1、白塞病合并骶髂关节炎 是真的吗？,绵阳市中心医院风湿免疫科 邹晋梅,病史介绍,入院查体,体温36.3 脉搏:86次/分 呼吸:20次/分 血压114/68mmHg 神清合作，面部及背部可见多个痤疮样皮损，皮肤粘膜无出血、浅表淋巴结未见肿大，下唇粘膜可见2个溃疡，上覆白苔，直径约0.3cm，咽部无充血，扁桃体无肿大，心肺查体未见异常，腹部软； 右手2、4指近端指间关节有肿胀压痛，跟腱无肿胀压痛，骶髂关节无压痛及叩痛，腰椎活动度尚可，腰2-5棘突有压痛，schober征阴性，枕墙距0cm，胸廓活动度大于2.5cm，双下肢4字试验阴性。,关节照片,辅助检查,辅助检查,患者骶髂关节MRI,T1W1,S
2、TIR,T1W1,T1W1,STIR,(1)回盲部,白塞病诊断标准(1990),International Criteria for Behcets disease (ICBD)2006,oral aphthosis (OA) 1分genital aphthosis (GA) 2分skin manifestations,comprising pseudofolliculitis (PF) and erythema nodosum(EN) 1分eye lesions (anterior uveitis AU, posterior uveitis PU, retinal vasculitis RV
3、) 2分Vascular manifestations (VMs):superficial phlebitis, deep vein thrombosis, large vein thrombosis, arterial thrombosis, and aneurysm 1分Pathergy phenomenon (PP) 1分 A patient has to get 3 or more points to be diagnosed/classified as having BD.,入院诊断：1.白塞病并白塞肠病2.骶髂关节、脊柱病变原因待查,联科讨论,消化科内分泌科风湿免疫科讨论意见： 1
4、.符合肠白塞病诊断 2.注意排除代谢性骨病,治疗,甲强龙40mg ivgtt qd沙利度胺 50mg qn,进一步检查,患者骶髂关节及脊柱病变是否为白塞病导致的？,白塞病相关的关节炎及骶髂关节病变文献,治疗调整,益赛普 25mg iH biw 强的松减量为10mg 沙利度胺 50mg qn,2周后复诊,血常规结果： WBC:2.95109/L、NEUT%：28.8 % LYMPH53.9% 中性粒细胞绝对计数0.85*109/L、HBG:120g/L、PLT:35.00109/LCRP：15.3mg/L肝肾功正常,面部毛囊炎好转、关节肿痛缓解、腹泻停止,调整治疗,停用益赛普、沙利度胺加用瑞白、
5、利可君、地榆升白片,益赛普会导致血液系统异常吗？,新的病史补充,12年前患者曾患再生障碍性贫血,骨穿及骨髓活检结果,诊断,1.白塞病并白塞肠病2.再生障碍性贫血,再生障碍性贫血,简称再障，是一组由多种病因所致的骨髓功能障碍，以全血细胞减少为主要表现的综合征。发病与化学药物、放射线、病毒感染、免疫因素（胸腺瘤、系统性红斑狼疮和类风湿关节炎等，患者血清中可找到抑制造血干细胞的抗体）及遗传因素有关。各年龄组均可发病，但以青壮年多见；男性发病率略高于女性。根据起病和病程急缓分为急性和慢性再障。 骨髓检查可发现骨髓增生低下或重度低下，非造血细胞和脂肪细胞增多，一般在60%以上。,再障患者MRI,A,B,
6、A:T1W1椎体呈明显的浓白状弥漫性高信号;B:STIR椎体呈明显低信号,其强度明显低于背部肌肉C：T1Wl 显示L2 及S1 结节状低信号造血组织岛诊断学理论与实践2005 年第4 卷第2 期,C,该患者骶髂关节MRI,T1W1,STIR,T1W1,T1W1,STIR,白塞病与再障有关系吗？,AbstractThe aim of this study was to determine the clinical characteristics ofBehcets disease (BD) complicated with malignancy in Korea. Of 1,769 patien
7、ts with BD in our hospital, 32 patients (1.8%, 21 in solid cancer, 11 in hematologic malignancy) developed cancer. In 10 of the 32 subjects (31.3%), malignancy was diagnosed before or concomitantly with BD. Twelve cases (37.5%) occurred within the first 2 years of diseaseand9 cases (28.1%) occurred
8、5 years after the diagnosis of BD. Myelodysplastic syndrome (MDS) was the most common disease (n = 7) followed by thyroid cancer (n = 4), breast cancer, cervix cancer, stomach cancer, rectal cancer, hepatoma,aplasticanemia(n = 3, each), renal cell cancer, endometrial cancer, lymphoma (n = 1, each).
9、There were no significant differences in the clinical characteristic between patients with or without malignancy. Intestinal involvement were more frequent in patients with malignancy than those without, but was not statistically significant (p = 0.083). Our results demonstrate that MDSandthyroid ca
10、ncer are the most common hematologic diseaseandsolid cancer associated with BD, respectively.,Aplasticanemiawith trisomy 8andtrisomy 9 in intestinal behcets disease. Korean J Gastroenterol2010 Apr;55 (4): 256-60,治疗？,经 验 教 训,病史询问的重要性白塞病与恶性疾病、包括血液系统恶性疾病可能有关骶髂关节病变原因的鉴别（感染性疾病、骨关节疾病、内分泌代谢疾病、血液系统疾病和肿瘤 ）使用生物制剂时需监测血细胞风湿科医师应加强影像学知识的学习,THANKS,
