Effective Strategies for Managing Late-Stage Primary Biliary Cirrhosis?

Question

When a patient with primary biliary cirrhosis (PBC) begins to exhibit markedly abnormal liver function tests and is experiencing increasing pruritis that is not relieved by an H1 blocker, hydroxyzine, or cholestyramine, what other therapies may be beneficial? The patient is unable to tolerate doxepin.

Charla Bright, PA-C

Response from  Mary P. Ettari, MPH, PA-C 
A PA in family practice with Medical Partners of Martin County, Stuart, Florida. She is immediate past-president of the Florida Academy of Physician Assistants, past secretary of the American Academy of Physician Assistants, and presently a trustee of the PA Foundation.

PBC is an autoimmune disorder characterized by pruritis and fatigue. The cause is unknown but the mechanism of the disease produces a chronic inflammatory reaction in the liver that damages the interlobular bile ducts, leading to progressive cholestasis and finally cirrhosis.[1]

The disease affects mainly middle-aged women between the ages of 35 and 60 but can affect all ages and races. At least 30% of patients are initially asymptomatic. The diagnosis is usually made during routine bloodwork.[2]

The clinical course of the disease is divided into 4 stages. Stage I is characterized by lymphocytic destruction of the interlobular ducts, and during stage II, there is bile ductular proliferation. Stages III and IV have worsening fibrosis, with cirrhosis occurring in stage IV.[3]

Most cases of PBC are diagnosed on the basis of abnormal liver function tests (LFTs), which lead to further investigation and the ultimate diagnosis of PBC. The most common symptoms are fatigue and pruritis. Additional symptoms are xanthelasma on the eyelids, xanthoma on the palms of the hands and heels, neuropathy, and asymptomatic urinary tract infections (UTIs). As the disease worsens, the patient may develop esophageal varices, osteopenia and osteoporosis, and hepatocellular carcinoma. About 50% of patients present with an enlarged nontender liver, 25% with splenomegaly, 10% with hyperpigmentation, and fewer than 10% with jaundice alone.[2]

The presence of antimitochondrial antibody (AMA) in the serum is a hallmark in the diagnosis of PBC, although not all patients test positive.[3] Additionally, elevations of alkaline phosphatase, gammaglutamyl transpeptidase, and 5'-nucleotidase may be found. These enzymes may be substantially elevated; a less dramatic elevation of the serum aminotransferase may be seen. The conjugated fraction of hyperbilirubinemia may also be elevated.[1]

Medications used in the therapy of PBC are ursodeoxycholic acid 12-15 mg/kg each day in divided doses.[1] Treatment with immunosuppressive agents such as azathioprine and cyclosporine have not improved survival. A clinical trial evaluating methotrexate is currently being evaluated. Colchicine may improve liver function in terms of prothrombin time and serum albumin, but no large-scale studies have yet been done to show improved survival.[1]

Other strategies used to control pruritus include avoiding nylon or wool next to the skin, avoiding hot showers, and not getting overheated in bed at night. Ultraviolet light helps some, but sunburn must be avoided. The old standby of bicarbonate of soda in a cool bath is soothing, as is a post shampoo rinse of bicarbonate of soda. Avoidance of perfumed soaps, bath gels, and powder may also lessen pruritis.[4]

Liver transplantation remains the only cure for PBC and has a survival rate of greater than 70%.Posted 03/13/2001

References

Heathcote J. Primary biliary cirrhosis. In: Harrison's Online. New York, NY: McGraw-Hill Companies, Inc. 2000. Available at:
http://www.medscape.com/HOL/articles/2000/11/hol52/hol52-01.htmlBerkow R, Beers MH, Fletcher AJ, Bogin RM, eds. The Merck Manual. Whitehouse Station, NJ: Merck and Co., Inc. 2000. Available at:
http://www.merckhomeedition.com/home.htmlHeathcote J. Update on Primary biliary cirrhosis. Can J Gastroenterol. 2000;14:43-48.That dreaded itch. The PBC Foundation. Available at: http://www.nhtech.demon.co.uk/pbc/itching.html

Medscape Family Medicine/Primary Care.  2001;3(1) ©2001 Medscape


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