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idiopatische hypertrofische pachymeningitis (aandoening)
idiopatische hypertrofische pachymeningitis
Immunoglobulin G4 related pachymeningitis
IgG4-related pachymeningitis
Idiopathic hypertrophic pachymeningitis
A rare brain inflammatory disease with characteristics of thickening of the dura mater of the cranium or spine with at least two histopathological features of IgG4 (immunoglobulin G4) related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific cerebrospinal fluid 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.
Id762282007
StatusDefined
Associated morphologyinflammatoire morfologie
Finding sitestructuur van dura mater
Pathological processauto-immuunproces
PALGA thesaurus simple reference set for pathology
DHD Diagnosis thesaurus reference set
ICD-10 complex map reference set
TargetG03.9
RuleTRUE
AdviceALWAYS G03.9
CorrelationSNOMED CT source code to target map code correlation not specified