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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.
People with scleromyositis have symptoms of both systemic scleroderma and either polymyositis or dermatomyositis, and is therefore considered an overlap syndrome. Although it is a rare disease, it is one of the more common overlap syndromes seen in scleroderma patients, together with MCTD and Antisynthetase syndrome.
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 .
Balo concentric sclerosis: Central nervous system Unknown Probable Rare [57] Bickerstaff's encephalitis: Brain Anti-GQ1b Confirmed Rare [58] Chronic inflammatory demyelinating polyneuropathy: Peripheral nerves Various, including anti-MAG Confirmed 1-2 per 100,000 [59] Guillain-Barré syndrome: Peripheral nerves Various, including anti-GM1, anti ...
Some patients with MCTD may have scleroderma-like symptoms such as squared telangiectasia on the hands and face, periungual telangiectasia, sclerodactyly, and calcinosis cutis. [ 18 ] [ 10 ] Like systemic sclerosis , aberrant nailfold capillaroscopy with enormous capillaries, atypical forms, and low capillary density is a common hallmark of ...
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