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Paget’s Disease of Bone (PDB)

Definition:

Paget’s Disease of Bone (PDB) is a disorder of the bone remodeling process, in which the body absorbs old bone and forms abnormal new bone. [3]

Overview

Generally, Paget’s disease of bone (PDB) is a common condition characterized by focal areas of increased and disorganised bone remodelling.

Furthermore, It mostly affects the axial skeleton, and bones that are commonly involve include the pelvis, femur, tibia, lumbar spine, skull and scapula.

It is seldom diagnose before the age of 40, but gradually increases in incidence.

The disease is common in Caucasians from north­west and southern Europe but is rare in Scandinavians, Asians, Chinese and Japanese.

These ethnic differences persist after migration, supporting the importance of genetic factors in the aetiology, but the incidence of PDB has fall in some countries over the past 25 years, suggesting that environmental triggers also play a role. [1]

The body of a person with Paget’s disease might generate new bone in incorrect locations or remove old bone from its intended areas.

This process can lead to weakness in the bones, bone pain, arthritis, deformities, also fractures.

Many people with Paget’s disease do not realize that they have it, as symptoms are often either mild or undetectable.

If a person with Paget’s disease fractures a bone, it may take a long time to heal because of faults in the bone renewal process. [3]

Pathophysiology

Pathophysiology of Paget’s Disease of Bone (PDB)

In general, The primary abnormality is increase osteoclastic bone resorption, accompany by marrow fibrosis, increased vascularity of bone also increased osteoblast activity.

Moreover, Bone in PDB is architecturally abnormal and has reduced mechanical strength.

Osteoclasts in PDB are increase in number, are unusually large also contain characteristic nuclear inclusion bodies.

In detail, Genetic factors are important and mutations in the SQSTM1 gene are a common cause of classical PDB.

The presence of nuclear inclusion bodies in osteoclasts has fuelled speculation that PDB might cause by a slow virus infection with measles or distemper but the evidence is conflicting.

Besides this, Biomechanical factors may help determine the pattern of involvement, since PDB often starts at sites of muscle insertions into bone and, in some cases, localises to bones or limbs that have subjected to repetitive trauma or overuse.

Lastly, Involvement of subchondral bone can compromise the joint and predispose to OA (‘Pagetic arthropathy’). [1]

Sign & Symptoms

Many people are not aware that they have Paget’s disease because they do not experience symptoms.

They might also mistake any symptoms for other bone disorders, such as arthritis.

The most common symptoms that occur relate to;

  • Either Bone or joint pain,
  • Swelling of joints,
  • Tenderness,
  • Redness of the skin that covers the areas affected by Paget’s disease.

Some people only become aware of their presentation of Paget’s disease after experiencing a fracture in a weakened bone.

Paget’s disease most commonly occurs in the following bones:

  • Pelvis
  • Spine
  • Skull
  • Femur / Thigh bone
  • Tibia / Shin bone

Many major nerves in the body run through or alongside the bones, so abnormal bone growth might cause a bone to compress, pinch, or damage a nerve, triggering pain.

Complications:

While the outlook for people with Paget’s disease is generally good, it might lead to other health problems, including i.e.:

  • Arthritis
  • Hearing loss
  • Heart disease
  • Nervous system issues
  • Paget’s sarcoma, a type of cancer that occurs in 1 percent of people with Paget’s disease
  • Loose teeth
  • Vision problems [3]

Causes

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 i.e.:
  • 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 i.e.:
  • Paget’s disease occurs only rarely in people under 40 years of age.
  • It is more common as people age.
Ancestry i.e.:
  • 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 i.e.:
  • Some studies suggest that certain environmental exposures may play a role in the development of Paget’s disease.
  • 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. [4]

Diagnosis

  • The characteristic features are an elevated serum ALP and bone expansion on X­ ray, with alternating areas of radiolucency also osteosclerosis.
  • ALP is normal in about 5% of cases, usually because of monostotic involvement.
  • Radionuclide bone scanning is useful to define the presence and extent of disease.
  • If the bone scan is positive, X ­rays should be taken of an affect bone to confirm the diagnosis.
  • Bone biopsy is not usually require but may help to exclude osteosclerotic metastases in cases of diagnostic uncertainty. [1]

Treatment

There is no cure for Paget’s disease and no way to reverse its effects on bone.

Treatment focuses on relieving symptoms and preventing future complications.

Medications:

If you do not have pain or other symptoms, no treatment is required.

Your doctor may recommend simply monitoring your condition with regular office visits and periodic x-rays to watch for changes in the affected bone and to ensure that complications do not develop.

Nonsteroidal anti-inflammatory drugs (NSAIDs) i.e.:
  • Medications such as ibuprofen, naproxen and aspirin can help relieve mild bone pain that arises from Paget’s disease or from arthritis that may associated with the disease.
Assistive devices i.e.:
  • If your pelvis or leg is affect by the disease, using a cane can help relieve pain by decreasing the forces going through the bone.
  • Using a cane can also help prevent falls, so there is less risk of fracture in the bone a common complication of Paget’s disease.
  • Wearing a brace can help relieve pain by preventing malalignment of the affected bones.
Bisphosphonate medications i.e.:
  • When bone pain is more significant, medications call bisphosphonates are the treatment of choice.
  • These drugs block osteoclasts and can very effective in treating Paget’s disease.
  • There are several types of bisphosphonates.
  • Some are give by mouth (orally) and others are give as an injection (intravenously).
  • During treatment with bisphosphonates, your doctor will perform blood tests periodically to check your alkaline phosphatase level.
  • A falling alkaline phosphatase level and improvement in bone pain are indications that treatment is working.

Surgery:

In some cases, surgery may needed to treat the complications of Paget’s disease, including:

  • Bone fractures
  • Malalignment or deformity of bone
  • Severe arthritis

The surgical procedures used to treat fractures, malalignment, or arthritis in patients with Paget’s disease are similar to those used to treat similar conditions in people with normal bone.

These procedures may include:

Internal fixation i.e.:
  • This procedure can used to treat fractures in bone affected by the disease.
  • In internal fixation, bone fragments are first repositioned into their normal alignment, then held in place with screws, wires, pins, or metal plates attached to the outside of the bone.
Osteotomy i.e.:
  • An osteotomy can help relieve pain and restore alignment to weight-bearing joints that are affect by Paget’s disease, especially the knee and hip.
  • During the procedure, your doctor will remove a wedge of bone near the damaged joint in order to shift weight onto a healthier part of the joint.
Total joint replacement i.e.:
  • In this procedure, parts of an arthritic or damaged joint are remove and replace with a metal, plastic or ceramic device called a prosthesis.
  • The prosthesis is design to replicate the movement of a normal, healthy joint.
  • Surgery may also needed if an enlarged bone begins to compress nerves, especially in the spine or skull.
  • In the rare case of Paget’s sarcoma, surgery is almost always use to try to remove the tumor entirely.
  • Chemotherapy and radiation therapy may also be used.
  • Because Paget’s disease increases the blood supply to bones, your doctor may recommend taking bisphosphonates for a period of time before surgery to help reduce potential blood loss.
  • Bones affected by Paget’s disease may take longer to heal than normal bones.
  • A longer period of rehabilitation may also be necessary. [4]

Homeopathic Treatment

Homeopathic treatment for Paget’s disease is  natural, safe and without any side-effects, and one can enjoy immense relief from these frustrating symptoms.

It is a patient-oriented science and medicines are prescribed on the characteristics of the individual rather than just the symptoms of the disease.

Symphytum Officinale: 

  • Symphytum is one of the effective remedies especially for bone cancer.
  • There is pricking pain also soreness of periosteum.
  • The bones are broken.
  • Symphytum is effective for the reunion of bones.

Calcarea Phosphoricum:

  • Calcarea phos is also considered to be effective for bone cancer with soft, thin and brittle bones.
  • Weak bones that break easily.
  •  It also helps for the reunion of bones.

Ruta Graveolens:

  • Ruta graveolens is prescribed where the bone is brittle , sore and aching.
  • There is much restlessness.
  • Feeling of intense pain.
  •  The bones can be in wrist, ankle, back, upper limb or lower limb or any other bone when soreness is marked.
  • Burning sensation can also accompany the Bone Pains.
  • Touch worsens the pain.

Mezereum:

  • Mezereum is also the best remedy for leg pain arising in tibia bone.
  • It is mainly used to treat Bone Pains, especially the long bones, that get worse at night.
  • The bones may be swollen or inflamed.
  • The slightest touch may also worsen the Bone Pains.
  • The person requiring Mezereum may also show an increased sensitivity towards cold air. [5]

FAQs

Frequently Asked Questions

What is Paget’s Disease of Bone?

Paget’s Disease of Bone (in other words; PDB) is a disorder of the bone remodeling process, in which the body absorbs old bone and forms abnormal new bone.

Homeopathic Medicines used by Homeopathic Doctors in treatment of Paget’s Disease of Bone?

  • Symphytum Officinale
  • Calcarea Phosphoricum
  • Ruta Graveolens
  • Mezereum

What causes Paget’s Disease of Bone?

  • Genetics
  • more common as people age
  • Ancestry
  • Environmental factors

What are the symptoms of Paget’s Disease of Bone?

  • Bone or joint pain
  • Swelling of joints
  • Tenderness
  • Redness of the skin

References:

  1. Davidson’s Principles and Practice of Medicine (22nd edition) Ch. 25
  2. https://www.mayoclinic.org/diseases-conditions/pagets-disease-of-bone/diagnosis-treatment/drc-20350816
  3. https://www.medicalnewstoday.com/articles/177668.php
  4. https://orthoinfo.aaos.org/en/diseases–conditions/pagets-disease-of-bone
  5. https://www.linkedin.com/pulse/homoeopathy-bone-cancer-dr-ks-gopi