If you would like to know more about paget's disease symptoms and diagnosis, and about paget's disease treatment, read the following article for more information.
Paget's disease affects the structure of parts of one or more bones. The most common symptom is pain. Affected bones are weaker than normal and may become deformed. However, in most cases it causes no symptoms and does not need treatment. Treatment with a biphosphonate medicine is used in some cases to ease pain and/or to control the progression of the disease.
Understanding normal bone
Bone is a living tissue. There are two main types of cells within the hard bone material. One type called osteoblasts makes and lays down bone material. Another type called osteoclasts resorbs ('dissolves') particles of bone. These cells are active throughout life. They work in a balanced way to make and mould bone, repair damage, and keep the bone structure correctly 'woven'. There is a slow but constant turnover of bone.
What is Paget's disease?
Paget's disease of bone was first described by Sir James Paget in 1877. (It is a different condition to Paget's disease of the breast. This page is only about Paget's disease of bone.)
In Paget's disease the bone making process (the bone turnover) gets faster and out of control. Affected areas of bone form new bone material in an abnormal way. The newly formed bone is thicker than normal and the bone may become wider. However, the bone is not made properly and it is weak. This can cause deformity of the affected bone, particularly in weight bearing bones such as the leg bones which may bend. Affected bone is also more likely to fracture (break).
What causes Paget's disease of bone?
The underlying cause is a problem with the osteoclasts (one of the bone cells). In affected areas of bone there are abnormal osteoclasts which are bigger than normal. It is thought that these are wrongly 'programmed' in the way they mould bone. More bone than normal is resorbed ('dissolved') by the abnormal osteoclasts. As a response to this the osteoblasts increase in activity to make new bone material. But this increase in bone turnover leads to badly structured areas of bone that is wrongly 'woven'.
How the osteoclasts in a local area of bone become out of control is not known. One theory is that they may become infected with a virus which alters them in some way. There also seems to be some genetic factor. Up to 4 in 10 people with Paget's disease have a close relative (parent, mother, brother, sister) who also develops the disease. One theory is that your genetic make-up makes you prone to this disease, but it may need to be triggered by a certain virus.
Who gets Paget's disease of bone?
It mainly affects people aged over 50. Men and women are equally affected. One study found that up to 8 in 100 people in the UK over the age of 55 had some X-ray appearance of Paget's disease on one or more of their bones. It becomes more common with increasing age. Nearly 1 in 10 people in their 80s are thought to have some degree of Paget's disease. However, most people with Paget's disease have no symptoms and will not be aware they have the condition.
Which bones are affected?
One or more bones may be affected. The bones most commonly affected are the pelvis, the femur (upper leg), the vertebrae of the spine, and the skull. Other bones are less commonly affected.
What are the symptoms of Paget's disease of bone?
The abnormality of bone formation starts in one point on a bone. It then gradually spreads along the bone at up to 12 mm per year. This may lead to one or more of the following.
In many cases there are no symptoms
More than 9 in 10 people with Paget's disease have no symptoms. The abnormal structure of bone is localised and may never cause any problems. Sometimes it is found by chance when an X-ray is taken for another reason.
Pain
This is the most common symptom if symptoms occur. The pain is typically a deep seated ache of the bone that is usually not affected by rest or exercise. It is commonly worse at night. It may be mild but can become severe. Shooting pains from the affected area may also occur.
Deformity
A deformity may develop, depending on the site of the disease and the size of the affected part of the bone. Bowing of the upper leg is the most common when the femur (thigh bone) is affected. Another example is an odd shape to the head that may develop if the skull is affected.
Fractures
Affected bones are more liable to fracture (break). A fracture after a minor fall or injury may be the first indication that Paget's disease has developed.
Nerve compression
Abnormal overgrowth of a bone may press on nearby nerves. This can cause a variety of symptoms. For example, neuralgic pain (a different type of pain to the more common 'bone pain' of Paget's disease). Another quite common symptom if the skull is affected is deafness of one ear. This is caused by pressure on the ear nerve that goes through an affected skull bone.
Other possible problems include weakness of muscles supplied by a compressed nerve, or numbness of an area of skin supplied by a compressed nerve.
Arthritis (joint inflammation)
This may develop if an affected section of bone is next to a joint.
Other
The high bone turnover may cause high blood levels of various chemicals. Rarely, this leads to problems such as gout, or to a high blood calcium level which can cause various symptoms. Rarely, a bone cancer develops in an affected bone.
How is Paget's disease diagnosed?
- X-ray. A bone with Paget's disease can usually be seen quite easily on an X-ray. The X-ray may be done if the disease is suspected by your doctor (if you have pain, etc). More often, it is seen by chance when an X-ray is done for another reason.
- Bone scan. This may be done if an area of Paget's disease is found in one bone. This test involves an injection of a radioisotope ('radioactive chemical'). This is taken up into bone but much more readily by areas of Paget's disease with a high bone turnover. A scan then detects the level of radioactivity coming from each bone. High readings come from affected bones. Therefore this test can detect which bones are affected throughout the body. (See separate page called 'Bone Scan' for more details of this test.)
- Blood tests. A chemical in the blood called alkaline phosphatase can be measured in a blood sample. This chemical rises with increasing bone turnover. The level of this gives some idea of the activity of the disease and how extensive it is throughout the body. (This blood test is also useful to see how well treatment is working. The level falls when treatment is working and bone turnover falls.)
What is the treatment for Paget's disease?
No treatment is needed in many cases. In most cases the disease is found by chance, does not cause symptoms, and often does not need treatment. Treatment may be advised if symptoms such as pain develops. Also, treatment may be advised if you do not have symptoms, but where the site of the disease may cause problems as the disease progresses. For example, if you have an area of Paget's disease at the base of the skull. Progression of the disease here, if left untreated, may result in compression of the ear nerve and cause deafness.
Your specialist will advise on whether treatment is necessary.
Bisphosphonate medicines
If treatment is advised, a bisphosphonate medicine is the usual treatment given. There are several types which include: etidronate, pamidronate, tiludronate, risedronate, clodronate, alendronate, and ibandronate. Others are under investigation for use in Paget’s disease.
Bisphosphonates work by reducing the abnormal bone turnover. This results in any new bone formation to be more normal in structure. They have an effect on existing disease to reduce pain although this may take several months of treatment. Treatment is good at preventing further progression of the disease.
A course of treatment usually lasts several weeks or months. This often has a knock-on effect for several months or years. Most of the bisphosphonates are taken as tablets, but pamidronate is given by injection.
If you take bisphosphonate tablets, it is vital that you take them exactly as instructed. Read the page that comes with the tablets for details on how to take the tablets. For example, one instruction will be not to take the tablets with food or drink containing calcium. This is because the absorption of bisphosphonates from the gut is affected by calcium.
Like all medicines, bisphosphonate medicines may cause side-effects but generally they are well tolerated and work well. Tell your doctor if you have side-effects when taking a bisphosphonate as an alternative one may be better.
Other treatments
Various other treatments may be advised, depending on your circumstances. For example:
- Painkillers will help if the disease is causing pain. The type of painkillers used may be different for bone pain and neuralgic pain - two different types of pain which may occur.
- Calcitonin is a medicine that also reduces bone turnover. It was used before biphosphonates were developed. It is less effective than these newer medicines and is not used so much now.
- Surgery may be needed if the disease has caused deformity or damaged a joint. For example, joint replacement surgery may be an option if a bone near to a joint is affected and has caused bad arthritis.
Further help and information
National Association for the Relief of Paget's Disease
323 Manchester Road, Walkden, Worsley, Manchester, M28 3HH
Tel: 0161 799 4646 Web: www.paget.org.uk
Tel: 0161 799 4646 Web: www.paget.org.uk
©EMIS and PIP 2005 Updated: May 2005 CHIQ Accredited
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