原發(fā)性中樞神經(jīng)系統(tǒng)血管炎培訓(xùn)課件

1、原發(fā)性中樞神經(jīng)系統(tǒng)血管炎原發(fā)性中樞神經(jīng)系統(tǒng)血管炎原發(fā)性中樞神經(jīng)系統(tǒng)血管炎培訓(xùn)課件Pathology主要侵犯軟腦膜和腦實(shí)質(zhì)的中小血管的血管炎性病變，動(dòng)脈為主，靜脈少見(jiàn)，大血管少見(jiàn)。病理分型1、肉芽腫性血管炎 granulomatous angiitis 2、壞死性血管炎 necrotizing vasculitis 3、淋巴細(xì)胞浸潤(rùn)性血管炎 lymphocytic vasculitis 最終導(dǎo)致血管阻塞，相應(yīng)供血區(qū)域缺血壞死原發(fā)性中樞神經(jīng)系統(tǒng)血管炎3Pathology主要侵犯軟腦膜和腦實(shí)質(zhì)的中小血管的血管炎性臨床分型：GACNS, BACNS, Atypical PACNS GACNS ：慢性或

2、亞急性（常6m,至少3m），陣發(fā)性頭痛，精神智能改變，可伴有各種中樞神經(jīng)系統(tǒng)損害表現(xiàn)。BACNS：女性多見(jiàn)，急性起病，頭痛表現(xiàn)突出。血管造影有血管狹窄和近端血管擴(kuò)張，CSF正常，預(yù)后良好。Atypical PACNS：PACNS表現(xiàn)，病理非肉芽腫性，或累及靜脈、大動(dòng)脈、脊髓動(dòng)脈等。預(yù)后各異。原發(fā)性中樞神經(jīng)系統(tǒng)血管炎4臨床分型：GACNS, BACNS, Atypical PClinical Features頭痛精神紊亂/癡呆/嗜睡 卒中樣表現(xiàn)/TIA 癲癇/行為異常 局限性運(yùn)動(dòng)和感覺(jué)異常 共濟(jì)失調(diào) 顱神經(jīng)癥狀 視物改變 錐體外系癥狀脊髓病受累表現(xiàn) 臨床表現(xiàn)缺乏特異性，取決于病變累及的部位和范圍

3、原發(fā)性中樞神經(jīng)系統(tǒng)血管炎5Clinical Features頭痛臨床表現(xiàn)缺乏特異性，取Comparison of BACNS and GACNSBACNSGACNSPrevalenceFemale-predominantMale-predominantDisease onsetAcuteChronicClinical courseMonophasicRecurrentCSF findingsNormalAbnormalAbnormal angiography100%15%Diagnostic modalityAngiographyCNS biopsyTreatmentGC and Ca-Blo

4、ckersGC and CTXOutcomesExcellentGood原發(fā)性中樞神經(jīng)系統(tǒng)血管炎6Comparison of BACNS and GACNSDiagnose1、中樞神經(jīng)系統(tǒng)血管炎（除外其他疾病）2、除外系統(tǒng)性血管炎有提示意義的檢查：CSF、CT、MRI、血管造影。除外性檢查：腫瘤篩查、感染篩查、免疫篩查等最終診斷：病理活檢原發(fā)性中樞神經(jīng)系統(tǒng)血管炎7Diagnose1、中樞神經(jīng)系統(tǒng)血管炎（除外其他疾病）有提示Mimics of Primary Angiitis of the Central Nervous SystemCTDInfectionNeoplasmMiscellaneo

5、us結(jié)節(jié)性多動(dòng)脈炎CTD相關(guān)急性肉芽腫性血管炎Wegeners 肉芽腫巨細(xì)胞動(dòng)脈炎高安動(dòng)脈炎白塞氏病SLE結(jié)節(jié)病淋巴瘤樣肉芽腫病病毒HIV/帶皰/巨細(xì)胞/單皰霍奇金或非霍奇金淋巴瘤煙霧病MSTTP動(dòng)脈粥樣硬化血栓/血栓形成抗磷脂綜合征擬交感神經(jīng)類(lèi)藥物細(xì)菌白血病真菌左房粘液瘤立克次氏體惡性血管內(nèi)皮細(xì)胞瘤其他CNS原發(fā)腫瘤/轉(zhuǎn)移瘤原發(fā)性中樞神經(jīng)系統(tǒng)血管炎8Mimics of Primary Angiitis of Nikolov NP et al. (2006) Diagnosis and treatment of vasculitis of the central nervous system

6、in a patient with systemic lupus erythematosus Nat Clin Pract Rheumatol 2: 627633 doi:10.1038/ncprheum0337Diagnostic approach to CNS vasculitis原發(fā)性中樞神經(jīng)系統(tǒng)血管炎9Nikolov NP et al. (2006) DiagnCSF: 中度淋巴細(xì)胞和蛋白升高，非感染性血管炎表現(xiàn)。ESR、CPR等炎性指標(biāo)通常正?；蜉p度升高，否則提示系統(tǒng)性血管炎MRI:敏感性77-100%，特異性差，表現(xiàn)為雙側(cè)、多灶性，廣泛分布于皮質(zhì)、深白質(zhì)、軟腦膜的梗死灶?？捎谢虿话?/p>

7、有增強(qiáng)，如果有軟腦膜血管的增強(qiáng)病灶，則是取病理的好地方。DSA:血管炎典型表現(xiàn)，中小動(dòng)脈的節(jié)段性狹窄，“串珠樣”改變。敏感性95%，其中65%有典型表現(xiàn)，特異性只有26%，必須與其他能引起血管攣縮或狹窄的疾病鑒別。Biopsy:診斷金標(biāo)準(zhǔn)，但仍有25%假陰性率。對(duì)于沒(méi)有影像學(xué)提示的，建議開(kāi)顱楔形取非優(yōu)勢(shì)半球顳葉皮質(zhì)，連帶軟腦膜。病理標(biāo)本仍應(yīng)該進(jìn)行病原學(xué)檢查。原發(fā)性中樞神經(jīng)系統(tǒng)血管炎10CSF: 中度淋巴細(xì)胞和蛋白升高，非感染性血管炎表現(xiàn)。ESRTreatment激素首選，首劑量1mg/kg/d。癥狀緩解后減量，連續(xù)2-3個(gè)月。對(duì)于典型GACNS，早期加用CTX，在癥狀緩解后，仍可持續(xù)1年，或換

8、用抗代謝藥物，如依木蘭或MTX。對(duì)于BACNS，可加用CCB，如維拉帕米240mg/Qd。癥狀重的可用激素沖擊。對(duì)于不典型PACNS，癥狀重則加用CTX。預(yù)后：早期激素治療，預(yù)后大多良好。原發(fā)性中樞神經(jīng)系統(tǒng)血管炎11Treatment激素首選，首劑量1mg/kg/d。癥狀緩解The EndThanks for your attention原發(fā)性中樞神經(jīng)系統(tǒng)血管炎12The EndThanks for your attenti原發(fā)性中樞神經(jīng)系統(tǒng)血管炎13原發(fā)性中樞神經(jīng)系統(tǒng)血管炎13A: T2 Profound signal abnormalities in the white matter ar

9、e compatible with ischemic or demyelinating disease. B: FLAIR The white matter signal abnormalities are more obvious. C: DW No frank restricted diffusion is seen, militating against acute/subacute infarction. D: Enhanced T1 striking enhancement along the perivascular regions (arrows) and slightly al

10、ong the meninges. There is no fusiform, nodular, or ring-like enhancement as would be expected with demyelinating disease. E: Enhanced coronal T1 section through the trigones of the lateral ventricles shows dramatic perivascular enhancement (arrows).原發(fā)性中樞神經(jīng)系統(tǒng)血管炎14A: T2 Profound signal abnormal原發(fā)性中樞神經(jīng)系統(tǒng)血管炎15原發(fā)性中樞神經(jīng)系統(tǒng)血管炎15原發(fā)性中樞神經(jīng)系統(tǒng)血管炎16原發(fā)性中樞神經(jīng)系統(tǒng)血管炎16Leptomeningeal arteries show severe tra