Introduction to Paget disease of bone

Paget disease of bone is a focal bone disorder characterised by excessive bone resorption by osteoclasts, and a compensatory but disorganised increase in bone formation by osteoblasts. Paget disease can progress and deform bones in one or more parts of the skeleton, causing bone pain, bony deformities, neurological syndromes, osteoarthritis and arthropathies. The cause is unknown. Distinguishing between bone pain due to Paget disease and joint pain due to associated osteoarthritis can be difficult. Paget-related pain typically occurs at rest, particularly at night.

The prevalence of Paget disease increases with age. It has a mean age of onset of around 55 years, and affects approximately 2 to 4% of people over 55 years. It most commonly affects Caucasian people of British origin. The prevalence of Paget disease declined by 60% from 1974 to 1994, and appears to be continuing to decline.

Siblings and children of patients with Paget disease have a slightly higher risk of developing the disease. Consider familial screening with measurement of serum alkaline phosphatase concentration after the age of 40 years.