What is Scleroderma?
The term scleroderma literally translates to “hard skin.” It is an autoimmune disease in which the body’s immune system mistakenly attacks its own connective tissues. Scleroderma involves an overproduction of collagen and presents itself in two major forms:
The milder of the two
Affects limited areas of the skin and muscle
Can cause excessive scarring, swollen, tight or hard skin.
Potentially involves joints, blood vessels and major organs
Can cause serious, sometimes life-threatening damage to the lungs, heart, kidneys, esophagus, and gastrointestinal tract.
What are the symptoms?
Among the early signs of scleroderma is Raynaud’s phenomenon (pronounced RAY-KNOWDS), which is identified by fingers that become very sensitive to cold, causing the fingers to change colors. The changes of color are caused by the spasm and narrowing of the blood vessels. This reaction can be caused by emotional stress as well. There are two types of Raynaud’s Phenomenon:
Raynaud’s phenomenon is a common condition and most people with Raynaud’s phenomenon will NOT develop scleroderma. These individuals have what is referred to as primary Raynaud’s.
While only a small percentage of people with Raynaud’s phenomenon develop scleroderma, almost all scleroderma patients have Raynaud’s symptoms. This is referred to as secondary Raynaud’s phenomenon, so-named because it is secondary to the scleroderma.
Additionally, fingers and hands that become stiff and puffy can also be early indicators of scleroderma.
What causes scleroderma?
At the moment there is no known cause of scleroderma and no way of preventing this disease. It is more common in females than males, yet it affects all genders, all ages and all racial groups. The peak of the disease onset occurs in the fourth and fifth decades of life. Familial scleroderma is rare, and there is no evidence of genetic inheritance.
How many people suffer from Scleroderma?
According to Dr. Fredrick Wigley, M.D., physician-scientist and Director of the Johns Hopkins Scleroderma Center, there are a variety of different components involved in determining how many people actually suffer from scleroderma. “The estimate for systemic sclerosis,” he explains, “therefore vary from about 30 cases/million to about 400 cases/million.” But, “most feel the overall prevalence of systemic sclerosis is about 200-280 cases/million.”
He adds, “the term scleroderma includes cases of systemic sclerosis, localized scleroderma and ‘scleroderma spectrum of disease’… and, if one includes all the groups, the prevalence estimates are higher: one study reporting over 3000 cases/million.”
Dr. Wigley also explains that some areas and populations are believed to have a greater prevalence of scleroderma than others. “Most believe there are more cases in the USA and Australia than in Japan.” And, “certain populations have a very high prevalence compared to others: the Choctaw Indians were reported to have 658.6 cases/million.”
How does scleroderma affect the patients who are living with this disease?
The symptoms and effects of this disease vary greatly from patient to patient. When the skin is affected, the symptoms are visible, but, when only internal organs are affected, they are unseen. For some, scleroderma can be life-threatening due to the severity of organ involvement, and, for others, their symptoms can be managed with the right treatment.
The effects of scleroderma also go beyond just the physical. Many scleroderma patients find that the emotional toll this disease takes can be equally as debilitating.
Because they don’t always appear to be sick, patients sometimes struggle to communicate to their family and peers that what they are dealing with is serious. Additionally, due to severe loss of mobility in their hands and other scleroderma related issues, many patients are forced to leave their jobs and file for disability.