Autoimmune liver disease | Pediatric Oncall Journal

REVIEW ARTICLE

Year : July-September 2014 | Volume : 11 | Issue : 3

DOI : 10.7199/ped.oncall.2014.53

AUTOIMMUNE LIVER DISEASE

Rodrigo Liberal¹, Diego Vergani¹, Giorgina Mieli-Vergani².
¹Institute of Liver Studies, King’s College London, UK, ²Paediatric Liver,GI and Nutrition Centre, School of Medicine at King’s College Hospital, London, UK.

Abstract

Autoimmune liver disorders in childhood include autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (ASC). These inflammatory liver disorders are characterised histologically by interface hepatitis, biochemically by elevated transaminase levels and serologically by autoantibodies and increased levels of immunoglobulin G. AIH is particularly aggressive in children and progresses rapidly unless immunosuppressive treatment is started promptly. With appropriate treatment some 80% of patients achieve remission and long-term survival. For non-responders and difficult-to-treat patients, novel and more effective therapeutic approaches are sought. ASC responds to the same treatment used for autoimmune hepatitis in regards to parenchymal inflammation, but bile duct disease progresses in about 50% of cases, leading to a worse prognosis and a higher liver transplantation requirement; moreover, it has a high recurrence rate after transplant. Progression of liver disease and recurrence after transplant are more common in patients with associated poorly controlled inflammatory bowel disease, which therefore needs to be treated aggressively and effectively.

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