Below I’ve included a few slides and some notes; my notes may have errors of omission or transcription.

Primary Sclerosing Cholangitis: Beyond Anecdotal Medicine  Jim Squires

Key points:

• MMP-7 is emerging as better biomarker than alk phos or GGT
• Patients with PSC-IBD often have PUCAI scores which underestimates severity of IBD activity. Even PSC-IBD patients in “clinical remission” often have disease activity.
• PSC-IBD phenotype includes pancolitis (often with rectal sparing and backwash ileitis
• Long-term prognosis is associated with level of GGT values
• Prognosis: ~70% have event-free survival at 5 years
• Adult prognosis models are inadequate due to frequent differences between disease in children and disease in adults.  Adults also have more comorbidities: obestiy, smoking, alcohol and medications
• SCOPE index is a useful prognostic model for children (scores of 3 or less indicate very low risk of disease progression over next 5 years)
• Actigall is current first line treatment in children based on biochemical improvement (no long term proof of efficacy); vancomycin has only anecdotal evidence of effectiveness

Related blog posts:

• Easy Advice for Pediatric Hepatologists: PSC Guidelines from AGA  Best practice advice 6: “Surveillance for cholangiocarcinoma should not be performed in PSC patients with small-duct PSCs or those younger than age 20.”
• Ursodeoxycholic Acid for Pediatric PSC
• Active Colitis More Likely in Children with PSC-IBD
• Big Study of PSC in Children
• PSC -Natural History Study (pediatric)
• Primary Sclerosing Cholangitis (PSC) –Natural History Study
• Why does PSC increase the risk of colorectal cancer in UC?
• Should We Care About Subclinical Primary Sclerosing Cholangitis with Inflammatory Bowel Disease?
• Staying current with PSC
• Primary Sclerosing Cholangitis 2016 | gutsandgrowth
• Population-Based Outcomes for Primary Sclerosing Cholangitis
• PSC 2013 Review