Abstract
Primary sclerosing cholangitis is a chronic progressive disease of the biliary tract. There are many differences be-tween adult primary sclerosing cholangitis and juvenile type.
Major part of pediatric PSC patients will develop inflammatory bowel disease (IBD). The phenotype of IBD in patients with PSC-IBD differs from those without associated PSC and is characterized by a milder course and typical findings of pancolitis, rectal sparing and backwash ileitis.
Endoscopic activity of the disease is more often present despite the absence of symptoms suggesting subclini-cal inflammation. The course of PSC associated with Crohn's disease is usually less severe than that with ulcerative colitis (UC).
Compared to patients with an isolated form of UC, patients with PSC-UC are more often treated with amino-salicylates monotherapy. Likewise, these patients have been reported to have a lower risk of undergoing colectomy or requiring steroids, thiopurines, or biologic therapy than patients without associated PSC.
Recent data suggest that none of the current treatment modalities can stop the progression of PSC, and significant number of patients will end up with liver failure requiring a liver transplant.