1、白塞病 (Behcets disease),,Behcets disease,,Company Logo,OVERVIEW,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、中枢神经系统及 大血管受累者预后不佳。,,Company Logo,Type,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,,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 (A
2、Dhthous 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，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,,白塞病口腔溃疡,,Company Logo,,Company Lo
3、go,,Company Logo,,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, 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
4、(70% vs. 41.7%，p0.001),针刺反应 (注射部位出现脓疱疹）,,Company Logo,诊断标准,1989年白塞病国际诊断标准(ISG),主要条件：复发性口腔溃疡或庖疹性溃疡，3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,,Company Logo,EULAR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
5、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。,,Company Logo,EULAR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。,,Company Lo
6、go,EULAR关于白塞病治疗的建议,5.无循证医学证据提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。,,Company Logo,EULAR关于白塞病治疗的建议,9.白塞病皮肤和粘膜受累的治疗方法取决于医生和患者所认为
7、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。,,EULAR关于白塞病治疗的建议,1Any patient with BD and inflammatory eye disease affecting the posterior segment should be on a treatment regim
8、e 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), it is recommended that either ciclosporine A or infliximab be used in co
9、mbination 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 acute deep vein thrombosis in BD immunosuppressive agents such as cortic
10、osteroids, azathioprine, cyclophosphamide or ciclosporine A are recommended. For the management of pulmonary and peripheral arterial aneurysms, cyclophosphamide and corticosteroids are recommended.,,Company Logo,EULAR关于白塞病治疗的建议,4. Similarly there are no controlled data on, or evidence of benefit fro
11、m uncontrolled experience with anticoagulants, antiplatelet or antifibrinolytic 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 inv
12、olvement 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 be managed with colchicine7. There are no controlled data to guide the management of CN
13、S involvement in BD. For parenchymal involvement agents to be tried may include corticosteroids, IFNa, azathioprine, cyclophosphamide, methotrexate and TNFa antagonists. For dural sinus thrombosis corticosteroids Are recommended.,,Company Logo,EULAR关于白塞病治疗的建议,8 Ciclosporine A should not be used in B
14、D patients with central nervous 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 should be treated according to the dominant or codominan
15、t lesions present. Topical measures (ie, local corticosteroids) should be the first line of treatment for isolated oral and genital 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.,,Thank You !,
