Our body is made up of complex body parts, and bones help to provide definite shape and structure to the body. Usually, bones undergo a periodic recycling process in which the new bone tissue replaces the old bone tissue. Paget’s disease is a developmental bone disorder that interferes with the bone regulatory process. Moreover, it makes the bone fragile. Some of the frequent sites affected by Paget’s disease include the skull, pelvis, spine, and legs.
Paget’s disease is thought to be an inherited disorder. However, the exact cause is yet unknown. The risk of Paget’s disease increases with advancing age. Some of the complications may include bone fracture, problems in the spine, and hearing loss.
Often, the treatment includes bisphosphonate therapy to strengthen the bones. Surgical interventions may be required for patients with bone complications. Today’s article will highlight the features of Paget’s disease. Furthermore, we will discuss the symptoms and treatment of Paget’s disease.
What is Paget’s disease?
Paget’s disease, also known as Osteitis Deformans, is a condition characterized by cellular remodeling and deformity of one or more bones in the body. it is the second most common type of bone disorder after osteoporosis. (1)
Often, in this disorder, new bone is generated in incorrect locations. Moreover, old bone is removed from areas where it provides support. Such incidents can lead to the weakening of the bones. Additionally, it increases the risk of bone pain, arthritis, and fractures. The prevalence of Paget’s disease is around one million in the United States. Moreover, it tends to occur in older adults.
What causes Paget’s disease?
The exact cause of Paget’s disease is not definite. However, it is believed to be an inherited disorder. The American College of Rheumatology states that in 30% of the cases, more than one family member has this disorder. (2)
Other possible causes of Paget’s disease may include –
- Measles virus infection
- Environmental factors
- Genetic mutations
What are the symptoms of Paget’s disease?
Often, people do not notice the effects of Paget’s disease until it becomes severe. Some of the common symptoms of Paget’s disease involve –
- Swelling of the joints
- Tenderness and redness around the skin that covers the affected area
- Weak bones
- Increased frequency of bone fractures (3)
- Joint stiffness and pain
- Problems with the spine, skull, thigh bone and shin
- Compression of nerves located close to the affected bones
- Neuronal damage which may cause triggering pain
Some of the complications of Paget’s disease include –
- Loss of hearing
- Heart disease
- Development of bony sarcoma – a type of bone cancer (4)
- Loose teeth
- Vision problems
- Issues with the nervous system
How is Paget’s disease diagnosed?
The diagnosis of Paget’s disease includes extensive physical and radiographic evaluation. Some of the diagnostic steps to identify Paget’s disease are listed below –
- A complete history of the patient is taken to rule out genetic connections. Additionally, a physical examination is performed to determine the abnormalities of the bone.
- A series of bone scans and laboratory studies are done to determine the extent of the condition. Bone scans can reveal the type of bone deformity, abnormalities with bone remodeling, and imbalance in a bone deposition. A classic ‘cotton wool’ appearance in the skull scan is characteristic of Paget’s disease. (5)
- An alkaline phosphatase blood test can quickly identify Paget’s disease.
- X-ray studies of the bone are performed for confirmation of the condition.
What is the treatment of Paget’s disease?
Treatment typically controls and reduces the impact of symptoms. However, there is no definitive cure for Paget’s disease.
- Often, the first line of treatment for Paget’s disease is bisphosphonate therapy. It helps to strengthen the weak bones in the body. Moreover, it reduces the breakdown of the dislocated bone.
- Vitamin D and calcium supplements are also prescribed. The ideal recommended dose of calcium is 1200 mg, and that of vitamin D is 600 IU per day for people between the age of 51 – 70 years. (6)
- People older than 70 years should consume 800 IU of vitamin D per day. Additionally, exposure to sunlight is essential to generate natural vitamin D production in the body.
It is essential to note that the bisphosphonate and calcium supplements should be consumed one after the other at least two hours apart.
- Surgical intervention is necessary to repair bone fractures. Osteotomy is a standard surgical procedure performed to correct the weak bone.
- Exercise may help to maintain skeletal health. However, it is essential to consult the physician before starting any exercise program.
Take away message
Paget’s disease is a developmental disorder that interferes with the remodeling of the bone – a process through which the body absorbs and regenerates bone.
Typically, this condition leads to the weakening of the bone and increases the risk of injury and fracture. Often, people with Paget’s disease do not notice the symptoms until it becomes severe. Some of the common symptoms include – joint pain and swelling, redness of the skin, weakening of the bone, and nerve damage.
The exact cause of Paget’s disease is not known. However, it is associated with measles infection, genetic mutation, and environmental factors. Paget’s disease is common in older males. There is no cure for Paget’s disease. However, treatment of Paget’s disease often includes medications including bisphosphonate, calcium, and vitamin D supplement. In some cases, surgical procedures like osteotomy may be performed to repair bone fractures.