Primary Sclerosing Cholangitis

Features of inflammatory bowel disease differed between children with primary sclerosing cholangitis and those without it, similar to adult patients, according to study results.

A single liver stiffness measurement and changes in liver stiffness over time correlated with hepatic decompensation in patients with primary sclerosing cholangitis, researchers found. However, changes in liver stiffness occurred slowly in those without advanced fibrosis or decompensation.

SAN ANTONIO — At this year’s National Liver Congress, Gideon M. Hirschfield, MD, presented real-life cases of patients with primary sclerosing cholangitis along with an overview of how autoimmune liver disease may change over time from initial presentation.

CHICAGO — Large duct injury in pediatric patients with primary sclerosing cholangitis and autoimmune sclerosing cholangitis correlated with a distinct pattern of hepatic gene expression, which may limit benefits from T cell-directed immunosuppressive therapy, according to a study presented at NASPGHAN 2019.

Children with inflammatory bowel disease and primary sclerosing cholangitis may have milder disease activity, but the lack of symptoms does not always correlate with an absence of mucosal inflammation, according to published findings.

Autoimmune liver diseases include primary biliary cholangitis, primary sclerosing cholangitis, and pediatric diseases such as biliary atresia and progressive familial intrahepatic cholestasis. Researchers continue to investigate and develop potential treatments for both the diseases and their debilitating complications.

Immunic announced the first patient enrolled in a proof-of-concept clinical trial of IMU-838 for the treatment of primary sclerosing cholangitis, according to a press release.