The Role Of Exosomes In The Treatment Of Paget’s Disease

Paget’s disease is a disease that affects the cellular remodelling and deformity of the bones of the body. The bones affected by this disease often have an abnormal remodelling, at the microscopic level. These abnormal remodelling of the bones causes the bone to breakdown excessively, and to also have a disorganized new bone formation. As this disease progresses, the bones affected would become more fragile and prone to fracture. Paget’s disease mostly affects the disease of the pelvis, skull, spine and the extremities. The exact cause of this disease is not known yet, although studies have shown that it might be due to genetic and acquired factors. Some of the factors that increase the chances of developing this disease include age, family history, and so on. This disease can lead to other complications such as fractures, hearing abnormality, and pinched nerves in the spine. The pinched nerves in the spine are due to the abnormal new bone formation. Studies have also shown that this disease occurs more in people that have a British descent. In addition, this disease occurs more in people that are over the age of 55. It has also been shown that Paget’s disease is more prevalent in men than women. The treatment of this disease varies in people, as people present with different symptoms. The focus of the treatment of this disease is on reducing and possibly eliminate the pain and discomfort associated with it. Exosomes are a potentially effective treatment option for Paget’s disease.

What Are The Signs And Symptoms Of Paget’s Disease?

Majority of the people affected by Paget’s disease do not present with any symptoms. However, when they do, the most common symptom is bone pain. Patients also have weak and bones that are prone to fracture, because of the fast remodelling process. This leads to fractures, deformities, and pains in the bone. The symptoms presented by the patient would be dependent on the parts of the body affected. Below are some of the symptoms of Paget’s disease;

Pelvis: Patients with Paget’s disease of the bone in the pelvis would experience hip pain.
Skull: Patients with Paget’s disease of the skull might manifest with loss of hearing and headaches. This is mostly because of the overgrowth of the bones of the skull.
Leg: The Paget’s disease of the bones of the leg, might cause the bone to bend. Patients might present with bowlegs. This can put a lot of pressure on the legs, and excess stress on the surrounding bone joints, which might eventually lead to the osteoarthritis of the knee or hip.
Skin temperature may be increased over areas of active disease.
Deafness and ringing in the ears may occur due to compression of a nerve (which supplies the ears) in the brain by a deformed bone. This is a rare complication.
Spinal deformity may occur due to compression of the spine. This is also a rare complication.

What Are the Causes Of Paget’s Disease?

The exact cause of Paget’s disease is unknown. However, researchers have suggested that it might be caused by a combination of genetics and environmental factors. Asides from these two, below are some factors that could lead to the development of Paget’s disease;

Infections: Paget’s disease could be caused by infections. One of the prevalent organisms that cause this disease is the slow virus. These viruses can persist in the body for many years before the patient would become symptomatic. Some of the diseases associated with Paget’s disease include respiratory syncytial virus, measles virus and so on.
Genetics: Some genes have been identified to contribute to the development of Paget’s disease. According to statistics, about fifty per cent of all cases of Paget’s disease is due to genetics. The people affected by genetics usually inherited the gene responsible for this disease from their parents. The gene is named the “SQSTM1” gene. The gene encodes for the protein that regulates the action of osteoclastic cells in the body. Osteoclast cells are cells that break down the bone tissues. Meanwhile, another set of patients have a mutation of “RANK”. RANK is a receptor on the osteoclasts, that is responsible for breaking down the bone tissues.
Lifestyle factors: There’s some evidence that poor diet or a bone injury early in life may act as triggers for the later development of Paget’s in people who also have genetic risk factors.

What Are the Risk Factors Of Paget’s Disease?

There are some factors that increase the risk of having Paget’s disease. Some of these factors are;

Gender: According to statistics, it has been noted that men are more affected by the disease, as compared to women.
Age: This disease is more predominant in people above the age of It rarely occurs in young people. Naturally, as people grow, the actions of the osteoclast cells surpasses that of the osteoblast cells. In other words, more bone cells are broken down, than the cells formed. However, Paget’s disease makes the situation worse.
Race: It has been observed that this disease is more prevalent in Europe, especially in England. In addition, some cases have also been recorded in Scotland and Greece
Family: People who have had a relative or sibling that got affected by this disease have a high chance of having Paget’s disease.

What Are The Complications Of Paget’s Disease?

Paget’s disease is one that progresses gradually. Below are some of the complications that might occur when not well managed;

Bone carcinoma: This occurs in less than one per cent of people affected by the disease.
Cardiac failure:
Hypercalcemia:

Treatment of Paget disease of the Bone

The focus of the treatment of this disease is on reducing and possibly eliminate the pain and discomfort associated with it.

Bisphosphonates, Calcitonin drugs are usually administered to reduce bone turnover, improve bone pain and restore normal bone histology.
Non-steroidal anti-inflammatory drugs (NSAIDs) and paracetamol may be effective for pain.
Surgery is performed on those that have had fractures, or severe cases of arthritis, and other bone deformities.
Orthotic devices, sticks and walkers may be useful for the disease of the legs if it causes problems with walking.

References

Colman, A. (2008). Induced Pluripotent Stem Cells and Human Disease. Cell Stem Cell, 3(3), pp.236-237.

SHIMIZU, M. (2011). Origin of Paget’s cells in Paget’s disease. Skin Cancer, 26(1), pp.21-27.

Wagner, G. and Sachse, M. (2011). Extramammary Paget disease – clinical appearance, pathogenesis, management. JDDG: Journal der Deutschen Dermatologischen Gesellschaft, 9(6), pp.448-454.