loading...| Preferred Name |
Systemic sclerosis |
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| Synonyms |
Systemic scleroderma |
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| Definitions |
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) (see these terms). A third subset of SSc has also been observed, called limited Systemic Sclerosis (lSSc) or systemic sclerosis sine scleroderma (see these terms). |
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| ID |
http://www.orpha.net/ORDO/Orphanet_90291 |
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| alternative_term |
Systemic scleroderma |
|
| definition |
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) (see these terms). A third subset of SSc has also been observed, called limited Systemic Sclerosis (lSSc) or systemic sclerosis sine scleroderma (see these terms). |
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| definition_citation |
Orphanet |
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| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=90291 |
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| has_age_of_onset |
Adult |
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| has_inheritance |
Not applicable Multigenic/multifactorial |
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| hasDbXref |
MedDRA:10042953 ICD-10:M34.8 ICD-10:M34.0 ICD-11:4A42 MeSH:D012595 ICD-10:M34.1 OMIM:181750 ICD-10:M34.9 ICD-10:M34.2 UMLS:C0036421 |
|
| label |
Systemic sclerosis |
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| notation |
ORPHA:90291 |
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| part_of |
http://www.orpha.net/ORDO/Orphanet_182228 http://www.orpha.net/ORDO/Orphanet_101944 http://www.orpha.net/ORDO/Orphanet_801 http://www.orpha.net/ORDO/Orphanet_567558 |
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| prefixIRI |
ORDO:Orphanet_90291 |
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| prefLabel |
Systemic sclerosis |
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| present_in |
Australia AND has_annual_incidence_average_value : 204.0 AND has_annual_incidence_range : >1 / 1000 Taiwan, Province of China AND has_annual_incidence_average_value : 1.09 AND has_annual_incidence_range : 1-9 / 100 000 Japan AND has_point_prevalence_average_value : 4.6 AND has_point_prevalence_range : 1-9 / 100 000 Australia AND has_point_prevalence_average_value : 23.3 AND has_point_prevalence_range : 1-5 / 10 000 Taiwan, Province of China AND has_point_prevalence_average_value : 5.63 AND has_point_prevalence_range : 1-9 / 100 000 Japan AND has_annual_incidence_average_value : 72.0 AND has_annual_incidence_range : 6-9 / 10 000 Greece AND has_point_prevalence_average_value : 15.4 AND has_point_prevalence_range : 1-5 / 10 000 United States AND has_point_prevalence_average_value : 30.0 AND has_point_prevalence_range : 1-5 / 10 000 Italy AND has_annual_incidence_average_value : 1.7 AND has_annual_incidence_range : 1-9 / 100 000 Europe AND has_point_prevalence_average_value : 15.4 AND has_point_prevalence_range : 1-5 / 10 000 Greece AND has_annual_incidence_average_value : 110.0 AND has_annual_incidence_range : >1 / 1000 Spain AND has_annual_incidence_average_value : 230.0 AND has_annual_incidence_range : >1 / 1000 France AND has_point_prevalence_average_value : 17.3 AND has_point_prevalence_range : 1-5 / 10 000 Spain AND has_point_prevalence_average_value : 27.7 AND has_point_prevalence_range : 1-5 / 10 000 Canada AND has_point_prevalence_average_value : 44.3 AND has_point_prevalence_range : 1-5 / 10 000 Italy AND has_point_prevalence_average_value : 22.2 AND has_point_prevalence_range : 1-5 / 10 000 United Kingdom AND has_point_prevalence_average_value : 8.8 AND has_point_prevalence_range : 1-9 / 100 000 |
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| treeView |
http://www.orpha.net/ORDO/Orphanet_182228 http://www.orpha.net/ORDO/Orphanet_101944 http://www.orpha.net/ORDO/Orphanet_801 http://www.orpha.net/ORDO/Orphanet_567558 |
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| subClassOf |