白塞病 ppt課件

1、白塞病(Behcet‘s disease),www.themegallery.com,Behcet‘s disease,www.themegallery.com,Company Logo,OVERVIEW,白塞病(BD)是一種以反復(fù)口腔潰瘍、外陰潰瘍、眼炎及皮膚損害為主要臨床特征的自身免疫性疾病。也可累及血管、神經(jīng)系統(tǒng)、消化道、關(guān)節(jié)、肺等器官病情呈反復(fù)發(fā)作和緩解的交替過程大部分患者預(yù)后良好，眼、中樞神經(jīng)系統(tǒng)及大血管受累者

2、預(yù)后不佳。,www.themegallery.com,Company Logo,Type,神經(jīng)型：有中樞或周圍神經(jīng)受累者；,血管型：有大、中型動(dòng)脈、靜脈受累者；,胃腸型：有胃腸道潰瘍、出血、穿孔等,www.themegallery.com,Company Logo,基本癥狀,1、口腔潰瘍：幾乎100％患者均有復(fù)發(fā)性、痛性口腔潰瘍(ADhthous ulceration，阿弗他潰瘍)，多數(shù)患者為首發(fā)癥狀。2、生殖器潰瘍：約75％

3、患者出現(xiàn)生殖器潰瘍，病變與口腔潰瘍基本相似，但出現(xiàn)次數(shù)少。潰瘍深大，疼痛劇，愈合慢。3、眼炎：約50％患者有眼炎，雙眼各組織均可累及，男性多于女性。 葡萄膜炎(色素膜炎)最常見。4、皮膚病變：皮損發(fā)生率高，可達(dá)80％。98％，表現(xiàn)多種多樣，有結(jié)節(jié)性紅斑、膿皰疹、丘疹、痤瘡樣皮疹等。5、血管損害：本病的基本病變?yōu)檠苎?，全身大小血管均可累及，約10％～20％患者合并大中血管炎，是致死致殘的主要原因。其他：神經(jīng)

4、系統(tǒng)損害，消化道損害，肺部損害等,www.themegallery.com,白塞病口腔潰瘍,www.themegallery.com,Company Logo,www.themegallery.com,Company Logo,www.themegallery.com,Company Logo,www.themegallery.com,Company Logo,診斷,BD病人中陽性率高于正常人群 (58.66% vs. 18.51%,

5、OR = 6.245) Pirim I, Atasoy M, Ikbal M, et al. HLA class I and class II genotyping in patients with BD: a regional study of eastern part of Turkey. Tissue Antigens. 2004;64(3):293-7 是目前診斷白塞病唯一的特異性體征； 57.9%的BD患者針

6、刺反應(yīng)陽性 男性患者的陽性率明顯高于女性 (70% vs. 41.7%，p<0.001),針刺反應(yīng)(注射部位出現(xiàn)膿皰疹）,www.themegallery.com,Company Logo,診斷標(biāo)準(zhǔn),1989年白塞病國(guó)際診斷標(biāo)準(zhǔn)(ISG),主要條件：復(fù)發(fā)性口腔潰瘍或庖疹性潰瘍，>3次/年次要條件：復(fù)發(fā)性外陰潰瘍(經(jīng)醫(yī)生確診或本人確認(rèn)有把握的外陰潰瘍或疤痕) 眼?。浩咸涯ぱ?、視網(wǎng)膜血管炎、裂隙燈下的玻璃體內(nèi)有

7、細(xì)胞出現(xiàn)。 皮膚病變：結(jié)節(jié)紅斑、假性毛囊炎、丘疹性膿皰疹等。 針刺反應(yīng)（+） 具備主要條件，加上次要條件4項(xiàng)中任何2項(xiàng),www.themegallery.com,Company Logo,EULAR關(guān)于白塞病治療的建議,1.有眼后極受累的炎性眼病的白塞病患者應(yīng)使用包括硫唑嘌呤和全身激素在內(nèi)的治療方案。2.如果白塞病患者有嚴(yán)重眼部疾?。ǘx是：在10/10尺度下視

8、力降低2行或/和視網(wǎng)膜病變包括視網(wǎng)膜血管炎或黃斑受累），建議使用環(huán)孢素A或類克聯(lián)合硫唑嘌呤和激素，也可使用α-干擾素聯(lián)合或不聯(lián)合激素治療。,www.themegallery.com,Company Logo,EULAR關(guān)于白塞病治療的建議,3. 尚無肯定證據(jù)來指導(dǎo)白塞病大血管受累的治療。對(duì)于有急性深靜脈血栓形成的白塞病患者，推薦使用免疫抑制劑如激素、硫唑嘌呤、環(huán)磷酰胺或環(huán)孢素A。有肺動(dòng)脈或外周動(dòng)脈瘤的白塞病，推薦使用環(huán)磷酰胺和激素。

9、4.相類似的是，尚無對(duì)照資料或非對(duì)照資料提示，使用抗凝療法、抗血小板或抗纖溶藥治療白塞病深靜脈血栓形成或動(dòng)脈損害后作為抗凝治療會(huì)帶來好處。,www.themegallery.com,Company Logo,EULAR關(guān)于白塞病治療的建議,5.無循證醫(yī)學(xué)證據(jù)提示白塞病胃腸道受累有有效治療方法。在進(jìn)行手術(shù)前（除急診外），應(yīng)嘗試使用藥物如柳氮磺吡啶、激素、硫唑嘌呤、腫瘤壞死因子拮抗劑及反應(yīng)停。 6. 在多數(shù)白塞病患者，關(guān)節(jié)炎能使用秋水仙

10、堿治療。7. 無對(duì)照資料指導(dǎo)白塞病中樞神經(jīng)受累治療。對(duì)于腦實(shí)質(zhì)受累，應(yīng)嘗試的藥物包括激素、α-干擾素、硫唑嘌呤、環(huán)磷酰胺、甲氨蝶呤和腫瘤壞死因子拮抗劑。對(duì)于腦硬膜竇血栓形成，推薦使用激素。8.環(huán)孢素A不用于合并中樞神經(jīng)受累的白塞病患者，除非有眼內(nèi)炎癥。,www.themegallery.com,Company Logo,EULAR關(guān)于白塞病治療的建議,9.白塞病皮膚和粘膜受累的治療方法取決于醫(yī)生和患者所認(rèn)為的嚴(yán)重程度。粘膜皮膚

11、受累的治療應(yīng)根據(jù)同時(shí)存在的其他損害情況。僅有口腔和外生殖潰瘍的一線治療是局部措施（如局部激素）。痤瘡樣損害常僅因影響美容受到關(guān)注，因此，對(duì)于尋常型痤瘡用局部措施即可。當(dāng)出現(xiàn)明顯的結(jié)節(jié)紅斑損害時(shí)，應(yīng)使用秋水仙堿。白塞病的小腿潰瘍可能有多種原因，治療應(yīng)該有計(jì)劃性，對(duì)于耐受患者，可使用硫唑嘌呤、α-干擾素和腫瘤壞死因子α拮抗劑。,www.themegallery.com,EULAR關(guān)于白塞病治療的建議,1．Any patient with B

12、D and inflammatory eye disease affecting the posterior segment should be on a treatment regime that includes azathioprine and systemic corticosteroids.2. If the patient has severe eye disease defined as > 2 lines of

13、 drop in visual acuity on a 10/10 scale and/or retinal disease (retinal vasculitis or macular involvement), it is recommended that either ciclosporine A or infliximab be used in combination with azathioprine and corticos

14、teroids; alternatively IFNa with or without corticosteroids could be used instead.3. There is no firm evidence to guide the management of major vessel disease in BD. For the management of acute deep vein thrombosis in

15、BD immunosuppressive agents such as corticosteroids, azathioprine, cyclophosphamide or ciclosporine A are recommended. For the management of pulmonary and peripheral arterial aneurysms, cyclophosphamide and corticosteroi

16、ds are recommended.,www.themegallery.com,Company Logo,EULAR關(guān)于白塞病治療的建議,4. Similarly there are no controlled data on, or evidence of benefit from uncontrolled experience with anticoagulants, antiplatelet or antifibrinolyti

17、c agents in the management of deep vein thrombosis or for the use of anticoagulation for the arterial lesions of BD.5. There is no evidence-based treatment that can be recommended for the management of gastrointestinal

18、 involvement of BD. Agents such as sulfasalazine, corticosteroids, azathioprine, TNFa antagonists and thalidomide should be tried first before surgery, except in emergencies.6. In most patients with BD, arthritis can b

19、e managed with colchicine7. There are no controlled data to guide the management of CNS involvement in BD. For parenchymal involvement agents to be tried may include corticosteroids, IFNa, azathioprine, cyclophosphamid

20、e, methotrexate and TNFa antagonists. For dural sinus thrombosis corticosteroidsAre recommended.,www.themegallery.com,Company Logo,EULAR關(guān)于白塞病治療的建議,8 Ciclosporine A should not be used in BD patients with central nervous

21、 system involvement unless necessary for intraocular inflammation.9 The decision to treat skin and mucosa involvement will depend on the perceived severity by the doctor and the patient. Mucocutaneous involvement sho

22、uld be treated according to the dominant or codominant lesions present. Topical measures (ie, local corticosteroids) should be the first line of treatment for isolated oral and genital ulcers. Acne-like lesions are usual

23、ly of cosmetic concern only. Thus, topical measures as used in acne vulgaris are sufficient. Colchicine should be preferred when the dominant lesion is erythaema nodosum. Leg ulcers in BD might have different causes. Tre