中枢神经系统血管炎

1、中枢神经系统血管炎第一页，共三十九页。第二页，共三十九页。第三页，共三十九页。第四页，共三十九页。第五页，共三十九页。Classification of CNS vasculitisINFECTIOUS VASCULITIS- Spirochetal (syphilis)- Mycobacterial- Fungal- Rickettsial- Bacterial (purulent) meningitis- Viral- Other organismsNECROTIZING VASCULITIDES- Classic polyarteritis nodosa- Wegeners granul

2、omatosis- 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-

3、 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 vascul

4、itides- 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第六页，共三十九页。Histor

5、y1922 Harbitzs first report.1959 Gravioto and Feigins extensive autopsy descriptions1970s Primary CNS angiitis, Granulomatous angiitis of the CNS, isolated CNS angiitis.1980s High dose steroid and Cyclophosphamide started.Prognosis is very poor without treatment. Mortality is almost 100% without tre

6、atment第七页，共三十九页。Pathology of the isolated CNS vasculitisThe 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.The

7、size is 2-300 micron.第八页，共三十九页。Animal ModelsIntrvanous injection of Mycoplasma gallisepticum in turkeys produced similar damage as human vasculitis. 第九页，共三十九页。第十页，共三十九页。第十一页，共三十九页。Clinical PresentationAUTOPSYBIOPSYSYMPTOMS OR CASES CASES SIGNS (N = 45) (N = 26)_Altered mentation 3976%1142%Headache 2

8、9641350Hemiparesis 20441142 Stupor or coma 1942 415Dysphasia 14311142Seizures 1329 831“Eye signs”1533 312Paraparesis1124 415Ataxia 818 935Fever 818 312Papilledema 920 1 4Weight Loss 818 0 0 第十二页，共三十九页。Diagnostic Testing-1Labs: CBCAnti-BM abs, ANCA, ACE, SSA, SSB, FANA, RF, Cryoglobulin, etcESR, C-re

9、active proteinNormal ESR for man is age/2, for women is (age +10)/2.Corrected ESR = ESR (standard Hct-actual Hct) x 1.75. Standard Hct is 45 for man, 42 for women.第十三页，共三十九页。Initial ESR (n=47)Less than 20 mm/hr 22 47%21-40 14 30%41-60 7 15%61-80 3 6% 81 1 2%第十四页，共三十九页。Diagnostic studies for CNS vasc

10、ulitisTESTSENSITIVITY 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

11、% 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 patc

12、hy nature of the can be due to other causesdisease and small tissuesample 第十五页，共三十九页。 Biller“VASCULITIS” Look-Alikes on Cerebral Angiography_CONDITIONAUTHOR(S)_Neoplastic angioendotheliosis Witt et al.Spasm after subarachnoid hemnorrhageFerris and LevineAtherosclerosisFerris and LevineOral contracep

13、tive use Irey et al.Hypertension with pheochromocytomaALrmstrong and Hayes, Postpartum Garner et al. Eclampsia Trommer, Homer, andMigraineSchon and HarrisonPostcoital headache (?)Kapoor, Kendall, TraumaSuwanwela and Surgical manipulation of intracranial arteriesKhodadad“Reversible cerebral segmental

14、 vasoconstriction”Call et al.Sumatriptan and isometheptane第十六页，共三十九页。Diagnostic Testing-3CSF: Very sensitive, but not specific90% abnormal第十七页，共三十九页。Differential Diagnosis1. CVA2. MS3. Infection4. Tumor5. Specific / systemic vasculitis6. Toxic7. Leukodystrophy8. MERRF, MELAS9. Hypertensive encephalo

15、pathy第十八页，共三十九页。第十九页，共三十九页。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第二十页，共三十九页。第二十一页，共三十九页。第二十二页，共三十九页。第二十三页，共三十九页。第二十四页，共三十九页。第二十五页，共三十九页。第二十六页，共三十九页。第二十七页，共三十九页。第二十八页，共三十九页。第二十九页，共三十九页。第三十页，共三十九页。第三十一页，共三十九页。第三十二页，共三十九页。第三十三页，共三十九页。第三十四页，共三

16、十九页。第三十五页，共三十九页。MELAS 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第三十六页，共三十九页。第三十七页，共三十九页。Treatment for CNS vasculitis CYTOXIC AGENT CORTICOSTEROIDS_Induction Cyclophosphamide 2mg/

17、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 suc