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
- 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