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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 ...
Patients with systemic sclerosis also display hypomethylation of the collagen genes COL23A1 and COL4A2. This overexpression of these collagen genes leads an overproduction of collagen characteristic to tissue fibrosis. The TGF-β signaling pathway and Wnt/β-catenin signaling pathway also play an important role in this disease.
In those with systemic disease, life expectancy can be affected, and this varies based on subtype. [3] Death is often due to lung, gastrointestinal, or heart complications. [3] About three per 100,000 people per year develop the systemic form. [3] The condition most often begins in middle age. [1] Women are more often affected than men. [1]
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".
ANAs are found in many disorders, as well as some healthy individuals. These disorders include: systemic lupus erythematosus (SLE), rheumatoid arthritis, Sjögren syndrome, scleroderma, polymyositis, dermatomyositis, primary biliary cirrhosis, drug induced lupus, autoimmune hepatitis, multiple sclerosis, discoid lupus, thyroid disease, antiphospholipid syndrome, juvenile idiopathic arthritis ...
High dietary salt intake may activate a novel molecular pathway that could trigger autoimmune diseases such as multiple sclerosis (MS), a new study finds. High salt intake may trigger mechanism ...
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 .
The constitutive activation of NF-κB, not only in CD, is in particular caused by alanine (A20) deficiency. [9] NFκB pathway is tightly regulated through multiple posttranslational mechanisms including ubiquitination. Mutations in these regulatory pathways often cause diseases connected with malfunctions of NF-κB.