Study: Impact of Clinical manifestations and outcome of ulce
Primary sclerosing cholangitis (PSC) is an idiopathic, inflammatory, and fibrotic disease characterized by strictures and dilatations of both the intra- and extrahepatic bile ducts. A total of 60%–80% of PSC patients have concomitant inflammatory bowel disease (IBD), with ulcerative colitis (UC) being the most common form and less frequently Crohn's disease (CD). However, pediatric IBD with PSC is uncommon, affecting 9.9% of children with UC and 0.6% of patients with CD.

Children with UC-PSC are at an increased risk of active endoscopic and histologic disease in the absence of symptoms and present significant pancolitis with a right-sided predominance, macroscopic rectal-sparing, and a higher risk of colorectal cancer. Ileocolonoscopy should be conducted in IBD patients with concurrent PSC annually or at least biannually, starting from the time of PSC diagnosis.
Children with PSC-IBD have more serious growth impairments than children with UC or unclassified IBD.6

In pediatric PSC-IBD, 28% of patients presents a unique phenotype characterized by an overlap with autoimmune hepatitis (AIH). The overlap syndrome of PSC and AIH is more common in children than in adults and induces milder colitis activity and may be treated with steroids and immunomodulators.

In conclusion, pediatric UC-PSC has a distinct phenotype, including milder bowel disease, less treatment with steroids or infliximab, lower rate of IBD-related admission and surgery, more common pancolitis and rectal-sparing, higher risk of colorectal cancer, and greater growth impairment. Annual or at least biannual ileocolonoscopy is recommended. PSC and AIH overlap syndrome is more common in children and can be treated with steroids and immunomodulators.