A 74-year-old woman had her LFTs checked routinely as part of her annual hypertension screen. Her medical history includes sciatica, hypothyroidism, mild depression and psoriasis. Her current medications include amiloride 5mg once daily, atenolol 50mg nightly, calcipotriol, furosemide 40mg once daily, sertraline 50mg once daily and levothyroxine.
Her LFTs revealed raised GGT and ALP of 92 and 184 units/L respectively. Further history was taken and revealed that the patient did not drink alcohol and had never taken a statin or other medication potentially affecting her LFTs. However, she did report undergoing a blood transfusion many years ago and also thought that her mother possibly had 'liver cancer' but the details of this were unclear.
The patient denied any abdominal pain and no stigmata of chronic liver disease were noted. She was a smoker, and had reported some fatigue over a couple of months but her hypothyroidism was uncontrolled and recent adjustments had been made to her thyroxine in order to normalise her TSH.
Repeat LFTs four months later showed that both ALP and GGT had increased to 196 and 135 units/L respectively. Based on this, an ultrasound scan and full liver screen were organised. The ultrasound scan was reported as normal; however, the liver screen revealed a positive M2 and anti-mitochondrial antibody. Her cholesterol was also high, at 7.5mmol/L – her cardiovascular risk warranted statin treatment.
The patient was subsequently referred as a case of primary biliary cirrhosis (PBC) to the hepatology department; appropriate treatment has been commenced.
Primary biliary cirrhosis
PBC has an estimated prevalence of 12.9 per 100,000 population. It is more common in females around the fifth decade of life.1 Fatigue is the most common symptom but other symptoms can include pruritus, jaundice and right upper quadrant pain. Look for stigmata of chronic liver disease on examination.
PBC is often diagnosed following further investigation of abnormal LFTs, namely raised ALP and GGT, because the disease involves the biliary tree. Anti-mitochondrial antibody is highly specific for the condition. There is a strong association with other autoimmune conditions, particularly thyroid disease.
Other basic primary care investigations can include ultrasound scan of the abdomen to look for other causes of the deranged LFTs and to see if there is evidence of cirrhosis. Further investigations in secondary care may involve CT or MRI. A liver biopsy may also be indicated to stage the disease.
Treatment focuses on slowing disease progression and alleviating symptoms. Fatigue is difficult to treat; however, pruritus can be treated with sedating antihistamines, emollients and drugs that sequester bile salts to prevent bile salt deposition in the skin.
Ursodeoxycholic acid is used to slow disease progression, with mixed reports as to whether it improves survival. Other treatments involve altering the autoimmune component of the condition, for example with methotrexate.
Transplant is also performed, although the autoimmune process affects the transplanted liver and other problems such as rejection are likely.
- Dr Singh is a GP in Northumberland
1. Selmi C, Invernizzi P, Keeffe EB et al. Epidemiology and pathogenesis of primary biliary cirrhosis. J Clin Gastroenterol 2004; 38 (3): 264-71.