Paget's disease of bone

What is Paget’s disease of bone?

Paget's disease of bone is a metabolic bone disorder (a problem with the way bone tissue constantly breaks itself down and regrows) that leads to reshaping of an area of bone.

Symptoms

Many people with Paget's disease have no symptoms and only find out they have the disease by chance when having an x-ray. When symptoms occur they may include:

• Bone and joint pain.
• Deformity of a bone such as bowing of the leg, skull enlargement or curvature of the spine.
• Muscle weakness due to bones pressing on nerves.
• Bone fractures resulting from minor injury (bone affected by Paget's disease has a disorganised structure and is mechanically weak).
• Arthritis (when Paget’s affects bone around a joint).
• Deafness, vision disturbance, dizziness and tinnitus when enlargement of skull bones puts pressure on vital nerves.
• A “hot spot” over an area of affected bone due to high blood supply.

Causes and risk factors

The origins of Paget's disease of bone are unknown, although genetics plays a part (more than 40 per cent of those affected have a family history of the condition) and environmental factors are probably also important (some believe that a virus infection may trigger the disease). In the condition the body's normal process of continuously breaking down old bone and replacing it is disrupted. This results in increased and irregular bone formation that causes bones to expand.

The deformed bones become larger and structurally abnormal, making them weak and more likely to fracture. The bones most often affected are those in the central axis of the body and include those of the spine, pelvis, legs, skull and collarbone. In the majority of cases more than one area of bone is affected.

Paget's disease normally affects older people and is rare under the age of 40. It's more common in men and more widespread in Europe, North America and Australia. It's rare in people of African or Asian origin.

The condition occurs more often in the UK than anywhere else in the world, with up to 1 million people affected here.

Treatment snd recovery

Since the disease is of unknown origin it's impossible to prevent and treatment is a matter of managing symptoms when they occur, and preventing complications such as fracture. Painkillers and anti-inflammatory medication can be used to relieve pain.

Drugs to protect bone strength and slow the progress of the disease may be recommended. There is an increased risk of bone tumours forming in bone affected by Paget’s disease, so those with the condition should be regularly monitored.

Advice and support

• National Association for the Relief of Paget's Disease
• Tel: 0161 799 4646
• Email: director@paget.org.uk
• Website: www.paget.org.uk