中枢神经系统血管炎PPT讲稿.ppt
中枢神经系统血管炎第1页，共41页，编辑于2022年，星期三第2页，共41页，编辑于2022年，星期三第3页，共41页，编辑于2022年，星期三第4页，共41页，编辑于2022年，星期三第5页，共41页，编辑于2022年，星期三第6页，共41页，编辑于2022年，星期三Classification of CNS vasculitisINFECTIOUS VASCULITIS-Spirochetal(syphilis)-Mycobacterial-Fungal-Rickettsial-Bacterial(purulent)meningitis-Viral-Other organismsNECROTIZING VASCULITIDES-Classic polyarteritis nodosa-Wegeners granulomatosis-Allergic Angitis and granulomatosis(Churg-Strauss)-Necrotizing systemic vasculitis-overlap syndrome-Lymphomatoid granulomatosisVASCULITIS ASSOCIATED WITH COLLAGEN VASCULAR DISEASES-Systemic lupus erythematosus-Rheumatoid arthritis-Scleroderma-Sjogrens syndromeGIANT CELL ARTERITIDES-Takayasus arteritis-Temporal(cranial)arteritisVASCULITIS ASSOCIATED WITH OTHER SYSTEMIC DISEASES-Behcets disease-Ulcerative colitis-Sarcoidosis-Relapsing polychondritis-Kohlmeier-Degos diseaseHYPERSENSITIVITY VASCULITIDES-Henoch-Schonlein purpura-Drug-induced vasculitides-Chemical vasculitides-Essential mixed cryoglobulinemiaMISCELLANEOUS-Vasculitis associated with neoplasia-Vasculitis associated with radiation-Cogans syndrome-Dermatomyositis-polymyositis-X-linked lymphoproliferative syndrome-Thromboangiitis obliterans-Kawasaki syndromePRIMARY CNS VASCULITIS第7页，共41页，编辑于2022年，星期三Historyn n1922 Harbitzs first report.n n1959 Gravioto and Feigins extensive autopsy descriptionsn n1970s Primary CNS angiitis,Granulomatous angiitis of the CNS,isolated CNS angiitis.n n1980s High dose steroid and Cyclophosphamide started.n nPrognosis is very poor without treatment.Mortality is almost 100%without treatment第8页，共41页，编辑于2022年，星期三Pathology of the isolated CNS vasculitisnThe essential feature is a giant cell,granulomatous inflammation of the small arteries and veins,which exhibits a nearly constant affinity for the vessels of the leptomeninges and the branches that arise from them to penetrate the cortex.nThe size is 2-300 micron.第9页，共41页，编辑于2022年，星期三Animal ModelsnIntrvanous injection of Mycoplasma ngallisepticum in turkeys produced similar n ndamage as human vasculitis.第10页，共41页，编辑于2022年，星期三第11页，共41页，编辑于2022年，星期三第12页，共41页，编辑于2022年，星期三Clinical PresentationAUTOPSYAUTOPSYBIOPSYBIOPSYSYMPTOMS ORSYMPTOMS OR CASES CASES CASESCASES SIGNS SIGNS (N=45)(N=45)(N=26)(N=26)_Altered mentationAltered mentation 393976%76%111142%42%HeadacheHeadache 2929646413135050HemiparesisHemiparesis 20204444111142 42 Stupor or comaStupor or coma 19194242 4 41515DysphasiaDysphasia 1414313111114242SeizuresSeizures 13132929 8 83131“Eye signs”“Eye signs”15153333 3 31212ParaparesisParaparesis11112424 4 41515AtaxiaAtaxia 8 81818 9 93535FeverFever 8 81818 3 31212PapilledemaPapilledema 9 92020 1 1 4 4Weight LossWeight Loss 8 81818 0 0 0 0 第13页，共41页，编辑于2022年，星期三Diagnostic Testing-1nLabs:CBCtAnti-BM abs,ANCA,ACE,SSA,SSB,FANA,RF,Cryoglobulin,etctESR,C-reactive proteintNormal ESR for man is age/2,for women is(age+10)/2.tCorrected ESR=ESR (standard Hct-actual Hct)x 1.75.Standard Hct is 45 for man,42 for women.第14页，共41页，编辑于2022年，星期三Initial ESR(n=47)nLess than 20 mm/hr 22 47%n21-40 14 30%n41-60 7 15%n61-80 3 6%n 81 1 2%第15页，共41页，编辑于2022年，星期三Diagnostic studies for CNS vasculitisTEST SENSITIVITY ESTIMATED SPECIFICITYCT33-50%Data not available(even lbiopsy-proven cases)no pathognomonic findingsMRI50-100%Data not available(It approaches 100%in histo-no patholognomonic findings logically confirmed cases,and is lowest in those diagnosedonly by angiography)ANGI-30-100%22%ography(It is less than 40%in Assessed in only one study but histologically confirmed may be higher if vasculitis is cases,and 100%in reports secondary to other causes arenot supported by histology)excluded)BIOPSY75%80%(The negativity can be due The same pattern of inflammation to the patchy nature of the can be due to other causesdisease and small tissuesample 第16页，共41页，编辑于2022年，星期三 BillerBiller“VASCULITIS”Look-Alikes on Cerebral AngiographyVASCULITIS”Look-Alikes on Cerebral Angiography_CONDITIONCONDITIONAUTHOR(S)AUTHOR(S)_Neoplastic angioendotheliosisNeoplastic angioendotheliosis Witt et al.Witt et al.Spasm after subarachnoid hemnorrhageSpasm after subarachnoid hemnorrhageFerris and LevineFerris and LevineAtherosclerosisAtherosclerosisFerris and LevineFerris and LevineOral contraceptive useOral contraceptive use Irey et al.Irey et al.HypertensionHypertension with pheochromocytoma with pheochromocytomaALrmstrong and Hayes,ALrmstrong and Hayes,Postpartum Postpartum Garner et al.Garner et al.Eclampsia Eclampsia Trommer,Homer,and Trommer,Homer,andMigraineMigraineSchon and HarrisonSchon and HarrisonPostcoital headache (?)Postcoital headache (?)Kapoor,Kendall,Kapoor,Kendall,TraumaTraumaSuwanwela and Suwanwela and Surgical manipulation of intracranial arteriesSurgical manipulation of intracranial arteriesKhodadadKhodadad“Reversible cerebral segmental vasoconstriction”“Reversible cerebral segmental vasoconstriction”Call et al.Call et al.Sumatriptan and isometheptaneSumatriptan and isometheptane第17页，共41页，编辑于2022年，星期三Diagnostic Testing-3n nCSF:Very sensitive,but not specificn n90%abnormal第18页，共41页，编辑于2022年，星期三Differential Diagnosisn n1.CVA1.CVAn n2.MS2.MSn n3.Infection3.Infectionn n4.Tumor4.Tumorn n5.Specific/systemic vasculitis5.Specific/systemic vasculitisn n6.Toxic6.Toxicn n7.Leukodystrophy7.Leukodystrophyn n8.MERRF,MELAS8.MERRF,MELASn n9.Hypertensive encephalopathy9.Hypertensive encephalopathy第19页，共41页，编辑于2022年，星期三第20页，共41页，编辑于2022年，星期三7-26-087-26-087-26-087-26-088-3-0882 y/o WF with no PMH admitted because of MS change7-26-087-26-08第21页，共41页，编辑于2022年，星期三第22页，共41页，编辑于2022年，星期三第23页，共41页，编辑于2022年，星期三第24页，共41页，编辑于2022年，星期三第25页，共41页，编辑于2022年，星期三第26页，共41页，编辑于2022年，星期三第27页，共41页，编辑于2022年，星期三第28页，共41页，编辑于2022年，星期三第29页，共41页，编辑于2022年，星期三第30页，共41页，编辑于2022年，星期三第31页，共41页，编辑于2022年，星期三第32页，共41页，编辑于2022年，星期三第33页，共41页，编辑于2022年，星期三第34页，共41页，编辑于2022年，星期三第35页，共41页，编辑于2022年，星期三第36页，共41页，编辑于2022年，星期三MELAS DNA testingMELAS DNA testingMELAS 3243-tRNA leu 3243GMELAS 3271-tRNA leu T3271CMELAS 3252-tRNA leu A3252GMELAS 3256-tRNA leu C3256TMELAS 3291-tRNA leu T3291CMELAS 13,513-ND5 G13513A第37页，共41页，编辑于2022年，星期三第38页，共41页，编辑于2022年，星期三Treatment for CNS vasculitis CYTOXIC AGENT CORTICOSTEROIDS_Induction Cyclophosphamide 2mg/kd daily Prednisolone 1mg/kg daily therpay by mouth(max 150mg);lower (max 80mg);Reduce weekly to 4 6 mo dose by 25mg if 60 years WBC 10mg/day by 6 months must be 4.0 x 10/1Maintenance Azathioprine 2mg/kg daily Prednisolone 5-10mg/day therapy6 24 mo MTXEscalation Acute severe disease with creatinine 500 umol/1 or pulmonary therapy hemorrhage;Consider 7 10 plasma exchange treatment over 14 days such that 60 ml/kg of plasma is exchanged for 4.5%or 5%human albumin solution or consider three pulses of methylprednisolone,15 mg/kg daily for 3 days.These patients(if under 60 years)may also require 2.5mg/kg daily of cyclophosphamide.第39页，共41页，编辑于2022年，星期三Thank you!第40页，共41页，编辑于2022年，星期三第41页，共41页，编辑于2022年，星期三
