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Systemic scleroderma, or systemic sclerosis, is an autoimmune rheumatic disease characterised by excessive production and accumulation of collagen, called fibrosis, in the skin and internal organs and by injuries to small arteries. There are two major subgroups of systemic sclerosis based on the extent of skin involvement: limited and diffuse ...
As of 2012, the five-year survival rate for systemic scleroderma was about 85%, whereas the 10-year survival rate was just under 70%. [43] This varies according to the subtype; while localized scleroderma rarely results in death, the systemic form can, and the diffuse systemic form carries a worse prognosis than the limited form.
Anti-Scl-70 (also called anti-topoisomerase I after the type I topoisomerase target [1]) is an anti-topoisomerase antibody-type of anti-nuclear autoantibodies, seen mainly in diffuse systemic scleroderma (with a sensitivity of 28–70%), but is also seen in 10–18% of cases of the more limited form of systemic scleroderma called CREST syndrome. [2]
Anti Scl-70 antibodies (also called anti-topoisomerase I after the type I topoisomerase target [1]) is a type of antinuclear autoantibody seen mainly in diffuse systemic scleroderma, but is also seen the more limited form of systemic scleroderma called CREST syndrome. [2]
CREST syndrome, also known as the limited cutaneous form of systemic sclerosis (lcSSc), is a multisystem connective tissue disorder. The acronym "CREST" refers to the five main features: calcinosis , Raynaud's phenomenon , esophageal dysmotility , sclerodactyly , and telangiectasia .
Sclerodactyly is one component of the limited cutaneous form of systemic sclerosis (lcSSc), also known as CREST syndrome (CREST is an acronym that stands for calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.) [4] Sclerodactyly is also one component of Huriez Syndrome, along with palmoplantar ...
The idea behind the "mixed" disease is that this specific autoantibody is also present in other autoimmune diseases such as systemic lupus erythematosus, polymyositis, scleroderma, etc. MCTD was characterized as an individual disease in 1972 by Sharp et al., [3] [4] and the term was introduced by Leroy [5] in 1980.
Anti-centromere antibodies are found in approximately 60% of patients with limited systemic scleroderma and in 15% of those with the diffuse form of scleroderma. The specificity of this test is >98%. Thus, a positive anti-centromere antibody finding is strongly suggestive of limited systemic scleroderma.