Increased cholestatic enzymes in two patients with long-term history of ulcerative colitis: consider primary biliary cholangitis not always primary sclerosing cholangitis

BMJ Case Rep. 2017 Sep 25:2017:bcr2017220824. doi: 10.1136/bcr-2017-220824.

Abstract

Several hepatobiliary disorders have been reported in ulcerative colitis (UC) patients with primary sclerosing cholangitis (PSC) being the most specific. Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, rarely occurs in UC. We present two PBC cases of 67 and 71 years who suffered from long-standing UC. Both patients were asymptomatic but they had increased cholestatic enzymes and high titres of antimitochondrial antibodies (AMA)-the laboratory hallmark of PBC. After careful exclusion of other causes of cholestasis by MRI/magnetic resonance cholangiopancreatography (MRCP), virological and microbiological investigations, a diagnosis of PBC associated with UC was established. The patients started ursodeoxycholic acid (13 mg/kg/day) with complete response. During follow-up, both patients remained asymptomatic with normal blood biochemistry. Although PSC is the most common hepatobiliary manifestation among patients with UC, physicians must keep also PBC in mind in those with unexplained cholestasis and repeatedly normal MRCP. In these cases, a reliable AMA testing can help for an accurate diagnosis.

Keywords: inflammatory bowel disease; liver disease; ulcerative colitis.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Autoantibodies / blood
  • Cholagogues and Choleretics / therapeutic use
  • Cholangiopancreatography, Endoscopic Retrograde*
  • Cholestasis / enzymology*
  • Colitis, Ulcerative / complications
  • Colitis, Ulcerative / enzymology*
  • Diagnosis, Differential
  • Female
  • Humans
  • Liver Cirrhosis, Biliary / diagnosis*
  • Liver Cirrhosis, Biliary / drug therapy
  • Liver Cirrhosis, Biliary / physiopathology
  • Liver Function Tests*
  • Male
  • Treatment Outcome
  • Ursodeoxycholic Acid / therapeutic use

Substances

  • Autoantibodies
  • Cholagogues and Choleretics
  • Ursodeoxycholic Acid