突尼斯behcet病伴眼部损害的特征:单中心研究和文献综述

F. Ajili , S. Bellakhal , N. Ben Abdelhafidh , A. Mrabet , B. Zouari , A. Maalej , B. Louzir , J. Laabidi , S. Othmani
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引用次数: 3

摘要

behet病是一种多系统炎症性疾病,其特征是复发性口腔和生殖器溃疡、皮肤病变和葡萄膜炎。behaperet病的诊断是基于临床标准的。该疾病的病因尚不清楚,但族裔患病率和不同地理区域同一族裔群体患病率的巨大差异表明,环境触发了一种遗传决定的疾病。患者与方法回顾性分析1995 ~ 2010年我院内科收治的150例behaperet病患者的病历。对确认眼部受累的患者进行分析,并与未受累的患者进行比较。结果在研究的150份病历中,85例患者被纳入研究。33例(38.5%)患者有眼部受累。眼部BD诊断发病的平均年龄为35.3岁。男女比例为5.6。双眼受累26例(78.8%)。葡萄膜炎是最常见的眼部病变(31例,93.9%)。全葡萄膜炎是最常见的解剖部位(n = 21, 63.6%)。对比伴有或不伴有眼部受累的BD患者,眼部和神经系统表现有统计学意义的相关性(p = 0.03)。所有眼部受累的患者均接受皮质类固醇治疗。免疫抑制(IS)治疗28例(84.8%)。环磷酰胺作为一线治疗使用最多(71.4%)。环磷酰胺-硫唑嘌呤是采用最多的方案(28.5%)。在出现抵抗或复发的情况下,根据BD的其他表现,用另一种IS代替第一次使用的IS。33例患者中有7例在其他治疗方法失败后接受了英夫利昔单抗(IFX)治疗。结论通过早期治疗和定期监测,可改善BD患者的预后。根据葡萄膜炎的解剖形态、严重程度和相关的眼外表现来调整治疗方案是很重要的。
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Caractéristiques de la maladie de Behçet avec atteinte oculaire en Tunisie : étude monocentrique et revue de la littérature

Background

Behçet's disease is a multisystemic inflammatory disease characterized by recurrent oral and genital ulcers, skin lesions and uveitis. The diagnosis of Behçet's disease is based on clinical criteria. The etiology of the disease is unknown but the wide variations of ethnic prevalence and of the prevalence in the same ethnic group in different geographic areas indicate environmental triggering of a genetically determined disorder.

Patients and methods

A retrospective analysis of the medical charts of 150 Behçet's disease patients seen in our internal medicine department between 1995 and 2010 was undertaken. Patients with confirmed ocular involvement were analyzed and compared with those without ocular involvement.

Results

Among the 150 medical charts studied, 85 patients were included in the study. Thirty-three patients (38.5%) had ocular involvement. Mean age at ocular BD diagnosis onset were 35.3. Male to female ratio was 5.6. Ocular involvement was bilateral in 26 patients (78.8%). Uveitis was the most common ocular lesion (n = 31 patients, 93.9%). Panuveitis was the most common anatomical location (n = 21, 63.6%). The comparison of patients treated for BD with or without ocular involvement showed a statistically significant association between ocular and neurological manifestations (p = 0.03). All patients with ocular involvement were treated with corticosteroids. Immunosuppressive (IS) treatments were used in 28 patients (84.8%). Cyclophosphamide was the most used as first-line treatment (71.4%). Cyclophosphamide relayed by azathioprine was the most adopted protocol (28.5%). In case of resistance or relapse and depending on the other manifestations of the BD, the IS used in first intention was replaced by another one. Seven of the 33 patients had received treatment with infliximab (IFX) after failure of other therapeutic lines.

Conclusion

Ocular prognosis in the BD can be improved by early treatment and regular monitoring. It is important to adjust the therapeutic protocol to the anatomic form, to the severity of uveitis and to the extra-ocular manifestations associated.

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Pathologie-biologie
Pathologie-biologie 医学-病理学
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