Metabolic bone disease in primary biliary cholangitis (primary biliary cirrhosis)
- Steven Flamm, MD
Steven Flamm, MD
- Chief, Liver Transplantation Program
- Professor of Medicine
- Feinberg School of Medicine
- Northwestern University
- Raoul Poupon, MD
Raoul Poupon, MD
- Professor of Hepatology and Gastroenterology
- University Pierre et Marie Curie
- UPMC, Sorbonne University, Paris, France
- Section Editors
- Clifford J Rosen, MD
Clifford J Rosen, MD
- Section Editor — Bone Disease
- Professor of Nutrition
- University of Maine
- Professor of Medicine
- Tufts University School of Medicine
- Keith D Lindor, MD
Keith D Lindor, MD
- Section Editor — Alcoholic and Metabolic Liver Disease
- Professor of Medicine, Mayo Clinic College of Medicine
- Dean, College of Health Solutions
- Arizona State University
Hepatic osteodystrophy refers to the structural and metabolic bone changes seen in patients with chronic liver disease and includes both osteoporosis and osteomalacia. Osteoporosis is a skeletal condition characterized by low bone mass, whereas osteomalacia is a disorder of decreased mineralization of newly formed osteoid at sites of bone turnover. Both disorders put patients at risk for fractures. (See "Pathogenesis of osteoporosis" and "Epidemiology and etiology of osteomalacia" and "Clinical manifestations, diagnosis, and treatment of osteomalacia".)
This topic will review metabolic bone disease in patients with primary biliary cholangitis (previously referred to as primary biliary cirrhosis). The discussion that follows is consistent with the 2003 guidelines issued by The American Gastroenterological Association (AGA) regarding osteoporosis in patients with liver disease [1,2].
Other issues related to metabolic bone disease, including metabolic bone disease following liver transplantation and a detailed discussion of treatment approaches for osteoporosis, are discussed elsewhere. (See "Osteoporosis after solid organ or stem cell transplantation" and "Overview of the management of osteoporosis in postmenopausal women" and "Evaluation and treatment of premenopausal osteoporosis" and "Treatment of osteoporosis in men".)
Estimates of the prevalence of osteoporosis among patients with primary biliary cholangitis (PBC) range from 14 to 52 percent . One reason that osteoporosis is common in PBC is that many patients with PBC are postmenopausal women, a group already at increased risk for osteoporosis. In addition to age and postmenopausal status, other factors associated with an increased risk for osteoporosis among patients with PBC include lower weight, shorter height, more advanced fibrosis on liver biopsy, hypogonadism, and increasing severity and duration of PBC [3-7]. The prevalence of osteoporosis appears to be decreasing over time, likely related to improved treatment for PBC. In one study, the prevalence of osteoporosis between 1987 and 1995 was 37 percent, whereas between 2002 and 2007 it was 21 percent, though the difference did not reach statistical significance.
Osteoporosis has been shown to be a risk factor for vertebral and nonvertebral fractures in women with PBC , and the prevalence of fractures among patients with PBC is approximately 10 to 20 percent . In a study of 185 women with PBC, fractures were seen in 21 percent. Risk factors for fractures included osteoporosis, menopause, age, and height. When compared with the general population, the absolute increase in fracture risk is moderately increased. In a population-based study, patients with PBC had a twofold increased risk of any fracture compared with the general population, with an absolute excess fracture rate of 12.5 per 1000 person-years .
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