What is the definition of Paget Disease of the Bone?

Paget disease is a disorder that involves abnormal bone destruction and regrowth. This results in deformity of the affected bones.

What are the alternative names for Paget Disease of the Bone?

Osteitis deformans

What are the causes for Paget Disease of the Bone?

The cause of Paget disease is unknown. It may be due to genetic factors, but also could be due to a viral infection early in life.

The disease occurs worldwide, but is more common in Europe, Australia, and New Zealand. The disease has become much less common over the last 50 years.

In people with Paget disease, there is an abnormal breakdown of bone tissue in specific areas. This is followed by abnormal bone formation. The new area of bone is larger, but weaker. The new bone is also filled with new blood vessels.

The affected bone may only be in one or two areas of the skeleton, or in many different bones in the body. It more often involves bones of the arms, collarbones, legs, pelvis, spine, and skull.

What are the symptoms for Paget Disease of the Bone?

Most people with the condition have no symptoms. Paget disease is often diagnosed when an x-ray is done for another reason. It may also be discovered when trying to find the cause of high blood calcium levels.

If they do occur, symptoms may include:

  • Bone pain, joint pain or stiffness, and neck pain (the pain may be severe and be present most of the time)
  • Bowing of the legs and other visible deformities
  • Enlarged head and skull deformities
  • Fracture
  • Headache
  • Hearing loss
  • Reduced height
  • Warm skin over the affected bone

What are the current treatments for Paget Disease of the Bone?

Not all people with Paget disease need to be treated. People who may not need treatment include those who:

  • Only have mildly abnormal blood tests
  • Have no symptoms and no evidence of active disease

Paget disease is commonly treated when:

  • Certain bones, such as weight-bearing bones, are involved and the risk of fracture is higher.
  • Bony changes are getting worse quickly (treatment can reduce the risk of fractures).
  • Bony deformities are present.
  • A person has pain or other symptoms.
  • The skull is affected. (This is to prevent hearing loss.)
  • The calcium levels are elevated and causing symptoms.

Drug therapy helps prevent further bone breakdown and formation. Currently, there are several classes of drugs used to treat Paget disease. These include:

  • Bisphosphonates: These drugs are the first treatment, and they help decrease bone remodeling. Medicines are commonly taken by mouth, but can also be given through a vein (intravenously).
  • Calcitonin: This hormone is involved in bone metabolism. It may be given as a nasal spray (Miacalcin), or as an injection under the skin (Calcimar or Mithracin).

Acetaminophen (Tylenol) or nonsteroidal anti-inflammatory drugs (NSAIDs) may also be given for pain. In severe cases, orthopedic surgery may be needed to correct a deformity or fracture.

What are the support groups for Paget Disease of the Bone?

People with this condition may benefit from taking part in support groups for people with similar experiences.

What is the outlook (prognosis) for Paget Disease of the Bone?

Most of the time, the condition can be controlled with medicines. A small number of people may develop a cancer of the bone called osteosarcoma. Some people will need joint replacement surgery.

What are the possible complications for Paget Disease of the Bone?

Complications may include:

  • Bone fractures
  • Deafness
  • Deformities
  • Heart failure
  • Hypercalcemia
  • Paraplegia
  • Spinal stenosis

When should I contact a medical professional for Paget Disease of the Bone?

Call your health care provider if you develop symptoms of Paget disease.

X-ray

REFERENCES

Ralston SH. Paget disease of bone. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 233.

Singer FR. Paget's disease of bone. In: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Adult and Pediatric. 7th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 72.

  • Condition: Osteoporosis, Paget's Disease, and Myeloma and Cancers for the Reduction of Adverse Skeletal-Related Events (SREs)
  • Journal: Bone
  • Treatment Used: Zoledronate
  • Number of Patients: 0
  • Published —
This article discusses the use of zoledronate in the treatment of osteoporosis, Paget's disease, and in myeloma and cancers to reduce adverse skeletal related events (SREs).
  • Condition: Paget's disease of bone
  • Journal: Osteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA
  • Treatment Used: Zoledronate
  • Number of Patients: 0
  • Published —
This article discusses management of Paget's disease of the bone.