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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.
Primary lateral sclerosis, progressive muscle weakness in the voluntary muscles. Primary sclerosing cholangitis, a hardening of the bile duct by scarring and repeated inflammation. Systemic sclerosis (progressive systemic scleroderma), a rare, chronic disease which affects the skin, and in some cases also blood vessels and internal organs.
As of 2012, the five-year survival rate for systemic scleroderma was about 85%, whereas the 10-year survival rate was just under 70%. [44] 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 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]
3.3 per 100,000 (adults), 50 per 100,000 (children) [90] Thrombotic thrombocytopenic purpura: ADAMTS13 autoantibodies Confirmed 1-2 per million [91] Antiphospholipid syndrome: Antiphospholipid antibodies Confirmed 40-50 per 100,000 [92] Paroxysmal nocturnal hemoglobinuria: None specific, mutation causes self-cells to become susceptible to ...
The scars are diffuse with inflammation, distal oligodendrogliopathy, microglial activation and loss of myelin-associated glycoprotein (MAG). It is considered atypical and an overlap between MS and Balo concentric sclerosis. The scars do not surround the blood vessels, and a rim of preserved myelin appears around the vessels.
[4] [5] [6] The ability of FXII to bind to negatively charged surfaces and activate coagulation forms the basis of the aPTT test, in which artificial materials act as a surface for contact activation. This test is used to measure the contact activation pathway (intrinsic pathway) and the common pathway of clotting. [7]
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 .