Ulcerative colitis may be accompanied by all of the following, except:
a. Extraintestinal manifestations of ulcerative colitis include arthritis, ankylosing spondylitis, erythema nodosum, pyoderma gangrenosum, and primary sclerosing cholangitis.
b. Arthritis, particularly of the knees, ankles, hips, and shoulders, occurs in about 20% of patients, typically in association with increased activity of the intestinal disease.
c. Ankylosing spondylitis occurs in 3% to 5% of patients and is most prevalent in patients who are HLA-B27 positive or have a family history of ankylosing spondylitis.
d. Erythema nodosum arises in 10% to 15% of patients with ulcerative colitis and often occurs in conjunction with peripheral arthropathy.
e. Pyoderma gangrenosum typically presents on the pretibial region as an erythematous plaque that progresses into an ulcerated, painful wound.
f. Arthritis, ankylosing spondylitis, erythema nodosum, and pyoderma gangrenosum typically improve or completely resolve after colectomy.
g. Primary sclerosing cholangitis (PSC) occurs in 5% to 8% of patients with ulcerative colitis.
h. Most patients with inflammatory bowel disease who develop PSC are younger than 40 years of age, and the majority are men.
i. Genetics likely play a role because patients with ulcerative colitis who have the HLA-B8 or HLA-DR3 haplotype are 10 times more likely to develop PSC.
j. Patients with PSC and ulcerative colitis typically have a more quiescent disease course; however, the risk for colon cancer in these patients is up to five times greater than in patients with ulcerative colitis alone.
k. These tumors are more likely to arise proximal to the splenic flexure. Primary sclerosing cholangitis may be asymptomatic and diagnosed only by abnormal laboratory studies, or it may present with symptoms of obstructive jaundice and abdominal pain.
l. The disease is progressive and ultimately fatal unless liver transplantation is undertaken. Colectomy has no effect on the course of PSC.