loading...| Preferred Name |
Systemic Scleroderma |
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| Synonyms |
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| Definitions |
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. |
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| ID |
http://purl.obolibrary.org/obo/NCIT_C72070 |
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| ALT_DEFINITION |
A disease that is marked by hardening and thickening of skin, connective tissue that surrounds other tissues and organs, and blood vessels. 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. |
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| code |
C72070 |
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| Contributing_Source |
Cellosaurus CTRP NICHD |
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| definition |
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. |
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| Display_Name |
Systemic Scleroderma |
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| in_subset |
http://purl.obolibrary.org/obo/NCIT_C90259 http://purl.obolibrary.org/obo/NCIT_C116977 http://purl.obolibrary.org/obo/NCIT_C118468 |
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| label |
Systemic Scleroderma |
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| Legacy Concept Name |
Systemic_Scleroderma |
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| Preferred_Name |
Systemic Scleroderma |
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| prefixIRI |
NCIT:C72070 |
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| prefLabel |
Systemic Scleroderma |
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| Semantic_Type |
Disease or Syndrome |
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| UMLS_CUI |
C0036421 |
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| subClassOf |