Review Question - QID 108652

The most likely diagnosis in this patient is ulcerative colitis, which is associated with an increased risk of developing primary sclerosing cholangitis (PSC).

Ulcerative colitis is a form of inflammatory bowel disease (IBD) that is characterized by inflammation extending proximally from the rectum through the colon in a contiguous fashion. Unlike Crohn's disease, another form of IBD, ulcerative colitis does not involve the terminal ileum and does not have areas of normal mucosa (“skip lesions”). Crypt abscesses and pseudopolyps are common biopsy findings in ulcerative colitis. Clinically, ulcerative colitis can present with bloody diarrhea, colicky abdominal pain, fever, and occasionally large joint arthritis. Initial treatment for ulcerative colitis is mesalamine. Ulcerative colitis is associated with an increased risk of PSC, a chronic inflammatory cholestatic disease affecting the intrahepatic and extrahepatic bile ducts. This disease leads to insidious onset of biliary obstruction, jaundice, pruritis, weight loss, and fatigue. Importantly, PSC increases the risk of cholangiocarcinoma, a rare but deadly cancer of the gallbladder.

Incorrect Answers:
Answer 1: Primary biliary cirrhosis (PBC) is an inflammatory disease of the intrahepatic bile ducts that classically affects young females with autoimmune disorders. Unlike in PSC, the extrahepatic bile ducts are spared in PBC. Common symptoms include fatigue, pruritis, and jaundice. PBC is not associated with ulcerative colitis.

Answer 3: Intestinal strictures are areas of focal narrowing of the gastrointestinal tract that lead to obstructive symptoms. Strictures are sometimes seen in Crohn's disease but not ulcerative colitis.

Answer 4: Perianal fistulae are inflammation-induced connections between the gastrointestinal tract and the perianal skin surface. Fistulae are seen in Crohn's disease but not ulcerative colitis.

Answer 5: Aphthous ulcers are painful oral lesions that are seen in Crohn's disease and to a much less common extent in ulcerative colitis. However, ulcerative colitis is much more commonly associated with PSC.

Bullet Summary:
Ulcerative colitis is characterized by continuous inflammatory lesions extending proximally from the rectum. It is associated with an increased risk of PSC, a chronic cholestatic condition causing intrahepatic and extrahepatic biliary obstruction.