" /> Systemic Scleroderma - CISMeF





Preferred Label : Systemic Scleroderma;

NCIt synonyms : Scleroderma, Diffuse; Diffuse Sclerosis; Diffuse Scleroderma; Scleroderma, Systemic;

NCIt related terms : SSc, Diffuse Sclerosis; Systemic Sclerosis; Systemic sclerosis, unspecified;

NCIt 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.;

Alternative definition : NCI-GLOSS: A disease that is marked by hardening and thickening of skin, connective tissue that surrounds other tissues and organs, and blood vessels.; NICHD: 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.;

Codes from synonyms : CDR0000613766; CDR0000613765; M34.9;

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29/04/2024


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