770687001: Vasculitis due to adenosine deaminase 2 deficiency (disorder)

• SNOMED CT Concept\Clinical finding (finding)\...
• \General finding of soft tissue\Blood vessel finding\Vascular disorder\Vasculitis\Autoimmune vasculitis (disorder)\Vasculitis due to adenosine deaminase 2 deficiency
• \General finding of soft tissue\Disorder of soft tissue\Vascular disorder\Vasculitis\Autoimmune vasculitis (disorder)\Vasculitis due to adenosine deaminase 2 deficiency
• \Cardiovascular finding\Disorder of cardiovascular system (disorder)\Cardiovascular system hereditary disorder\Vasculitis due to adenosine deaminase 2 deficiency
• \Cardiovascular finding\Disorder of cardiovascular system (disorder)\Inflammatory disorder of the cardiovascular system\Vasculitis\Autoimmune vasculitis (disorder)\Vasculitis due to adenosine deaminase 2 deficiency
• \Cardiovascular finding\Disorder of cardiovascular system (disorder)\Vascular disorder\Vasculitis\Autoimmune vasculitis (disorder)\Vasculitis due to adenosine deaminase 2 deficiency
• \Cardiovascular finding\Blood vessel finding\Vascular disorder\Vasculitis\Autoimmune vasculitis (disorder)\Vasculitis due to adenosine deaminase 2 deficiency
• \Disease\Genetic disease\Monogenic autoinflammatory syndrome (disorder)\Type I interferonopathy\Vasculitis due to adenosine deaminase 2 deficiency
• \Disease\Genetic disease\Hereditary disease\Hereditary disorder by system\Hereditary disorder of immune system\Vasculitis due to adenosine deaminase 2 deficiency
• \Disease\Genetic disease\Hereditary disease\Hereditary disorder by system\Cardiovascular system hereditary disorder\Vasculitis due to adenosine deaminase 2 deficiency
• \Disease\Genetic disease\Hereditary disease\Autosomal hereditary disorder\Autosomal recessive hereditary disorder\Vasculitis due to adenosine deaminase 2 deficiency
• \Disease\Disorder of immune function (disorder)\Autoinflammatory disease (disorder)\Monogenic autoinflammatory syndrome (disorder)\Type I interferonopathy\Vasculitis due to adenosine deaminase 2 deficiency
• \Disease\Disorder of immune function (disorder)\Hereditary disorder of immune system\Vasculitis due to adenosine deaminase 2 deficiency
• \Disease\Disorder of immune function (disorder)\Immunodeficiency disorder\Primary immune deficiency disorder\Vasculitis due to adenosine deaminase 2 deficiency
• \Disease\Disorder of immune function (disorder)\Autoimmune disease\Autoimmune vasculitis (disorder)\Vasculitis due to adenosine deaminase 2 deficiency
• \Disease\Disorder of body system\Disorder of immune structure (disorder)\Inflammatory disorder of immune system\Autoinflammatory disease (disorder)\Monogenic autoinflammatory syndrome (disorder)\Type I interferonopathy\Vasculitis due to adenosine deaminase 2 deficiency
• \Disease\Disorder of body system\Inflammation of specific body systems\Inflammatory disorder of immune system\Autoinflammatory disease (disorder)\Monogenic autoinflammatory syndrome (disorder)\Type I interferonopathy\Vasculitis due to adenosine deaminase 2 deficiency
• \Disease\Disorder of body system\Inflammation of specific body systems\Inflammatory disorder of the cardiovascular system\Vasculitis\Autoimmune vasculitis (disorder)\Vasculitis due to adenosine deaminase 2 deficiency
• \Disease\Disorder of body system\Hereditary disorder by system\Hereditary disorder of immune system\Vasculitis due to adenosine deaminase 2 deficiency
• \Disease\Disorder of body system\Hereditary disorder by system\Cardiovascular system hereditary disorder\Vasculitis due to adenosine deaminase 2 deficiency
• \Disease\Disorder of body system\Disorder of cardiovascular system (disorder)\Cardiovascular system hereditary disorder\Vasculitis due to adenosine deaminase 2 deficiency
• \Disease\Disorder of body system\Disorder of cardiovascular system (disorder)\Inflammatory disorder of the cardiovascular system\Vasculitis\Autoimmune vasculitis (disorder)\Vasculitis due to adenosine deaminase 2 deficiency
• \Disease\Disorder of body system\Disorder of cardiovascular system (disorder)\Vascular disorder\Vasculitis\Autoimmune vasculitis (disorder)\Vasculitis due to adenosine deaminase 2 deficiency
• \Disease\Inflammatory disorder\Inflammation of specific body structures or tissue\Inflammation of specific body systems\Inflammatory disorder of immune system\Autoinflammatory disease (disorder)\Monogenic autoinflammatory syndrome (disorder)\Type I interferonopathy\Vasculitis due to adenosine deaminase 2 deficiency
• \Disease\Inflammatory disorder\Inflammation of specific body structures or tissue\Inflammation of specific body systems\Inflammatory disorder of the cardiovascular system\Vasculitis\Autoimmune vasculitis (disorder)\Vasculitis due to adenosine deaminase 2 deficiency
• \Disease\Inflammatory disorder\Inflammation of specific body structures or tissue\Inflammation of specific body organs\Vasculitis\Autoimmune vasculitis (disorder)\Vasculitis due to adenosine deaminase 2 deficiency
• \Disease\Disorder of soft tissue\Vascular disorder\Vasculitis\Autoimmune vasculitis (disorder)\Vasculitis due to adenosine deaminase 2 deficiency

Status: current, Not sufficiently defined by necessary conditions definition status (core metadata concept). Date: 31-Jan 2019. Module: SNOMED CT core

Descriptions:

Id	Description	Lang	Type	Status	Case?	Module
3702118014	Vasculitis due to ADA2 deficiency	en	Synonym (core metadata concept)	Active	Only initial character case insensitive (core metadata concept)	SNOMED CT core
3702119018	Vasculitis due to adenosine deaminase 2 deficiency	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
3702120012	Vasculitis due to adenosine deaminase 2 deficiency (disorder)	en	Fully specified name	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
3702121011	Vasculitis due to ADA2 (adenosine deaminase 2) deficiency	en	Synonym (core metadata concept)	Active	Only initial character case insensitive (core metadata concept)	SNOMED CT core
3702122016	A rare genetic systemic and rheumatologic disease due to adenosine deaminase-2 inactivating mutations, combining variable features of auto inflammation, vasculitis, and a mild immunodeficiency. Variable clinical presentation includes chronic or recurrent systemic inflammation with fever, livedo reticularis or racemosa, early-onset ischemic or hemorrhagic strokes, peripheral neuropathy, abdominal pain, hepatosplenomegaly, portal hypertension, cutaneous polyarteritis nodosa, variable cytopenia and immunoglobulin deficiency.	en	Definition	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
3702123014	A rare genetic systemic and rheumatologic disease due to adenosine deaminase-2 inactivating mutations, combining variable features of auto inflammation, vasculitis, and a mild immunodeficiency. Variable clinical presentation includes chronic or recurrent systemic inflammation with fever, livedo reticularis or racemosa, early-onset ischaemic or haemorrhagic strokes, peripheral neuropathy, abdominal pain, hepatosplenomegaly, portal hypertension, cutaneous polyarteritis nodosa, variable cytopenia and immunoglobulin deficiency.	en	Definition	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core

Expanded Value Set

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