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IgG4-related pachymeningitis

ORPHA:449427 · Clinical subtype · Subtype of disorder

HPO 表現型（共 39 項）

Very frequent (99-80%)(2)

  • Abnormal cerebrospinal fluid morphologyHP:0002921
  • Abnormal dura mater morphologyHP:0010652

Frequent (79-30%)(6)

  • Elevated circulating C-reactive protein concentrationHP:0011227
  • HeadacheHP:0002315
  • Increased circulating IgG4 concentrationHP:0032300
  • Increased CSF protein concentrationHP:0002922
  • Lower limb muscle weaknessHP:0007340
  • PainHP:0012531

Occasional (29-5%)(28)

  • Abnormal lung morphologyHP:0002088
  • Abnormal optic nerve morphologyHP:0000587
  • Abnormal spinal dura mater morphologyHP:0009744
  • Abnormal temporal bone morphologyHP:0009911
  • Abnormality of cervical plexusHP:3000035
  • Abnormality of the brachial nerve plexusHP:0045052
  • Abnormality of the cervical spineHP:0003319
  • Abnormality of the clivusHP:0010558
  • AphasiaHP:0002381
  • ConfusionHP:0001289
  • Cranial nerve paralysisHP:0006824
  • DiplopiaHP:0000651
  • DysphagiaHP:0002015
  • DyspneaHP:0002094
  • HallucinationsHP:0000738
  • Low back painHP:0003419
  • LymphadenitisHP:0002840
  • Mental deteriorationHP:0001268
  • Neck painHP:0030833
  • NephritisHP:0000123
  • PancreatitisHP:0001733
  • ParaparesisHP:0002385
  • ParotitisHP:0011850
  • SeizureHP:0001250
  • SinusitisHP:0000246
  • Somatic sensory dysfunctionHP:0003474
  • Spinal cord compressionHP:0002176
  • Visual lossHP:0000572

Very rare (<4-1%)(3)

  • Abnormal skull morphologyHP:0000929
  • Increased total eosinophil countHP:0001880
  • Reduced circulating complement concentrationHP:0004431