703802001: Acquired angioedema due to C1 inhibitor autoantibody (disorder)

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

\Oedema\Disorder characterized by edema\Angioedema and/or urticaria (disorder)\...

\Autoimmune urticaria and/or angioedema (disorder)\Autoimmune angioedema (disorder)\Acquired angio-oedema due to the presence of neutralising antibodies against C1 inhibitor.

\Angioedema\Angioedema due to disorder of kinin metabolism\A rare non-histaminic angioedema characterised by potentially life-threatening episodes of oedema of subcutaneous and/or mucosal tissues without urticaria, caused by excessive consumption of C1 esterase inhibitor (C1-INH) in the context of lymphoproliferative or autoimmune diseases. Patients typically present in the fourth decade of life or later and without a family history of angioedema. Clinical manifestation includes nonpitting oedema of the skin predominantly involving the face, but also the limbs or genitals, as well as abdominal pain due to involvement of the gastrointestinal mucosa and severe oedema of the upper airway and oral mucosa. Laboratory examination shows low C1-INH activity and low C3, C4, and C1q levels. Autoantibodies to C1-INH are frequently detectable.\Acquired angio-oedema due to the presence of neutralising antibodies against C1 inhibitor.
\Angioedema\Autoimmune angioedema (disorder)\Acquired angio-oedema due to the presence of neutralising antibodies against C1 inhibitor.

\Disease\Disorder of immune function (disorder)\Autoimmune disease\Autoimmune urticaria and/or angioedema (disorder)\Autoimmune angioedema (disorder)\Acquired angio-oedema due to the presence of neutralising antibodies against C1 inhibitor.
\Disease\Disorder characterized by edema\Angioedema and/or urticaria (disorder)\Autoimmune urticaria and/or angioedema (disorder)\Autoimmune angioedema (disorder)\Acquired angio-oedema due to the presence of neutralising antibodies against C1 inhibitor.
\Disease\Disorder characterized by edema\Angioedema and/or urticaria (disorder)\Angioedema\Angioedema due to disorder of kinin metabolism\A rare non-histaminic angioedema characterised by potentially life-threatening episodes of oedema of subcutaneous and/or mucosal tissues without urticaria, caused by excessive consumption of C1 esterase inhibitor (C1-INH) in the context of lymphoproliferative or autoimmune diseases. Patients typically present in the fourth decade of life or later and without a family history of angioedema. Clinical manifestation includes nonpitting oedema of the skin predominantly involving the face, but also the limbs or genitals, as well as abdominal pain due to involvement of the gastrointestinal mucosa and severe oedema of the upper airway and oral mucosa. Laboratory examination shows low C1-INH activity and low C3, C4, and C1q levels. Autoantibodies to C1-INH are frequently detectable.\Acquired angio-oedema due to the presence of neutralising antibodies against C1 inhibitor.
\Disease\Disorder characterized by edema\Angioedema and/or urticaria (disorder)\Angioedema\Autoimmune angioedema (disorder)\Acquired angio-oedema due to the presence of neutralising antibodies against C1 inhibitor.

Status: current, Not sufficiently defined by necessary conditions definition status (core metadata concept). Date: 31-Jul 2014. Module: SNOMED CT core

Descriptions:

Expanded Value Set

Outbound Relationships	Type	Target	Active	Characteristic	Refinability	Group	Values
Acquired angio-oedema due to the presence of neutralising antibodies against C1 inhibitor.	Is a	A rare non-histaminic angioedema characterised by potentially life-threatening episodes of oedema of subcutaneous and/or mucosal tissues without urticaria, caused by excessive consumption of C1 esterase inhibitor (C1-INH) in the context of lymphoproliferative or autoimmune diseases. Patients typically present in the fourth decade of life or later and without a family history of angioedema. Clinical manifestation includes nonpitting oedema of the skin predominantly involving the face, but also the limbs or genitals, as well as abdominal pain due to involvement of the gastrointestinal mucosa and severe oedema of the upper airway and oral mucosa. Laboratory examination shows low C1-INH activity and low C3, C4, and C1q levels. Autoantibodies to C1-INH are frequently detectable.	true	Inferred relationship	Some
Acquired angio-oedema due to the presence of neutralising antibodies against C1 inhibitor.	Associated morphology	A raised, erythematous papule or cutaneous plaque, usually representing short-lived dermal edema.	false	Inferred relationship	Some	2
Acquired angio-oedema due to the presence of neutralising antibodies against C1 inhibitor.	Occurrence	Adulthood	false	Inferred relationship	Some	1
Acquired angio-oedema due to the presence of neutralising antibodies against C1 inhibitor.	Associated morphology	Angioedema	true	Inferred relationship	Some	1
Acquired angio-oedema due to the presence of neutralising antibodies against C1 inhibitor.	Occurrence	Any period of life commencing after birth, but before death.	true	Inferred relationship	Some	1
Acquired angio-oedema due to the presence of neutralising antibodies against C1 inhibitor.	Is a	Autoimmune angioedema (disorder)	true	Inferred relationship	Some
Acquired angio-oedema due to the presence of neutralising antibodies against C1 inhibitor.	Pathological process (attribute)	Autoimmune process	true	Inferred relationship	Some	1
Acquired angio-oedema due to the presence of neutralising antibodies against C1 inhibitor.	Causative agent (attribute)	Kallidin I	true	Inferred relationship	Some	1

Inbound Relationships

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Characteristic

Refinability

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Id	Description	Lang	Type	Status	Case?	Module
3009860016	Acquired angio-oedema due to the presence of neutralising antibodies against C1 inhibitor.	en	Definition	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
3009869015	Acquired angioedema due to the presence of neutralizing antibodies against C1 inhibitor.	en	Definition	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
3009158015	Acquired angio-oedema due to C1 inhibitor autoantibody	en	Synonym (core metadata concept)	Active	Only initial character case insensitive (core metadata concept)	SNOMED CT core
3009454012	Acquired angioedema due to C1 inhibitor autoantibody (disorder)	en	Fully specified name	Active	Only initial character case insensitive (core metadata concept)	SNOMED CT core
3009619014	Acquired angioedema due to C1 inhibitor autoantibody	en	Synonym (core metadata concept)	Active	Only initial character case insensitive (core metadata concept)	SNOMED CT core
5956171000241112	angiœdème acquis dû à des autoanticorps anti C1	fr	Synonym (core metadata concept)	Active	Only initial character case insensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module
5956181000241114	angiœdème acquis dû à des autoanticorps inhibiteurs de C1	fr	Synonym (core metadata concept)	Active	Only initial character case insensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module