Behcet's Disease Unit, Rheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Kargar Avenue, Tehran, Iran.
Int J Rheum Dis. 2010 Oct;13(4):367-73. doi: 10.1111/j.1756-185X.2010.01549.x.
To identify the clinical picture of Behcet's disease in a large cohort of patients (6500) in Iran, over a period of 35 years, and compare them with other large series from around the world.
Patients with Behcet's disease from all over Iran were seen in the Behcet's Disease Research Unit by a multidisciplinary team (rheumatologists, dermatologists, and ophthalmologists). Diagnosis was based on 'expert opinion'. Data were collected on a standardized data sheet (105 items), and stored in an electronic database. Data were updated at each follow-up.
Male to female ratio was 1.22 : .00. The mean age at onset was 26 years ± 11.3. The frequency of symptoms were: oral aphthosis 97.3%, genital aphthosis 64.6%, skin manifestations 64.9% (pseudofolliculitis 54.5%, erythema nodosum 22.5%, other lesions 7%), pathergy phenomenon 52.5%, ophthalmologic manifes-tations 56.8% (anterior uveitis 41.2%, posterior uveitis 44.9%, retinal vasculitis 32.1%), joint manifestations 37.4% (arthralgia 17.2%, monoarticular arthritis 7.6%, oligoarthritis 16.8%, ankylosing spondylitis 2%), neurological manifestations 3.8% (central manifestations 3.5%, mononeuritis multiplex 0.3%), gastrointestinal manifestations 7.4%, vascular involvement 8.3% (phlebitis 5.7%, superficial phlebitis 2.2%, large vein thrombosis 1.1%, arterial thrombosis 0.154%, aneurysm 0.5%), epididymitis 4.7%, cardiac involvement 0.6%, and pulmonary involvement 0.9%. Sedimentation rate was normal in 46.5% of patients. Abnormal urine sediment was detected in 12.2%. HLA-B5 was present in 53.3% and HLA-B51 in 47.9% of patients.
Behcet's disease is mainly seen in young people. The most frequent symptoms are mucocutaneous, ocular and joint manifestations. Comparison with large series did not show major differences.
在伊朗一个由 6500 名患者组成的大样本中识别白塞病的临床特征，并与世界各地的其他大型系列研究进行比较。
通过多学科团队（风湿病学家、皮肤科医生和眼科医生）在白塞病研究单位中观察来自伊朗各地的白塞病患者。诊断基于“专家意见”。在标准化数据表（105 项）上收集数据，并存储在电子数据库中。每次随访时更新数据。
男女比例为 1.22：0.00。发病年龄的平均值为 26 岁±11.3。症状频率为：口腔阿弗他溃疡 97.3%，生殖器阿弗他溃疡 64.6%，皮肤表现 64.9%（假性毛囊炎 54.5%，结节性红斑 22.5%，其他病变 7%），针刺现象 52.5%，眼部表现 56.8%（前葡萄膜炎 41.2%，后葡萄膜炎 44.9%，视网膜血管炎 32.1%），关节表现 37.4%（关节痛 17.2%，单关节炎 7.6%，少关节炎 16.8%，强直性脊柱炎 2%），神经系统表现 3.8%（中枢表现 3.5%，多发性单神经炎 0.3%），胃肠道表现 7.4%，血管受累 8.3%（静脉炎 5.7%，浅表静脉炎 2.2%，大静脉血栓形成 1.1%，动脉血栓形成 0.154%，动脉瘤 0.5%），附睾炎 4.7%，心脏受累 0.6%，肺部受累 0.9%。46.5%的患者血沉正常。12.2%的患者尿液沉淀物异常。53.3%的患者存在 HLA-B5，47.9%的患者存在 HLA-B51。
白塞病主要发生在年轻人中。最常见的症状是黏膜皮肤、眼部和关节表现。与大型系列研究相比，没有发现重大差异。
