Paget's Disease of the Bone (Osteitis Deformans)

What is Paget’s disease of the bone (osteitis deformans)

Paget’s disease of the bone is a rare chronic bone disorder in which there's excessive and disordered bone turnover with both excessive resorption and formation. This is the process by your body breaks down old bone and creates new bone. A person with Paget’s disease of the bone is more likely to experience bone fractures (breaks). When the disease is around a joint, it can cause arthritis.

Which bones are affected by Paget’s disease of the bone (osteitis deformans)?

Any of your bones can be affected by Paget’s disease. However, your pelvis, skull, spine and leg bones (femur and tibia) are most commonly affected by Paget’s disease. Paget’s disease can affect one bone (monoostotic) or many bones (polyostotic).

Who does Paget’s disease of the bone (osteitis deformans) affect?

Anyone can develop Paget’s disease of the bone, but it’s more common in people who are over the age of 50. People who are from northern Europe or people who are from countries settled by European immigrants are more likely to develop Paget’s disease.

How common is Paget’s disease of the bone (osteitis deformans)?

The number of cases of Paget’s disease of the bone varies from country to country Paget’s disease of the bone is more common in some European countries. Approximately 1% of the population in the United States has Paget’s disease of the bone.

Is Paget’s disease of the bone (osteitis deformans) cancer?

Paget’s disease of the bone isn’t a form of cancer. However, in very rare cases, Paget’s disease of the bone can cause bone cancer (osteosarcoma).

Other conditions have the name of Paget’s that aren’t bone diseases, including:

• Paget’s disease of the breast (also known as Paget’s disease of the nipple): This is a rare form of breast cancer in which cancer cells form in or around your nipple.
• Paget’s disease of the vulva: This is a rare form of skin cancer that forms on the vulva.

These Paget’s diseases are distinct from Paget’s disease of the bone, and they aren’t related.

What causes Paget’s disease of the bone (osteitis deformans)?

Researchers and healthcare providers don’t know the exact cause of Paget’s disease of the bone. They believe it’s caused by a combination of genetic and environmental factors.

What are the symptoms and signs of Paget’s disease of the bone (osteitis deformans)?

Many people who have Paget’s disease of the bone don’t realize that they have it. They may experience no symptoms, or the symptoms may be mild. When a person has symptoms, they're similar to symptoms of other bone conditions, like arthritis.

Symptoms of Paget’s disease of the bone depend on which bones are affected, and Paget’s disease usually gets worse slowly over time if it isn’t treated. Symptoms and signs of Paget’s disease of the bone can include:

• Bone or joint pain: This could be caused by Paget’s disease or by arthritis, which is a complication of Page’s disease of the bone. When the disease is adjacent (next to) to a joint, osteoarthritis can develop.
• Larger head size, bowing of the arm or leg or curvature of the spine: Bone deformations like these can happen in advanced cases of Paget’s disease of the bone.
• Bone fractures: Paget’s disease can weaken your bones, which makes it more likely that you will experience bone fractures (breaks).
• Headaches and hearing loss: These symptoms can happen if Paget’s disease is affecting your skull.

How is Paget’s disease of the bone (osteitis deformans) diagnosed?

Because Paget’s disease of the bone often has no symptoms or very mild symptoms, it’s often diagnosed by chance (incidentally) after healthcare providers perform an X-ray or blood test to for another reason. Providers often use X-rays to make the final diagnosis of Paget’s disease of the bone.

What tests will be done to diagnose Paget’s disease of the bone (osteitis deformans)?

The following tests help to diagnose Paget’s disease of the bone:

• Alkaline phosphatase blood test: A high level of alkaline phosphatase in your blood could be a sign that you have Paget’s disease of the bone. Sometimes this blood test is what first alerts healthcare providers that someone could have Paget’s disease.
• X-rays: With X-ray images, healthcare providers can see if you have bones affected by Paget’s disease. The affected bones look different in the images than unaffected bones.
• Bone scan: Providers sometimes use bone scans to see which bone(s) are affected by Paget’s disease and how mild or severe it is. During a bone scan, a safe amount of radioactive liquid is injected into your vein. The liquid then flows through your bloodstream and into your bones and makes areas of the bone affected by Paget’s disease appear “bright” on the images your bones.

What treatment is used for Paget’s disease of the bone (osteitis deformans)?

Treatments for Paget’s disease of the bone can include:

• Medication: Many medications can treat Paget’s disease of the bone. The most common type is bisphosphonates. The most effective bisphosphonate is zoledronate (Reclast®). Often, one dose of this drug can result in long-lasting remission of the disease but it can’t correct bone deformities.
• Surgery:You might need surgery to fix or improve complications of Paget’s disease of the bone. Some surgeries include knee or hip replacements, surgery to realign deformed bone and surgery to enable bone fractures to heal in a better position.

Is there a cure for Paget’s disease of the bone (osteitis deformans)?

There's currently no cure for Paget’s disease of the bone, but it’s treatable. The sooner Paget’s disease can be diagnosed and treated, the less likely that you’ll experience complications from the disease.

How long will I have Paget’s disease of the bone (osteitis deformans)?

Paget’s disease of the bone is a chronic (lifelong) condition but it’s treatable. It often gets worse slowly over time if it isn’t treated.

What are the risk factors for developing Paget’s disease of the bone (osteitis deformans)?

Unfortunately, there’s nothing you can do to prevent Paget’s disease of the bone. There are some things that are considered risk factors for developing Paget’s disease of the bone, including:

• Your age: People over the age of 50 are more likely to develop Paget’s disease of the bone.
• Your national origin: Paget’s disease of the bone is more common in European populations, including those living in England, Italy and Spain. It’s rare among Scandinavians and non-European immigrants living in Europe.
• Your sex: People assigned male at birth are slightly more likely to develop Paget’s disease of the bone than people assigned female at birth.
• Your family history: Paget’s disease of the bone can sometimes run in families. If you have a family member who has the disease, you may be more likely to develop it.

What is the prognosis (outlook) for Paget’s disease of the bone (osteitis deformans)?

The prognosis (outlook) for Paget’s disease of the bone is excellent if it's diagnosed and treated early in the course of the disease before complications such as arthritis, fractures and hearing loss have occurred.

How do I take care of myself if I have Paget’s disease of the bone?

If you have Paget’s disease of the bone and take medication, it’s important to follow your healthcare provider’s instructions for taking your medicine.

While medication and surgery are the only methods that can treat Paget’s disease of the bone, there are things you can do to help keep your skeletal system healthy in general, including:

• Maintain a healthy weight: Having obesity can cause extra pressure on your joints. Try to maintain a healthy weight to keep your joints and bones healthy.
• Exercise: Physical activity can help keep your skeletal system healthy and can help maintain your joint mobility. Be sure to talk with your healthcare provider before you begin an exercise routine. Certain exercises could put too much pressure on your bones that are affected by Paget’s disease.
• Get enough calcium and vitamin D: Calcium and vitamin D help keep bones strong and healthy. Be sure you're consuming enough calcium and vitamin D in your diet and/or through supplements.

When should I see my healthcare provider?

If you have an elevated alkaline phosphatase blood level, you need an evaluation from a specialist to determine if it’s coming from your bones. If you have an X-ray that’s suggestive of Paget’s disease, you should have an evaluation.

A note from Cleveland Clinic
Paget’s disease of the bone can be difficult to identify because it often develops without any symptoms. Without signs that something is wrong, your bones can slowly become damaged without you knowing. Because of this, it’s important to know the risk factors for Paget’s disease of the bone, especially if you have had a bone break or are experiencing symptoms of arthritis. Paget’s disease of the bone could be the cause. If you experience any symptoms of Paget’s disease of the bone, reach out to your healthcare provider.