What Is Paget’s Disease Of Bone

Overview

Paget’s disease of bone is a rare chronic bone disorder (1 in 12), an excessive bone turnover called osteoclast (a cell that absorbs deteriorated bone and later causes bone fracture if disturbed).¹

Osteitis Deformans also refers to this rare disorder. Paget's diagnosis and treatment depend on the affected area, such as the pelvis, the skull, the spine, and the leg. Paget’s bone disease can either affect one bone (monoostotic) or many bones (polyostotic).

The affected bones become weak (osteoporosis) and get a fracture. Arthritis is a disorder that sometimes collides with Paget’s disease. When the bone condition invades the joint, it is automatically diagnosed with arthritis. 

Osteoarthritis is a factor that can diagnose Paget’s bone disease that weakens a bone site, whether it is genetic inheritance or environmental material. However, not all patients having an autosomal dominant trait can be diagnosed with this bone disease.²  

Who can get this bone disease?

The autosomal dominant trait can be present in any individual approaching their 50s. Yet, the highest percentages of occurrence are males since birth, with 1 in 12. 

You should be cautious, as this disorder affects the bone, but there is also another classification called Paget’s disease on breasts. 

Causes of paget’s disease of bone

The research narrows down to two factors: the genetic component and the environmental element triggering this bone aliment. We know that bone cells are generated (old bone cells are getting replaced to make way for the new healthy ones). 

Paget’s bone disease occurs when osteoclast and osteoblast (responsible for generating new cells) are not coping anymore, and the cells produced are immature due to excessive production.² 

Environmental factors:

Regarding this factor, it is said that paramyxovirus infection is a viral infection) stimulates Paget's bone disease. The infection is the primary cause which a person gets along with measles. Research debunks that there is no association between any viral infection and Paget’s disease.³

Paget’s bone disease is common in European and Northern family heritages.

The environmental factor is most underrated as researchers are having trouble locating the specific single trace that triggers the bone disease. There are insights related to this.⁴

• Food as in diet- rich in saturated fat and lack of vitamin D
• Stress
• Ageing factors and their infections
• Cigarette smoke-  childhood exposure to wood burning promotes a slow stimulant 
• Lack of Vitamin D- poor diet cannot support bone structure 

Genetic factor:

SQSTM1 is a protein that remodels healthy bone production. The SQSTM1 has turned into a mutation responsible and localised with the gene. The diagnosis is necessary as the bone disease is asymptomatic (no symptoms are visible).⁴

• A familial genetic 
• The disease is sporadic (that are in a few places)
• The disease clusters within families 
• The genetic factor is poorly understood

The site where paget’s disease occurs 

In most cases, patients are asymptomatic. The disease weakens the bones at a slower rate. The affected bones are identified as follows.²  

• Fractured bone- The bone is brittle (osteoclast), allowing the bone to fracture. 
• Hearing loss- The disease affects the skull from the enlargement of the bone and disturbs the nerves connected to the skull, leading to the patient being deaf. 
• Headaches- Paget’s bone disease does target the cartilage that is part of the skull. 
• Joint pain- also known as osteoarthritis, the abnormal growth of the osteoblast pressures the affected bone and forces it to wear and tear action that causes joint pain. 
• Bone deformation- It occurs as the bone disease gradually deteriorates the bones.  
• The pelvis- Due to the wear and tear action, the affected bone is inflated, causing pain in the pelvis and its thighbone. 

The diagnosis stage

1. A radiologist can start by investigating the patient's concern. Performing a biopsy is taking a bone sample and running tests to validate a sign that corresponds to Paget’s bone disease. The process required inserting a needle and numbing the area where the sample was from.
2. Another direct diagnosis would be a patient lying on a bed, and another radiologist would capture the X-ray to analyse it with the medical history file.
3. Another visual scan like the scintigraphy. Before the scan, the patient needed to digest a radioactive substance into the bloodstream for a good visual.
4. The blood test is a good indicator of the rising level of alkaline phosphatases (ALP). The disease enlarges the bones, which induces vitamin D deficiency, and the ALP detected high levels as osteoblast and osteoclast damages the bone site and causes ALP to rise abnormally.⁵ 

Management and treatment

• To manage osteoarthritis conditions, the patient should engage their muscles and joint movements. Regularly exercising regularly strengthens joints. The proper regime and consulting a physiotherapist are advised.  
• Being overweight puts pressure on the bones, so losing weight is another way to reduce pressure on the bones. 
• Paracetamol is the popular choice to relieve pain towards osteoarthritis. However, the GP needs to prescribe them. 
• Vitamin D balances calcium intake and is a supportive system for the bones; incorporating it into your routine is required.
• The osteoporosis drug (Bisphosphonates) is the popular choice for the treatment so far. The drug’s function is specific to suppress bone turnover, which is the main characteristic of bone disease. The drug is administered via injection to restore the bone state. The treatment is efficient by inhibiting bone turnover, preventing signs like hearing loss, possible fracture, and bone deformity, and reducing osteoarthritis complications.⁶
• Surgery is only the resort due to complications of the disease. This invasive treatment method does relieve osteoarthritis. 
• The surgery allows for the replacement of the damaged site and the enforcement of an artificial bone. 
• For surgery fixes such as thigh bone fractures, the surgery process uses metal rods to support the bone.

FAQs

Can paget’s disease of bone be prevented?

The data on Paget’s bone disease is not yet enough to know how to prevent the disease effectively. Taking healthy steps, sufficient amounts of calcium and vitamin D, and maintaining weight can work toward improving joint mobility.  

Who is at risk of paget's bone disease?

The targeted people are older individuals. Looking in-depth, people with northern European heritage are prone to develop the disease. The disease can be present within the gene to which the disease is tested within the family. 

How common is paget's bone disease?

Paget's bone disease is rare. 1 in 12 of the population in the UK over the age of 55.

When should I see a doctor?

 There should be knowledge of family disease traits whenever doing the annual visits. The patient complaining of joint pain can consult a GP and request for a blood test appointment.  

Summary

This article focuses on Paget’s disease, a chronic bone disease. The bone is deteriorating slowly. Within the site, bone cells are altered and caught up with fragile states of the bone. However, it simultaneously dysregulates the cycle of the cells. Any bone can be affected by Paget’s bone disease and the most common sites are the pelvis, skull spine and leg bones. Older age groups, usually the age above 50 are at risk of developing Paget’s bone disease. Therefore, taking care of the dieting process and exercising right at any age is beneficial to support your bones.