Influence of gender on Behçet's Disease phenotype and irreversible organ damage: Data from the International AIDA Network Behçet's Disease Registry

Jurgen Sota; Gaafar Ragab; Ibrahim Almaglouth; Giuseppe Lopalco; Abdurrahman Tufan; Haner Direskeneli; Andrea Hinojosa-Azaola; Henrique Ayres Mayrink Giardini; Silvana Guerriero; Paola Triggianese; Petros P. Sfikakis; Matteo Piga; Piero Ruscitti; Marcello Govoni; Annamaria Iagnocco; Francesco Carubbi; José Hernández-Rodríguez; Ahmed Hatem Laymouna; Ayman Abdel-Monem Ahmed Mahmoud; Mahmoud Ghanema; Aos A. Aboabat; Kazi Nur Asfina; Fehaid Alanazi; Maria Morrone; Veronica Spedicato; Hamit Kucuk; Riza Kardas; Fatma Alibaz Öner; Gizem Sevik; Jiram Torres-Ruiz; Perla Ayumi Kawakami-Campos; Isabelle Parente De Brito Antonelli; Rosanna Dammacco; Maria Sole Chimenti; Katerina Arida; Alberto Floris; Martina Gentile; Francesca Ruffilli; Elisa Bellis; Alessia Alunno; Gerard Espinosa; Stefano Gentileschi; Carla Gaggiano; Antonio Vitale; Valeria Caggiano; Roberta Lopez; Maria Tarsia; Sara Monti; Gülen Hatemi; Alican Karakoç; Micol Frassi; Roberto Giacomelli; Samar Tharwat; Maissa Thabet; Francesco Ciccia; Giacomo Emmi; Ombretta Viapiana; Ali Şahin; Gian Domenico Sebastiani; Ezgi Deniz Batu; Seza Ozen; Seher Sener; Daniela Opris-Belinski; Stefania Costi; Alessandro Conforti; Marco Cattalini; Elena Bartoloni; Nurullah Akkoç; Ozgul Soysal Gunduz; Giovanni Conti; Armin Maier; Annarita Giardina; Francesca Li Gobbi; Paola Parronchi; Piercarlo Sarzi Puttini; Luciana Breda; Amato De Paulis; Ester Carreño; Francesco La Torre; Ewa Więsik-Scewczyk; Alejandra De-La Torre; Germán Mejía-Salgado; Farhad Shahram; Serena Guiducci; Maria Cristina Maggio; Emma Aragona; Donato Rigante; Alessandro Ciavarro; Fatos Önen; Şükran Erten; Antonella Insalaco; Emanuela Del Giudice; Patrizia Barone; Francesca Gicchino; Antonio Brucato; Alberto Lo Gullo; Angela Mauro; Anastasios Karamanakos; Alberto Balistreri; Maria Antonietta Mazzei; Bruno Frediani; Claudia Fabiani; Luca Cantarini

doi:10.1016/j.jbspin.2024.105819

Influence of gender on Behçet's Disease phenotype and irreversible organ damage: Data from the International AIDA Network Behçet's Disease Registry

Jurgen Sota, Gaafar Ragab, Ibrahim Almaglouth, Giuseppe Lopalco, Abdurrahman Tufan, Haner Direskeneli, Andrea Hinojosa-Azaola, Henrique Ayres Mayrink Giardini, Silvana Guerriero, Paola Triggianese, Petros P. Sfikakis, Matteo Piga, Piero Ruscitti, Marcello Govoni, Annamaria Iagnocco, Francesco Carubbi, José Hernández-Rodríguez, Ahmed Hatem Laymouna, Ayman Abdel-Monem Ahmed Mahmoud, Mahmoud GhanemaAos A. Aboabat, Kazi Nur Asfina, Fehaid Alanazi, Maria Morrone, Veronica Spedicato, Hamit Kucuk, Riza Kardas, Fatma Alibaz Öner, Gizem Sevik, Jiram Torres-Ruiz, Perla Ayumi Kawakami-Campos, Isabelle Parente De Brito Antonelli, Rosanna Dammacco, Maria Sole Chimenti, Katerina Arida, Alberto Floris, Martina Gentile, Francesca Ruffilli, Elisa Bellis, Alessia Alunno, Gerard Espinosa, Stefano Gentileschi, Carla Gaggiano, Antonio Vitale, Valeria Caggiano, Roberta Lopez, Maria Tarsia, Sara Monti, Gülen Hatemi, Alican Karakoç, Micol Frassi, Roberto Giacomelli, Samar Tharwat, Maissa Thabet, Francesco Ciccia, Giacomo Emmi, Ombretta Viapiana, Ali Şahin, Gian Domenico Sebastiani, Ezgi Deniz Batu, Seza Ozen, Seher Sener, Daniela Opris-Belinski, Stefania Costi, Alessandro Conforti, Marco Cattalini, Elena Bartoloni, Nurullah Akkoç, Ozgul Soysal Gunduz, Giovanni Conti, Armin Maier, Annarita Giardina, Francesca Li Gobbi, Paola Parronchi, Piercarlo Sarzi Puttini, Luciana Breda, Amato De Paulis, Ester Carreño, Francesco La Torre, Ewa Więsik-Scewczyk, Alejandra De-La Torre, Germán Mejía-Salgado, Farhad Shahram, Serena Guiducci, Maria Cristina Maggio, Emma Aragona, Donato Rigante, Alessandro Ciavarro, Fatos Önen, Şükran Erten, Antonella Insalaco, Emanuela Del Giudice, Patrizia Barone, Francesca Gicchino, Antonio Brucato, Alberto Lo Gullo, Angela Mauro, Anastasios Karamanakos, Alberto Balistreri, Maria Antonietta Mazzei, Bruno Frediani, Claudia Fabiani, Luca Cantarini

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Abstract

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.

Original language	English
Pages (from-to)	1-8
Number of pages	8
Journal	Joint Bone Spine
Volume	92
DOIs	https://doi.org/10.1016/j.jbspin.2024.105819
Publication status	Published - 2025

Keywords

Behçet's disease

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10.1016/j.jbspin.2024.105819

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Sota, J., Ragab, G., Almaglouth, I., Lopalco, G., Tufan, A., Direskeneli, H., Hinojosa-Azaola, A., Mayrink Giardini, H. A., Guerriero, S., Triggianese, P., Sfikakis, P. P., Piga, M., Ruscitti, P., Govoni, M., Iagnocco, A., Carubbi, F., Hernández-Rodríguez, J., Laymouna, A. H., Mahmoud, A. A.-M. A., ... Cantarini, L. (2025). Influence of gender on Behçet's Disease phenotype and irreversible organ damage: Data from the International AIDA Network Behçet's Disease Registry. Joint Bone Spine, 92, 1-8. https://doi.org/10.1016/j.jbspin.2024.105819

@article{e8c2586f0169482fba0760bd1666aca1,

title = "Influence of gender on Beh{\c c}et's Disease phenotype and irreversible organ damage: Data from the International AIDA Network Beh{\c c}et's Disease Registry",

abstract = "Objectives Gender impact on phenotypical expression of Beh{\c c}et'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 Beh{\c c}et'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.",

keywords = "Beh{\c c}et's disease, Beh{\c c}et's disease",

author = "Jurgen Sota and Gaafar Ragab and Ibrahim Almaglouth and Giuseppe Lopalco and Abdurrahman Tufan and Haner Direskeneli and Andrea Hinojosa-Azaola and {Mayrink Giardini}, {Henrique Ayres} and Silvana Guerriero and Paola Triggianese and Sfikakis, {Petros P.} and Matteo Piga and Piero Ruscitti and Marcello Govoni and Annamaria Iagnocco and Francesco Carubbi and Jos{\'e} Hern{\'a}ndez-Rodr{\'i}guez and Laymouna, {Ahmed Hatem} and Mahmoud, {Ayman Abdel-Monem Ahmed} and Mahmoud Ghanema and Aboabat, {Aos A.} and Asfina, {Kazi Nur} and Fehaid Alanazi and Maria Morrone and Veronica Spedicato and Hamit Kucuk and Riza Kardas and {Alibaz {\"O}ner}, Fatma and Gizem Sevik and Jiram Torres-Ruiz and Kawakami-Campos, {Perla Ayumi} and {Parente De Brito Antonelli}, Isabelle and Rosanna Dammacco and Chimenti, {Maria Sole} and Katerina Arida and Alberto Floris and Martina Gentile and Francesca Ruffilli and Elisa Bellis and Alessia Alunno and Gerard Espinosa and Stefano Gentileschi and Carla Gaggiano and Antonio Vitale and Valeria Caggiano and Roberta Lopez and Maria Tarsia and Sara Monti and G{\"u}len Hatemi and Alican Karako{\c c} and Micol Frassi and Roberto Giacomelli and Samar Tharwat and Maissa Thabet and Francesco Ciccia and Giacomo Emmi and Ombretta Viapiana and Ali {\c S}ahin and Sebastiani, {Gian Domenico} and Batu, {Ezgi Deniz} and Seza Ozen and Seher Sener and Daniela Opris-Belinski and Stefania Costi and Alessandro Conforti and Marco Cattalini and Elena Bartoloni and Nurullah Akko{\c c} and Gunduz, {Ozgul Soysal} and Giovanni Conti and Armin Maier and Annarita Giardina and {Li Gobbi}, Francesca and Paola Parronchi and {Sarzi Puttini}, Piercarlo and Luciana Breda and {De Paulis}, Amato and Ester Carre{\~n}o and {La Torre}, Francesco and Ewa Wi{\c e}sik-Scewczyk and {De-La Torre}, Alejandra and Germ{\'a}n Mej{\'i}a-Salgado and Farhad Shahram and Serena Guiducci and Maggio, {Maria Cristina} and Emma Aragona and Donato Rigante and Alessandro Ciavarro and Fatos {\"O}nen and {\c S}{\"u}kran Erten and Antonella Insalaco and {Del Giudice}, Emanuela and Patrizia Barone and Francesca Gicchino and Antonio Brucato and {Lo Gullo}, Alberto and Angela Mauro and Anastasios Karamanakos and Alberto Balistreri and Mazzei, {Maria Antonietta} and Bruno Frediani and Claudia Fabiani and Luca Cantarini",

year = "2025",

doi = "10.1016/j.jbspin.2024.105819",

language = "English",

volume = "92",

pages = "1--8",

journal = "Joint Bone Spine",

issn = "1297-319X",

publisher = "Expansion Scientifique Francaise",

}

Sota, J, Ragab, G, Almaglouth, I, Lopalco, G, Tufan, A, Direskeneli, H, Hinojosa-Azaola, A, Mayrink Giardini, HA, Guerriero, S, Triggianese, P, Sfikakis, PP, Piga, M, Ruscitti, P, Govoni, M, Iagnocco, A, Carubbi, F, Hernández-Rodríguez, J, Laymouna, AH, Mahmoud, AA-MA, Ghanema, M, Aboabat, AA, Asfina, KN, Alanazi, F, Morrone, M, Spedicato, V, Kucuk, H, Kardas, R, Alibaz Öner, F, Sevik, G, Torres-Ruiz, J, Kawakami-Campos, PA, Parente De Brito Antonelli, I, Dammacco, R, Chimenti, MS, Arida, K, Floris, A, Gentile, M, Ruffilli, F, Bellis, E, Alunno, A, Espinosa, G, Gentileschi, S, Gaggiano, C, Vitale, A, Caggiano, V, Lopez, R, Tarsia, M, Monti, S, Hatemi, G, Karakoç, A, Frassi, M, Giacomelli, R, Tharwat, S, Thabet, M, Ciccia, F, Emmi, G, Viapiana, O, Şahin, A, Sebastiani, GD, Batu, ED, Ozen, S, Sener, S, Opris-Belinski, D, Costi, S, Conforti, A, Cattalini, M, Bartoloni, E, Akkoç, N, Gunduz, OS, Conti, G, Maier, A, Giardina, A, Li Gobbi, F, Parronchi, P, Sarzi Puttini, P, Breda, L, De Paulis, A, Carreño, E, La Torre, F, Więsik-Scewczyk, E, De-La Torre, A, Mejía-Salgado, G, Shahram, F, Guiducci, S, Maggio, MC, Aragona, E, Rigante, D, Ciavarro, A, Önen, F, Erten, Ş, Insalaco, A, Del Giudice, E, Barone, P, Gicchino, F, Brucato, A, Lo Gullo, A, Mauro, A, Karamanakos, A, Balistreri, A, Mazzei, MA, Frediani, B, Fabiani, C & Cantarini, L 2025, 'Influence of gender on Behçet's Disease phenotype and irreversible organ damage: Data from the International AIDA Network Behçet's Disease Registry', Joint Bone Spine, vol. 92, pp. 1-8. https://doi.org/10.1016/j.jbspin.2024.105819

TY - JOUR

T1 - Influence of gender on Behçet's Disease phenotype and irreversible organ damage: Data from the International AIDA Network Behçet's Disease Registry

AU - Sota, Jurgen

AU - Ragab, Gaafar

AU - Almaglouth, Ibrahim

AU - Lopalco, Giuseppe

AU - Tufan, Abdurrahman

AU - Direskeneli, Haner

AU - Hinojosa-Azaola, Andrea

AU - Mayrink Giardini, Henrique Ayres

AU - Guerriero, Silvana

AU - Triggianese, Paola

AU - Sfikakis, Petros P.

AU - Piga, Matteo

AU - Ruscitti, Piero

AU - Govoni, Marcello

AU - Iagnocco, Annamaria

AU - Carubbi, Francesco

AU - Hernández-Rodríguez, José

AU - Laymouna, Ahmed Hatem

AU - Mahmoud, Ayman Abdel-Monem Ahmed

AU - Ghanema, Mahmoud

AU - Aboabat, Aos A.

AU - Asfina, Kazi Nur

AU - Alanazi, Fehaid

AU - Morrone, Maria

AU - Spedicato, Veronica

AU - Kucuk, Hamit

AU - Kardas, Riza

AU - Alibaz Öner, Fatma

AU - Sevik, Gizem

AU - Torres-Ruiz, Jiram

AU - Kawakami-Campos, Perla Ayumi

AU - Parente De Brito Antonelli, Isabelle

AU - Dammacco, Rosanna

AU - Chimenti, Maria Sole

AU - Arida, Katerina

AU - Floris, Alberto

AU - Gentile, Martina

AU - Ruffilli, Francesca

AU - Bellis, Elisa

AU - Alunno, Alessia

AU - Espinosa, Gerard

AU - Gentileschi, Stefano

AU - Gaggiano, Carla

AU - Vitale, Antonio

AU - Caggiano, Valeria

AU - Lopez, Roberta

AU - Tarsia, Maria

AU - Monti, Sara

AU - Hatemi, Gülen

AU - Karakoç, Alican

AU - Frassi, Micol

AU - Giacomelli, Roberto

AU - Tharwat, Samar

AU - Thabet, Maissa

AU - Ciccia, Francesco

AU - Emmi, Giacomo

AU - Viapiana, Ombretta

AU - Şahin, Ali

AU - Sebastiani, Gian Domenico

AU - Batu, Ezgi Deniz

AU - Ozen, Seza

AU - Sener, Seher

AU - Opris-Belinski, Daniela

AU - Costi, Stefania

AU - Conforti, Alessandro

AU - Cattalini, Marco

AU - Bartoloni, Elena

AU - Akkoç, Nurullah

AU - Gunduz, Ozgul Soysal

AU - Conti, Giovanni

AU - Maier, Armin

AU - Giardina, Annarita

AU - Li Gobbi, Francesca

AU - Parronchi, Paola

AU - Sarzi Puttini, Piercarlo

AU - Breda, Luciana

AU - De Paulis, Amato

AU - Carreño, Ester

AU - La Torre, Francesco

AU - Więsik-Scewczyk, Ewa

AU - De-La Torre, Alejandra

AU - Mejía-Salgado, Germán

AU - Shahram, Farhad

AU - Guiducci, Serena

AU - Maggio, Maria Cristina

AU - Aragona, Emma

AU - Rigante, Donato

AU - Ciavarro, Alessandro

AU - Önen, Fatos

AU - Erten, Şükran

AU - Insalaco, Antonella

AU - Del Giudice, Emanuela

AU - Barone, Patrizia

AU - Gicchino, Francesca

AU - Brucato, Antonio

AU - Lo Gullo, Alberto

AU - Mauro, Angela

AU - Karamanakos, Anastasios

AU - Balistreri, Alberto

AU - Mazzei, Maria Antonietta

AU - Frediani, Bruno

AU - Fabiani, Claudia

AU - Cantarini, Luca

PY - 2025

Y1 - 2025

N2 - 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.

AB - 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.

KW - Behçet's disease

UR - http://hdl.handle.net/10807/301816

U2 - 10.1016/j.jbspin.2024.105819

DO - 10.1016/j.jbspin.2024.105819

M3 - Article

SN - 1297-319X

VL - 92

SP - 1

EP - 8

JO - Joint Bone Spine

JF - Joint Bone Spine

ER -

Influence of gender on Behçet's Disease phenotype and irreversible organ damage: Data from the International AIDA Network Behçet's Disease Registry

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