring inflammation and fibrosis. For most patients PSC is diagnosed incidentally and only 10-15% experience itching, abdominal pain, or fever. Diagnostic criteria include increased ALP levels, bile-duct strictures detected using MRCP, and exclusion of secondary sclerosing cholangitis. PSC is closely related to inflammatory bowel disease (IBD). Approximately 25% of PSC patients are diagnosed with IBD characterized by typical symptoms. However, about 65% of patients with PSC have asymptomatic IBD, which is diagnosed only by endoscopic procedures and histological analysis. Case report: A 29-year-old man attended a gastroenterology consultation due to transaminase elevation (ALT 216 IU/L, AST 72 IU/L). Abdominal ultrasound was performed and revealed markedly hyperechogenic portal vessels, liver normal in size, homogeneous, with increased echogenicity. The ultrasound elastography score was 7,3 kPa which refers to minimal scarring (F1). MRCP scan showed a local dilatation up to 0,47 cm in the right hepatic duct as a result of a short stricture. Several short strictures were also observed in segment 6. Both CBD and CHD were approximately 0,4 cm wide. ANCA antibodies were found positive. Summarising the findings, the PSC diagnosis was confirmed. For further examination, a colonoscopy was performed and a biopsy was taken. After histological analysis, ulcerative colitis (UC) was diagnosed. Ursodeoxycholic acid and Mesalazine treatment was started. During the course of treatment, the transaminases normalized, pruritus disappeared, endoscopic and histological remission of ulcerative colitis was achieved. Discussion with conclusion: Some patients diagnosed with PSC may have a co-existing asymptomatic inflammatory bowel disease. This includes UC, and less commonly Crohn‘s disease. Therefore, patients with PSC must be investigated for the diagnosis of IBD. Unfortunately, there is currently no effective medical treatment for PSC. Liver transplantation is considered the only option.