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Systemic Scleroderma (CUI C0036421) Suggest changes to this concept
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Terms & Properties

Concept Unique Identifier (CUI): C0036421

NCI Thesaurus Code: C72070  (see NCI Thesaurus info)

Semantic Type: Disease or Syndrome

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.

GARD Definition: 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.

- this information is from GARD/ORDR/NCATS.

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

NICHD Definition: 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.

CSP Definition: 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.

MSH Definition: 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.

Synonyms & Abbreviations: (see Synonym Details)
Diffuse Scleroderma
Diffuse Sclerosis
Scleroderma syndrome
Scleroderma, Diffuse
Scleroderma, Systemic [Disease/Finding]
Scleroderma, systemic
SCLEROSIS, SYSTEMIC
SS - Systemic sclerosis
SSc, Diffuse Sclerosis
systemic scleroderma
Systemic sclerosis (disorder)
Systemic sclerosis [scleroderma]
Systemic sclerosis, unspecified
systemic sclerosis

External Source Codes: 
NCI Thesaurus Code C72070 (see NCI Thesaurus info)

Other Properties: Property Definitions
Name Value Source
ACTIVE 1 SNOMEDCT_US
AN note specific indentations and SCLERODERMA, LOCALIZED MSH
AQL BL CF CI CL CN CO DG DH DI DT EC EH EM EN EP ET GE HI IM ME MI MO NU PA PC PP PS PX RH RT SU TH UR VE VI MSH
CASE_SIGNIFICANCE_ID 900000000000017005 SNOMEDCT_US
CASE_SIGNIFICANCE_ID 900000000000448009 SNOMEDCT_US
CHARACTERISTIC_TYPE_ID 900000000000011006 SNOMEDCT_US
Contributing_Source CTRP NCI
Contributing_Source NICHD NCI
CTV3ID Xa1jE SNOMEDCT_US
DATE_CREATED 2013-01-09T00:04:00 GARD
DATE_CREATED 2015-01-18T00:03:00 GARD
DATE_FIRST_PUBLISHED 2008-02-13 PDQ
DATE_LAST_MODIFIED 2010-02-08 PDQ
DATE_LAST_MODIFIED 2013-01-09T00:04:00 GARD
DATE_LAST_MODIFIED 2015-01-18T00:03:00 GARD
DC 1 MSH
DEFINITION_STATUS_ID 900000000000074008 SNOMEDCT_US
DID 4008-0097 CSP
DISEASE_IDENTIFIER_ID 16180 GARD
DISEASE_IDENTIFIER_ID 16181 GARD
DISEASE_IDENTIFIER_ID 29545 GARD
DX 19710101 MSH
EFFECTIVE_TIME 20020131 SNOMEDCT_US
EXCLUDES1 circumscribed scleroderma (L94.0) ICD10CM
EXCLUDES1 neonatal scleroderma (P83.8) ICD10CM
HAS_GARD_PAGE true GARD
HN 1971 MSH
IAN DEFAULT ICD10
ICA Use additional code to identify manifestations, as: {lung involvement (517.2); myopathy (359.6)} ICD9CM
ICE Acrosclerosis; CRST syndrome; Progressive systemic sclerosis; Scleroderma ICD9CM
IDENTIFIER_SOURCE Orp GARD
IDENTIFIER_TYPE_ID 1 GARD
INCLUSION_TERM scleroderma ICD10
IS_ACTIVE true GARD
IS_RARE true GARD
IS_SPANISH false GARD
LAST_REVIEW_ACTION Synonym Added GARD
LAST_REVIEW_DATE 2006-03-14T00:00:00 GARD
MDA 19990101 MSH
MESH_DEFINITION 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. NDFRT
MESH_DUI D012595 NDFRT
MESH_NAME Scleroderma, Systemic NDFRT
MESH_UI M0019543 NDFRT
MMR 20130708 MSH
MN C17.300.799 MSH
MN C17.800.784 MSH
MODIFIER_ID 900000000000451002 SNOMEDCT_US
NCI_THESAURUS_CODE C72070 PDQ
NDFRT_KIND DISEASE_KIND NDFRT
NICHD_Hierarchy_Term Systemic Sclerosis NCI
NUI N0000002701 NDFRT
ORDER_NO 17977 ICD10CM
ORDER_NO 17986 ICD10CM
ORIG_STY Disease/diagnosis PDQ
PM 1971; SCLERODERMA was heading 1963-70 MSH
PRIMARY_PATH 10078638$10074472$10010761$10028395$Systemic scleroderma$Connective tissue disorders NEC$Connective tissue disorders (excl congenital)$Musculoskeletal and connective tissue disorders MDR
PRIMARY_SOC 10028395 MDR
PT_IN_VERSION 10.0 MDR
PT_IN_VERSION 10.1 MDR
PT_IN_VERSION 11.0 MDR
PT_IN_VERSION 11.1 MDR
PT_IN_VERSION 12.0 MDR
PT_IN_VERSION 12.1 MDR
PT_IN_VERSION 13.0 MDR
PT_IN_VERSION 13.1 MDR
PT_IN_VERSION 14.0 MDR
PT_IN_VERSION 14.1 MDR
PT_IN_VERSION 15.0 MDR
PT_IN_VERSION 15.1 MDR
PT_IN_VERSION 16.0 MDR
PT_IN_VERSION 16.1 MDR
PT_IN_VERSION 17.0 MDR
PT_IN_VERSION 17.1 MDR
PT_IN_VERSION 18.0 MDR
PT_IN_VERSION 18.1 MDR
PT_IN_VERSION 19.0 MDR
PT_IN_VERSION 19.1 MDR
PT_IN_VERSION 20.0 MDR
PT_IN_VERSION 20.1 MDR
PT_IN_VERSION 8.0 MDR
PT_IN_VERSION 8.1 MDR
PT_IN_VERSION 9.0 MDR
PT_IN_VERSION 9.1 MDR
RARE_DISEASE_URL http://rarediseases.info.nih.gov/gard/12427/systemic-sclerosis/resources/1 GARD
RARE_DISEASE_URL http://rarediseases.info.nih.gov/gard/9748/systemic-scleroderma/resources/1 GARD
RXAUI 3236879 RXNORM
RXAUI 3236880 RXNORM
RXAUI 3236930 RXNORM
RXAUI 3243234 RXNORM
RXAUI 3243235 RXNORM
RXCUI 1022426 RXNORM
SNOMED_CID 89155008 NDFRT
SOS Excludes: circumscribed scleroderma (701.0) ICD9CM
SUBSET_MEMBER 447562003~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPADVICE~ALWAYS M34.9 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPTARGET~M34.9 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS M34.9 | DESCENDANTS NOT EXHAUSTIVELY MAPPED SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~M34.9 SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~Xa1jE SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
TERMUI T037315 MSH
TERMUI T037317 MSH
TERMUI T540313 MSH
TERMUI T540315 MSH
TH GHR (2014) MSH
TH NLM (1971) MSH
TH NLM (1997) MSH
TH NLM (2004) MSH
TYPE_ID 900000000000003001 SNOMEDCT_US
TYPE_ID 900000000000013009 SNOMEDCT_US

Additional Concept Data:  (none)

URL to Bookmark: https://ncim-stage.nci.nih.gov/ncimbrowser/ConceptReport.jsp?dictionary=NCI Metathesaurus&code=C0036421

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