what-is-pagets-disease
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What is Paget's disease?

What is Paget's disease?

Last Reviewed : 12/29/2020
What is Paget's disease?

  • Paget’s disease of bone is a chronic disorder that results in enlarged and deformed bones in one or more regions of the skeleton. Excessive bone breakdown and formation can cause the bone to weaken. As a result, bone pain, arthritis, noticeable deformities and fractures can occur.

Prevalence in the population ranges from 1.5 percent to 8 percent depending on the person’s age and area of the world where he or she lives. Men and Women are affected equally. A combination of environmental and genetic factors contribute to the disease. Several genes are linked to the disease.

Symptoms:

Bone pain is the most common symptom, it often localizes to areas adjacent to the joints. Any bone can be affected in Paget’s disease. Paget’s disease occurs most frequently in the spine, skull, pelvis and bones of the lower extremities. Some patients will have only one affected bone, while others may have two, three, or more affected bones.

Headaches and hearing loss may occur when Paget’s disease affects the skull. Pressure on nerves may also occur when the skull or spine is affected.

Deformities of bone such as an increased head size, bowing of a limb, or curvature of the spine may occur in advanced cases. These deformities are due to enlargement and/or weakening of the affected bones.

Bones weakened by Paget’s disease can break more easily than healthy bones. Damage to the cartilage of joints adjacent to the affected bone may lead to arthritis.

Diagnosis:

During the physical exam, doctor will examine areas of the body that are causing pain. Then X-ray and bone scan along with blood tests can be done to confirm the diagnosis. 

  • X-rays. X-ray images of your bones can show areas of bone resorption, enlargement of the bone and deformities that are characteristic of Paget’s disease of bone, such as bowing of your long bones.
  • Bone scan. In a bone scan, radioactive material is injected into your body. This material travels to the spots on your bones most affected by Paget’s disease of bone, so they light up on the scan images.

People who have Paget’s disease of bone usually have elevated levels of alkaline phosphatase in their blood, which can be revealed by a blood test.  Patients who have a very small area of involved bone may have a normal test but a bone scan may be abnormal.

The goal of treatment is to normalize Paget’s disease activity for a prolonged period of time. If you don’t have symptoms, you may not need treatment. However, if the disease is active as indicated by an elevated alkaline phosphatase level and is affecting high-risk sites in your body, such as your skull or spine, you might need treatment to prevent complications, even if you don’t have symptoms.

Bisphosphonates are the most common treatment for Paget’s disease of bone. Some bisphosphonates are taken by mouth, while others are given by injection. Oral bisphosphonates are generally well-tolerated, but may irritate your gastrointestinal tract. Also, an adequate dietary calcium intake (1000-1500 mg daily) and vitamin D intake (400 units) are recommended during bisphosphonate use. 

If Bisphosphonates cannot be tolerated, calcitonin (Miacalcin), a naturally occurring hormone involved in calcium regulation and bone metabolism can be used. Calcitonin can be administer to yourself by injection or nasal spray. Side effects may include nausea, facial flushing and irritation at the injection site.  It is generally less effective than bisphosphonates.

In rare cases, surgery might be required to:

  • Help fractures heal
  • Replace joints damaged by severe arthritis
  • Realign deformed bones
  • Reduce pressure on nerves

The course of Paget’s disease varies greatly and may from completely stable to rapid progression. In general, symptoms progress slowly in affected bones. Treatment can control Paget’s disease and lessen symptoms but is not a cure. If treatment is given before major changes have occurred, outcome is good. If left untreated, Paget’s disease can cause serious complications, depending on which bones are affected.

 

Reference: National Osteoporosis Foundation. 

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