Overview
Paget’s disease of the bone is the most common type of Paget’s disease. Named after Sir James Paget in 1877, the disease affects approximately one million Americans and twice as many men as women, according to the National Institute of Health. The disease is characterized by abnormal bone remodeling resulting in deformed bones and increased risk of fractures, arthritis and other complications.
What is Paget’s Disease?
Paget’s disease is a chronic skeletal disease affecting the process of bone turnover by osteoblasts and osteoclasts. Osteoblasts are responsible for building new bone while osteoclasts break down bone, a normal and ongoing process in the body. In the early stages of the disease, more bone is absorbed than is laid down, which results in the body compensating by building new bone at a faster than normal rate. This results in large and dense bones that are weak and brittle. Bones most often affected by the disease include the skull, spine and bones of the arms and legs.
Symptoms and Complications
The majority of individuals with Paget’s disease do not exhibit any symptoms. When symptoms are present, the most common is pain in the affected bone region. Other conditions that arise from abnormal bone remodeling include damage to joint cartilage, nerve compression from enlarged bones and increased fracture and bone deformity risk. Cartilage damage can lead to osteoarthritis and nerve compression can cause serious pain, depending on the nerve affected. In individuals with heart problems, heart failure is a concern because the heart must work harder to deliver blood to the affected areas.
Causes and Risk Factors
The cause of Paget’s disease is unknown, but several risk factors exist that increase the chance of developing the disease. Individuals over the age of 40 are at an increased risk, with more men than women diagnosed with the disease. Paget’s disease tends to run in families, suggesting a genetically inheritable component to the disease. As of 2010, researchers are looking into possible gene mutations as well as the possibility of a viral infection as the cause of Paget’s disease.
Diagnosis
Paget’s disease is diagnosed by a physician using imaging techniques and blood tests. X-rays and bone scans provide visual images of the bones to assess the bones involved, extent of damage and other complications such as fractures or deformities. Blood tests analyze the serum for elevated levels of an enzyme called alkaline phosphatase, which is normally produced by the body as a result of the bone formation process.
Treatment
Paget’s disease generally has a good outlook with treatments focused on managing bone remodeling activity and complications as well as relieving any associated pain. Two classes of medications are often prescribed as treatment for Paget’s: bisphosphonates and calcitonin. These medications work to decrease the turnover of new bone. This increases the strength and structural integrity of bones as well as decreases the pain associated with the disease. Anti-inflammatory and pain-relieving medications are most often used to treat the arthritic complications associated with the disease.
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