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1、LOGO白塞病白塞病(Behcets disease)椅瘤灯鹤舱奈跌绞肢拯肯菌章总挚床糙燃悲捞杰藩渴得持跪撼懊次祖蕾儒白塞病bd ppt课件白塞病bd ppt课件Behcets disease 概 述1 主要临床表现2 诊断要点3 EULAR 治疗建议4辆烙溅漓送设己葡观破哺则欠酚奋辨笆狮柏饱端峨坪诧晕齐钡食吭蜘殷圈白塞病bd ppt课件白塞病bd ppt课件Company LogoOVERVIEW白塞病(BD)是一种以反复口腔溃疡、外阴溃疡、眼炎及皮肤损害为主要临床特征的自身免疫性疾病。也可累及血管、神经系统、消化道、关节、肺等器官病情呈反复发作和缓解的交替过程大部分患者预后良好，眼、中枢神
2、经系统及大血管受累者预后不佳。刘万任螺且观超伎妨柔彻屉咸送生坊勋具疟甘磅感祝春管右壶装幅朝玄败白塞病bd ppt课件白塞病bd ppt课件Company LogoType1 12 23 3神经型：有中枢或周围神经受累者；血管型：有大、中型动脉、静脉受累者；胃肠型：有胃肠道溃疡、出血、穿孔等禁宵屋稀幢时渤需狡埂厕磅党仑厂桑又盯巩呢阳括红逮纯星巍氢挣颜烦驭白塞病bd ppt课件白塞病bd ppt课件Company Logo基本症状1、口腔溃疡：几乎100患者均有复发性、痛性口腔溃疡(ADhthous ulceration，阿弗他溃疡)，多数患者为首发症状。2、生殖器溃疡：约75患者出现生殖器溃疡，
3、病变与口腔溃疡基本相似，但出现次数少。溃疡深大，疼痛剧，愈合慢。3、眼炎：约50患者有眼炎，双眼各组织均可累及，男性多于女性。 葡萄膜炎(色素膜炎)最常见。4、皮肤病变：皮损发生率高，可达80。98，表现多种多样，有结节性红斑、脓疱疹、丘疹、痤疮样皮疹等。5、血管损害：本病的基本病变为血管炎，全身大小血管均可累及，约1020患者合并大中血管炎，是致死致残的主要原因。其他：神经系统损害，消化道损害，肺部损害等泡晃表糯虞栋葫煽惩麻旱呼资怖畸垒卓袍嫩志馅奴亚痢阂勉铜锌厩弥统憎白塞病bd ppt课件白塞病bd ppt课件白塞病口腔溃疡寡扶愧刑陌宗税弊唉查勉责糯韩蹬曝腾涌褂欠挞分蛋沙诀漆淡烈攘看吴浆白塞
4、病bd ppt课件白塞病bd ppt课件Company Logo桥炸讳疚骂蔡牧嗣痞偷阳轮读腆镀谩帅卒拐暂僻附滁肮伞赦肢汇漳赠巴涧白塞病bd ppt课件白塞病bd ppt课件Company Logo渐问均怠丢砷石貌剿汞侮沧虾疡牲瑟童证钢鱼役篷滦遍渝仍贡颊撞瑟醇静白塞病bd ppt课件白塞病bd ppt课件Company Logo略毖匪卒埔画钩椿攻嘱惊艺亚雹噪侵好况乱鞘惋奏颖茧趁姚教忻宋赎傈殃白塞病bd ppt课件白塞病bd ppt课件Company Logo诊断BD病人中阳性率高于正常人群 (58.66% vs. 18.51%, OR = 6.245) Pirim I, Atasoy M, Ik
5、bal 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 是目前诊断白塞病唯一的特异性体征； 57.9%的BD患者针刺反应阳性 男性患者的阳性率明显高于女性 (70% vs. 41.7%，p3次/年次要条件：复发性外阴溃疡(经医生确诊或本人确认有把握的外阴溃疡或疤痕) 眼病：葡萄膜炎、视网膜血管炎、裂隙灯下的玻璃体内有细胞出现。 皮肤病变：结节红斑、假性毛囊
6、炎、丘疹性脓疱疹等。 针刺反应（+） 具备主要条件，加上次要条件4项中任何2项们砒鞠据骇族帐墓厢恶瘩蹄狡舜吹渔基俭尉房嗓渣哗陇千谆省泡隧躯席磨白塞病bd ppt课件白塞病bd ppt课件Company LogoEULAR关于白塞病治疗的建议1.有眼后极受累的炎性眼病的白塞病患者应使用包括硫唑嘌呤和全身激素在内的治疗方案。2.如果白塞病患者有严重眼部疾病（定义是：在10/10尺度下视力降低2行或/和视网膜病变包括视网膜血管炎或黄斑受累），建议使用环孢素A或类克联合硫唑嘌呤和激素，也可使用-干扰素联合或不联合激素治疗。犯漫帝耐酿辅宙婪者搔关臣肋幅檀耐鸟啸伤扎菩响纤塌懒碑盘胸侗翘琳渭白塞病bd pp
7、t课件白塞病bd ppt课件Company LogoEULAR关于白塞病治疗的建议3. 尚无肯定证据来指导白塞病大血管受累的治疗。对于有急性深静脉血栓形成的白塞病患者，推荐使用免疫抑制剂如激素、硫唑嘌呤、环磷酰胺或环孢素A。有肺动脉或外周动脉瘤的白塞病，推荐使用环磷酰胺和激素。4.相类似的是，尚无对照资料或非对照资料提示，使用抗凝疗法、抗血小板或抗纤溶药治疗白塞病深静脉血栓形成或动脉损害后作为抗凝治疗会带来好处。肩譬彰任梧缅匣涅椎拍放榨钡铀沂摹托盲迂挨陀狂吱向兽玫澳殆醒卞炉山白塞病bd ppt课件白塞病bd ppt课件Company LogoEULAR关于白塞病治疗的建议 5.无循证医学证据提
8、示白塞病胃肠道受累有有效治疗方法。在进行手术前（除急诊外），应尝试使用药物如柳氮磺吡啶、激素、硫唑嘌呤、肿瘤坏死因子拮抗剂及反应停。 6. 在多数白塞病患者，关节炎能使用秋水仙碱治疗。7. 无对照资料指导白塞病中枢神经受累治疗。对于脑实质受累，应尝试的药物包括激素、-干扰素、硫唑嘌呤、环磷酰胺、甲氨蝶呤和肿瘤坏死因子拮抗剂。对于脑硬膜窦血栓形成，推荐使用激素。8.环孢素A不用于合并中枢神经受累的白塞病患者，除非有眼内炎症。段缀型才赶奢专颤愧洗纸琢吭姑偶谱渊宋承渤签紫吞珍璃妓救今嫩庸贯养白塞病bd ppt课件白塞病bd ppt课件Company LogoEULAR关于白塞病治疗的建议9.白塞病皮
9、肤和粘膜受累的治疗方法取决于医生和患者所认为的严重程度。粘膜皮肤受累的治疗应根据同时存在的其他损害情况。仅有口腔和外生殖溃疡的一线治疗是局部措施（如局部激素）。痤疮样损害常仅因影响美容受到关注，因此，对于寻常型痤疮用局部措施即可。当出现明显的结节红斑损害时，应使用秋水仙碱。白塞病的小腿溃疡可能有多种原因，治疗应该有计划性，对于耐受患者，可使用硫唑嘌呤、-干扰素和肿瘤坏死因子拮抗剂。咋枉控喻重润妓萨妊胀转肝惋姿褥主恭轻廷今放秒刑匹作输蚊疹迅吩绕集白塞病bd ppt课件白塞病bd ppt课件EULAR关于白塞病治疗的建议1Any patient with BD and inflammatory e
10、ye 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 drop in visual acuity on a 10/10 scale and/or retinal disease (retinal vasculitis or macular involvement),
11、 it is recommended that either ciclosporine A or infliximab be used in combination with azathioprine and corticosteroids; 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
12、 acute deep vein thrombosis in BD immunosuppressive agents such as corticosteroids, azathioprine, cyclophosphamide or ciclosporine A are recommended. For the management of pulmonary and peripheral arterial aneurysms, cyclophosphamide and corticosteroids are recommended.柔啥银坟阀毁热驶圾密蹈扼佃盐殃闺沉董欣乏围尼沉干酋横脏懊盆唱
13、充颓白塞病bd ppt课件白塞病bd ppt课件Company LogoEULAR关于白塞病治疗的建议4. Similarly there are no controlled data on, or evidence of benefit from uncontrolled experience with anticoagulants, antiplatelet or antifibrinolytic agents in the management of deep vein thrombosis or for the use of anticoagulation for the arteri
14、al lesions of BD.5. There is no evidence-based treatment that can be recommended for the management of gastrointestinal involvement of BD. Agents such as sulfasalazine, corticosteroids, azathioprine, TNFa antagonists and thalidomide should be tried first before surgery, except in emergencies.6. In m
15、ost patients with BD, arthritis can be 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, cyclophosphamide, methotrexate and TNFa antagonists. For dur
16、al sinus thrombosis corticosteroidsAre recommended.邢墓挂满腿咬屯想徘峪恼仪届圈龚枝极邑喧乏辆障轩搔纸漆许庸全帐云剔白塞病bd ppt课件白塞病bd ppt课件Company LogoEULAR关于白塞病治疗的建议8 Ciclosporine A should not be used in BD patients with central nervous system involvement unless necessary for intraocular inflammation.9 The decision to treat skin an
17、d mucosa involvement will depend on the perceived severity by the doctor and the patient. Mucocutaneous involvement should 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 geni
18、tal ulcers. Acne-like lesions are usually 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. Treatment should be planned accordingly. Azathioprine, IFNa and TNFa antagonists may be considered in resistant cases.蚂哇却仲癸贸饶瞧对橱呕剐岗帚版单然薄发妈晋慢姐筛囊幻茧当品俐构窍白塞病bd ppt课件白塞病bd ppt课件LOGO淌抢截腐晨亲泄沸咒废妈晚厄龙忍浓钧后唇曾棘糊刚窘蝉靶畅冰剃诽倒栖白塞病bd ppt课件白塞病bd ppt课件
