关键词: Behçet’s syndrome Clinical phenotype Cluster Gender

来  源:   DOI:10.1007/s12026-024-09498-1

Abstract:
Behçet\'s syndrome (BS) is a complex, multi-systemic disorder with a global occurrence, notably concentrated along the Silk Road. This study aimed to investigate gender-specific expressions and clinical phenotypes in BS patients within the Eastern Black Sea Region of Turkey. A total of 290 BS patients were retrospectively analyzed between January 2013 and December 2023. Demographic characteristics, clinical manifestations, medical treatment, and pathergy test results were obtained from a review of medical records. The mean age was 45.79 ± 13.05, with a male-to-female ratio of 48.6:51.4. Male patients had higher papulopustular lesions (p < 0.001) and ocular involvement (p = 0.036), while females showed more frequent genital ulcers (p = 0.032). Medication usage showed gender-based variations, notably higher corticosteroid, azathioprine, and tumor necrosis factor-alpha inhibitor (TNFi) use in males (p < 0.001). Cluster analysis revealed five distinct clusters, each with unique features and gender predominance. Cardiovascular type, ocular type, and skin type predominantly featured male patients, while joint involvement type and neurologic and mucosal involvement type were more prevalent among female patients with BS. This research contributes valuable insights into the gender-related clinical variations of BS within a specific geographic region, fostering a more comprehensive understanding of this challenging syndrome. The identification of distinct clinical phenotypes facilitates the development of tailored treatment strategies, potentially leading to improved outcomes for patients with BS.
摘要:
Behçet综合征(BS)是一种复杂的,具有全球性发生的多系统疾病,特别集中在丝绸之路沿线。这项研究旨在调查土耳其黑海地区东部BS患者的性别特异性表达和临床表型。对2013年1月至2023年12月期间的290例BS患者进行了回顾性分析。人口特征,临床表现,医疗,和pathergy测试结果是从医疗记录的审查中获得的。平均年龄为45.79±13.05,男女比例为48.6:51.4。男性患者有较高的丘疹脓疱病变(p<0.001)和眼部受累(p=0.036),而女性表现出更频繁的生殖器溃疡(p=0.032)。药物使用显示基于性别的差异,特别是更高的皮质类固醇,硫唑嘌呤,和男性使用肿瘤坏死因子-α抑制剂(TNFi)(p<0.001)。聚类分析显示了五个不同的聚类,每个人都有独特的特征和性别优势。心血管类型,眼型,皮肤类型主要以男性患者为特征,而在女性BS患者中,关节受累类型和神经系统和粘膜受累类型更为普遍。这项研究为特定地理区域内与性别相关的BS临床变异提供了宝贵的见解,促进对这种具有挑战性的综合症的更全面的理解。不同临床表型的识别有助于制定量身定制的治疗策略。可能导致改善BS患者的预后。
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