性别对白塞病表型及不可逆器官损害的影响：来自国际AIDA网络白塞病注册中心的数据
Influence of gender on Behçet's disease phenotype and irreversible organ damage: Data from the International AIDA Network Behçet's Disease Registry.
作者信息
Sota Jurgen, Ragab Gaafar, AlMaglouth Ibrahim, Lopalco Giuseppe, Tufan Abdurrahman, Direskeneli Haner, Hinojosa-Azaola Andrea, Mayrink Giardini Henrique Ayres, Guerriero Silvana, Triggianese Paola, Sfikakis Petros P, Piga Matteo, Ruscitti Piero, Govoni Marcello, Iagnocco Annamaria, Carubbi Francesco, Hernández-Rodríguez José, Laymouna Ahmed Hatem, Mahmoud Ayman Abdel-Monem Ahmed, Ghanema Mahmoud, Aboabat Aos A, Asfina Kazi Nur, Alanazi Fehaid, Morrone Maria, Spedicato Veronica, Kucuk Hamit, Kardas Riza, Alibaz Öner Fatma, Sevik Gizem, Torres-Ruiz Jiram, Kawakami-Campos Perla Ayumi, Parente de Brito Antonelli Isabelle, Dammacco Rosanna, Chimenti Maria Sole, Arida Katerina, Floris Alberto, Gentile Martina, Ruffilli Francesca, Bellis Elisa, Alunno Alessia, Espinosa Gerard, Gentileschi Stefano, Gaggiano Carla, Vitale Antonio, Caggiano Valeria, Lopez Roberta, Tarsia Maria, Monti Sara, Hatemi Gülen, Karakoç Alican, Frassi Micol, Giacomelli Roberto, Tharwat Samar, Thabet Maissa, Ciccia Francesco, Emmi Giacomo, Viapiana Ombretta, Şahin Ali, Sebastiani Gian Domenico, Batu Ezgi Deniz, Ozen Seza, Sener Seher, Opris-Belinski Daniela, Costi Stefania, Conforti Alessandro, Cattalini Marco, Bartoloni Elena, Akkoç Nurullah, Gunduz Ozgul Soysal, Conti Giovanni, Maier Armin, Giardina Annarita, Li Gobbi Francesca, Parronchi Paola, Sarzi Puttini Piercarlo, Breda Luciana, De Paulis Amato, Carreño Ester, La Torre Francesco, Więsik-Scewczyk Ewa, de-la Torre Alejandra, Mejía-Salgado Germán, Shahram Farhad, Guiducci Serena, Maggio Maria Cristina, Aragona Emma, Rigante Donato, Ciavarro Alessandro, Önen Fatos, Erten Şükran, Insalaco Antonella, Del Giudice Emanuela, Barone Patrizia, Gicchino Francesca, Brucato Antonio, Lo Gullo Alberto, Mauro Angela, Karamanakos Anastasios, Balistreri Alberto, Mazzei Maria Antonietta, Frediani Bruno, Fabiani Claudia, Cantarini Luca
机构信息
Rheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences Department of Medical Sciences, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory, and Autoimmune Diseases (RITA) Center], Siena, Italy.
Rheumatology and Clinical Immunology Unit, Internal Medicine Department, Faculty of Medicine, Cairo University, Giza, Egypt; Faculty of Medicine, Newgiza University, 6th of October City, Egypt.
出版信息
Joint Bone Spine. 2025 Mar;92(2):105819. doi: 10.1016/j.jbspin.2024.105819. Epub 2024 Nov 15.
OBJECTIVES
Gender impact on phenotypical expression of Behçet's disease (BD) has been specifically investigated only in a few large-scale studies. The main goal of the study was to examine gender differences in a large cohort of patients affected by BD.
METHODS
Data were retrieved from the International AIDA Network Registry for BD. We assessed differences between males and females in terms of Behçet's syndrome Overall Damage Index (BODI), differences in the disease manifestations at onset and in the cumulative manifestations throughout disease course, as well as differences in the cardiovascular risk. Finally, predictive factors leading to major organ involvement were investigated.
RESULTS
In total, 1024 BD patients (567 males, 457 females) were enrolled in the study, with a male-to-female ratio of 1.24/1. Males displayed a significantly higher mean±SD BODI (1.92±2.09) at the last follow-up, compared to female patients (1.25±1.87) (P<0.0001). Uveitis (P<0.0001) and vascular involvement (P=0.0076) were significantly more frequent among males whereas female patients were significantly over-represented in arthralgia (P<0.0001), arthritis (P=0.00025), isolated headache (P<0.0001), central nervous system (CNS) involvement (P=0.040), and gastrointestinal involvement (P=0.00046). Regarding cardiovascular risk, no differences between the two groups emerged (P=0.617). Four variables were associated with the development of major organ involvement: male gender (OR=2.104, P=0.001), current treatment with biologic agents (OR=2.257, P=0.0003), origin from endemic countries (OR=2.661, P=0.0009), and disease duration (OR=1.002, P=0.024).
CONCLUSION
BD displays a more severe course among males. This subgroup develops more irreversible damage and presents more frequently ocular and vascular involvement during disease course. On the other hand, female patients are prone to experience articular involvement, headache, CNS and gastrointestinal involvement. These data suggest the existence of a gender-driven disease expression.
目的
仅在少数大规模研究中专门调查了性别对白塞病（BD）表型表达的影响。本研究的主要目的是在一大群BD患者中检查性别差异。
方法
数据取自国际白塞病AIDA网络注册中心。我们评估了男性和女性在白塞综合征总体损伤指数（BODI）方面的差异、发病时疾病表现和整个病程中累积表现的差异，以及心血管风险的差异。最后，研究了导致主要器官受累的预测因素。
结果
共有1024例BD患者（567例男性，457例女性）纳入研究，男女比例为1.24/1。在最后一次随访时，男性的平均±标准差BODI（1.92±2.09）显著高于女性患者（1.25±1.87）（P<0.0001）。葡萄膜炎（P<0.0001）和血管受累（P=0.0076）在男性中明显更常见，而女性患者在关节痛（P<0.0001）、关节炎（P=0.00025）、孤立性头痛（P<0.0001）、中枢神经系统（CNS）受累（P=0.040）和胃肠道受累（P=0.00046）中明显占比过高。关于心血管风险，两组之间未出现差异（P=0.617）。四个变量与主要器官受累的发生相关：男性性别（OR=2.104，P=0.001）、当前使用生物制剂治疗（OR=2.257，P=0.0003）、来自流行国家（OR=2.661，P=0.0009）和病程（OR=1.002，P=0.024）。
结论
BD在男性中病程更严重。该亚组在病程中会出现更多不可逆损伤，眼部和血管受累也更频繁。另一方面，女性患者更容易出现关节受累、头痛、CNS和胃肠道受累。这些数据表明存在性别驱动的疾病表现。
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