Symptomatic Therapy

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The most prevalent symptoms affecting patients with PBC are fatigue and pruritus. Both can be extremely debilitating and significantly impact on patients' quality of life.

Fatigue

Unfortunately, to date, no good therapy exists to manage fatigue in patients with PBC. There are many anecdotal reports that UDCA improves fatigue, and pilot studies of methotrexate (MTX) suggested that it may be effective for this purpose as well. Although targeted therapy may not markedly affect fatigue in PBC, it is important to ensure that there are no other contributing factors. Hypothyroidism is commonly associated with PBC and should be excluded. Fatigue is extremely common in the general population and is often multifactorial. It is important to take a good sleep history and to identify and correct any bad habits that may be worsening fatigue. Some common problems include

TABLE 121-1. Strategies for Prevention and Management of Primary Biliary Cirrhosis Symptoms and Complications

Problem

Management Strategy

Fatigue

Ensure no contributing factors, exclude

hypothyroidism

Pruritis

Cholestyramine 1 pkt before and after breakfast

Rifampicin 150 mg bid

Naltrexone 50 mg od (use cautiously)

Ultraviolet light exposure

Sialoadenitis/Sjogren's

Artificial teardrops

syndrome

Water, sugarless gum, pills with ++ water

Regular dental follow-up

Pilocarpine

Osteoporosis

All—calcium 1,500 mg/d + vitamin D 800 lU/d

If osteoporosis—bisphosphonates

Hormone replacement therapy (use cautiously)

Calcitonin

Hepatocellular carcinoma

Ultrasound screening every 6 mo for cirrhotics

Esophageal varices

Screen once platelets < 200,000/pL

p-Blocker/band ligation therapy as needed

Hypercholesterolemia

Not assisted with heart disease

Cholestyramine (first line)

"Statins" safe (if needed)

excessive caffeine and/or nicotine use, obesity with or without sleep apnea, use of sedatives that may impair sleep quality, and lack of exercise. Often improvement of one or more of these factors may make the difference between manageable and unmanageable fatigue.

Pruritus

Although the specific cause of pruritus is unknown, there is a range of therapeutic options for this troublesome symptom. Although bile acids per se are not likely the cause of pruritus, there is clearly a pruritogen in bile. Consequently, the use of cholestyramine as a binding agent is generally very successful for cholestasis-induced pruritus. It is given before and after breakfast to coincide with maximal gallbladder emptying. Although effective, it is important to warn patients that it may cause constipation and that it will bind all medications taken within 4 hours of ingestion, including UDCA. If patients take cholestyramine in the morning, it is best that they take UDCA, calcium, and vitamin D in the evening. If cholestyramine is not well tolerated or is ineffective, the anti-tuberculous medication rifampicin can be used. Although rifampicin can occasionally cause a hepatitis, this is generally seen only when used in combination with isoniazid. At the dose of 150 mg twice daily, adverse effects (aside from orange urine) are not generally seen. However, a recent report of three cases of rifampicin-induced hepatitis in patients with PBC stresses the importance of clinical follow-up in all patients on this medication. Opiate antagonists can be used if the above two medications are ineffective. It is believed that endogenous opioids may be overproduced in the liver in PBC and other chronic cholestatic conditions. Although reportedly effective, these agents should be used with caution because severe opiate withdrawal-type reactions have been reported. Generally, naltrexone is used at a dose of 50 mg daily. Some patients have also reported improved fatigue with opiate antagonists. Antihistamines should not be used to treat PBC-induced pruritus because they will not be effective and may contribute to fatigue. Exposure to ultraviolet light in the absence of sunblock is often helpful for pruitus as well. If pruritus is intractable and unresponsive to all agents, consideration of liver transplantation on this basis alone should be given. It is noteworthy that pruritus often improves as the disease progresses.

Sjogren's Syndrome

A symptom that is often underappreciated by physicians is sialoadenitis or full-blown Sjogren's syndrome. This is present in up to 93% of patients with PBC and can be quite troublesome. Patients may not report symptoms of dry eyes or dry mouth unless directly asked. Dry eyes can usually be managed with artificial teardrops. It is important that patients with complaints of dry mouth regularly see a periodontist to ensure that they do not develop gingival disease. If drinking water and chewing sugarless gum are inadequate, use of pilocarpine and other standard therapies for Sjogren's syndrome has been reported with good success. All affected individuals should be advised to swallow pills with plenty of water while standing up. Finally, women should be asked directly if they have problems with vaginal dryness because they rarely report this spontaneously. Management with lubricants is usually adequate.

In addition to the specific symptoms of PBC, it is also worthwhile to consider associated diseases. Rheumatoid arthritis; Raynaud's phenomenon with or without scleroderma; calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia (CREST syndrome); thyroiditis; and celiac disease are all associated with PBC and should be investigated as necessary.

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