IgG4相关中枢和周围神经系统病变

1.IgG4-RDwasfirstrecognisedinJapanasadiseaseaffectingthepancreas.

2.Themeanageatdiagnosisis55–59years,andthemale-to-femaleratiorangesfrom1:0?64to1:0?77.

3.Themostabundantcellsatsitesoftissueinvolvement,however,areCD4+Tcells.

4.TwomajorinteractionsbetweencellsoftheB-lymphocytelineageandCD4+TlymphocytesdrivethepathophysiologyofIgG4-RD.

5.CurrentthinkingaboutthepathophysiologyofIgG4-RDholdsthattheCD4+Tcellsorchestratingthediseasearesustainedbycontinuousantigen-presentationbyBcellsandplasmablasts.

6.Ironically,IgG4mighthaveonlyacounter-regulatorymechanismindiseasepathophysiology.Twoclinicalpresentationsare







































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