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scleroderma p. 485
labs/findings p. 718, 722
autoantibody p. 113
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Scleroderma refers to systemic sclerosis, a rare autoimmune disorder in which normal tissue is replaced with thick, dense connective tissue. It affects the skin, blood vessels and internal organs. Now, there are two main types of scleroderma: limited cutaneous systemic scleroderma, better known as CREST syndrome; and diffuse cutaneous systemic scleroderma. The word scleroderma comes from the Greek words ‘sclero,’ meaning hard, and ‘derma,’ meaning skin, which points to the hard skin as its most visible feature.
So, normally, when there’s an infection in the body, macrophages will eat some of the invading organisms and break them down. In addition to destroying the pathogen, they also present a fragment of the pathogen, called an antigen, to naive T cells. When the naive T-cells bind to this presented antigen, they mature into T-helper cells, also called CD4+ T-cells, and they go on to help and recruit more immune cells. The T-helper cells release cytokines, which increase the activity of macrophages and attract nearby neutrophils. Macrophages also release cytokines, like TGF-β, that tell fibroblasts to repair damaged tissue after the infection by laying down collagen.
Scleroderma affects women three times more often than men. The exact pathology of scleroderma is not completely understood, but generally, it’s thought that some individuals have a genetic predisposition to scleroderma which is triggered by external triggers. Some known triggers include viral infection by cytomegalovirus and parvovirus B19; exposure to silica dust, organic solvents, and vinyl chloride; and drugs and medications like cocaine, bleomycin, and pentazocine.
Scleroderma, also known as systemic sclerosis, is a chronic systemic autoimmune disease characterized by progressive fibrosis of skin and internal organs such as the gastrointestinal tract, lungs, heart, and kidneys. The exact cause is unknown but is associated with autoimmune processes that lead to excessive collagen production in individuals with a genetic predisposition. Scleroderma can be either limited or diffuse, with symptoms varying according to the organs involved. Treatment options involve immunosuppressants and medications to relieve symptoms and slow the progression of the disease.
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