中枢神经系统血管炎

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1、Central Nervous System VasculitisJames Jinxing Wang,MD,PhDClinical Assistant ProfessorUniversity of TNMemphis,TNClassification of CNS vasculitisINFECTIOUS VASCULITIS-Spirochetal(syphilis)-Mycobacterial-Fungal-Rickettsial-Bacterial(purulent)meningitis-Viral-Other organismsNECROTIZING VASCULITIDES-Cla

2、ssic 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-Sjogren

3、s 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 vasculi

4、tides-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 VASCULITISPat

5、hology 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 micro

6、n.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.Initial ESR(n=47)nLess than 20 mm/hr 22 47%n21-40

7、 14 30%n41-60 7 15%n61-80 3 6%n 81 1 2%Diagnostic studies for CNS vasculitisTESTSENSITIVITY 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

8、,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 negativi

9、ty can be due The same pattern of inflammation to the patchy nature of the can be due to other causesdisease and small tissuesample Biller7-26-087-26-087-26-087-26-088-3-0882 y/o WF with no PMH admitted because of MS change7-26-087-26-08MELAS 3243-tRNA leu 3243GMELAS 3271-tRNA leu T3271CMELAS 3252-t

10、RNA leu A3252GMELAS 3256-tRNA leu C3256TMELAS 3291-tRNA leu T3291CMELAS 13,513-ND5 G13513ATreatment 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

11、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.Thank you!