Abstract
Sclerosing cholangitis (SC) is a rare chronic disorder characterised by inflammation
and progressive obliterative fibrosis of the intrahepatic and/or extrahepatic bile
ducts.
Diagnosis is based on cholangiogram showing bile duct dilatation, narrowing and obliteration
of the biliary tree, and histologically, on the presence of inflammatory bile duct
damage leading to periductal fibrosis.
In children the most common SC is associated with strong autoimmune features, overlapping
with those of autoimmune hepatitis (AIH); this form is known as autoimmune sclerosing
cholangitis, ASC.
Conversely, primary SC (PSC), a condition in which the term “primary” indicates that
aetiology and pathogenesis are unknown, is rare in paediatrics. Secondary SC (SSC)
defines a cholangiopathy associated with an identifiable aetiology such as immunodeficiencies,
infections or haematological disorders. ASC and PSC are strongly associated with inflammatory
bowel disease (IBD).
ASC responds biochemically well to immunosuppressive drugs and ursodeoxycholic acid
(UDCA). Primary forms are exclusively managed with oral UDCA, while in the secondary
forms the medical treatment depends on the underlying aetiology.
Despite treatment, SC often progresses to biliary cirrhosis and end-stage liver disease
requiring liver transplantation. The disease can recur after transplant.
Better understanding of pathogenic mechanisms and better treatment modalities are
needed to improve the prognosis of this invalidating hepatic disorder.
Keywords
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Article info
Publication history
Published online: July 18, 2021
Accepted:
June 23,
2021
Received in revised form:
June 8,
2021
Received:
March 18,
2021
Identification
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© 2021 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.
