Disease or Syndrome
systemic scleroderma
sys·tem·ic scle·ro·der·ma [ si-stem-ik skleer-uh-dur-muh, skler- ]
Subclass of:
Connective Tissue Diseases;
Dermatologic disorders
Also called:
Scleroderma
Definitions related to systemic scleroderma:
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(systemic sclerosis) A heterogeneous disorder characterized by small vessel vasculopathy, autoantibodies, and fibroblast dysfunction which results in hardening and thickening of body tissue. The clinical manifestations vary with the majority of the patients having skin thickening and involvement of internal organs.NICHD Pediatric TerminologyU.S. National Cancer Institute, 2021
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(systemic sclerosis) Systemic sclerosis (SSc) is a generalized disorder of small arteries, microvessels and connective tissue, characterized by fibrosis and vascular obliteration in the skin and organs, particularly the lungs, heart, and digestive tract. There are two main subsets of SSc: diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc). A third subset of SSc has also been observed, called limited Systemic Sclerosis (lSSc) or systemic sclerosis sine scleroderma.OrphaNetINSERM, 2021
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A chronic disorder, possibly autoimmune, marked by excessive production of collagen which results in hardening and thickening of body tissues. The two types of systemic scleroderma, limited cutaneous and diffuse cutaneous are classified with focus on the extent of affected skin. A relationship exists between the extent of skin area affected and degree of internal organ/system involvement. Systemic scleroderma can manifest itself in pulmonary fibrosis, Raynaud's syndrome, digestive system telangiectasias, renal hypertension and/or pulmonary hypertension.NCI ThesaurusU.S. National Cancer Institute, 2021
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A disease that is marked by hardening and thickening of skin, connective tissue that surrounds other tissues and organs, and blood vessels.NCI Dictionary of Cancer TermsU.S. National Cancer Institute, 2021
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Systemic disorder of the connective tissue; manifested by hardening and thickening of the skin, by abnormalities involving the microvasculature and larger vessels, and by fibrotic degenerative changes in various body organs including the heart, lungs, kidneys, and gastrointestinal tract.CRISP ThesaurusNational Institutes of Health, 2006
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(scleroderma, systemic) A chronic multi-system disorder of CONNECTIVE TISSUE. It is characterized by SCLEROSIS in the SKIN, the LUNGS, the HEART, the GASTROINTESTINAL TRACT, the KIDNEYS, and the MUSCULOSKELETAL SYSTEM. Other important features include diseased small BLOOD VESSELS and AUTOANTIBODIES. The disorder is named for its most prominent feature (hard skin), and classified into subsets by the extent of skin thickening: LIMITED SCLERODERMA and DIFFUSE SCLERODERMA.NLM Medical Subject HeadingsU.S. National Library of Medicine, 2021
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Systemic sclerosis is a rare chronic disease of unknown cause characterized by diffuse fibrosis and vascular abnormalities in the skin, joints, and internal organs (especially the esophagus, lower gastrointestinal tract, lungs, heart, and kidneys). Common symptoms include Raynaud phenomenon, polyarthralgia, dysphagia, heartburn, and...Merck & Co., Inc., 2020
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Systemic sclerosis (SSc) is a systemic connective tissue disease. Characteristics of systemic sclerosis include essential vasomotor disturbances; fibrosis; subsequent atrophy of the skin (see the image below), subcutaneous tissue, muscles, and internal organs (eg, alimentary tract, lungs, heart, kidney, CNS); and immunologic disturbances...WebMD, 2019
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Systemic scleroderma is an autoimmune disorder that affects the skin and internal organs. It is characterized by the buildup of scar tissue (fibrosis) in the skin and other organs. The fibrosis is caused by the body's production of too much collagen, which normally strengthens and supports connective tissues. The signs and symptoms of...National Center for Advancing Translational Sciences
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Systemic sclerosis (SSC), also known as scleroderma, is a multisystem, autoimmune disease, characterized by functional and structural abnormalities of small blood vessels, fibrosis of skin and internal organs, and production of autoantibodies.Athenahealth, Inc., 2019
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