Paget’s Disease

General Illness Information

Common Name: Paget’s disease

Medical Term: Deforming osteitis, deforming osteoporosis, deforming osteodystrophy

Description:

Paget’s disease occurs when the mechanism of restoring the bones of the body is damaged.

Bone is a living tissue that is constantly destroyed and restored, this process is called “bone remodeling.” In the bones affected by Paget’s disease, the process of remodeling is broken, which leads to a disruption in the structure of the bones, the bone is not restored properly, forming “weak” bones that are deformed, overstretched, bent and prone to fractures.

Paget’s disease can occur in any bone of the body. In one third of the patient, only one bone is affected, but the average number of damaged bones is about three. The bones often involved in the process are the pelvis, spine, skull and long tubular bones of the hands and feet.

Up to 3% of Europeans over 40 are affected by Paget’s disease, and the prevalence of the disease increases with age. The disease is twice as common in men than in women.

Causes:

The causes of Paget’s disease are still unknown.

The most common opinion is about the viral nature of the disease (slow viral infection). A sick person can be a carrier of a virus without any signs of the disease for years, after which, as a result of the influence of provoking factors, symptoms appear. One of the most significant viral infections is measles.

Also,hereditary predisposition does matter. Some experts believe that Paget’s disease is to some extent a family one. Therefore, it is recommended that brothers, sisters and children with Paget’s disease take a biochemical blood test every two to three years to control the level of alkaline phosphatase in the blood, and if necessary make a radiography of the bones.

These reasons are not mutually exclusive.

Signs & Symptoms:

Symptoms of Paget’s disease depend on the damaged bones and severity of the disease.

Symptoms of Paget’s disease include pain around the damaged bone and osteoarthritis if the disease occurs near the joint of the bone. Pain associated with a damaged bone in Paget’s disease is usually described as continuous, dull, aching and, in contrast to osteoarthritis, often worse at rest and after rest. Stiffness, limitation of mobility in the affected joints also increase after rest.

Bones in different parts of the body become heavily thickened and brittle, which leads to the destruction of bones supporting the weight of the body. Fractures can occur even with minor injuries, the back becomes stooped and deformed.

Incorrect growth of the bones of the skull, resulting in a characteristic appearance of the head, and the spinal column can put pressure on the surrounding nerves, the damage of which can lead to serious consequences, such as constant loss of hearing or vision, headaches or nerve damage to the spinal cord, causing tingling and numbness in the hands and feet, the feeling of “creeping crawl” on the body. In very rare cases, a severe form of Paget’s disease can cause heart failure or malignancy of the process with the development of a bone tumor.

Risk factors:

Factors that increase the risk of Paget’s disease:

• Age. People over 40 are at high risk;
• Sex. Men are more predisposed to the disease than women;
• Nationality. Paget’s disease is common in England, Scotland, central Europe and Greece, as well as in countries inhabited by immigrants from Europe. The disease is rare in Scandinavia and Asia;
• Family history. If you have a close relative with this disease, the probability of developing a disease is higher.

Diagnosis & Treatment:

Diagnosis

Radiography and blood test for serum alkaline phosphatase, which is formed as a result of the process of bone remodeling, are used to diagnose the disease. The level of serum alkaline phosphatase is often much higher than normal. Regular blood tests are necessary to monitor any changes. Scintigraphy of the bones of the skeleton is important in establishing the exact place of bone damage, it provides visualization of the entire skeleton.

Treatment

If you do not have symptoms, you may not need treatment. If the disease is in an active stage, your doctor may recommend a treatment to prevent complications, even if you have no symptoms.

Medications

Medications for osteoporosis – bisphosphonates – are the most common treatment for Paget’s disease. Some bisphosphonates are taken orally, others are injected. Oral forms are usually well tolerated, but they can irritate the gastrointestinal tract. Options include:

• Aldendronate;
• Ibandonate;
• Pamidronate;
• Risedronate;
• Zolbidronic acid.

Rarely, treatment with bisphosphonates is associated with severe muscle, joint or bone pain, which may not pass after the drugs are cancelled. Bisphosphonates can also increase the risk of a rare disease in which necrosis of the jaw part occurs against the background of a dental disease or after some teeth have been removed.

If you can not take bisphosphonates, the doctor can prescribe calcitonin, a hormone involved in the regulation of calcium metabolism. Calcitonin is given by injection or as a nasal spray. Side effects include nausea, redness of the face and irritation of the area of administration.

Surgery

In rare cases, surgical treatment may be required to:

1. help cure fractures;
2. perform joint prosthetics in severe arthritis;
3. rebuild deformed bones;
4. reduce pressure on nerve endings.

Often Paget’s disease leads to the formation of a large number of vessels in the area of damaged bones, which increases the risk of serious blood loss during surgery. If you are planning surgery, the doctor may prescribe additional medications to reduce the activity of the disease and the amount of blood loss.

Possible complications

In most cases, the disease progresses slowly. Paget’s disease can be effectively treated in almost all people. Possible complications include:

• Fractures and deformations. Affected bones break more easily. Additional vessels, formed during formation of bone tissue, bleed profusely during operations. The bones of the legs may flex, which can lead to impaired walking ability.
• Osteoarthritis. Deformed bones lead to an increase in the load on the joints, which can lead to osteoarthritis.
• Heart failure. The extensive development of Paget’s disease can increase the work of the heart for adequate blood supply to the affected areas. In people with previous heart disease, increased stress can lead to the development of heart failure.
• Bone cancer. Bone cancer develops less than 1% of people with Paget’s disease.

Prevention

To reduce the risk of complications, try the following steps:

• Avoid falls. Wear low-heeled shoes with a non-slip sole, remove cords from household appliances, do not allow slippery surfaces that can lead to falls. Maintain bright lighting in the rooms, install handrails outside and inside the shower, make sure you can easily get out of your bed.
• Eat well. Make sure that your diet contains enough calcium and vitamin D. This is especially important if you take bisphosphonates. Reconsider your diet, ask your doctor if you need vitamins and supplements.
• Exercise regularly. Regular exercises are necessary to maintain normal mobility of joints and muscle strength. Talk to your doctor before starting the exercise program to determine the correct type, duration and intensity of the exercise. Some activities may be excessive for the affected bones.

Posted by RxMed