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Navigating Paget's Disease - Unveiling the Enigma

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Paget's disease, also called osteitis deformans, is a bone disorder that causes abnormal resorption and deposition of the bone.

Medically reviewed by

Dr. Anuj Gupta

Published At September 26, 2023
Reviewed AtFebruary 8, 2024

Introduction

Old bone is gradually reabsorbed during the recycling process, and new bone formation occurs. But in the case of Paget's disease, this normal cycle is disturbed, leading to abnormal resorption and bone formation. This is commonly seen in backbones, skull bones, bones of the legs, and hips. Abnormal bone formation exerts pressure on the surrounding tissues, including nerves and blood vessels. This causes neurological problems and other complications.

What Are the Symptoms Of Paget's Disease?

Patients with Paget's disease do not express any symptoms, and most patients complain of bone pain. In Paget's disease, the new bone formed is fragile and improperly organized, leading to fractures and bony deformities. Paget‘s disease can be limited to a single bone, or it can widely spread over the bones, and the symptoms include:

  • Pain in the pelvic or hip region due to the involvement of hip bones.

  • Oversized skull leading to loss of vision and tooth.

  • Loss of sensation and pain can be seen in the body's extremities due to the compression of nerve roots. The compression is because of abnormal bone deposition.

  • Wear and tear of the joints could lead to osteoarthritis of the joints.

  • The bone deformity can lead to an abnormal curvature of the long bones, especially the spine and leg bones. This causes a bowleg appearance.

Which Bones Are Impacted by Paget's Disease of the Bone?

Paget disease can damage any bone in the body. However, the most common areas of the human body impacted by Paget's disease are the pelvis, head, spine, and leg bones. Paget's disease can damage either one or more bones.

Is Paget's Disease of the Bones Cancerous?

Paget's bone disease is not a kind of cancer. However, malignancies of the bone can extremely rarely result from Paget's disease of the bone.

What Are the Complications of Paget's Disease?

The complications of Paget's disease include:

  • Eyes: If Paget's disease in the head affects the optic nerves, there would be a loss of vision. However, this occurs in rare situations.

  • Oral Cavity: Patients will complain of loose teeth if the upper and lower jaw bones get affected by Paget's disease. The supporting bone structure will be progressively lost when the tooth becomes mobile.

  • Cancers: Patients with Paget's disease are more prone to cancerous conditions such as osteosarcoma. This is a type of cancer that begins in the bones and is commonly seen in knee bones and shoulder bones.

  • Nerves: The increasing bone mass exerts pressure on the surrounding nerves in the brain and spinal cord. This leads to neurological problems like paralysis and dementia (memory loss).

  • Kidneys: Calcium in the body is processed through the kidneys. When there is a considerable destruction of bone, it leads to elevated calcium levels in the blood. This excess amount of calcium can be precipitated as kidney stones, leading to obstruction of urine flow.

  • Cardiac Problems: Atherosclerosis (accumulation of plaque along the inner walls of the arteries) occurs due to the deposition of extra calcium in the blood along the walls of cardiac arteries. This can lead to heart failure.

  • Bone Disorders: Arthritis (painful and swollen joints) is common in people with Paget's disease. Irregular bone formation can lead to friction between the joints leading to inflammation of the joints.

Who Is At Risk of Developing Paget's Disease?

Certain conditions, called risk factors, can increase the chance of getting the disease. Risk factors indicate that the person is more likely to get the disease. The following are the risk factors that cause Paget disease of the bone:

  • Family History: If a person is suffering from Paget's disease, there are more chances for the patient's relatives to acquire Paget's disease.

  • Age: Elderly people whose age is above 50 years are more likely to be affected by Paget's disease.

  • Gender: Males are more likely to get affected by Paget's disease when compared to females.

  • Nationality: People in European countries are mostly affected by Paget's disease. It is a rare condition to be seen in Asian continents.

How to Diagnose Paget's Disease?

A healthcare professional carries out the diagnosis in the following ways:

1. Examination of the Patient: During the direct physical examination, the patient can disclose the areas of pain. Depending upon the condition, the doctor advises the imaging tests and laboratory tests

2. Imaging Tests:

  • X-rays: X-rays can reveal the presence of bone deformities such as bending of the spine, bowing of legs, and abnormal bone deposition, which are the characteristic features of Paget's disease.

  • Bone Scans: Bone scans are advised to determine the seriousness of the disease. They are indicated in moderate and severe cases. During the bone scan, radioactive material is given intravenously to the patient. This radioactive material flows through the blood circulation and reaches the affected bones. The affected bones take up the radioactive material and appear as bright images on scanning.

3. Laboratory Diagnosis: Elevated levels of alkaline phosphatase are an indication of Paget's disease. Doctors consider this blood test the primary diagnostic tool for confirming Paget's disease.

How to Treat Paget's Disease?

Presently, there is no cure for Paget's disease. However, treatment is mainly directed towards symptom relief and prevention of complications. Early detection of the disease can give a satisfactory treatment outcome. Treatment is carried out according to the severity of the disease, and this includes:

1. Medications or Drugs: Commonly prescribed drugs are Bisphosphonates. They are administered either intravenously or taken as oral pills. Side effects of bisphosphonates include gastric irritation. Commercially available intravenous bisphosphonates are:

  • Ibandronate.

  • Pamidronate.

  • Zoledronic acid.

Commercially available oral bisphosphonates include:

  • Risedronate.

  • Alendronate.

2. Surgery: Surgery is advised in severe cases of Paget's disease to fix the bony deformities like fractures, relieve the pressure on the surrounding nerves, realigning the joints. In extreme conditions, knee joint and hip joint replacements are advised. One of the severe complications of the surgery is the bleeding. Paget's disease causes the deformed bones to produce more blood vessels, and this is the reason for the excessive loss of blood during the surgery.

When to Visit a Physician?

Consult a physician if individuals suffer from the following:

  • Discomfort in the joints and bones.

  • Weakness and tingling sensation in a limb.

  • Skeletal abnormalities.

  • Loss of hearing, particularly if isolated to one side.

Conclusion

Paget's disease is a bone disorder that progresses slowly and is symptomless. Identifying the risk factors and regular check-ups with the doctor could help diagnose the disease in the initial stages. If diagnosis and treatment are made in the early stages, the prognosis would be better, and the complications of Paget's disease could be avoided.

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Dr. Anuj Gupta
Dr. Anuj Gupta

Spine Surgery

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