Paget’s Disease of Bone

Diagnosis

During the physical exam your doctor will examine areas of your body that are causing pain. To confirm a diagnosis of Paget’s disease of bone, imaging and laboratory tests are commonly used. Clinical assessment focuses on affected bone sites, pain characteristics, deformity, and any neurologic symptoms that might suggest spinal or skull involvement.

Imaging tests

X-rays are often the first indication of Paget’s disease of bone and may be found incidentally when imaging is done for other reasons. Typical X-ray findings include areas of bone breakdown, enlargement of affected bones, and deformities such as bowing of long bones.

A bone scan involves injection of a small amount of radioactive tracer that accumulates in the most active areas of disease; these spots appear “hot” on scan images and help map the extent of involvement.

Lab tests

People with Paget’s disease of bone commonly have elevated alkaline phosphatase (ALP) levels on blood testing. ALP is a useful marker of disease activity and can guide decisions about treatment and monitoring.

More Information

• Alkaline phosphatase (ALP) blood test

• Bone scan

• X-ray

Treatment

If you are asymptomatic, immediate treatment may not be necessary. Treatment is generally recommended when the disease is active (for example, when ALP is elevated) or when affected sites are at higher risk for complications — particularly the skull, spine, pelvis, or weight-bearing long bones. Treating active disease at high-risk sites can reduce pain and prevent complications such as fracture, deformity, and neurologic compression.

Medications

Osteoporosis drugs from the bisphosphonate class are the most commonly used medications for Paget’s disease of bone and work by decreasing excessive bone turnover. These drugs are available both as intravenous infusions and as oral tablets. When taken orally, bisphosphonates are usually well tolerated but can cause gastric irritation in some patients.

Intravenous bisphosphonates commonly used:

• Zoledronic acid (Zometa, Reclast)

• Pamidronate (Aredia)

• Ibandronate (Boniva)

Oral bisphosphonates commonly used:

• Alendronate (Fosamax, Binosto)

• Risedronate (Actonel, Atelvia)

Potential adverse effects and cautions:

• Rare reports of severe musculoskeletal pain that may not resolve after stopping therapy

• Small risk of osteonecrosis of the jaw, most often associated with active dental disease or oral surgery — dental evaluation and care are recommended before high-dose or IV therapy when possible

If bisphosphonates are not tolerated or contraindicated, calcitonin (Miacalcin) is an alternative. Calcitonin can be self-administered by injection or as a nasal spray; side effects include nausea, flushing, and local irritation at injection sites.

Surgery

Surgery is infrequently required, but may be necessary to:

• Help fractures heal

• Replace joints severely damaged by arthritis

• Realign deformed bones to improve function or reduce pain

• Reduce pressure on nerves caused by deformity or overgrowth

Because Paget’s disease can cause increased blood vessel formation in affected bones, there is a heightened risk of significant blood loss during operations involving those bones. To reduce this risk, doctors may prescribe medications to quiet disease activity before surgery.