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Paget's disease

Introduction

Paget’s disease of the bone

Paget’s disease is named after James Paget, an English surgeon, who first described the condition’s symptoms in 1877. A number of other conditions were also named after James Paget, including:

To avoid confusion, healthcare professionals often use the term "Paget’s disease of the bone" to distinguish this condition from the ones above. However, for ease of reading, the rest of this topic will use the term "Paget’s disease" to refer to "Paget’s disease of bone".

Paget’s disease is a condition where the normal cycle of bone renewal and repair is disrupted. In some cases, this can cause the affected bone (or bones) to become weakened and deformed.

Bone pain is the most common symptom of Paget’s disease. It most commonly affects the pelvis or spine and it is usually worse when lying down.

However, in many cases Paget’s disease does not cause any noticeable symptoms, and it is only diagnosed during tests for an unrelated medical condition or when an affected bone is fractured.

See the encylopaedia pages on symptoms of Paget’s disease for more information.

There is no cure for the condition but the symptoms can be controlled by painkillers and a range of medications that help regulate bone growth.

Following treatment for Paget’s disease many people will have long periods of remission (where symptoms disappear) provided that the disease is picked up at an early stage. If the disease has progressed to an advanced stage by the time it is discovered, treatment is much less effective.

Complications of Paget’s disease are uncommon, but they can be potentially serious.

They include:

How common is Paget's disease?

After osteoporosis (brittle bones), Paget’s disease is the second most common type of bone disease.

Paget’s disease occurs among people of white British descent, possibly due to genetic factors. It is very rare among other ethnic groups, such as Asians and Africans.

Paget’s disease is most widespread in Britain and it is also relatively widespread in countries where there have been high levels of migration from Britain, such as Australia, New Zealand and South Africa, and to a lesser extent the USA.

There is also a marked variation in the number of cases of Paget’s disease in different regions of Britain. For example, the condition is less common in the south of the country, where an estimated 1 in 100 people over the age of 55 is affected, and it is more widespread in the north, particularly in the county of Lancashire where 1 in 50 people of this age may be affected. 

Who is affected by Paget’s disease?

Paget’s disease is an age-related condition. It is estimated that 1-2% of white adults aged over 55 have the condition. This figure increases to about 7% for white people who are over 80 years old.

Paget’s disease is thought to be caused by a combination of genetic and environmental factors. See the encycopaedia pages on causes of Paget’s disease for more information.

 

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Symptoms

Paget’s disease usually affects the bones of the pelvis or spine. Other areas of the body that can also be affected include the skull, the shoulders and the long bones in the arms and legs – particularly the thigh bone.

Bone pain

Bone pain is the most common symptom of Paget’s disease. The pain has been described as a dull, constant, "boring" sensation deep within the affected part of the body. The pain is usually worse at night when you are lying down.

As Paget’s disease progresses, you may experience deformities in the affected bones, such as twisted or misshaped limbs or scoliosis (curvature of the spine). Affected bones can sometimes fracture (break), particularly the longer bones in your arms and legs.

Symptoms of broken bones include:

  • pain and swelling in the affected area
  • bruising or discoloured skin around the bone or joint
  • the limb or body part being bent at an unusual angle (angulation)

Other symptoms

Paget’s disease can cause a range of other symptoms depending on which bones are affected by the condition. These are described below.

The skull

If Paget’s disease develops inside the bones of your skull it can cause the following symptoms:

  • hearing impairment – which can be total or partial
  • vertigo – feeling very dizzy or the sensation that you are moving when standing still
  • headaches
  • tinnitus – a constant buzzing or ringing noise in your ears

The joints

Abnormal bone growth can cause damage to nearby cartilage. Cartilage is the thick, spongy tissue that cushions your joints. Cartilage damage can lead to progressive joint damage, which is known as osteoarthritis.

Symptoms of osteoarthritis include:

  • pain
  • stiffness – which is worse when you wake up in the morning, but usually improves within about 30 minutes of starting to move
  • difficulty moving your affected joints

The nerves

Many of the major nerves in your body run through, or alongside, your bones. Abnormal bone growth can result in a bone compressing (squeezing), "pinching" or damaging a nerve.

Depending on where the affected nerve is and its main purpose, this can lead to a wide range of symptoms. Possible symptoms affecting the nerves (neurological symptoms) include:

  • pain that travels from the base of your spine down into your legs (sciatica)
  • pain that travels from your neck into your arms and chest (cervical radiculopathy)
  • numbness or tingling in the affected limbs
  • partial loss of movement in your limbs
  • loss of balance
  • bowel incontinence or urinary incontinence (the loss of bowel or bladder control)

When to seek medical advice

You should always visit your GP if you:

  • have persistent bone pain
  • notice deformities in any of your bones
  • experience any neurological symptoms, such as numbness or tingling
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Causes

Bone remodelling

To get a better understanding of how Paget’s disease affects your bones, it is useful to understand how they are kept healthy throughout life.

It is often assumed that once a person’s bones reach adult size, they do not change, but this is not the case.

In a similar way to skin, bone cells undergo a continuous cycle of regeneration, where old bone is removed and is replaced by new bone. This cycle is known as bone remodelling.

Bone is made up of a protein called collagen and a mineral called hydroxyapatite. Two specialised cells are responsible for bone renewal and repair. These are known as:

  • osteoclasts – cells that absorb old bones
  • osteoblasts – cells that lay down new bone

In Paget’s disease, something goes wrong with the osteoclast cells and they begin to absorb collagen at a much faster rate than normal. The osteoblasts attempt to compensate for this by producing new bone, but because they have to work at a faster speed than normal, the bone that they produce becomes weak and unstable.

Over time, this disruption to the bone remodelling process can lead to bones becoming bigger and weaker than normal.

Possible causes of Paget’s disease

The exact cause of Paget’s disease is unknown, but it is likely that the condition develops as result of a combination of genetic and environmental factors.

These are described below.

Genetic factors

People who inherit certain mutated genes from their parents could go on to develop Paget's disease. The most important of these is a gene called SQSTM1. People with a mutation in this gene have a greatly increased risk of developing Paget’s disease in later life. Seven other genes have now been identified which also increase the risk of Paget’s disease substantially.

In about 15% of cases, Paget’s disease runs in families. If you have a close relative with Paget’s disease, such as a parent, brother or sister, you are seven to eight times more likely to develop the condition.

Environmental factors

The evidence that environmental factors may play a role in Paget’s disease is based on the fact that the number of people who are affected by the condition has fallen sharply over the last 50 years in Britain and New Zealand. In contrast, the number of people affected in some other countries, such as Italy, has remained static.

The severity of symptoms also seems to be decreasing, as up to 60 years ago it was not uncommon for people to experience very severe symptoms in multiple bones. However, this is no longer the case.

The reasons for these differences are unclear.

In Britain, a possible reason for the decrease in the number of people who are affected by Paget’s disease might be the significant changes that have been seen in the ethnic mix of the population over the past 50 years. An influx of people from Asia and Africa, where Paget’s disease is rare, could account in part for the decreasing frequency of the disease.

Another theory is that Paget’s disease might be caused by a slow virus infection.

Viruses that have been suggested as possible triggers for Paget’s disease include:

  • measles virus – a common childhood infection
  • canine distemper virus – a virus that is responsible for distemper which is a type of viral infection that affects animals, mainly dogs
  • respiratory syncitial virus – which causes respiratory infections during childhood

Due to vaccines, both measles and distemper are now uncommon infections. This could account for the decrease in cases of Paget's disease.

Another theory is that people with rural lifestyles, who regularly come into contact with farmyard animals, and who in the past had high levels of physical activity, may be at increased risk of developing Paget’s disease. As the number of people living this type of lifestyle is much less common than it was, it could offer an alternative explanation for the fall in cases of Paget’s disease.

Improvements in nutrition and a reduction in the occurrence of rickets (a bone disorder caused by a vitamin deficiency) over the past 50 to 100 years have also been suggested as possibly accounting for the decreasing severity of Paget’s disease.

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Diagnosis

It is sometimes possible to diagnose Paget’s disease in its early stages, leading to early treatment. However, it is not yet known whether this is effective in preventing the most serious complications, such as hearing loss and bone deformity.

Serum alkaline phosphatase (SAP)

In many cases, Paget’s disease is first diagnosed during a blood test for another, unrelated condition.

This occurs because the abnormal pattern of bone remodelling caused by Paget’s disease leads to a rise in levels of a chemical called serum alkaline phosphatise (SAP) in your blood. The high levels of SAP can be detected with a blood test.

X-rays and bone scans

Paget’s disease can often be confirmed by taking X-rays of the affected bones. This can help to determine whether the bones have become enlarged due to Paget’s disease.

As well as having an X-ray, a bone scan may also be recommended. This involves having a small amount of a harmless radioactive substance injected into your blood. This substance collects in the areas of bone where there is the most activity, such as where there is abnormal bone remodelling.

A special camera, known as a gamma camera, is used to highlight where the radiation has collected. A bone scan is a useful way of pinpointing exactly where abnormal bone remodelling is taking place.

Further testing

Further testing is usually only required if:

  • You develop abnormalities that affect one or more joints.
  • You are experiencing neurological symptoms (symptoms that affect your nerves, such as pain, numbness or tingling).
  • A diagnosis of bone cancer is suspected.

In these circumstances, tests that can be used are:

  • A bone biopsy – where a sample of bone is taken under local or general anaesthetic.
  • computer tomography (CT) scan – where a series of X-rays of the affected bone are taken to show a detailed three-dimensional image.
  • magnetic resonance imaging (MRI) scan – another type of scan that uses a strong magnetic field and radio waves to build up an image of the inside of the affected bone.
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Treatment

If a blood test reveals that you have Paget’s disease but you are not experiencing any symptoms, a policy of "watchful waiting" is likely to be recommended. This means that you will not receive treatment immediately, but your progress will be carefully monitored using regular blood tests.

Treatment is usually recommended if you have symptoms that are associated with Paget’s disease, such as bone pain or neurological symptoms (those that affect the nerves, causing symptoms such as pain, numbness and tingling).

The short-term aim of treatment for Paget’s disease is to relieve symptoms. The long-term aim is to prevent the condition from getting worse and to reduce the risk of complications developing.

Treatment options for Paget’s disease include:

  • medication to help regulate bone remodelling
  • medication to relieve pain
  • surgery

Regulating bone remodelling

There are a number of different medications that can be used to help regulate bone growth in cases of Paget’s disease. These are outlined below.

Bisphosphonates

Bisphosphonates are the first choice medications that are used to regulate bone growth. These medicines work by suppressing (controlling) the actions of the osteoclasts cells, which means that the bone remodelling process should return to normal.

A type of bisphosphonate called risedronate is usually recommended because it has proved to be effective in treating Paget’s disease. Risedronate is available in tablet form and most people are advised to take one tablet a day over the course of a two-month period.

It is usually recommended that risedronate is taken first thing in the morning, 30 minutes before eating or drinking anything (other than plain water).

Take your risedronate tablet while you are standing or sitting up and avoid lying down for at least 30 minutes after taking it. This will help prevent heartburn (burning chest pain and discomfort).

Side effects of risedronate include:

If you are unable to take risedronate because you are unable to stay upright for 30 minutes, there are alternative bisphosphonates, such as pamidronate, that can be used. Pamidronate is given by injection, usually once a week over the course of six weeks.

The most common side effects of pamidronate include:

  • mild, flu-like symptoms, such as high temperature
  • joint pain
  • headaches
  • chills

However, these side effects usually pass within 48 hours of receiving an injection.

Zoledronic acid

Zoledronic acid is a new type of bisphosphonate that is increasingly being used as an alternative to risedronate or pamidronate.

Zoledronic acid is a long-acting medication so you will only need to be given one injection a year.

Flu-like symptoms, such as chills, fever and joint pain are common after receiving your first injection, although these side effects should pass within three to four days.

Calcitonin

If you have low levels of calcium in your blood (hypocalcemia), you will probably be unable to use bisphosphonates safely. This is because bisphosphonates can lower your calcium levels further which could be potentially dangerous.

In such circumstances, an alternative medication called calcitonin may be recommended. Calcitonin is a synthetic (man-made) version of a hormone that is known to prevent bone loss. It is given by injection once a day. The recommended course will depend on how well you respond to treatment.

If you require a relatively long course of treatment, training can be given so that you can administer the injections yourself.

Common side effects of calcitonin include:

  • headaches
  • changes in how things taste
  • nausea
  • abdominal pain
  • vomiting 
  • diarrhoea
  • fatigue
  • dizziness

If you experience feelings of dizziness when taking calcitonin, avoid driving or operating machinery.

Painkillers

In most cases, painkillers that are available over the counter, such as paracetamol or ibuprofen, should be effective in relieving the symptoms of bone pain. If your symptoms continue, your GP can prescribe more powerful painkillers.

Physiotherapy

Some people with Paget’s disease find that they benefit from having physiotherapy, particularly those who have had fractures or other types of damage in the bones of their legs or spine.

A physiotherapist will be able to advise you about the use of equipment that is designed to reduce the weight placed on affected bones. This should help to reduce pain and make everyday physical activities easier. Examples include:

  • walking devices – such as a cane or walking frame
  • orthoses – insoles that are made out of plastic and fit inside your shoe to hep support your feet
  • spine braces – designed to support the spine in the correct position

Some physiotherapists also provide energy-based therapy which uses different types of energy, such as electric currents or impulses to stimulate the nervous system. The electric impulses are thought to be effective in easing pain and promoting healing in some people.

The physiotherapist will also be able to teach you a number of exercises that can:

  • improve your muscle strength
  • maintain the range of movement and flexibility in your joints
  • increase your physical stamina

See the encyclopaedia topic about physiotherapy for more information and advice.

Surgery

If you develop a fracture in a bone, surgery may be required to realign the bones so that the affected bone is able to heal properly. See the encyclopaedia pages about treating broken bones for more information.

If you experience severe osteoarthritis (joint inflammation and damage), surgery may be required to repair or replace a damaged joint. Surgical options for osteoarthritis include:

  • an arthroplasty – where the damaged joint is removed and replaced with an artificial joint; the most commonly performed arthoplasties are hip replacements and knee replacements
  • arthrodesis – where the joint is fused into a in a permanent position
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Complications

Hypercalcaemia

The abnormally rapid process of bone remodelling that is associated with Paget’s disease can sometimes lead to a build-up of calcium in the blood. The medical term for this is hypercalcaemia.

Hypercalcaemia only occurs in people with Paget’s disease who have been confined to bed after having an operation or following a fracture. However, even in these circumstances, hypercalcaemia is quite a rare complication.

Symptoms of hypercalcaemia include:

It is also relatively common for people with hypercalcaemia to develop kidney stones.

Hypercalcaemia can be treated using a combination of medication to lower blood calcium levels and bisphosphonates to slow down bone remodelling.

Heart failure

In severe cases of Paget’s disease, the blood vessels inside the bones can become damaged. This means that the heart has to work harder to pump blood around the body. In rare cases, the heart may no longer be able to pump enough blood around the body to meet demand. This is known as heart failure and it is a very rare complication of Paget’s disease.

Symptoms of heart failure include:

  • breathlessness (dyspnoea) – when you are more active than normal or sometimes when you are resting
  • extreme tiredness and weakness
  • oedema – swelling in the legs, ankles and feet

Heart failure can be treated using a combination of medications designed to reduce strain on the heart while also helping it to work more efficiently. In some cases, surgery may be recommended to repair or replace a damaged heart valve.

See the encyclopaedia topic about Heart failure for more information.

Sarcoma

Sarcoma is a type of cancer that starts in the bone cells. Very rarely it can occur as a complication of Paget’s disease. It is estimated that less than 1 in 1,000 people with Paget’s disease will go on to develop sarcoma later in life, usually many years after Paget’s disease was first diagnosed.

Symptoms of sarcoma include:

  • bone pain which is very similar to the pain that is associated with Paget’s disease itself; it is a dull ache that is usually worse at night
  • swelling around the affected bone
  • a noticeable lump in the affected bone

Sarcoma is usually a very aggressive type of cancer and it is often necessary to remove the affected bone to prevent the cancer spreading to other parts of the body.

See the encyclopaedia topics about sarcoma and amputation for more information.

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