Gastroenterology Education and CPD for trainees and specialists

1. Which one of the following statements regarding PBC is true?

1. Pruritus is the commonest symptom
2. Pruritus is always generalised
3. The likely causes of pruritus include accumulation of bile acids in the skin or it may be related to retention of opioid agonist substrates
4. Serum transaminases are normally significantly raised
5. AMA is normally positive in 85% of cases

Explanation :Fatigue as opposed to pruritus is the most common symptom in PBC. Fatigue does not correlate with the severity, histologic stage, or duration of PBC. . The likely causes of pruritus include accumulation of bile acids in the skin or it may be related to retention of opioid agonist substrates and up regulation of opioid receptors. Pruritus can be local or diffuse, usually worse at night and is often exacerbated by contact with wool, other fabrics, heat, or pregnancy Transaminases are only slightly elevated. Marked elevations of transaminases levels over 200-300U/l are distinctly unusual. Consider other or dual pathologies AMA is found in nearly 95% of patients with PBC. It is a highly disease-specific autoantibody and is found in less than 1% of normal controls. The presence or absence of antibody, rather than the titres of antibody level, is most important.

2. Which one of the following statements regarding treatement of PBC is false?

1. UDCA in a dose of 13-15 mg/kg/day is the only approved therapy
2. UDCA has been shown to improve liver biochemistry but it does not slow histologic progression
3. UDCA therapy does not improve fatigue, pruritus.
4. Bile acid sequestrants should be used as initial therapy for patients with PBC who have pruritus
5. Rifampicin, Oral opiate antagonists such as naltrexone and Sertraline all are recommended for treatment of pruritus

Explanation :UDCA in a dose of 13-15 mg/kg/day is the only approved therapy UDCA has been shown to improve liver biochemistry, slows histologic progression, reduced need for liver transplantation and improved survival. UDCA therapy does not improve fatigue, pruritus, cholestasis associated bone disease, or autoimmune features found in association with PBC. Bile acid sequestrants should be used as initial therapy for patients with PBC who have pruritus. The recommended dose of cholestyramine is 4 g per dose to a maximum of 16 g/day given 2-4 hours before or after UDCA Rifampicin is given for pruritus in doses of 150-300 mg twice daily . It may cause hepatitis and hepatic failure, haemolysis, renal impairment, and alteration in drug metabolism. Thus rifampicin treatment needs regular follow-up of blood tests Oral opiate antagonists such as naltrexone 50mg daily is another treatment option. Sertraline (75-100 mg daily) can be tried when other measures fail

3. Which one of the following statements regarding treatement of PBC is false?

1. Serum bilirubin exceeding 8.5mg/dl (150 umol/l) or evidence of decompensated liver disease are indications of liver transplant
2. Patients are not in increased risk from cardiovascular disease
3. First degree female relatives including sisters and daughters should be screened
4. Pruritus improves once cirrhosis is developed
5. a.Normally serum lipids are only mildly raised

Explanation :Serum bilirubin exceeding 8.5mg/dl (150 umol/l),evidence of decompensated liver disease, and intractable symptoms like pruritus, incapacitating fatigue or severe osteoporosis are all indications of liver transplant. Serum lipids may be strikingly elevated in PBC. Levels of HDL cholesterol are disproportionately elevated compared to LDL cholesterol. This is of little consequence as retrospective studies suggest that there is no increased risk of cardiovascular disease in patients with PBC and hypercholesterolemia. However, in patients at high risk of cardiovascular disease, it may be appropriate to use cholesterol lowering drugs. (Statins are safe in PBC) Family members of patients with PBC are at increased risk of developing PBC, particularly among first degree female relatives including sisters and daughters.Screening is usually done by measuring the serum alkaline phosphatase level and if elevated by assessing for AMA. The value of screening these individuals for PBC has not been established, however Once pruritus occurs in PBC, it is unlikely to disappear completely without treatment until a patient develops cirrhosis and liver failure

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