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Systemic scleroderma is an autoimmune or connective tissues disease. It is characterized by thickening of the skin caused by accumulation of collagen, and by injuries to the smallest arteries. There are two overlapping forms. Limited cutaneous scleroderma is limited to the skin on the face, hands and feet. Diffuse cutaneous Survival is determined by the severity of visceral disease. Prognosis is difficult to predict until the disease differentiates into recognizable subsets. Patients with limited cutaneous scleroderma have a good prognosis, with 10-year survival of 75%, although <10% develop pulmonary arterial hypertension after 10 to 20 years. Patients with diffuse cutaneous scleroderma have a 10-year survival of 55%. Death is most often from pulmonary, heart and kidney involvement, although survival has greatly improved with effective treatment for kidney failure. Immunosuppressive drugs are used, although glucocorticoids have limited application. Annual incidence is 19 per million, and prevalence is 19-75 per 100,000, with a female: male ratio of 3:1, and 8:1 in mid to late childbearing years. Incidence is twice as high among African Americans, and the Choctaw Native Americans in Oklahoma have the highest prevalence in the world (469/100,000). There is some hereditary association, some suggestion of immune reaction (molecular mimicry) to a virus, and some cases caused by toxins. Diffuse Scleroderma - affects the skin as well as the heart, lungs, GI tract, and kidneys.
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Patients with larger amounts of cutaneous involvement are more likely to have involvement of the internal tissues and organs. Most patients (over 80%) have vascular symptoms and Raynaud's phenomenon, which leads to attacks of discoloration of the hands and feet in response to cold. Raynaud's normally affects the fingers and toes. Systemic scleroderma and Raynaud's can cause painful ulcers on the fingers or toes which are known as digital ulcers. Calcinosis (deposition of calcium in lumps under the skin) is also common in systemic scleroderma, and is often seen near the elbows, knees or other joints.Diagnosis is by clinical suspicion, presence of autoantibodies (specifically anti-centromere and anti-scl70/anti-topoisomerase antibodies) and occasionally by biopsy. Of the antibodies, 90% have a detectable anti-nuclear antibody. Anti-centromere antibody is more common in the limited form (80-90%) than in the diffuse form (10%), and anti-scl70 is more common in the diffuse form (30-40%) and in African-American patients (who are more susceptible to the systemic form).
Limited Scleroderma - mostly affects the skin of the face, neck and distal elbows and knees and late in the disease causes isolated pulmonary hypertension. CREST syndrome (Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, Telangiectasias) is associated with limited scleroderma.There is a slight increase in the risk of cancer with systemic sclerosis.In the skin, systemic sclerosis causes hardening and scarring. The skin may appear tight, reddish or scaly. Blood vessels may also be more visible. Where large areas are affected, fat and muscle wastage may weaken limbs and affect appearance. Also, patients report substantial, even severe and recurrent itching of large skin areas, the source of much affliction as the condition worsens. There is much variation in severity between patients, with some having scleroderma of only a limited area of the skin (such as the fingers) and little involvement of the underlying tissue; while others have progressive skin involvement Diffuse scleroderma can cause musculoskeletal, pulmonary, gastrointestinal, renal and other complications.
One of the suspected mechanisms behind the autoimmune phenomenon is the existence of microchimerism, i.e. fetal cells circulating in maternal blood, triggering an immune reaction to what is perceived as "foreign" material.A distinct form of scleroderma and systemic sclerosis may develop in patients with chronic renal failure. This entity, nephrogenic fibrosing dermopathy or nephrogenic systemic fibrosis, has been linked to the exposure to gadolinium-containing radiocontrast. In 1980 the American College of Rheumatology agreed upon diagnostic criteria for scleroderma. Other conditions may mimic systemic sclerosis by causing hardening of the skin. Diagnostic hints that another disorder is responsible include the absence of Raynaud's phenomenon, a lack of abnormalities in the skin on the hands, a lack of internal organ involvement, and a normal antinuclear antibodies test result. There is no clear obvious cause for scleroderma and systemic sclerosis. Genetic predisposition appears to be limited: genetic concordance is small; still, there often is a familial predisposition for autoimmune disease. Polymorphisms in COL1A2 and TGF-β1 may influence severity and development of the disease. There is limited evidence implicating cytomegalovirus (CMV) as the original epitope of the immune reaction, as well as parvovirus B19.Organic solvents and other chemical agents have been linked with scleroderma. Bleomycin (a chemotherapeutic agent) and possibly taxane chemotherapy may cause scleroderma, and occupational exposure to solvents has been linked with an increased risk of systemic sclerosis.
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This page was last updated on February 23, 2024