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1、Central Nervous System Vasculitis,James Jinxing Wang, MD, PhD Clinical Assistant Professor University of TN Memphis, TN ,Classification of CNS vasculitis,INFECTIOUS VASCULITIS - Spirochetal (syphilis) - Mycobacterial - Fungal - Rickettsial - Bacterial (purulent) meningitis - Viral - Other organisms
2、NECROTIZING VASCULITIDES - Classic polyarteritis nodosa - Wegeners granulomatosis - Allergic Angitis and granulomatosis (Churg-Strauss) - Necrotizing systemic vasculitis-overlap syndrome - Lymphomatoid granulomatosis VASCULITIS ASSOCIATED WITH COLLAGEN VASCULAR DISEASES - Systemic lupus erythematosu
3、s - Rheumatoid arthritis - Scleroderma - Sjogrens syndrome GIANT CELL ARTERITIDES - Takayasus arteritis - Temporal (cranial) arteritis,VASCULITIS ASSOCIATED WITH OTHER SYSTEMIC DISEASES - Behcets disease - Ulcerative colitis - Sarcoidosis - Relapsing polychondritis - Kohlmeier-Degos disease HYPERSEN
4、SITIVITY VASCULITIDES - Henoch-Schonlein purpura - Drug-induced vasculitides - Chemical vasculitides - Essential mixed cryoglobulinemia MISCELLANEOUS - Vasculitis associated with neoplasia - Vasculitis associated with radiation - Cogans syndrome - Dermatomyositis-polymyositis - X-linked lymphoprolif
5、erative syndrome - Thromboangiitis obliterans - Kawasaki syndrome PRIMARY CNS VASCULITIS,History,1922 Harbitzs first report. 1959 Gravioto and Feigins extensive autopsy descriptions 1970s Primary CNS angiitis, Granulomatous angiitis of the CNS, isolated CNS angiitis. 1980s High dose steroid and Cycl
6、ophosphamide started. Prognosis is very poor without treatment. Mortality is almost 100% without treatment,Pathology of the isolated CNS vasculitis,The essential feature is a giant cell, granulomatous inflammation of the small arteries and veins, which exhibits a nearly constant affinity for the ves
7、sels of the leptomeninges and the branches that arise from them to penetrate the cortex. The size is 2-300 micron.,Animal Models,Intrvanous injection of Mycoplasma gallisepticum in turkeys produced similar damage as human vasculitis.,Clinical Presentation,AUTOPSY BIOPSY SYMPTOMS OR CASES CASES SIGNS
8、 (N = 45) (N = 26) _ Altered mentation 39 76% 11 42% Headache 29 64 13 50 Hemiparesis 20 44 11 42 Stupor or coma 19 42 4 15 Dysphasia 14 31 11 42 Seizures 13 29 8 31 “Eye signs” 15 33 3 12 Paraparesis 11 24 4 15 Ataxia 8 18 9 35 Fever 8 18 3 12 Papilledema 9 20 1 4 Weight Loss 8 18 0 0,Diagnostic Te
9、sting-1,Labs: CBC Anti-BM abs, ANCA, ACE, SSA, SSB, FANA, RF, Cryoglobulin, etc ESR, C-reactive protein Normal 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.,Initial ESR (n=47),Less than 20 mm/hr 22 47%
10、21-40 14 30% 41-60 7 15% 61-80 3 6% 81 1 2%,Diagnostic studies for CNS vasculitis,TEST SENSITIVITY ESTIMATED SPECIFICITY CT 33-50% Data not available (even lbiopsy-proven cases) no pathognomonic findings MRI 50-100% Data not available (It approaches 100% in histo- no patholognomonic findings logical
11、ly confirmed cases, and is lowest in those diagnosed only 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 are not supported by histology) excl
12、uded) BIOPSY 75% 80% (The negativity can be due The same pattern of inflammation to the patchy nature of the can be due to other causes disease and small tissue sample,Biller,“VASCULITIS” Look-Alikes on Cerebral Angiography _ CONDITION AUTHOR(S) _ Neoplastic angioendotheliosis Witt et al. Spasm afte
13、r subarachnoid hemnorrhage Ferris and Levine Atherosclerosis Ferris and Levine Oral contraceptive use Irey et al. Hypertension with pheochromocytoma ALrmstrong and Hayes, Postpartum Garner et al. Eclampsia Trommer, Homer, and Migraine Schon and Harrison Postcoital headache (?) Kapoor, Kendall, Traum
14、a Suwanwela and Surgical manipulation of intracranial arteries Khodadad “Reversible cerebral segmental vasoconstriction” Call et al. Sumatriptan and isometheptane,Diagnostic Testing-3,CSF: Very sensitive, but not specific 90% abnormal,Differential Diagnosis,1. CVA 2. MS 3. Infection 4. Tumor 5. Spec
15、ific / systemic vasculitis 6. Toxic 7. Leukodystrophy 8. MERRF, MELAS 9. Hypertensive encephalopathy,7-26-08,7-26-08,7-26-08,7-26-08,8-3-08,82 y/o WF with no PMH admitted because of MS change,7-26-08,7-26-08,MELAS DNA testing,MELAS 3243-tRNA leu 3243G MELAS 3271-tRNA leu T3271C MELAS 3252-tRNA leu A
16、3252G MELAS 3256-tRNA leu C3256T MELAS 3291-tRNA leu T3291C MELAS 13,513-ND5 G13513A,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
17、60 years WBC 10mg/day by 6 months must be 4.0 x 10 /1 Maintenance Azathioprine 2mg/kg daily Prednisolone 5 - 10mg/day therapy 6 24 mo MTX Escalation 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!,