Davatchi Fereydoun, Chams-Davatchi Cheyda, Shams Hormoz, Nadji Abdolhadi, Faezi Tahereh, Akhlaghi Massoomeh, Sadeghi Abdollahi Bahar, Ashofteh Farimah, Ghodsi Zahra, Mohtasham Negin, Shahram Farhad
Behcet's Disease Unit, Rheumatology Research Center, Tehran University of Medical Sciences, Shariati Hospital, Tehran, Iran.
Int J Rheum Dis. 2016 Jan;19(1):95-103. doi: 10.1111/1756-185X.12691. Epub 2015 Aug 10.
This is an analysis of adult Behcet's disease (BD) in Iran, from the Iran Registry of 7187 BD patients, gathered from 1975 to 2014, among which 6075 were adults (84.5%).
Patients were seen by a multidisciplinary team of experts. The diagnosis was by expert opinion, not by any specific classification/diagnosis criteria. However, 96.8% of them were classified by the International Criteria for Behcet's Disease (ICBD). Adult BDs were patients who had their first manifestation at the age of 16 or later.
Males constituted 56% (male/female ratio 1.3/1). The mean age at onset was 28.3 ± 8.7, mean duration 10.8 ± 8.2, and mean follow-up 5.0 ± 6.3. Oral aphthosis was seen in 97.5%, genital aphthosis 65.7%, skin manifestations 64.6% (pseudofolliculitis 53.2%, erythema nodosum 23.9%), ocular manifestations 58.1% (anterior uveitis 41.1%, posterior uveitis 45%, retinal vasculitis 33.6%, cataract 24.4%), joint manifestations 39.4% (arthralgia 18.9%, monoarthritis 9.1%, oligoarthritis 17.8%, ankyloing spondylitis 2%), gastrointestinal manifestations 7% (gastroduodenitis 2.3%, peptic ulcer 1.2%, diarrhea 2.1%, rectorrhagia 1.0%, abdominal pain-nausea 1.8%), neurological manifestations 10.6% (central 3.7%, peripheral 0.3%, headache 7.6%), vessel involvement (large vessel 1.7% with large vein thrombosis 1.1% and arterial involvement 0.7%, phlebitis 6.6%, superficial phlebitis 2.3%), epididymitis 4.6%, pulmonary manifestations 1% and cardiac manifestations 0.6%. Positive pathergy test was seen in 52.3%, human leukocyte antigen (HLA)-B5 in 54%, HLA-B51 in 48.9%, and high erythrocyte sedimentation rate in 52.8% of patients. By International Study Group (ISG) criteria 77.9% were classified, compared to ICBD revised criteria with 96.9%. The specificity of ISG was 99.2% and ICBD 97.2%.
Results are near the nationwide surveys from Japan, China, Korea and Germany.
这是一项对伊朗成年白塞病（BD）患者的分析，数据来自于1975年至2014年收集的7187例BD患者的伊朗登记处，其中6075例为成年人（84.5%）。
患者由多学科专家团队诊治。诊断依据专家意见，而非任何特定的分类/诊断标准。然而，其中96.8%的患者依据国际白塞病诊断标准（ICBD）进行了分类。成年BD患者是指首次出现症状在16岁及以后的患者。
男性占56%（男女比例为1.3/1）。发病的平均年龄为28.3±8.7岁，平均病程为10.8±8.2年，平均随访时间为5.0±6.3年。口腔溃疡的发生率为97.5%，生殖器溃疡为65.7%，皮肤表现为64.6%（假性毛囊炎53.2%，结节性红斑23.9%），眼部表现为58.1%（前葡萄膜炎41.1%，后葡萄膜炎45%，视网膜血管炎33.6%，白内障24.4%），关节表现为39.4%（关节痛18.9%，单关节炎9.1%，少关节炎17.8%，强直性脊柱炎2%），胃肠道表现为7%（胃十二指肠炎症2.3%，消化性溃疡1.2%，腹泻2.1%，直肠出血1.0%，腹痛伴恶心1.8%），神经表现为10.6%（中枢性3.7%，周围性0.3%，头痛7.6%），血管受累（大血管1.7%，大静脉血栓形成1.1%，动脉受累0.7%，静脉炎6.6%，浅表静脉炎2.3%），附睾炎4.6%，肺部表现1%，心脏表现0.6%。52.3%的患者针刺反应试验呈阳性，54%的患者人类白细胞抗原（HLA）-B5阳性，48.9%的患者HLA-B51阳性，52.8%的患者红细胞沉降率升高。按照国际研究组（ISG）标准，77.9%的患者得到分类，而按照ICBD修订标准则为96.9%。ISG的特异性为99.2%，ICBD为97.2%。
研究结果与日本、中国、韩国和德国的全国性调查结果相近。
