Thickening and decreased flexibility of the skin of the fingers and other body parts is characteristic of scleroderma. Raynaud's phenomenon is almost always present, although it should be noted that about 4% of the general population also has Raynaud and most of these people will never develop scleroderma. Painful ulcers can occur at the fingertips. The fingers can gradually become fixed in a bent or flexed position. Arthritis (joint pain and swelling) and muscle weakness may also occur. Esophagus involvement can cause heartburn, regurgitation and difficulty swallowing. Small intestine involvement may cause an intestinal obstruction and reduce the ability to absorb food. The lung or heart involvement can cause shortness of breath. The involvement of the kidneys can manifest itself as a severe arterial hypertension crisis and reduced kidney function (renal failure). Some patients have a significant weight loss, while others have a persistent pruritus (itchy skin). Fatigue and decreased ability to carry out daily activities are pervasive. Scleroderma is a major cause of physical and moral suffering and decreased quality of life.
Raynaud's phenomenon is when blood vessels in the fingers constrict or narrow in response to cold or emotional stress. The resulting disruption in blood circulation causes a series of color changes in the skin: white, blanched or pale, when circulation is reduced or stopped; blue as the affected part lacks oxygen from decreased blood flow; and then red or flushed as blood flow returns. Raynaud's phenomenon affects about 4% of the general population. It is present in over 90% of patients with scleroderma. "Crises" can last anywhere from a few minutes to a few hours and tend to worsen with time. Although, there is currently no cure for Raynaud's phenomenon, many common-sense preventative measures can be taken by those affected. The most obvious is minimizing exposure to cold, wear gloves or mittens and avoid smoking. A number of different medications can also help to prevent, reduce the frequency and minimize the effects of Raynaud’s phenomenon. For more information, see the booklet « Winter at last » .
Calcinosis is calcium deposits that occur at the distal ends of the fingers, tendons, joint capsules or pre-articular (fingers, elbows) and, in some rare cases, at the anterior surface of the legs or buttocks. When the accumulation is close to the surface of the skin, it can produce a spontaneous discharge (drainage) with or without infection, because this opening to the skin is a new gateway for bacteria. Sometimes the accumulation of calcium is harmful and requires drainage. It might be tempting for patients to intervene on themselves with household tools, such as knives or needles, to accelerate drainage. Here a word of cautious is in order: these tools are usually not properly sterile, and by doing so the patient might introduce harmful bacteria in his/her skin, thus causing an infection that might otherwise be avoided. A calcinosis drainage must only be performed by a surgeon, under antibiotics. Reference : About scleroderma in Quebec.
The role of the immune system is to protect the body from foreign invaders, such as viruses and bacteria. Sometimes, however, this system goes awry and the immune response, which normally targets only outside elements, turns against the body’s own cells, attacking them, resulting in the production of large quantities of autoantibodies. Some studies suggest that scleroderma is an autoimmune disease. The main evidence for this notion is the fact that SSc patients have specific autoantibodies. In the case of scleroderma, fibroblast cells (a type of cell in the body which produces collagen and is important in wound healing) are abnormally overactivated and overproduce collagen that spreads throughout the skin and various internal organs, causing inflammation, leading to tissue fibrosis (scarring).
At present, it is unclear what exactly triggers this autoimmune disease. Research is being conducted to find out if there is a genetic susceptibility to scleroderma, but as yet the disease does not appear to be hereditary. There is no direct cure for scleroderma. Because the exact cause is unknown, any treatment is patient-specific and aimed at making symptoms of the disease more tolerable. Thus, it is imperative to continue research to find the underlying causes of this disease, develop new treatments to adequately relieve patients and, ultimately, find a cure that will allow patients to overcome scleroderma.
Scleroderma can make it difficult to maintain good oral hygiene practices, which in turn may lead to dental problems such as tooth decay and gum disease. These conditions may be aggravated by the narrowing of the mouth, dry mouth, pain on chewing as well as limited manual dexterity when hands and fingers are affected.
To prevent these dental problems from occurring and learn different methods to help overcome these difficulties, I invite you to read the pamphlet Dental care in Scleroderma prepared by Mervyn Gornitsky, DDS, of the Department of Dentistry at Montreal’s SMBD Jewish General Hospital, and Sabrina Gravel, M.Sc., of the Canadian Scleroderma Research Group (CSRG).