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What Is Paget's Disease Of Bone? What Causes Paget's Disease?

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Paget's disease of bone, often just called Paget's disease or osteitis deformans, is a condition in which the normal cycle of bone growth is disrupted. The condition affects bone metabolism that allows for old bone to be recycled into new bone throughout life. This can cause bones to become weakened and deformed.
In Paget's disease of bone, the rate at which old bone is broken down and new bone is formed is altered. Over time, it may result in bones becoming fragile. Common symptoms of Paget's disease include bone pain and deformity.
The disease is named after Sir James Paget, the British surgeon who first described it in 1877.
According to Medilexicon's medical dictionary:
    Paget¬ s disease of bone is "a generalized skeletal disease, frequently familial, of older people in which bone resorption and formation are both increased, leading to thickening and softening of bones (the skull), and bending of weight-bearing bones."
Paget's disease of bone is more common with age. After osteoporosis (brittle bones), Paget's disease is the second most common type of bone disease. Many older people with discomfort in their bones and joints assume the discomfort is a natural part of aging. Often, they do not seek treatment. However, it is important to get treatment as soon as possible after symptoms appear in order to prevent the most serious complications of Paget's disease of bone.
The causes of Paget's disease are unknown, but it has been suggested that it could be triggered by a combination of environmental and genetic factors.
In most cases of Paget's disease, the disruption to bone growth that is associated with the condition is so mild that it causes no obvious symptoms. This is known as asymptomatic Paget's disease. In the rest of the cases, medication can help to control the symptoms and relieve pain. There is no cure for Paget's disease.
In the most serious cases of Paget's disease, surgery may be required.

What are the signs and symptoms of Paget's disease?

A symptom is something the patient feels and reports, while a sign is something other people, such as the doctor detect. For example, pain may be a symptom while a rash may be a sign.
Paget's disease of bone affects each person differently. Most people with Paget's disease have no symptoms.
Paget's disease usually affects the bones in the pelvis. Other areas that sometimes become infected include the spine, thigh bones, and skull, shin and arm bones.
Bone symptoms
Bone pain is the most common symptom of Paget's disease. The pain may be constant, aching and deep, and may be most severe at night.
Affected bones may be more vulnerable to damage and be easily fractured or broken.
As Paget's disease progresses, there may be deformities in the affected bones, such as twisted or misshaped limbs, or a curvature of the spine (scoliosis).
Joint symptoms
Abnormal bone growth can cause damage to nearby cartilage. Cartilage is the thick, spongy tissue that cushions the joints. Damage to the cartilage can lead to progressive damage of the joints. This wear and tear often leads to osteoarthritis in the affected joints, a condition that may cause pain, swelling and stiffness. Symptoms of osteoarthritis include:
  • pain
  • stiffness (which is worse when wake up in the morning, but usually improves within about 30 minutes of movement)
  • difficulty moving the affected joints
Nerve symptoms
Abnormal bone growth can result in a bone compressing, 'pinching' or damaging a nerve. Enlarged bones can compress the spinal cord or the nerves exiting the brain and spinal cord. Pain resulting from nerve compression is more severe than the bone pain associated with Paget's.
This can lead to a wide range of symptoms that will depend on where the affected nerve is. Possible neurological symptoms include:
  • blurred vision
  • headaches
  • hearing loss
  • drowsiness due to vascular steal syndrome of the skull
  • numbness, weakness, tingling in the affected limbs
  • pain that radiates from the base of the spine down into the legs (sciatica), or alternatively pain that travels from the neck into the arms and chest (cervical radiculopathy)
Other signs and symptoms of Paget's disease may include:
  • bone deformities, such as bowlegs and enlarged head size
  • fractures
  • neurological problems, such as hearing loss, headache and rarely, vision loss
  • warmth in the skin over the affected area
Seek medical advice if you have:
  • Symptoms of Paget's disease of bone, such as pain in the bones and joints
  • Symptoms of pinched nerves, such as pain, tingling and weakness
  • Bone deformities
People who have Paget's disease of bone, should contact their doctor if they experience:
  • Loss of bladder or bowel control accompanied by weakness in the legs, which can indicate severe spinal damage
  • Signs and symptoms of the disease affecting the skull (hearing or vision loss, double vision, facial weakness or numbness)
  • Sudden or severe pain or a change in the pain that has been experiencing

What are the causes of Paget¬ s disease of bone?

Bone remodeling
Bones do not stop growing, even in adults. Bone is a living tissue and is in a continual process of renewal. Bone cells undergo a continuous cycle of regeneration. During this constant process called remodeling, old bone is removed and replaced by new bone. Paget's disease of bone disrupts this process. Over time, the disruption in the bone remodeling process can lead to the bones to become bigger and weaker than usual.
The exact cause of Paget's disease of bone has not been identified. But scientists have discovered several genes that appear to be linked to the disorder. It is likely that the condition develops as result of a combination of genetic and environmental factors.
Some scientists believe Paget's is related to a viral infection in the bone cells that may be present for many years before problems appear.
Hereditary factors seem to influence whether a person is susceptible to the disease.
Genetic factors
There is indication that people who inherit certain mutated genes from their parents (the RANKL mutated gene and the SQSTM1 mutated gene) have an increased risk of developing Paget's disease. In addition, there is evidence that Paget's disease can run in families.
If you have a first degree relative with Paget's disease, such as a parent, brother or sister, you are more likely to develop the condition.
Environmental factors
Environmental factors may play a role in Paget's disease. This is based on the fact that the number of people who are affected by the condition has fallen sharply over the last fifty years. If Paget's disease was only caused by genetic mutations, there would not be fluctuations in the number of new cases recorded by the authorities each year.
One theory suggests the measles virus is an environmental factor. The mutated genes, RANKL and SQSTM1, cause the measles virus to stay in the body after the initial measles infection has passed. The virus may lay dormant for many years. It can be're-activated' and attack the cells, causing them to malfunction. Therefore, the fall in the number of cases of Paget's disease could be because of a corresponding fall in the number of measles infections resulting from the measles vaccination programs.
Further research is required in order to understand how environmental factors are linked to Paget's disease.

What are the risk factors of Paget¬ s disease?

A risk factor is something which increases the likelihood of developing a condition or disease. For example, obesity significantly raises the risk of developing diabetes type 2. Therefore, obesity is a risk factor for diabetes type 2. The risk factors of Paget¬ s disease include:
Age and heredity are the only known risk factors for Paget's disease of bone. In some cases, the disease can run in families. People that are older than 40, are more likely to develop Paget's disease.
Men are more commonly affected than are women.

What are the complications of Paget¬ s disease?

In most cases, the condition progresses slowly. It can be managed effectively in nearly all people and is rarely fatal. Possible complications include:
  • Osteoarthritis. A degenerative joint disease which is a common long-term complication of Paget's disease.
  • Heart failure. In rare cases, extensive Paget's disease may force the heart to pump blood to the affected areas of the body. This increased workload can lead to heart failure in people with pre-existing heart disease.
  • Sarcoma. A rare complication is a bone cancer known as sarcoma. Most cases of sarcoma that are associated with Paget's disease occur in people who are over 70.
  • Head. If the disease affects bones in the head, the patient may experience hearing loss, loss of teeth and, rarely, loss of vision.

How is Paget's disease diagnosed?

Detecting Paget's disease of bone early may help prevent serious complications. Paget's disease can often be diagnosed in its early stages.
Serum alkaline phosphatise (SAP)
Paget¬ s disease can be diagnosed through a blood test. This occurs because the abnormal pattern of bone remodeling caused by Paget's disease leads to a rise in levels of a chemical called serum alkaline phosphatise (SAP) in the blood.
People with a sibling or parent with Paget's disease should request an alkaline phosphatase blood test every two to three years after age 40.
In many cases, Paget's disease is first diagnosed when a person is having a blood test for another, unrelated condition.
X-ray images of the bones can show areas of bone re-absorption, enlargement of the bone and deformities that are characteristic of Paget's disease. Diagnosis of Paget's disease can be based on the bone X-ray findings.
Bone scan
In some cases, a bone scan may be recommended. Bone scans can detect Paget's disease before it can be seen on an X-ray. Bone scans are also used to determine which bones are affected. In a bone scan, a small amount of a harmless radioactive substance is injected into the patient's blood. The radioactive substance will travel to the areas of bone where there is most cellular activity, such as where there is abnormal bone remodeling. A special camera, known as a gamma camera, is then used to see where the radiation has travelled to.

What are the treatment options for Paget¬ s disease?

Patients who do not have symptoms may not need treatment. However, if the disease has been diagnosed and is active, it could affect high-risk sites in the body (skull or spine). The progress of the condition will be monitored using regular blood tests.
Treatment may be recommended to prevent complications. It is generally recommended when:
  • Patient experiences bone pain or neurological signs or symptoms related to Paget's disease.
  • Patient plans to have surgery to repair damage related to Paget's disease. In this case, medication will be prescribed to minimize blood loss during the operation.
  • Patient is at risk of serious, long-term complications due to the aggressiveness of the disease and the location of the affected bones. When the spine, skull, bones near major joints, and the long bones of the arms and legs are involved, it is more likely to result in complications.
  • Patient¬ s heart is overworked because of widespread Paget's disease.
There are two kinds of medications to treat Paget's disease of bone:
  • Bisphosphonates. These medications are commonly used to treat osteoporosis and increase bone density. They also reduce the activity of Paget's disease. Treatment with these agents helps restore more-normal-appearing bone and may produce long-term remission of Paget's disease. Bisphosphonates are currently the treatment of choice for Paget's disease. They should be avoided for patients who have serious kidney disease.
    Bisphosphonates can be given as oral medications or through a vein (intravenously). Oral bisphosphonates are generally well tolerated, but may irritate the gastrointestinal tract. Intravenous administration offers a more rapid response than oral medications do. It also provides an alternative for people who do not tolerate oral bisphosphonates. Common side effects of bisphosphonates include diarrhoea, and nausea.
    Bisphosphonates are usually prescribed for two to six months, depending on the drug used. There is a potential risk that bisphosphonates can cause birth defects, so women should avoid becoming pregnant while taking the medication.
  • Zoledronic acid. It is a newer type of bisphosphonate given by injection. Zoledronic acid is a long-acting medication and is given by one injection a year.
    Flu-like symptoms, such as chills, fever and joint pain, are common after receiving the first injection, although these side effects should pass within three to four days.
    Zoledronic acid should not be used during pregnancy.
  • Calcitonin. People who cannot tolerate bisphosphonates may be prescribed calcitonin (Miacalcin). It is a naturally occurring hormone involved in calcium regulation and bone metabolism. It is administered by injection.
    Side effects may include nausea, facial flushing and irritation at the injection site. Stopping treatment with calcitonin usually results in a rapid reactivation of the disease and recurrence of symptoms.
    Regular blood tests measure the alkaline phosphatase level to monitor the response to these medications. If therapy is effective, the alkaline phosphatase level will decrease and may return to normal.
Arthritis treatment
Paget's disease may also require treatment to reduce pain or treat the inflammation associated with arthritis. Treatment options include:
  • Nonsteroidal anti-inflammatory drugs (NSAIDs) . NSAIDs alleviate pain and reduce the inflammation that is often the source of pain in arthritis related to Paget's disease. Long-term use of NSAIDs or use of more than one NSAID can cause side effects, such as ulcers, heartburn, nausea and stomach bleeding. Large doses of NSAIDs can lead to kidney problems.
  • Acetaminophen. Acetaminophen (paracetamol, Tylenol) may provide some relief of pain. But it does not improve inflammation. If taken at the maximum dose for extended periods and especially when combined with regular alcohol intake, acetaminophen may damage the liver.
Surgery In rare cases, surgery may be required to help fractures heal, to replace joints damaged by severe arthritis or to realign deformed bones. If Paget's disease affects the spine or the skull, surgery may be needed to reduce pressure on nerves and prevent serious complications.
Paget's disease often causes the body to produce an excessive number of blood vessels to the affected bones. This change increases the risk of serious blood loss during an operation. Medications may be prescribed to reduce the activity of the disease to reduce blood loss during surgery.
Dietary supplements
Regular vitamin D and calcium supplements may be prescribed, because both of these substances can strengthen bones.
Change in lifestyle
The following steps may reduce the risk of complications from Paget's disease of bone:
  • Eating well. Diet should include adequate levels of calcium and of vitamin D, which facilitates the absorption of calcium. This is especially important during treatment with bisphosphonates. The general recommendations are of at least 1,000 milligrams (mg) a day of calcium up to age 50, and 1,200 mg a day after age 50. Recommendations are of 400 international units (IU), or 10 micrograms (mcg), a day of vitamin D up to age 70, and then the intake is increased to 600 IU (15 mcg) daily. Review diet with your doctor.
  • Exercising regularly. Exercising on a regular basis is essential for maintaining joint mobility and bone strength. Focus on weight-bearing exercise, such as walking, jogging or dancing. These activities maximize the benefit to the bones. Include strength training in the exercise program because strong muscles help support damaged joints. Seek medical advice before beginning an exercise program to determine adequate type, duration and intensity of exercise. Some activities may stress affected bones.
  • Healthy weight. Excess body weight may cause additional damage to the joints. This may worsen pain in the joints. In case of surgery, excess weight also increases the risk of complications.
  • Preventing falls. Paget's disease increases risk of bone fractures. The use a cane or a walker may be recommended. Make adjustments at home, such as removing slippery floor coverings, using nonskid mats in the bathtub or shower, tucking away cords, and installing handrails on stairways and grab bars in the bathroom.

Coping with the condition

Even if symptoms are mild, living with a chronic condition such as Paget's disease of bone can be stressful. It is essential to focus on reducing the risk of complications by exercising regularly and eating a healthy diet. Be aware of any changes in the symptoms. Effective treatments are available. Joining a support group for people with Paget's disease may be helpful.
Written by Stephanie Brunner (B.A.)
View drug information on Miacalcin.
Copyright: Medical News Today
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