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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%. [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.
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.
In immunology, autoimmunity is the system of immune responses of an organism against its own healthy cells, tissues and other normal body constituents. [1] [2] Any disease resulting from this type of immune response is termed an "autoimmune disease".
HLA-DR2 (DR15 and DR16) are associated with scleroderma and systemic sclerosis. It has been found that patients with ATA that recognize the ET4 domain of topoisomerase were frequently HLA-DR2, [ 12 ] and in another population study it was found that DR-15 is associated with ATA in systemic sclerosis. [ 13 ]
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.
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 .
Loss-of-function mutations of IL-1Ra can develop fatal systemic inflammatory response syndrome. Another example is the inability of the anti-inflammatory cytokines, such as IL-10, to signal through its receptor. That, again, can lead to systemic inflammation and severe inflammatory bowel disease (IBD). This shows that even single-cytokine ...