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Primary biliary cholangitis and bone disease. Osteoporosis, characterized by compromised bone strength leading to fragility fractures, is a common event in patients with primary biliary cholangitis (PBC). Osteomalacia, defined by poor bone mineralization is very uncommon. The pathogenesis of osteoporosis is not well clarified, but it mainly results from low bone formation. Few reports have revealed increased bone resorption, particularly in end-stage disease. The prevalence of osteoporosis is about 35% in the most significant studies, and it depends on the diagnostic criteria and severity of liver damage. Osteoporosis is associated with age, postmenopausal status, duration of PBC and advanced histological stage. Bone densitometry is the common method for the diagnosis of osteoporosis and should be performed in all patients with PBC. Lateral X-rays of the dorsal and lumbar spine should also be carried out to disclose vertebral fractures. There is no specific treatment but bisphosphonates, especially alendronate and ibandronate, efficiently increases bone mass and prevents bone loss. Despite these positive effects on bone mass no clear results on decreasing the fracture rate have been demonstrated, probably because the low number of patients included in the trials. The potential value of new agents requires further evaluation.