Sclerosing Cholangitis in Children and Adolescents.

Sclerosing cholangitis in pediatric age is a severe disease, often associated with inflammatory bowel disease. It recognizes different etiologies. Management and prognosis depend on the underlying cause. A high proportion of patients have autoimmune features similar to those of autoimmune hepatitis and respond biochemically to immunosuppression, although bile duct disease progresses in half of them leading to liver transplant. The disease can recur after transplant. Severity of liver disease and risk of recurrence after transplant are linked to the severity of bowel disease.