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Abstract：
BACKGROUND: Behcet disease (BD) as a variable vessel vasculitis is mainly characterized by ocular involvement, genital and oral aphthosis, and erythema nodosum. However, major organ involvements including gastrointestinal involvement, nervous system, and vascular involvement are among the severe complications. Osteonecrosis is a rare complication of patients with BD. We aim to report the largest series of BD patients suffering from osteonecrosis.
METHODS: We have retrospectively reviewed all patients in Iran Behcet\'s Disease Registry and reported those with osteonecrosis. Patients\' medication and clinical features, symptoms, and details of osteonecrosis will also be presented. Furthermore, previously reported cases will also be reviewed.
RESULTS: Seven thousand eight hundred thirty-one patients were diagnosed with BD and registered. 18 patients developed ON with an incidence of 0.22%. The most common involvement during the disease progression was oral aphthosis which appeared in 100% of patients followed by ocular involvement in 85.7% and skin involvement in 71.4%. Vascular, ocular, and nervous system involvements are significantly higher in BD patients with osteonecrosis than the other BD patients. For the management of acute episode of uveitis, deep vein thrombosis, severe gastrointestinal involvement, arterial involvement, nervous system Involvement, and joint involvement high dose of glucocorticoids is indicated.
CONCLUSIONS: ON tends to appear as a multifocal involvement in BD patients, hence, after diagnosis of ON in one joint other possible sites of ON should be investigated.
METHODS: We have retrospectively reviewed all patients in Iran Behcet\'s Disease Registry and reported those with osteonecrosis. Patients\' medication and clinical features, symptoms, and details of osteonecrosis will also be presented. Furthermore, previously reported cases will also be reviewed.
RESULTS: Seven thousand eight hundred thirty-one patients were diagnosed with BD and registered. 18 patients developed ON with an incidence of 0.22%. The most common involvement during the disease progression was oral aphthosis which appeared in 100% of patients followed by ocular involvement in 85.7% and skin involvement in 71.4%. Vascular, ocular, and nervous system involvements are significantly higher in BD patients with osteonecrosis than the other BD patients. For the management of acute episode of uveitis, deep vein thrombosis, severe gastrointestinal involvement, arterial involvement, nervous system Involvement, and joint involvement high dose of glucocorticoids is indicated.
CONCLUSIONS: ON tends to appear as a multifocal involvement in BD patients, hence, after diagnosis of ON in one joint other possible sites of ON should be investigated.
摘要：
背景：作为可变血管血管炎的Behcet病（BD）的主要特征是眼部受累，生殖器和口腔口疮，和结节性红斑.然而,主要器官受累，包括胃肠道受累，神经系统,血管受累是严重并发症之一。骨坏死是BD患者的罕见并发症。我们的目标是报告最大的一系列患有骨坏死的BD患者。
方法：我们回顾性回顾了伊朗Behcet疾病登记处的所有患者，并报道了骨坏死患者。患者用药和临床特征，症状,骨坏死的细节也将介绍。此外，以前报告的病例也将得到审查。
结果：诊断为BD并登记的患者为7,800,31例。18例患者发生ON，发生率为0.22%。疾病进展过程中最常见的受累是口腔口疮，100％的患者出现，其次是眼部受累85.7％，皮肤受累71.4％。血管,眼,骨坏死BD患者的神经系统受累率明显高于其他BD患者。对于葡萄膜炎急性发作的治疗，深静脉血栓形成,严重的胃肠道受累,动脉受累,神经系统的参与，和关节受累高剂量的糖皮质激素表示。
结论：ON往往表现为BD患者的多灶性受累，因此，在一个关节中诊断为ON后，应调查其他可能的ON部位。
方法：我们回顾性回顾了伊朗Behcet疾病登记处的所有患者，并报道了骨坏死患者。患者用药和临床特征，症状,骨坏死的细节也将介绍。此外，以前报告的病例也将得到审查。
结果：诊断为BD并登记的患者为7,800,31例。18例患者发生ON，发生率为0.22%。疾病进展过程中最常见的受累是口腔口疮，100％的患者出现，其次是眼部受累85.7％，皮肤受累71.4％。血管,眼,骨坏死BD患者的神经系统受累率明显高于其他BD患者。对于葡萄膜炎急性发作的治疗，深静脉血栓形成,严重的胃肠道受累,动脉受累,神经系统的参与，和关节受累高剂量的糖皮质激素表示。
结论：ON往往表现为BD患者的多灶性受累，因此，在一个关节中诊断为ON后，应调查其他可能的ON部位。
