What is Scleroderma?
Scleroderma is not a single condition but a group of rare autoimmune conditions in which connective tissues and skin, the fibers supporting the body, progressively harden and tighten. It may or may not run in families. No genes that could be linked to scleroderma have been identified. It affects more women than men in the age group 30 to 60 years. Rarely found in children, it affects one person in a population of 4000.
What are Signs and Symptoms of Scleroderma?
The most prevalent signs of scleroderma include:
- Raynaud’s phenomenon
- Skin changes
- Gastroesophageal reflux disease or GERD
Raynaud’s phenomenon is the first sign to look for in case of localized scleroderma. In case of systemic scleroderma, the changes in skin are sudden and rapid. It goes on to worsen within the next year or two.
Who Gets Scleroderma?
It is difficult to diagnose scleroderma because the symptoms match other autoimmune disorders. Children are more commonly diagnosed with localized scleroderma, while adults are more commonly diagnosed with systemic scleroderma. The scleroderma risk is 4 times more among women than men. In addition, other factors like ethnicity and race may also influence the severity and pattern of this disease.
The exact hereditary link of scleroderma is not yet identified, but it is believed that families with rheumatic diseases are predisposed to it. Although it can strike any age group, middle-aged people are at a greater risk of getting scleroderma.
What Causes Scleroderma?
Scleroderma is known to occur when there is too much production of collagen in the body and its accumulation takes place. Body’s connective tissues are made by collagen, which is a form of protein. It is believed that the immune system in the body plays a possible role in abnormal collagen production. But how the immune system gets triggered is not well understood.
When an individual’s immune system in the body attacks itself, the result is over-production of collagen and inflammation. While the mechanism of scleroderma is known, the exact cause it yet not understood properly. Experts are still studying the issue to determine the cause of scleroderma.
There is no genetic evidence of scleroderma, but there is speculation of a genetic role in the disease. While certain genes are implicated, the environment too appears to play a critical role. Susceptible individuals are at a greater risk of this disease.
Scleroderma, according to NIH, is not a single disease but a group of conditions. So, it is actually a rheumatic disease as well as the disease of the connective tissue. The conditions grouped under rheumatic disease are characterized by the following:
- Pain and inflammation in the joints
- Pain and inflammation in the muscles
- Pain and inflammation in the fibrous tissue
The disease of connective tissue affects tendons, cartilage, and skin.
What are the Risk Factors for Scleroderma?
Certain ethnic and racial groups are at a higher risk of scleroderma. These include African Americans and Native Americans. Some Native American groups are at least 20 times more at risk than the general population. Women are at least 4 times more likely than men to be the victims of this disorder. Additionally, those exposed to silica dust, industrial solvents, and chemotherapy drugs are at a higher risk.
How is Scleroderma Diagnosed?
Scleroderma syndrome is diagnosed on the basis of clinical features accompanying the illness that are detected. Additionally, blood tests in nearly all patients show anti-nuclear antibodies and autoimmunity (ANAs). The antibody anticentromere is detected in almost every case of CREST or limited form of sclerosis that is systemic sclerosis. Additionally, antibody anti-scl70 is frequently found in those patients who show signs of diffuse systemic sclerosis.
There could possibly be several other tests in order to determine signs of any other internal disease. So, lower and upper gastrointestinal tests may be required in order to examine bowels and stomach. Tests to determine the functioning of lungs and pressure in the arteries of the lungs and heart may also be required. These may require pulmonary test to know the functioning of the lung, x-rays of chest, echocardiograms, EKG, and heart catheterization.
How is Scleroderma Treated?
Since scleroderma is not a single disease, there is no single treatment. Treatments to control blood pressure with the use of drugs are important to prevent kidney failure. Colchicine is found useful in controlling inflammation. The following lotions help in relieving skin itching:
- Bag balm
Aspirin in low doses can be used to reduce finger blood clots. There are medications to help mild Raynaud’s by opening up arteries other than protection and hand warming for patients suffering from finger tip ulceration. Typical depressants like Prozac can at times help circulation. Surgical procedures can be needed in severe cases of Raynaud’s phenomenon.
Heartburn and esophagus irritation can be helped with medication. Antacids are also helpful. It helps to avoid caffeine and cigarettes to diminish cancer risk. Bacteria causing diarrhea and constipation can be treated with medication. The symptoms can be reduced with increased fiber and fluid intake.
From the discussion above, it is clear that scleroderma is not a single disease but a group of diseases affecting the joints, connective tissue, and skin characterized by pain and inflammation. It is caused due to too much production and collection of collagen, a form of protein in the body. But scleroderma can be diagnosed and treated.