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白塞病的临床表现及治疗结果:单中心经验

The clinical manifestations and treatment outcomes of Behçet's disease: A single-center experience.

作者信息

Hama Hussein Karokh F, Abdullah Hiwa O, Ahmed Shaho F, Qadir Aso N, Asaad Hoshmand R, Fattah Fattah H, Hama Amin Bnar J, Gharib Dana T, Mohammed Shvan H, Salih Rawezh Q, Abdalla Berun A, Kakamad Fahmi H, Mohammed Shorsh A

机构信息

Smart Health Tower Sulaimani Iraq.

Department of Gastroenterology Kurdistan Center for Gastroenterology and Hepatology Sulaimani Iraq.

出版信息

Health Sci Rep. 2024 Jul 24;7(7):e2238. doi: 10.1002/hsr2.2238. eCollection 2024 Jul.

DOI:10.1002/hsr2.2238
PMID:39055614
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11269204/
Abstract

BACKGROUND AND AIMS

Behçet's disease is a chronic, multisystemic, and relapsing inflammatory disorder. It lacks a permanent cure, the focus of treatment is on mitigating symptoms, decreasing the frequency and severity of relapses, and preventing life-threatening complications. This study aims to report the experience of a single center in managing patients with Behçet's disease and discuss the treatment outcomes.

METHODS

This study was a retrospective case series conducted over 2 years. All cases were clinically diagnosed according to the International Criteria for Behçet's Disease. The extracted data were demographics, family history, clinical findings, criteria scores, treatment, and outcomes.

RESULTS

A total of 31 patients were included, consisting of 13 males (42%) and 18 females (58%). Most cases were over the age of 30, and both genders were nearly equally distributed among age groups. The most commonly affected site was the oral cavity, observed in 96.77% of cases. Genital, cutaneous, and vascular involvements were more common in males, while females were more likely to have oral, ocular, and musculoskeletal involvements. For various treatment regimens, oral, cutaneous, vascular, and musculoskeletal involvements showed complete response in all cases. Among cases with genital involvement, complete response was achieved in seven cases (41.2%), while four cases (23.5%) showed only partial response, and six cases (35.3%) experienced recurrence. In cases with ocular involvement, only partial responses were observed.

CONCLUSION

Oral, cutaneous, vascular, and musculoskeletal involvements may have a higher likelihood of a complete response to treatment regimens. However, genital involvement may be the most recurrent manifestation, followed by ocular involvement.

摘要

背景与目的

白塞病是一种慢性、多系统、复发性炎症性疾病。它缺乏根治方法,治疗重点在于缓解症状、降低复发频率和严重程度以及预防危及生命的并发症。本研究旨在报告单中心管理白塞病患者的经验并讨论治疗结果。

方法

本研究是一项为期2年的回顾性病例系列研究。所有病例均根据白塞病国际标准进行临床诊断。提取的数据包括人口统计学、家族史、临床发现、标准评分、治疗及结果。

结果

共纳入31例患者,其中男性13例(42%),女性18例(58%)。大多数病例年龄超过30岁,各年龄组中男女分布几乎相等。最常受累部位是口腔,96.77%的病例出现该症状。男性生殖器、皮肤和血管受累更为常见,而女性更易出现口腔、眼部和肌肉骨骼受累。对于各种治疗方案,口腔、皮肤、血管和肌肉骨骼受累在所有病例中均显示完全缓解。在生殖器受累病例中,7例(41.2%)达到完全缓解,4例(23.5%)仅部分缓解,6例(35.3%)复发。在眼部受累病例中,仅观察到部分缓解。

结论

口腔、皮肤、血管和肌肉骨骼受累对治疗方案完全缓解的可能性可能更高。然而,生殖器受累可能是最易复发的表现,其次是眼部受累。

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Front Med (Lausanne). 2022 Dec 2;9:987393. doi: 10.3389/fmed.2022.987393. eCollection 2022.
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Behçet Disease: An Update for Dermatologists.白塞病:皮肤科医生的最新知识。
Am J Clin Dermatol. 2021 Jul;22(4):477-502. doi: 10.1007/s40257-021-00609-4. Epub 2021 Jun 1.
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Treatment of Behçet's Disease: An Algorithmic Multidisciplinary Approach.白塞病的治疗:一种多学科算法治疗方法。
Front Med (Lausanne). 2021 Apr 28;8:624795. doi: 10.3389/fmed.2021.624795. eCollection 2021.
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Experimental Therapeutic Solutions for Behcet's Disease.白塞病的实验性治疗方案
J Exp Pharmacol. 2021 Feb 12;13:127-145. doi: 10.2147/JEP.S265645. eCollection 2021.
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Evaluation of different classification criteria in children with Behcet disease: results from a single referral center.评价不同分类标准在儿童白塞病中的应用:来自单一转诊中心的结果。
Expert Rev Clin Immunol. 2020 Nov;16(11):1093-1097. doi: 10.1080/1744666X.2021.1834853. Epub 2020 Oct 23.
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Predictive factors for work-day loss in Behçet's syndrome: A multi-center study.白塞病工作日损失的预测因素:一项多中心研究。
Int J Rheum Dis. 2020 Feb;23(2):240-246. doi: 10.1111/1756-185X.13771. Epub 2019 Dec 19.
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Prevalence and demographic characteristics of Behcet disease in Colombia: data from the national health registry 2012-2016.哥伦比亚贝赫切特病的患病率和人口统计学特征:2012-2016 年国家健康登记数据。
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Behçet's disease: A comprehensive review with a focus on epidemiology, etiology and clinical features, and management of mucocutaneous lesions.白塞病:一篇全面综述,重点关注流行病学、病因学、临床特征以及黏膜皮肤病变的管理。
J Dermatol. 2016 Jun;43(6):620-32. doi: 10.1111/1346-8138.13381. Epub 2016 Apr 14.
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