Curr Hepatology Rep (2017) 16:198–204 DOI 10.1007/s11901-017-0353-y AUTOIMMUNE, CHOLESTATIC, AND BILIARY DISEASES (S GORDON AND C BOWLUS, SECTION EDITORS) Autoimmune Sclerosing Cholangitis: a Review of the Overlap between Autoimmune Hepatitis and Primary Sclerosing Cholangitis in Children 1 2 1,3 C. Jaramillo & P. L. Valentino & M. Deneau Published online: 11 July 2017 Springer Science+Business Media, LLC 2017 Abstract response to immunosuppression. Rates of liver transplantation Purpose of Review Pediatric patients with autoimmune liver for ASC are identical to those of PSC. disease (ALD) frequently demonstrate clinical, histopatholog- Summary Rather than specifically labeling patients as ASC, ic, cholangiographic, and serologic overlap between primary the most important tasks for clinicians managing ALD are to sclerosing cholangitis and autoimmune hepatitis, termed auto- detect treatable autoimmune lobular inflammation in PSC pa- immune sclerosing cholangitis (ASC). Several large, collabo- tients and to identify cholangiopathy in AIH patients with rative studies have shed light on the epidemiology and natural persistent biochemical abnormalities prior to therapeutic history of ASC in the last 5 years. A detailed genetic and escalation. environmental description of the pathogenesis remains lack- . . ing however. Here, we review recent advancements in knowl- Keywords PSC ASC Primary autoimmune sclerosing edge on
Current Hepatology Reports – Springer Journals
Published: Jul 11, 2017
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