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Scleroderma
Scleroderma
or Systemic Sclerosis is a chronic multisystem disorder which is
manifested by thickening of the skin caused by an accumulation of connective
tissue and by involvement in visceral organs including the gastrointestinal
tract, lungs, heart, and kidneys.
This disease involves the immune system, the vascular
endothelial cells, and activation of fibroblasts which cause excessive
collagen production. This excessive connective tissue leads to injury
of the vascular lining, decreased circulation and ischemia. There is evidence
that cell mediated immunity attacks the vascular endothelium leading to
fibrosis and scarring.
In the lower 2/3 of the esophagus is found increased
collagen and fibrosis scarring. There is atrophy of the muscular lining,
which effects motility of the esophagus. Ulcers form as a result of the
superimposed peptic esophagitis and scleroderma. With chronic acid reflux,
there is a progression of the esophageal mucosa to metaplasia known as
Barrett's esophagitis, which is a premalignant state.
A case study was done at the University of Texas-Health
Science Center in San Antonio. They studied 13 young women who had been
diagnosed with Scleroderma.
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