What is scleroderma?
Scleroderma is a chronic disease that affects connective tissue, the tissue that supports and holds organs together and is also found in the joints.
The hallmark of scleroderma is thick and hardened skin caused by excessive production of collagen, the main component of scar tissue. This scar tissue can accumulate in and damage organs, including the heart and blood vessels, the lungs, stomach, and kidneys.
What causes scleroderma?
Scleroderma is an autoimmune disease, meaning it is caused by an overactive immune system that mistakenly attacks the body’s own tissues. The excessive inflammation damages the skin and affected organs.
Scleroderma is a complex condition, and the underlying disease process is not well-understood. It is thought that the disease results from a combination of genetic and environmental factors. But it is not contagious, infectious, cancerous, or malignant.
Who can develop scleroderma?
Women are three to four times more likely to develop scleroderma than men. Disease onset typically occurs between ages 25 to 55, but can also be found in children.
Types of scleroderma
This disease is broadly classified as either localized scleroderma or systemic scleroderma. The main difference between the two is the degree of internal organ involvement.
People with the localized form of the disease tend to have symptoms affecting skin only, although it can also spread to the muscles, joints, and bones. Those with the systemic form are more likely to have internal organ involvement and damage. Localized scleroderma is more common in children, whereas systemic scleroderma is more common in adults.
Both localized and systemic scleroderma can be further divided into various subtypes.
What are the symptoms of Scleroderma?
Symptoms of scleroderma vary among patients, and can range from very mild to being life-threatening depending on which parts of the body are affected and the extent of organ involvement. A mild case can become serious if not treated properly.
Possible co-existing conditions
Patients with scleroderma can be at greater risk of developing other conditions, such as Sjogren’s syndrome, erythromelalgia, fibromyalgia, and systemic lupus erythematosus.
Treatment and management of Scleroderma
Although scleroderma has no cure, several treatments can help make the condition more manageable. Medications include corticosteroids, non-steroidal anti-inflammatory medicines, and immunosuppressive therapies to manage the inflammation, and anti-fibrotic agents to block the formation of excess scar tissue.
Patients may also need treatments that specifically address symptoms resulting from organ damage. Physiotherapyand occupational therapy can also help improve the quality of life of patients with the condition.
Stem cell transplants are being investigated as a possible way to treating several autoimmune diseases, including scleroderma.
In scleroderma, the immune system mistakenly attacks healthy connective tissue. These attacks trigger damaging responses and lead to the buildup of collagen that causes hardened scar-like tissue to form.
There is currently no cure for scleroderma, but stem cell therapy may have the potential to alter or even “reset” the immune system and treat the disease.
What are stem cells?
During development, the cells in the body “differentiate,” or become specialized to be able to carry out a set and defined function. Once specialized, a cell generally cannot change into another type of cell.
But our stem cells, unlike the majority of the cells in our body, have the potential to develop into a range of different types of cells. These cells are able to maintain and repair tissues and organs. They can self-renew to produce more stem cells, and develop into the specialized cells of the tissues in which they are found. For example, they can replenish blood cells or repair the worn lining of cells of the small intestine. This specialization only occurs in stem cells under very specific circumstances, and in some organs — like the pancreas and heart — it is very difficult to trigger this change.
Types of stem cells
There are different types of stem cells with the potential to become different types of specialized cells, as stem cells have specific properties based on the tissue from which they are harvested. Stem cells are broadly split into two types: embryonic stem cells and adult stem cells.
Adult stem cells can be harvested from many tissues, but they are restricted in the type of cell they can become. There are many types, including:
• Hematopoietic stem cells are related to blood and immune cells
• Mesenchymal stem cells, which produce bone, muscle, and connective tissue, as well as regulating the immune response
• Neural stem cells that can produce certain types of nerve cells
• Epithelial stem cells that, for example, renew the walls of the small intestine
How could stem cells help in scleroderma?
In scleroderma a stem cell transplant might be able to modify or reset the immune response, potentially reducing or preventing disease progression. It is e possible that stem cells can be used to repair damage caused by scleroderma in tissues.