762282007: Immunoglobulin G4 related pachymeningitis (disorder)

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

\Central nervous system finding\Finding of meninges\Disorder of meninges\Meningitis\Pachymeningitis\A rare, brain inflammatory disease characterized by thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings and might be without systemic involvement or serum IgG4 elevation. Clinical manifestations are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness.
\Central nervous system finding\Disorder of the central nervous system (disorder)\Inflammatory disease of the central nervous system\Meningitis\Pachymeningitis\A rare, brain inflammatory disease characterized by thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings and might be without systemic involvement or serum IgG4 elevation. Clinical manifestations are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness.
\Central nervous system finding\Disorder of the central nervous system (disorder)\Disorder of meninges\Meningitis\Pachymeningitis\A rare, brain inflammatory disease characterized by thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings and might be without systemic involvement or serum IgG4 elevation. Clinical manifestations are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness.

\Disease\Disorder of immune function (disorder)\Autoimmune disease\A clinical disease characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells.\A rare, brain inflammatory disease characterized by thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings and might be without systemic involvement or serum IgG4 elevation. Clinical manifestations are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness.
\Disease\Disorder of body system\Inflammation of specific body systems\Inflammatory disease of the central nervous system\Meningitis\Pachymeningitis\A rare, brain inflammatory disease characterized by thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings and might be without systemic involvement or serum IgG4 elevation. Clinical manifestations are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness.
\Disease\Disorder of body system\Disorder of nervous system (disorder)\Disorder of the central nervous system (disorder)\Inflammatory disease of the central nervous system\Meningitis\Pachymeningitis\A rare, brain inflammatory disease characterized by thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings and might be without systemic involvement or serum IgG4 elevation. Clinical manifestations are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness.
\Disease\Disorder of body system\Disorder of nervous system (disorder)\Disorder of the central nervous system (disorder)\Disorder of meninges\Meningitis\Pachymeningitis\A rare, brain inflammatory disease characterized by thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings and might be without systemic involvement or serum IgG4 elevation. Clinical manifestations are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness.
\Disease\Inflammatory disorder\Inflammation of specific body structures or tissue\Inflammation of specific body systems\Inflammatory disease of the central nervous system\Meningitis\Pachymeningitis\A rare, brain inflammatory disease characterized by thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings and might be without systemic involvement or serum IgG4 elevation. Clinical manifestations are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness.
\Disease\Inflammatory disorder\Inflammation of specific body structures or tissue\Inflammation of specific body organs\Meningitis\Pachymeningitis\A rare, brain inflammatory disease characterized by thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings and might be without systemic involvement or serum IgG4 elevation. Clinical manifestations are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness.

Status: current, Sufficiently defined by necessary conditions definition status (core metadata concept). Date: 31-Jan 2022. Module: SNOMED CT core

Descriptions:

Id Description Lang Type Status Case? Module

5403917017 A rare, brain inflammatory disease characterized by thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings and might be without systemic involvement or serum IgG4 elevation. Clinical manifestations are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness. en Definition Active Entire term case sensitive (core metadata concept) SNOMED CT core

5403918010 A rare, brain inflammatory disease characterised by thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings and might be without systemic involvement or serum IgG4 elevation. Clinical manifestations are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness. en Definition Active Entire term case sensitive (core metadata concept) SNOMED CT core

3636309019 IgG4-related pachymeningitis en Synonym (core metadata concept) Active Entire term case sensitive (core metadata concept) SNOMED CT core

3636310012 Idiopathic hypertrophic pachymeningitis en Synonym (core metadata concept) Active Entire term case insensitive (core metadata concept) SNOMED CT core

4674044013 Immunoglobulin G4 related pachymeningitis en Synonym (core metadata concept) Active Only initial character case insensitive (core metadata concept) SNOMED CT core

4674046010 Immunoglobulin G4 related pachymeningitis (disorder) en Fully specified name Active Only initial character case insensitive (core metadata concept) SNOMED CT core

6056831000241110 pachyméningite liée aux immunoglobulines G4 fr Synonym (core metadata concept) Active Entire term case sensitive (core metadata concept) SNOMED CT Switzerland NRC maintained Module

6056841000241117 pachyméningite associée aux IgG4 (immunoglobulines G4) fr Synonym (core metadata concept) Active Entire term case sensitive (core metadata concept) SNOMED CT Switzerland NRC maintained Module

6056851000241119 pachyméningite hypertrophique idiopathique fr Synonym (core metadata concept) Active Entire term case insensitive (core metadata concept) SNOMED CT Switzerland NRC maintained Module

3450311001000119 Pachymeningitis, IgG4-assoziierte de Synonym (core metadata concept) Active Entire term case sensitive (core metadata concept) SNOMED CT Switzerland NRC maintained Module

Expanded Value Set

Outbound Relationships	Type	Target	Active	Characteristic	Refinability	Group	Values
A rare, brain inflammatory disease characterized by thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings and might be without systemic involvement or serum IgG4 elevation. Clinical manifestations are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness.	Is a	Pachymeningitis	true	Inferred relationship	Some
A rare, brain inflammatory disease characterized by thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings and might be without systemic involvement or serum IgG4 elevation. Clinical manifestations are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness.	Is a	Idiopathic disease	false	Inferred relationship	Some
A rare, brain inflammatory disease characterized by thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings and might be without systemic involvement or serum IgG4 elevation. Clinical manifestations are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness.	Associated morphology	inflammation	false	Inferred relationship	Some	1
A rare, brain inflammatory disease characterized by thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings and might be without systemic involvement or serum IgG4 elevation. Clinical manifestations are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness.	Finding site	Dura mater structure (body structure)	true	Inferred relationship	Some	1
A rare, brain inflammatory disease characterized by thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings and might be without systemic involvement or serum IgG4 elevation. Clinical manifestations are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness.	Associated morphology	Inflammatory morphology (morphologic abnormality)	true	Inferred relationship	Some	1
A rare, brain inflammatory disease characterized by thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings and might be without systemic involvement or serum IgG4 elevation. Clinical manifestations are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness.	Is a	A clinical disease characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells.	true	Inferred relationship	Some
A rare, brain inflammatory disease characterized by thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings and might be without systemic involvement or serum IgG4 elevation. Clinical manifestations are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness.	Pathological process (attribute)	Autoimmune process	true	Inferred relationship	Some	1

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Component annotation with string value reference set (foundation metadata concept)

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Id	Description	Lang	Type	Status	Case?	Module
5403917017	A rare, brain inflammatory disease characterized by thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings and might be without systemic involvement or serum IgG4 elevation. Clinical manifestations are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness.	en	Definition	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
5403918010	A rare, brain inflammatory disease characterised by thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings and might be without systemic involvement or serum IgG4 elevation. Clinical manifestations are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness.	en	Definition	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
3636309019	IgG4-related pachymeningitis	en	Synonym (core metadata concept)	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
3636310012	Idiopathic hypertrophic pachymeningitis	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
4674044013	Immunoglobulin G4 related pachymeningitis	en	Synonym (core metadata concept)	Active	Only initial character case insensitive (core metadata concept)	SNOMED CT core
4674046010	Immunoglobulin G4 related pachymeningitis (disorder)	en	Fully specified name	Active	Only initial character case insensitive (core metadata concept)	SNOMED CT core
6056831000241110	pachyméningite liée aux immunoglobulines G4	fr	Synonym (core metadata concept)	Active	Entire term case sensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module
6056841000241117	pachyméningite associée aux IgG4 (immunoglobulines G4)	fr	Synonym (core metadata concept)	Active	Entire term case sensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module
6056851000241119	pachyméningite hypertrophique idiopathique	fr	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module
3450311001000119	Pachymeningitis, IgG4-assoziierte	de	Synonym (core metadata concept)	Active	Entire term case sensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module