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Paget Diseases (PDB, osteitis deformans) : Symptoms, Causes, Diagnosis and Treatment

What is Paget Diseases?

Paget's disease of bone is a chronic disease of the skeleton. In healthy bone, a process called remodeling removes old pieces of bone and replaces them with new, fresh bone. Paget?s disease causes this process to shift out of balance, resulting in new bone that is abnormally shaped, weak, and brittle. Paget?s disease most often affects older people, occurring in approximately 2 to 3 percent of the population over the age of 55.

Many patients with Paget?s disease have no symptoms at all and are unaware they have the disease until x-rays are taken for some other reason. When bone pain and other symptoms are present, they can be related to the disease itself or to complications that arise from the disease?such as arthritis, bone deformity, and fractures.

In most cases, treatment for Paget?s disease involves taking medications to help slow or stop the progress of the disease. For patients who have complications, surgery may be needed to realign deformed bones or to help fractures heal.

Paget's disease can affect any bone in the skeleton. It appears most often in the spine, pelvis, long bones of the limbs, and skull. It can be present in just one bone or in several bones. It can affect the entire bone or just part of it.

Bone Remodeling

In normal bone, a process called remodeling takes place every day. Bone is absorbed and then reformed in response to the normal stresses on the skeleton. More specifically:

  • Cells of the bone called "osteoclasts" absorb bone.
  • Cells of the bone called "osteoblasts" make new bone.

In Paget's disease, osteoclasts are more active than osteoblasts. This means that there is more bone absorption than normal. The osteoblasts try to keep up by making new bone, but they overreact and make excess bone that is abnormally large, deformed, and fits together haphazardly.

Normal bone has a tight overlapping structure, like a well-constructed brick wall. Bone afflicted by Paget's disease has an irregular mosaic pattern, as though the bricks were just dumped in place. The end result is bones that are large and dense, but weak and brittle. The bone is prone to fractures, bowing, and deformities.

Paget Diseases is also known as PDB, osteitis deformans. Paget Diseases belongs under the category of Bone disease. monostotic, polyostotic are some common types of Paget Diseases. Generally Male and Female above 60 yrs. are the victim of the Paget Diseases. Seriousness of this disease is Medium.

Symptoms of Paget Diseases are :

  • Compression on neighboring nerves from enlarged bones
  • Deformity of bone
  • Fractures due to brittle bone.
  • Thickening skin on the breast
  • A lump in the breast
  • A flattened nipple
  • bloody nipple discharge
  • Flaky or scaly skin on your nipple
  • hardened skin of the areola
  • hardened skin of the nipple
  • redness of skin
  • itchy skin
  • Itchy skin, also known as pruritus, is an irritating and uncontrollable sensation that makes you want to scratch to relieve the feeling. The possible causes for itchiness include internal illnesses and skin conditions.

  • constipation
  • Gradual loss of sensation
  • People rely on their sense of touch to quickly pull away from a hot object or to feel changes in terrain under their feet. These are referred to as sensations.

    If you can?t feel as well, especially with your hands or feet, it?s known as impaired sensation. If you have impaired sensation, you may not feel anything at all. Or you may feel unusual sensations, such as:

    • numbness
    • tingling
    • weakness
    • pain

    Impaired sensation can lead to injury and balance problems.

    It can be a temporary occurrence that takes place after an injury or a chronic condition that results from diabetes or another illness. Sudden impaired sensation can be a medical emergency.

  • abdominal pain
  • Abdominal pain has many potential causes. The most common causes ? such as gas pains, indigestion or a pulled muscle ? usually aren't serious. Other conditions may require more-urgent medical attention.

    While the location and pattern of abdominal pain can provide important clues, its time course is particularly useful when determining its cause.

    Acute abdominal pain develops, and often resolves, over a few hours to a few days. Chronic abdominal pain may be intermittent, or episodic, meaning it may come and go. This type of pain may be present for weeks to months, or even years. Some conditions cause progressive pain, which steadily gets worse over time.

  • Loss of appetite
  • Fatigue is a constant state of tiredness, even when you?ve gotten your usual amount of sleep. This symptom develops over time and causes a drop in your physical, emotional, and psychological energy levels. You?re also more likely to feel unmotivated to participate in or do activities you normally enjoy.

    Some other signs of fatigue include feeling:

    • physically weaker than usual
    • tired, despite rest
    • as though you have less stamina or endurance than normal
    • mentally tired and moody

    Loss of appetite means you don?t have the same desire to eat as you used to. Signs of decreased appetite include not wanting to eat, unintentional weight loss, and not feeling hungry. The idea of eating food may make you feel nauseous, as if you might vomit after eating. Long-term loss of appetite is also known as anorexia, which can have a medical or psychological cause.

    It may be a warning sign from your body when you feel fatigue and loss of appetite together. Read on to see what conditions may cause these symptoms.

  • Fatigue/weakness
  • Weakness

    Weakness is when strength is decreased and extra effort is needed to move a certain part of the body or the entire body. Weakness is due to loss of muscle strength. Weakness can be a big part of why cancer patients feel fatigue.

    Fatigue

    Fatigue is an extreme feeling of tiredness or lack of energy, often described as being exhausted. Fatigue is something that lasts even when a person seems to be getting enough sleep. It can have many causes, including working too much, having disturbed sleep, stress and worry, not having enough physical activity, and going through an illness and its treatment.

    Causes

    Paget Diseases can be caused due to:

    The cause of Paget?s disease is not known, but doctors have identified a number of risk factors that make someone more likely to develop the disease. These include:

    • Genetics. Paget?s disease tends to run in families. In as many as 25 to 40 percent of cases, another relative will also have the disease.
    • Age. Paget's disease occurs only rarely in people under 40 years of age. It is more common as people age.
    • Ancestry. It is more common in people of Anglo-Saxon descent and those who live in certain geographic areas, such as the United States, England, Australia, New Zealand, and Western Europe. It is not common in Scandinavia, China, Japan, or India.
    • Environmental factors. Some studies suggest that certain environmental exposures may play a role in the development of Paget's disease. This has not been proven definitively, however.

    While there are no known ways to prevent Paget's disease from occurring, eating a healthy diet with sufficient calcium and vitamin D, and getting regular exercise, are important components in maintaining skeletal health and joint mobility.

    The exact cause of Paget?s disease is unknown. Researchers speculate that the cause of the disorder may be multifactorial (e.g. caused by the interaction of certain genetic and environmental factors). In most cases, no specific cause for Paget?s disease can be identified (sporadic).

    Research findings suggest that Paget?s disease may be related to a ?slow virus? infection of bone, a condition that is present for many years before symptoms appear.

    In approximately 15-30 percent of cases there is a family history of the disorder. Researchers have discovered several genes that may predispose individuals to developing Paget?s disease (genetic predisposition). Genes associated with this condition are the sequestosome 1 gene, the TNFRSFIIA gene that codes for the RANK protein, and the VCP gene. The exact role that these genes play in the development of the disorder is unknown. This hereditary factor may be the reason that family members are susceptible to the suspected virus.

    What kind of precaution should be taken in Paget Diseases?

    Paget?s Disease is unavoidable for most people with the disorder, but exercise can help to maintain skeletal health, avoid weight gain to relieve pressure on the joints and bones, and maintain joint mobility.

    People with the disease should talk to their physician before starting any exercise program, as placing extra stress on bones affected by Paget?s disease can lead to injury.

    How it can be spread?

    Does not spread

    Treatment for the Paget Diseases

    Diagnosis:

    Health care professionals make a diagnosis of Paget's disease based on the X-ray appearance. Doctors also may detect Paget's disease with other imaging tests, such as a bone scan, MRI scan, and CT scan to make a diagnosis. Alkaline phosphatase, an enzyme that comes from bone, is frequently elevated in the blood of people with Paget's disease as a result of the abnormal bone turnover of actively remodeling bone. This blood test is also referred to as the serum alkaline phosphatase (SAP) and is used to monitor the results of treatment of Paget's disease.

    The bone scan is particularly helpful in determining the extent of the involvement of Paget's disease as it provides an image of the entire skeleton. Bone that is affected by Paget's disease can easily be identified with bone scanning images. A bone biopsy is generally not necessary to make the diagnosis of Paget's disease. Bone biopsy would be considered if there was a suspicion of cancer or abnormal lesion in the involved bone.

    During the physical exam, your doctor will examine areas of your body that are causing you pain. He or she may also order X-rays and blood tests that can help confirm the diagnosis of Paget's disease of bone.

    Imaging tests

    Bone changes common to Paget's disease of bone can be revealed by:

    • X-rays. The first indication of Paget's disease of bone is often abnormalities found on X-rays done for other reasons. X-ray images of your bones can show areas of bone reabsorption, enlargement of the bone and deformities that are characteristic of Paget's disease of bone, such as bowing of your long bones.
    • Bone scan. In a bone scan, radioactive material is injected into your body. This material travels to the spots on your bones most affected by Paget's disease of bone, so they light up on the scan images.

    Lab tests

    People who have Paget's disease of bone usually have elevated levels of alkaline phosphatase in their blood, which can be revealed by a blood test.

    Treatment:

    If you don't have symptoms, you might not need treatment. However, if the disease is active ? indicated by an elevated alkaline phosphatase level ? and is affecting high-risk sites in your body, such as your skull or spine, your doctor might recommend treatment to prevent complications, even if you don't have symptoms.

    Medications

    Osteoporosis drugs (bisphosphonates) are the most common treatment for Paget's disease of bone. Some bisphosphonates are taken by mouth, while others are given by injection. Oral bisphosphonates are generally well-tolerated, but they may irritate your gastrointestinal tract. Examples include:

    • Alendronate (Fosamax)
    • Ibandronate (Boniva)
    • Pamidronate (Aredia)
    • Risedronate (Actonel)
    • Zoledronic acid (Zometa, Reclast)

    Rarely, bisphosphonate therapy has been linked to severe muscle, joint or bone pain, which might not resolve when the medication is discontinued. Bisphosphonates can also increase the risk of a rare condition in which a section of jawbone dies and deteriorates (osteonecrosis of the jawbone), usually associated with active dental disease or oral surgery.

    If you can't tolerate bisphosphonates, your doctor might prescribe calcitonin (Miacalcin), a naturally occurring hormone involved in calcium regulation and bone metabolism. Calcitonin is a drug that you administer to yourself by injection or nasal spray. Side effects may include nausea, facial flushing and irritation at the injection site.

    Surgery

    In rare cases, surgery might be required to:

    • Help fractures heal
    • Replace joints damaged by severe arthritis
    • Realign deformed bones
    • Reduce pressure on nerves

    Paget's disease of bone often causes the body to produce too many blood vessels in the affected bones, increasing the risk of serious blood loss during an operation.

    If you're scheduled for surgery that involves bones affected by Paget's disease, your doctor might prescribe medications to reduce the activity of the disease, which may help reduce blood loss during surgery.

    Possible complication with Paget Diseases

    In most cases, Paget's disease of bone progresses slowly. The disease can be managed effectively in nearly all people. Possible complications include:

    • Fractures and deformities. Affected bones break more easily. Extra blood vessels in these deformed bones cause them to bleed more during repair surgeries. Leg bones can bow, which can affect your ability to walk.
    • Osteoarthritis. Misshapen bones can increase the amount of stress on nearby joints, which can cause osteoarthritis.
    • Neurologic problems. When Paget's disease of bone occurs in an area where nerves pass through the bone, such as the spine and skull, the overgrowth of bone can compress and damage the nerve, causing pain, weakness or tingling in an arm or leg or hearing loss.
    • Heart failure. Extensive Paget's disease of bone may force your heart to work harder to pump blood to the affected areas of your body. Sometimes, this increased workload can lead to heart failure.
    • Bone cancer. Bone cancer occurs in about 1 percent of people with Paget's disease of bone.

    References:

    1 https://www.mayoclinic.org/diseases-conditions/pagets-disease-of-bone/symptoms-causes/syc-20350811 2 https://orthoinfo.aaos.org/en/diseases--conditions/pagets-disease-of-bone 3 https://www.medicalnewstoday.com/articles/177668#treatment 4 https://www.medicinenet.com/pagets_disease/article.htm#what_are_complications_of_paget#39s_disease 5 https://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Pagets-Disease-of-Bone 6 https://rarediseases.org/rare-diseases/pagets-disease/

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