IgG4-related pachymeningitis
ORPHA: 449427
Overview
human disease
Available Treatments (0)
No treatments linked yet
Clinical trials or compassionate use may be available — consult a specialist.
Clinical Presentation
Signs and symptoms associated with IgG4-related pachymeningitis, sourced from HPO and Orphanet clinical annotations.
Abnormal cerebrospinal fluid morphologyAbnormal dura mater morphologyHeadacheIncreased CSF protein concentrationLower limb muscle weaknessElevated circulating C-reactive protein concentrationPainIncreased circulating IgG4 levelNephritisSinusitisVisual lossAbnormal optic nerve morphologyDiplopiaHallucinationsSeizureMental deteriorationConfusionPancreatitisDysphagiaAbnormal lung morphologyDyspneaSpinal cord compressionAphasiaParaparesisLymphadenitisAbnormality of the cervical spineLow back painSomatic sensory dysfunctionCranial nerve paralysisAbnormal spinal dura mater morphologyAbnormal temporal bone morphologyAbnormality of the clivusParotitisNeck painAbnormality of the brachial nerve plexusAbnormality of cervical plexusAbnormal skull morphologyIncreased total eosinophil countReduced circulating complement concentration
Classification & Codes
Orphanet Code
ORPHA:449427IgG4-related pachymeningitis
| Orphanet | ORPHA:449427 |
| Treatments | 0 drug(s) |
| Symptoms on record | 39 signs |
| Status | published |
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO