PBC Information

Characteristics of PSC

70-80% of patients have IBD
- UC or Crohn's colitis
- Extensive involvement
- Mild disease
Men > women
Cholangiocarcinoma
Variable progression to cirrhosis
Intra hepatic vs extra hepatic

Small duct disease

Decreased risk with smoking

Epidemiology of PSC

Region	n	Population	Prevalence (per 100,000)
Olmstead, MN	22	125,000	13.6
Swansea, Wales	53	251,000	12.7
Oslo	17	130,000	8.5
Spain	43	19,230,000	0.2
Alaska (Natives)	0	--	0

What Causes PSC?

Genetics + Environment + ? = PSC

Why did I get PSC?

(Graphic illustrating that people who get PSC probably have genetics and live in enviroments that predispose them to get it)

(Untitled page with Graphic illustrating possible relationship between IBD, CD44 T cells and PSC)

MRC vs ERC

(Graphics comparing images obtained using MRC imaging technology to invasive procedure ERC)

Anti-neutrophil Antibodies

c-ANCA
p-ANCA
Atypical p-ANCA

PSC Natural History

(Three superimposed graphs, all showing declining probability of survival in the untreated disease over time)

Small Duct PSC

(Graph comparing the survival rates of patients diagnosed with small duct vs large duct PSC, with survival rates higher in small duct PSC)

PSC Complications

Choloangiocarcinoma
Colorectal cancer
Esophageal varices
Osteoporosis
Fat soluble vitamin deficiencies
Pancreatitis

UDCA for PSC (13-15 mg/kg)

(Graph illustrating UDCA dosed as for PBC vs placebo with very little difference in survival rates)

UDCA for PSC (17-23 mg/kg)

(Graph illustrating UDCA dosed higher than for PBC vs placebo with some improvement in survival rates)

UDCA decreases Colorectal Cancer in PSC

(Graph illustrating UDCA vs placebo significantly improving survival rates)

Liver Transplantation for PSC

(Graph illustrating post-transplantation survival rates similar to but slightly lower than those of PBC patients post-transplant)

PSC Research Goals

Improve diagnosis
- Currently requires ERC or MRC
- No specific blood test
Develop prognostic models
- Be able to predict course of disease in an individual
Design and Test New Treatments

Future Therapies

High dose UDCS (25-30 mg/kg)
Anti-TNF therapy
High dose immunotherapy
Anti-fibrotics

PSC Patient Resources

UC Davis PSC Patient Registry
California PSC Support Group
Yahoo PSC-Support Group
STOPSC
- 12 sites including UCSF - https://web.emmes.com/study/psc/index.html

Primary Sclerosing Cholangitis

Chris Bolus, MD, UC Davis