What is scleroderma?
Scleroderma is a collection of autoimmune diseases – this means that the immune system acts against the innate cells of the body. The immune system has mechanisms in place to detect and respond to foreign entities thus protecting the body from potential pathogens. In auto-immune diseases, the immune system mistakenly registers the tissues of one’s own body as foreign bodies and mounts an immune response against the body tissues.
The term scleroderma (derived from Greek to mean hard skin) is a collection of diseases that are characterized by abnormal fibrosis of skin. This means that the skin around certain parts of the body (depending on the variant of the disorder) begin to thicken.
Who can get scleroderma?
The disease is quite rare and there has been no defined criteria as to who gets it and why. However, there has been a significant predilection of the disease for women, with some experts estimating that 6 out of every 7 women who get the disease are women. The onset of the disease often commonly occurs between 35 and 50 years, however, the disease can also occur earlier or later in life.
What causes Scleroderma?
The exact cause of scleroderma is unknown. There are, however, some environmental factors which have been linked to scleroderma or scleroderma like illnesses. Researchers have identified Scleroderma – as well as other auto-immune diseases as having two roots. A genetic susceptibility (as the condition has been commonly found within multiple family members) and an environmental trigger that instigates the progression of the disease.
What are the symptoms of the disease?
The disease often begins with discoloration of the digits of the hands and the feet. The fingers and toes loose circulation and turn white on exposure to cold. This phenomenon typically precedes skin changes by a few months to years depending on the variant of the disorder. Other early symptoms may be painful joints, morning stiffness, or swollen hands.
The discoloration of the digits is known as Raynaud’s phenomenon and can occur independent of Scleroderma. As such the first specific sign that indicates a diagnosis of scleroderma is skin thickening that begins as swelling or puffiness of the hands and fingers. The skin later then takes on a leathery sheen as the disease further progresses. As the disease progresses, there is involvement with other organ systems and symptoms can range from gastro-intestinal, respiratory, and cardiac complaints.
Many individuals who have scleroderma develop a cluster of systems known as CREST syndrome which stands for calcinosis (where the digits are, Raynaud’s phenomenon, esophageal dysfunction (leading to difficulties in swallowing food), sclerodactyly (this is localized thickening and tightening of the skin of the fingers and toes) and telangiectasia (a condition whereby widened venules within the skin cause threadlike patterns within the skin)
There is currently no effective treatment for the disease. There are however, various modalities that been developed to manage the symptoms of the disease. These interventions include immunosuppressant drugs which repress the immune system and slow the disease progression such as cyclophosphamide and dexamethasone.
What are the side effects of the medication?
One of the main side effects of the medication is increased susceptibility to infection due to the mechanism of action of the various drugs. As the medication suppresses the immune system, the end result is that the activity and ability of the immune system to respond to infectious diseases is reduces.