中枢神经系统血管炎课件

CentralNervousSystemVasculitisJamesJinxingWang,MD,PhDClinicalAssistantProfessorUniversityofTNMemphis,TNjjxwang@CentralNervousSystemVasculi中枢神经系统血管炎课件中枢神经系统血管炎课件中枢神经系统血管炎课件中枢神经系统血管炎课件中枢神经系统血管炎课件ClassificationofCNSvasculitisINFECTIOUSVASCULITIS--Spirochetal(syphilis)--Mycobacterial--Fungal--Rickettsial--Bacterial(purulent)meningitis--Viral--OtherorganismsNECROTIZINGVASCULITIDES--Classicpolyarteritisnodosa--Wegener’sgranulomatosis--AllergicAngitisandgranulomatosis(Churg-Strauss)--Necrotizingsystemicvasculitis-overlapsyndrome--LymphomatoidgranulomatosisVASCULITISASSOCIATEDWITHCOLLAGENVASCULARDISEASES--Systemiclupuserythematosus--Rheumatoidarthritis--Scleroderma--Sjogren’ssyndromeGIANTCELLARTERITIDES--Takayasu’sarteritis--Temporal(cranial)arteritisVASCULITISASSOCIATEDWITHOTHERSYSTEMICDISEASES--Behcet’sdisease--Ulcerativecolitis--Sarcoidosis--Relapsingpolychondritis--Kohlmeier-DegosdiseaseHYPERSENSITIVITYVASCULITIDES--Henoch-Schonleinpurpura--Drug-inducedvasculitides--Chemicalvasculitides--EssentialmixedcryoglobulinemiaMISCELLANEOUS--Vasculitisassociatedwithneoplasia--Vasculitisassociatedwithradiation--Cogan’ssyndrome--Dermatomyositis-polymyositis--X-linkedlymphoproliferativesyndrome--Thromboangiitisobliterans--KawasakisyndromePRIMARYCNSVASCULITISClassificationofCNSvasculiHistory1922Harbitz’sfirstreport.1959GraviotoandFeigin’sextensiveautopsydescriptions1970sPrimaryCNSangiitis,GranulomatousangiitisoftheCNS,isolatedCNSangiitis.1980sHighdosesteroidandCyclophosphamidestarted.Prognosisisverypoorwithouttreatment.Mortalityisalmost100%withouttreatmentHistory1922Harbitz’sfirstrPathologyoftheisolatedCNSvasculitisTheessentialfeatureisagiantcell,granulomatousinflammationofthesmallarteriesandveins,whichexhibitsanearlyconstantaffinityforthevesselsoftheleptomeningesandthebranchesthatarisefromthemtopenetratethecortex.Thesizeis2-300micron.PathologyoftheisolatedCNSAnimalModelsIntrvanousinjectionofMycoplasmagallisepticuminturkeysproducedsimilardamageashumanvasculitis.

AnimalModelsIntrvanousinject中枢神经系统血管炎课件中枢神经系统血管炎课件ClinicalPresentation

AUTOPSY BIOPSYSYMPTOMSOR CASES CASESSIGNS (N=45) (N=26)_____________________________________________________________________

Alteredmentation 39 76% 11 42%Headache 29 64 13 50Hemiparesis 20 44 11 42Stupororcoma 19 42 4 15Dysphasia 14 31 11 42Seizures 13 29 8 31“Eyesigns” 15 33 3 12Paraparesis 11 24 4 15Ataxia 8 18 9 35Fever 8 18` 3 12Papilledema 9 20 1 4WeightLoss 8 18 0 0

ClinicalPresentation AUTOPDiagnosticTesting-1Labs:CBCAnti-BMabs,ANCA,ACE,SSA,SSB,FANA,RF,Cryoglobulin,etcESR,C-reactiveproteinNormalESRformanisage/2,forwomenis(age+10)/2.CorrectedESR=ESR–(standardHct-actualHct)x1.75.StandardHctis45forman,42forwomen.DiagnosticTesting-1Labs:CBCInitialESR(n=47)Lessthan20mm/hr2247%21-401430%41-60715%61-8036%>8112%InitialESR(n=47)Lessthan20DiagnosticstudiesforCNSvasculitisTEST SENSITIVITY ESTIMATEDSPECIFICITYCT 33-50% Datanotavailable (evenlbiopsy-provencases) nopathognomonicfindingsMRI 50-100% Datanotavailable (Itapproaches100%inhisto-nopatholognomonicfindings logicallyconfirmedcases, andislowestinthosediagnosed onlybyangiography)ANGI- 30-100% 22%ography (Itislessthan40%inAssessedinonlyonestudybut histologicallyconfirmedmaybehigherifvasculitisis cases,and100%inreportssecondarytoothercausesare notsupportedbyhistology)excluded)BIOPSY 75% 80% (Thenegativitycanbedue Thesamepatternofinflammation tothepatchynatureofthe canbeduetoothercauses diseaseandsmalltissue sample

DiagnosticstudiesforCNSvas

Biller“VASCULITIS”Look-AlikesonCerebralAngiography

____________________________________________

CONDITION AUTHOR(S)

___________________________________________________________

Neoplasticangioendotheliosis Wittetal.

Spasmaftersubarachnoidhemnorrhage FerrisandLevine

Atherosclerosis FerrisandLevine

Oralcontraceptiveuse Ireyetal.

Hypertension

withpheochromocytoma ALrmstrongandHayes,

Postpartum Garneretal.

Eclampsia Trommer,Homer,and

Migraine SchonandHarrison

Postcoitalheadache(?) Kapoor,Kendall,

Trauma Suwanwelaand

Surgicalmanipulationofintracranialarteries Khodadad

“Reversiblecerebralsegmentalvasoconstriction” Calletal.

Sumatriptanandisometheptane Biller“VASCULITIS”LoDiagnosticTesting-3CSF:Verysensitive,butnotspecific90%abnormalDiagnosticTesting-3CSF:VeryDifferentialDiagnosis1.CVA2.MS3.Infection4.Tumor5.Specific/systemicvasculitis6.Toxic7.Leukodystrophy8.MERRF,MELAS9.HypertensiveencephalopathyDifferentialDiagnosis1.CVA中枢神经系统血管炎课件7-26-087-26-087-26-087-26-088-3-0882y/oWFwithnoPMHadmittedbecauseofMSchange7-26-087-26-087-26-087-26-087-26-087-26-088-中枢神经系统血管炎课件中枢神经系统血管炎课件中枢神经系统血管炎课件中枢神经系统血管炎课件中枢神经系统血管炎课件中枢神经系统血管炎课件中枢神经系统血管炎课件中枢神经系统血管炎课件中枢神经系统血管炎课件中枢神经系统血管炎课件中枢神经系统血管炎课件中枢神经系统血管炎课件中枢神经系统血管炎课件中枢神经系统血管炎课件中枢神经系统血管炎课件MELASDNAtestingMELAS3243-tRNAleu3243G MELAS3271-tRNAleuT3271CMELAS3252-tRNAleuA3252GMELAS3256-tRNAleuC3256TMELAS3291-tRNAleuT3291CMELAS13,513-ND5G13513AMELASDNAtestingMELAS3243-tR中枢神经系统血管炎课件TreatmentforCNSvasculitis

CYTOXICAGENT CORTICOSTEROIDS_____________________________________________Induction Cyclophosphamide2mg/kddaily Prednisolone1mg/kgdailytherpay bymouth(max150mg);lower (max80mg);Reduceweeklyto4–6mo doseby25mgif>60yearsWBC 10mg/dayby6months mustbe>4.0x10/1MaintenanceAzathioprine2mg/kgdaily Prednisolone5-10mg/daytherapy 6–24mo MTXEscalation Acuteseverediseasewithcreatinine>500umol/1orpulmonarytherapyhemorrhage;Consider7–10plasmaexchangetreatmentover14 dayssuchthat60ml/kgofplasmaisexchangedfor4.5%or5%human albuminsolutionorconsiderthreepulsesofmethylprednisolone,15mg/kg dailyfor3days.Thesepatients(ifunder60years)mayalsorequire2.5mg/kg dailyofcyclophosphamide.TreatmentforCNSvasculitis Thankyou!Thankyou!中枢神经系统血管炎课件CentralNervousSystemVasculitisJamesJinxingWang,MD,PhDClinicalAssistantProfessorUniversityofTNMemphis,TNjjxwang@CentralNervousSystemVasculi中枢神经系统血管炎课件中枢神经系统血管炎课件中枢神经系统血管炎课件中枢神经系统血管炎课件中枢神经系统血管炎课件ClassificationofCNSvasculitisINFECTIOUSVASCULITIS--Spirochetal(syphilis)--Mycobacterial--Fungal--Rickettsial--Bacterial(purulent)meningitis--Viral--OtherorganismsNECROTIZINGVASCULITIDES--Classicpolyarteritisnodosa--Wegener’sgranulomatosis--AllergicAngitisandgranulomatosis(Churg-Strauss)--Necrotizingsystemicvasculitis-overlapsyndrome--LymphomatoidgranulomatosisVASCULITISASSOCIATEDWITHCOLLAGENVASCULARDISEASES--Systemiclupuserythematosus--Rheumatoidarthritis--Scleroderma--Sjogren’ssyndromeGIANTCELLARTERITIDES--Takayasu’sarteritis--Temporal(cranial)arteritisVASCULITISASSOCIATEDWITHOTHERSYSTEMICDISEASES--Behcet’sdisease--Ulcerativecolitis--Sarcoidosis--Relapsingpolychondritis--Kohlmeier-DegosdiseaseHYPERSENSITIVITYVASCULITIDES--Henoch-Schonleinpurpura--Drug-inducedvasculitides--Chemicalvasculitides--EssentialmixedcryoglobulinemiaMISCELLANEOUS--Vasculitisassociatedwithneoplasia--Vasculitisassociatedwithradiation--Cogan’ssyndrome--Dermatomyositis-polymyositis--X-linkedlymphoproliferativesyndrome--Thromboangiitisobliterans--KawasakisyndromePRIMARYCNSVASCULITISClassificationofCNSvasculiHistory1922Harbitz’sfirstreport.1959GraviotoandFeigin’sextensiveautopsydescriptions1970sPrimaryCNSangiitis,GranulomatousangiitisoftheCNS,isolatedCNSangiitis.1980sHighdosesteroidandCyclophosphamidestarted.Prognosisisverypoorwithouttreatment.Mortalityisalmost100%withouttreatmentHistory1922Harbitz’sfirstrPathologyoftheisolatedCNSvasculitisTheessentialfeatureisagiantcell,granulomatousinflammationofthesmallarteriesandveins,whichexhibitsanearlyconstantaffinityforthevesselsoftheleptomeningesandthebranchesthatarisefromthemtopenetratethecortex.Thesizeis2-300micron.PathologyoftheisolatedCNSAnimalModelsIntrvanousinjectionofMycoplasmagallisepticuminturkeysproducedsimilardamageashumanvasculitis.

AnimalModelsIntrvanousinject中枢神经系统血管炎课件中枢神经系统血管炎课件ClinicalPresentation

AUTOPSY BIOPSYSYMPTOMSOR CASES CASESSIGNS (N=45) (N=26)_____________________________________________________________________

Alteredmentation 39 76% 11 42%Headache 29 64 13 50Hemiparesis 20 44 11 42Stupororcoma 19 42 4 15Dysphasia 14 31 11 42Seizures 13 29 8 31“Eyesigns” 15 33 3 12Paraparesis 11 24 4 15Ataxia 8 18 9 35Fever 8 18` 3 12Papilledema 9 20 1 4WeightLoss 8 18 0 0

ClinicalPresentation AUTOPDiagnosticTesting-1Labs:CBCAnti-BMabs,ANCA,ACE,SSA,SSB,FANA,RF,Cryoglobulin,etcESR,C-reactiveproteinNormalESRformanisage/2,forwomenis(age+10)/2.CorrectedESR=ESR–(standardHct-actualHct)x1.75.StandardHctis45forman,42forwomen.DiagnosticTesting-1Labs:CBCInitialESR(n=47)Lessthan20mm/hr2247%21-401430%41-60715%61-8036%>8112%InitialESR(n=47)Lessthan20DiagnosticstudiesforCNSvasculitisTEST SENSITIVITY ESTIMATEDSPECIFICITYCT 33-50% Datanotavailable (evenlbiopsy-provencases) nopathognomonicfindingsMRI 50-100% Datanotavailable (Itapproaches100%inhisto-nopatholognomonicfindings logicallyconfirmedcases, andislowestinthosediagnosed onlybyangiography)ANGI- 30-100% 22%ography (Itislessthan40%inAssessedinonlyonestudybut histologicallyconfirmedmaybehigherifvasculitisis cases,and100%inreportssecondarytoothercausesare notsupportedbyhistology)excluded)BIOPSY 75% 80% (Thenegativitycanbedue Thesamepatternofinflammation tothepatchynatureofthe canbeduetoothercauses diseaseandsmalltissue sample

DiagnosticstudiesforCNSvas

Biller“VASCULITIS”Look-AlikesonCerebralAngiography

____________________________________________

CONDITION AUTHOR(S)

___________________________________________________________

Neoplasticangioendotheliosis Wittetal.

Spasmaftersubarachnoidhemnorrhage FerrisandLevine

Atherosclerosis FerrisandLevine

Oralcontraceptiveuse Ireyetal.

Hypertension

withpheochromocytoma ALrmstrongandHayes,

Postpartum Garneretal.

Eclampsia Trommer,Homer,and

Migraine SchonandHarrison

Postcoitalheadache(?) Kapoor,Kendall,

Trauma Suwanwelaand

Surgicalmanipulationofintracranialarteries Khodadad

“Reversiblecerebralsegmentalvasoconstriction” Calletal.

Sumatriptanandisometheptane Biller“VASCULITIS”LoDiagnosticTesting-3CSF:Verysensitive,butnotspecific90%abnormalDiagnosticTesting-3CSF:VeryDifferentialDiagnosis1.CVA2.MS3.Infection4.Tumor5.Specific/systemicvasculitis6.Toxic7.Leukodystrophy8.MERRF,MELAS9.HypertensiveencephalopathyDifferentialDiagnosis1.CVA中枢神经系统血管炎课件7-26-087-26-087-26-087-26-088-3-0882y/oWFwithnoPMHadmittedbecauseofMSchan