1. Practical Approaches to Alleviating Your PSC Symptoms
Marion Peters MD
University of California San Francisco

2. Early PSC PSC can be silent
15-40% of patients are asymptomatic at presentation
55% of patients have asymptomatic increase of liver enzymes

3. Prevalence of symptoms in PSC
Frequency, %
None
15–55
Fatigue
50–75
Pruritus
40–70
Jaundice
9–69
Abdominal pain
16–60
Weight loss
10–34
Fevers and chills
5–28
Hyperpigmentation 25
Silveira and Lindor 2008

4. Fatigue
Important to exclude other conditions e.g. hypothyroidism, anemia
Urso does not seem to help with fatigue
Fatigue cannot be explained by depressive symptoms
Fatigue may be centrally mediated: e.g. corticotrophin-releasing hormone or serotonergic neurotransmitter systems
Van Os et al. J Hepatol Jun;46(6):

5. Possible Causes of Pruritus- itching
Bile duct obstruction
Bile acids
Endogenous opioids
Histamine
Autotaxin-LPA

6. Management of Pruritus
Important to rule out biliary obstruction:
increase in serum bilirubin and/or worsening pruritus
progressive bile duct dilatation on imaging studies 
Start with ultrasound and liver tests
Recommend performing an ERC or MRCP if new change in US or LFTs

7. Cholangitis Symptoms
pain in the right upper quarter of the abdomen (pain may also occur in the chest, in the upper back, or the right shoulder) 
nausea and vomiting 
fever 
chills 
jaundice -yellowing of the skin and eyes 
low blood pressure 
lethargy 
decreased level of alertness 
Worse itching 
pale stools 
dark urine 

8. PSC: Treatment for infections
Narrowed or blocked bile ducts may cause bacterial infections
To prevent and treat these infections, people with PSC may take repeated courses of antibiotics or continue taking rotating antibiotics.
With any procedure that could cause an infection, such as an endoscopic procedure or abdominal surgery, antibiotics are needed

9. Pruritus- itching
Pruritus is common symptom in all forms of cholestatic liver diseases
The severity does not correlate with levels of
serum bile acid
Endogenous opioids
Histamine
Tryptamine
Substance P

10. Pruritus and Autotaxin
The severity does correlate with serum Autotaxin levels
Autotaxin is an enzyme that produces lysophosphatidic acid
Lysophosphatidic acid into skin of mice produces scratching
Patients with itching/ cholestasis have high LPA levels

11. Autotaxin LPA Itching
Kremer et al: Gastroenterology. 2010 

12. Pruritus - Treatment
Symptomatic
Dermatologic
Intestine
Liver

13. Management of Pruritus
Skin Therapy
Lower bathing water temperature
Fewer or lighter clothing and bed coverings
Minimize dry skin by using moisturizing soaps (e.g., Dove)
Apply topical moisturizers liberally (e.g., Eucerin cream)

14. Management of Pruritus
Stabilize mast cells in skin
Doxepin 25–50 mg PO daily taken at night
May induce sleepiness so best to take at night

15. Management of Pruritus
INTESTINE
Anion-Exchange Resins- binds bile salts
Cholestyramine or colestipol: 2-6 packets per day
start with 1 three times a day
Separated from other medications by 2 hours (especially ursodeoxycholic acid)

16. Management of Pruritus
LIVER
Decrease Bile Salts and increases bile flow
Ursodeoxycholic acid, 13 mg/kg/day at night
Hepatic Microsomal Enzyme Induction
Rifampin, 150 mg, PO, two- three times daily
Opioid Receptor Antagonists
Naltrexone, 12.5 mg PO daily to 50 mg PO daily
Naloxone and nalmefene are commonly available only for parenteral use

17. Monitoring PSC Yearly For Osteoporosis check 25-OH Vitamin D Calcium
Calcium  24 hour
urine Ca
Bone density
Vitamin A
For Osteoporosis check
Calcium
25-OH Vit D
HRT
Calcitonin
Bisphosphonates
Water Sol Vit A
HRT= hormone replacement therapy

18. Metabolic bone disease
Initial screening for osteopenia is recommended and thereafter at 2-3 year intervals depending upon result
Osteopenia is characterized by a T-score between 1 and 2.5 standard deviations below the density
Osteoporosis as a T-score beneath The incidence of osteoporosis in PSC is between 4 and 10%

19. Treatment of Osteoporosis
Vit D deficiency: bone pain or no symptoms
Check 25-OH vitamin D levels
If normal give calcium g with vitamin D
If low, vitamin D2 should be given to promote calcium absorption
If Vitamin D does not rise with D2 then 1,25-OH vitamin D is required (calcitriol)
In patients with proven osteoporosis bisphosphonates may be needed

20. PSC: Self Care Fatigue is common in PSC
Some treatment may decrease fatigue but your signs and symptoms may still persist. You might find relief with complementary and alternative treatments that have shown some benefit for fatigue, such as:
Exercise, such as walking 30 minutes most days of the week
Spending time with friends and family
Stress management techniques, such as meditation and relaxation exercises
Yoga

21. Nutrition support
Primary sclerosing cholangitis makes it difficult to absorb certain vitamins.
Check vitamins
Even with a healthy diet, patients may not get all the nutrients need.
Night time snacks
Your doctor may recommend vitamin supplements If the disease weakens your bones, you may take calcium and vitamin D supplements as well.

22. Health Maintenance Don't drink alcohol.
Get vaccinated against hepatitis A and B.
Use care with chemicals at home and at work.
Maintain a healthy weight.
Follow directions on all medications, both prescription and over-the-counter.
Make sure your pharmacist and any doctor prescribing for you know that you have a liver disease.
Talk to your doctor about any herbs or supplements you're taking since some can be harmful to your liver.