Osteoporosis in Primary Biliary Cirrhosis: Pathogenesis and Treatment

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Osteoporosis, characterized by loss of bone strength leading to fragility fractures, is a common event in patients who have primary biliary cirrhosis. Although its pathogenesis is not well known, it results mainly from low bone formation. There is no specific treatment, but bisphosphonates, especially alendronate, effectively increases bone mass and prevents bone loss. Despite these favorable effects on bone mass, no clear effects on decreasing the fracture rate are demonstrated, probably because of the low number of patients included in the trials. The potential usefulness of new agents requires further evaluation.

Section snippets

Prevalence of osteoporosis and fractures

The prevalence of bone disease in PBC has changed since the first description because of two main factors: the criteria used for diagnosing osteoporosis and osteomalacia and because patients now are diagnosed in the early stages of the disease and mostly are asymptomatic. In earlier studies, the incidence of bone disease was assessed by radiographs of the spine or by bone biopsy and histomorphometry [2], [3], [4], [5], [6], [7], [8], [9], [10], [11]. In the past 2 decades, however, bone mass

Pathogenesis

The mechanisms resulting in osteoporosis in patients who have PBC have not been elucidated completely because some studies indicate increased bone resorption whereas others suggest decreased bone formation [29]. Some histomorphometric reports have revealed increased bone resorption and turnover even in the absence of osteoporosis as an early feature of bone disease in PBC [7]. Reduced trabecular wall thickness and increased bone turnover is proportional to the severity of hepatic dysfunction

Diagnosis

Bone densitometry is the precise procedure for establishing the diagnosis of osteoporosis and should be performed in all patients who have PBC [56]. Additionally, risk factors for low bone mass and fractures should be evaluated, including alcohol abuse, smoking, body mass index lower than 19 kg/m2, early menopause (women under 45 years of age), secondary amenorrhea of more than 6 months, family history of bone fragility fractures and treatment with glucocorticoids (more than 5 mg of prednisone

General measures

A diagram for prevention and treatment of osteoporosis in PBC is shown in Fig. 2. The factors contributing to bone loss should, as far as possible, be reduced to a minimum by stopping alcohol intake and smoking. As much physical activity as possible and exercises aimed at improving the mechanics of spine are advisable.

Whenever possible, a balanced diet should be prescribed because patients who have advanced liver disease frequently have little appetite and are malnourished. Supplements of

Summary

Osteoporosis is a complication frequently observed in patients who have PBC. The pathogenesis of osteoporosis is characterized mainly by low bone formation, although increased bone resorption also is described, especially in cholestatic women who have advanced liver disease. Osteoporosis is associated with severity of liver disease, older age, and the duration of cholestasis. Patients who have osteoporosis and those who have a lumbar T score below −1.5 have a high risk for fractures,

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