89155008: Systemic sclerosis (disorder)

SNOMED CT Concept\Clinical finding (finding)\Disease\...

\Disorder of immune function (disorder)\Autoimmune disease\Autoimmune connective tissue disorder\A rare autoimmune connective tissue disorder characterized by abnormal hardening of the skin and sometimes other organs. It is classified into two main forms: localized scleroderma and systemic sclerosis (SSc), the latter comprising three subsets; diffuse cutaneous SSc (dcSSc), limited cutaneous SSc (lcSSc) and limited SSc (lSSc). Localized scleroderma is the cutaneous form of scleroderma characterized by fibrosis of the skin causing cutaneous plaques (morphea) or strips (linear scleroderma). Systemic sclerosis (SSc) is a generalized disorder characterized by fibrosis and vascular obliteration in the skin and organs, particularly, lungs, heart, and digestive tract. The exact cause of scleroderma is unknown. The disease originates from an autoimmune reaction, which leads to localized overproduction of collagen. In some cases, the condition is associated with exposure to chemicals. Other suggested causes include genetic and infectious mechanisms.\Systemic sclerosis

\Disorder of connective tissue (disorder)\Autoimmune connective tissue disorder\A rare autoimmune connective tissue disorder characterized by abnormal hardening of the skin and sometimes other organs. It is classified into two main forms: localized scleroderma and systemic sclerosis (SSc), the latter comprising three subsets; diffuse cutaneous SSc (dcSSc), limited cutaneous SSc (lcSSc) and limited SSc (lSSc). Localized scleroderma is the cutaneous form of scleroderma characterized by fibrosis of the skin causing cutaneous plaques (morphea) or strips (linear scleroderma). Systemic sclerosis (SSc) is a generalized disorder characterized by fibrosis and vascular obliteration in the skin and organs, particularly, lungs, heart, and digestive tract. The exact cause of scleroderma is unknown. The disease originates from an autoimmune reaction, which leads to localized overproduction of collagen. In some cases, the condition is associated with exposure to chemicals. Other suggested causes include genetic and infectious mechanisms.\Systemic sclerosis

\Systemic disease\Systemic sclerosis

Status: current, Sufficiently defined by necessary conditions definition status (core metadata concept). Date: 31-Jul 2019. Module: SNOMED CT core

Descriptions:

Expanded Value Set

Outbound Relationships	Type	Target	Active	Characteristic	Refinability	Group	Values
Systemic sclerosis	Is a	Disorder of fascia	false	Inferred relationship	Some
Systemic sclerosis	Is a	Multisystem disorder S-T	false	Inferred relationship	Some
Systemic sclerosis	Is a	Disorder of integument	false	Inferred relationship	Some
Systemic sclerosis	Is a	Collagen disease	false	Inferred relationship	Some
Systemic sclerosis	Finding site	Structure of skin region	false	Inferred relationship	Some
Systemic sclerosis	Associated morphology	Sclerosis (morphologic abnormality)	false	Inferred relationship	Some	1
Systemic sclerosis	Finding site	Connective tissue	false	Inferred relationship	Some
Systemic sclerosis	Finding site	Connective tissue structure	false	Inferred relationship	Some
Systemic sclerosis	Finding site	Structure of fascia (body structure)	false	Inferred relationship	Some	1
Systemic sclerosis	Is a	Disorder of skin (disorder)	false	Inferred relationship	Some
Systemic sclerosis	Is a	trouble multisytémique	false	Inferred relationship	Some
Systemic sclerosis	Is a	sclérodermie	false	Inferred relationship	Some
Systemic sclerosis	Finding site	Structure of collagen fibers of skin	false	Inferred relationship	Some
Systemic sclerosis	Is a	Lesion of soft tissue (disorder)	false	Inferred relationship	Some
Systemic sclerosis	Has definitional manifestation	Immune system finding	false	Inferred relationship	Some
Systemic sclerosis	Pathological process (attribute)	Autoimmune process	false	Inferred relationship	Some
Systemic sclerosis	Associated morphology	Sclerosis (morphologic abnormality)	false	Inferred relationship	Some	1
Systemic sclerosis	Finding site	Structure of fascia (body structure)	false	Inferred relationship	Some	1
Systemic sclerosis	Is a	Autoimmune disease	false	Inferred relationship	Some
Systemic sclerosis	Associated morphology	Fibrosis	false	Inferred relationship	Some
Systemic sclerosis	Associated morphology	Fibrosis	true	Inferred relationship	Some	1
Systemic sclerosis	Is a	Systemic disease	true	Inferred relationship	Some
Systemic sclerosis	Finding site	Connective tissue structure	true	Inferred relationship	Some	1
Systemic sclerosis	Pathological process (attribute)	Autoimmune process	true	Inferred relationship	Some	1
Systemic sclerosis	Is a	Autoimmune connective tissue disorder	false	Inferred relationship	Some
Systemic sclerosis	Is a	A rare autoimmune connective tissue disorder characterized by abnormal hardening of the skin and sometimes other organs. It is classified into two main forms: localized scleroderma and systemic sclerosis (SSc), the latter comprising three subsets; diffuse cutaneous SSc (dcSSc), limited cutaneous SSc (lcSSc) and limited SSc (lSSc). Localized scleroderma is the cutaneous form of scleroderma characterized by fibrosis of the skin causing cutaneous plaques (morphea) or strips (linear scleroderma). Systemic sclerosis (SSc) is a generalized disorder characterized by fibrosis and vascular obliteration in the skin and organs, particularly, lungs, heart, and digestive tract. The exact cause of scleroderma is unknown. The disease originates from an autoimmune reaction, which leads to localized overproduction of collagen. In some cases, the condition is associated with exposure to chemicals. Other suggested causes include genetic and infectious mechanisms.	true	Inferred relationship	Some

Inbound Relationships	Type	Active	Source	Characteristic	Refinability	Group
Pseudoatrophoderma colli	Is a	False	Systemic sclerosis	Inferred relationship	Some
sclérodermie avec atteinte organique ou systémique	Is a	False	Systemic sclerosis	Inferred relationship	Some
sclérodermie avec atteinte multisystémique	Is a	False	Systemic sclerosis	Inferred relationship	Some
Juvenile scleroderma	Is a	False	Systemic sclerosis	Inferred relationship	Some
Scleroderma variant	Is a	False	Systemic sclerosis	Inferred relationship	Some
Systemic sclerosis with limited cutaneous involvement	Is a	False	Systemic sclerosis	Inferred relationship	Some
Limited systemic sclerosis	Is a	True	Systemic sclerosis	Inferred relationship	Some
Systemic sclerosis, diffuse (disorder)	Is a	True	Systemic sclerosis	Inferred relationship	Some
Systemic sclerosis sine scleroderma	Is a	True	Systemic sclerosis	Inferred relationship	Some
Linear scleroderma	Is a	False	Systemic sclerosis	Inferred relationship	Some
Dilated cardiomyopathy due to systemic sclerosis	Is a	False	Systemic sclerosis	Inferred relationship	Some
Pericarditis secondary to scleroderma	Is a	True	Systemic sclerosis	Inferred relationship	Some
sclérose systémique due à des médicaments et des produits chimiques	Is a	False	Systemic sclerosis	Inferred relationship	Some
Cold flexed fingers	Is a	False	Systemic sclerosis	Inferred relationship	Some
[X]Other forms of systemic sclerosis	Is a	False	Systemic sclerosis	Inferred relationship	Some
Scleroderma NOS	Is a	False	Systemic sclerosis	Inferred relationship	Some
Lung disease with systemic sclerosis	Associated finding	False	Systemic sclerosis	Inferred relationship	Some
Lung disease with systemic sclerosis	Is a	True	Systemic sclerosis	Inferred relationship	Some
Occupational scleroderma (disorder)	Is a	True	Systemic sclerosis	Inferred relationship	Some
Cutaneous complication of systemic sclerosis (disorder)	Is a	False	Systemic sclerosis	Inferred relationship	Some
Scleroderma in newborn	Is a	False	Systemic sclerosis	Inferred relationship	Some
Family history: Scleroderma	Associated finding	False	Systemic sclerosis	Inferred relationship	Some	1
Cutaneous complication of systemic sclerosis (disorder)	Associated with	True	Systemic sclerosis	Inferred relationship	Some	1
Scleroderma-associated calcinosis (disorder)	Associated with	False	Systemic sclerosis	Inferred relationship	Some	2
Scleroderma-associated hypermelanosis (disorder)	Associated with	False	Systemic sclerosis	Inferred relationship	Some	2
Scleroderma-associated telangiectasia (disorder)	Associated with	False	Systemic sclerosis	Inferred relationship	Some	2
Scleroderma-associated nailfold telangiectasia (disorder)	Associated with	False	Systemic sclerosis	Inferred relationship	Some	2
Scleroderma-associated necrotizing vasculitis (disorder)	Associated with	True	Systemic sclerosis	Inferred relationship	Some	2
Poikiloderma due to scleroderma	Associated with	False	Systemic sclerosis	Inferred relationship	Some	2
Scleroderma-associated nail dystrophy (disorder)	Associated with	True	Systemic sclerosis	Inferred relationship	Some	4
Family history: Scleroderma	Associated finding	False	Systemic sclerosis	Inferred relationship	Some	1
Progressive systemic sclerosis (disorder)	Is a	True	Systemic sclerosis	Inferred relationship	Some
Hypothyroidism due to scleroderma	Due to	False	Systemic sclerosis	Inferred relationship	Some	2
Myopathy due to scleroderma	Associated with	False	Systemic sclerosis	Inferred relationship	Some	1
Dilated cardiomyopathy due to systemic sclerosis	Due to	False	Systemic sclerosis	Inferred relationship	Some
Pericarditis secondary to scleroderma	Due to	True	Systemic sclerosis	Inferred relationship	Some	3
Family history of systemic sclerosis (situation)	Associated finding	False	Systemic sclerosis	Inferred relationship	Some	1
Family history of systemic sclerosis (situation)	Associated finding	True	Systemic sclerosis	Inferred relationship	Some	1
Dilated cardiomyopathy due to systemic sclerosis	Associated with	False	Systemic sclerosis	Inferred relationship	Some	2
Polyneuropathy due to systemic sclerosis	Due to	True	Systemic sclerosis	Inferred relationship	Some	1
Reynolds syndrome (RS) is an autoimmune disorder characterized by the association of primary biliary cirrhosis (PBC) with limited cutaneous systemic sclerosis (lcSSc).	Is a	False	Systemic sclerosis	Inferred relationship	Some
Glomerulonephritis co-occurrent and due to scleroderma (disorder)	Due to	False	Systemic sclerosis	Inferred relationship	Some
Pediatric onset systemic sclerosis (disorder)	Is a	True	Systemic sclerosis	Inferred relationship	Some
A rare secondary neonatal autoimmune disease characterized by neonatal onset of erythematous skin lesions with a linear appearance that gradually become indurated and hyperpigmented and progressively present skin atrophy. Positive serum antibodies (in particular antinuclear antibodies and/or rheumatoid factor) may be associated.	Is a	False	Systemic sclerosis	Inferred relationship	Some
Renal involvement in scleroderma	Is a	True	Systemic sclerosis	Inferred relationship	Some
Scleroderma-associated calcinosis (disorder)	Due to	True	Systemic sclerosis	Inferred relationship	Some	2
Scleroderma-associated hypermelanosis (disorder)	Due to	True	Systemic sclerosis	Inferred relationship	Some	2
Scleroderma-associated telangiectasia (disorder)	Due to	True	Systemic sclerosis	Inferred relationship	Some	2
Scleroderma-associated nailfold telangiectasia (disorder)	Due to	True	Systemic sclerosis	Inferred relationship	Some	2
Dilated cardiomyopathy due to systemic sclerosis	Due to	True	Systemic sclerosis	Inferred relationship	Some	2
Gingival disease due to systemic sclerosis	Due to	True	Systemic sclerosis	Inferred relationship	Some	2
Disorder of skeletal muscle due to systemic sclerosis (disorder)	Due to	True	Systemic sclerosis	Inferred relationship	Some	2
Hypothyroidism due to systemic sclerosis (disorder)	Due to	True	Systemic sclerosis	Inferred relationship	Some	2
Systemic sclerosis caused by chemical (disorder)	Is a	True	Systemic sclerosis	Inferred relationship	Some

This concept is not in any reference sets

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Id	Description	Lang	Type	Status	Case?	Module
147833014	Systemic sclerosis	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
201443013	Systemic scleroderma	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
508372014	Thibierge-Weissenbach syndrome	en	Synonym (core metadata concept)	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
508375011	SS - Systemic sclerosis	en	Synonym (core metadata concept)	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
831989015	Systemic sclerosis (disorder)	en	Fully specified name	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
1043941000195118	sclerosi sistemica	it	Synonym (core metadata concept)	Active	Entire term case sensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module
30031000188113	sclérodermie	fr	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module
248661000172118	sclérose systémique	fr	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module
1008431000241115	sclérodermie systémique	fr	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module
1008591000241118	sclérodermie généralisée	fr	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module
3454461001000115	Systemische Sklerodermie	de	Synonym (core metadata concept)	Active	Only initial character case insensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module