Systemic Scleroderma malady
Genetic diseases, Rare diseases, Nephrological diseases, Respiratory diseases, Bone diseases, Skin diseases categories
Aliases & Descriptions for Systemic Scleroderma:
MalaCards categories: See all MalaCards categories (disease lists)
Global: Genetic diseases, Rare diseases
Anatomical: Nephrological diseases, Respiratory diseases, Bone diseases, Skin diseases
ICD10: 26 25
Rare renal diseases
Rare respiratory diseases
Rare systemic and rhumatological diseases
Rare skin diseases
Characteristics (Orphanet epidemiological data):48
Inheritance: Multigenic/multifactorial,Not applicable; Prevalence: 1-5/10000 (Europe); Age of onset: Adult; Age of death: adult
NIH Rare Diseases:42 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 systemic scleroderma usually begin with episodes of raynaud's phenomenon, which can occur weeks to years before fibrosis. this may be followed by puffy or swollen hands before the skin becomes thickened and hard. fibrosis can also affect internal organs and can lead to impairment or failure of the affected organs. the most commonly affected organs are the esophagus, heart, lungs, and kidneys.there are three types of systemic scleroderma, defined by the tissues affected in the disorder. limited scleroderma diffuse scleroderma sine scleroderma last updated: 1/30/2013
MalaCards based summary: Systemic Scleroderma, also known as systemic sclerosis, is related to connective tissue disease and pneumonia, and has symptoms including chondrocalcinosis, hyperkeratosis and edema. An important gene associated with Systemic Scleroderma is HOXD13 (homeobox D13). The drugs chloroquine and epoprostenol and the compounds phosphorus and warfarin have been mentioned in the context of this disorder. Affiliated tissues include skin, lung and heart, and related mouse phenotype homeostasis/metabolism.
Disease Ontology:8 A scleroderma that is characterized by fibrosis (or hardening) of the skin and major organs, as well as vascular alterations, and autoantibodies.
Genetics Home Reference:21 Systemic scleroderma is an autoimmune disorder that affects the skin and internal organs. Autoimmune disorders occur when the immune system malfunctions and attacks the body's own tissues and organs. The word "scleroderma" means hard skin in Greek, and the condition is characterized by the buildup of scar tissue (fibrosis) in the skin and other organs. The condition is also called systemic sclerosis because the fibrosis can affect organs other than the skin. Fibrosis is due to the excess production of a tough protein called collagen, which normally strengthens and supports connective tissues throughout the body.
Wikipedia:64 Systemic sclerosis or systemic scleroderma is an autoimmune or connective tissue disease. It is... more...
Symptoms:48 (show all 59)
HPO human phenotypes related to Systemic Scleroderma:(show all 45)
Drug clinical trials:
Inferred drug relations via UMLS61/NDF-RT40:
Data from LifeMap, the Embryonic Development and Stem Cells Database
Read about Systemic Scleroderma cell therapies at LifeMap Discovery.
Stem-cell-based therapeutic approaches for Systemic Scleroderma:
MalaCards organs/tissues related to Systemic Scleroderma:31
Skin, Lung, Heart, Kidney, Bone, Liver, T cells, Breast, Testes, Monocytes, Endothelial
Articles related to Systemic Scleroderma:(show top 50) (show all 207)
Search GEO for disease gene expression data for Systemic Scleroderma.
Compounds related to Systemic Scleroderma according to GeneCards Suite gene sharing:(show all 17)
26ICD10 via Orphanet
34MESH via Orphanet
47OMIM via Orphanet
57SNOMED-CT via Orphanet
62UMLS via Orphanet