You Only Live Twice: PSC as an Extraintestinal Manifestation of IBD

You Only Live Twice: PSC as an Extraintestinal Manifestation of IBD
In my first column, I discussed how my Crohn’s journey began with my primary sclerosing cholangitis (PSC) diagnosis almost 25 years ago. The National Organization for Rare Disorders (NORD) estimates that “60 to 80 percent of individuals with PSC also have inflammatory bowel disease (IBD), most often ulcerative colitis,” While the probability of PSC patients having this dual diagnosis is high, only 1.4 percent to 7.5 percent of IBD patients develop PSC. PSC is an autoimmune disease of the ducts that carry bile from the liver to the gallbladder and small intestine. Inflammation of the bile ducts causes them to stricture, or scar and narrow. Bile then accumulates in the liver, damaging the cells. Although the liver can regenerate, PSC causes scar tissue to form instead of healthy tissue. The buildup of scar tissue, or cirrhosis, results in irreversible damage and liver failure. According to NORD, medical literature first described PSC in 1867. Although doctors have not yet found a cure, some treatments can ease symptoms and slow the progression of the disease. One treatment is endoscopic balloon dilation. After my PSC diagnosis, my gastroenterologist (GI) performed an endoscopic retrograde cholangiopancreatography to assess my strictures. During the procedure, he inserted balloons to open the worst of the ducts. He performed the procedure as needed depending on the severity of my symptoms until I changed gastroenterologists after my Crohn's diagnosis in 2006. Two common medications to treat PSC are prednisone and ursodiol. As with the treatment for IBD, the corticosteroid prednisone acts as an anti-inflammatory. Because of the adverse effects of longterm prednisone use, my GI took me off prednisone and prescribed ursodiol. Ursodiol is a synthetic f
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