Risk for cancer development in familial Mediterranean fever and associated predisposing factors: an ambidirectional cohort study from the international AIDA Network registries

Antonio Vitale; Valeria Caggiano; Abdurrahman Tufan; Gaafar Ragab; Ezgi Deniz Batu; Piero Portincasa; Emma Aragona; Jurgen Sota; Giovanni Conti; Amato De Paulis; Donato Rigante; Alma Nunzia Olivieri; Ali Şahin; Francesco La Torre; Giuseppe Lopalco; Marco Cattalini; Maria Cristina Maggio; Antonella Insalaco; Petros P. Sfikakis; Elena Verrecchia; Derya Yildirim; Hamit Kucuk; Riza Can Kardas; Ahmed Hatem Laymouna; Mahmoud Ghanema; Moustafa Ali Saad; Seher Sener; Hulya Ercan Emreol; Seza Ozen; Nour Jaber; Mohamad Khalil; Agostino Di Ciaula; Carla Gaggiano; Giuseppe Malizia; Andrea Affronti; Serena Patroniti; Meri Romeo; Jessica Sbalchiero; Francesca Della Casa; Ilaria Mormile; Sara Silvaroli; Maria Francesca Gicchino; Neşe Çabuk Çelik; Maria Tarsia; Anastasios Karamanakos; José Hernández-Rodríguez; Paola Parronchi; Daniela Opris-Belinski; Patrizia Barone; Andreas Recke; Stefania Costi; Paolo Sfriso; Henrique A. Mayrink Giardini; Stefano Gentileschi; Ewa Wiesik-Szewczyk; Ibrahim Vasi; Roberta Loconte; Karina Jahnz-Różyk; Eduardo Martín-Nares; Jiram Torres-Ruiz; Alberto Cauli; Alessandro Conforti; Giacomo Emmi; Francesca Li Gobbi; Giovanni Rosario Biasi; Riccardo Terribili; Piero Ruscitti; Emanuela Del Giudice; Samar Tharwat; Antonio Luca Brucato; Benson Ogunjimi; Andrea Hinojosa-Azaola; Alberto Balistreri; Claudia Fabiani; Bruno Frediani; Luca Cantarini

doi:10.3389/fimmu.2024.1397890

Risk for cancer development in familial Mediterranean fever and associated predisposing factors: an ambidirectional cohort study from the international AIDA Network registries

Antonio Vitale, Valeria Caggiano, Abdurrahman Tufan, Gaafar Ragab, Ezgi Deniz Batu, Piero Portincasa, Emma Aragona, Jurgen Sota, Giovanni Conti, Amato De Paulis, Donato Rigante, Alma Nunzia Olivieri, Ali Şahin, Francesco La Torre, Giuseppe Lopalco, Marco Cattalini, Maria Cristina Maggio, Antonella Insalaco, Petros P. Sfikakis, Elena VerrecchiaDerya Yildirim, Hamit Kucuk, Riza Can Kardas, Ahmed Hatem Laymouna, Mahmoud Ghanema, Moustafa Ali Saad, Seher Sener, Hulya Ercan Emreol, Seza Ozen, Nour Jaber, Mohamad Khalil, Agostino Di Ciaula, Carla Gaggiano, Giuseppe Malizia, Andrea Affronti, Serena Patroniti, Meri Romeo, Jessica Sbalchiero, Francesca Della Casa, Ilaria Mormile, Sara Silvaroli, Maria Francesca Gicchino, Neşe Çabuk Çelik, Maria Tarsia, Anastasios Karamanakos, José Hernández-Rodríguez, Paola Parronchi, Daniela Opris-Belinski, Patrizia Barone, Andreas Recke, Stefania Costi, Paolo Sfriso, Henrique A. Mayrink Giardini, Stefano Gentileschi, Ewa Wiesik-Szewczyk, Ibrahim Vasi, Roberta Loconte, Karina Jahnz-Różyk, Eduardo Martín-Nares, Jiram Torres-Ruiz, Alberto Cauli, Alessandro Conforti, Giacomo Emmi, Francesca Li Gobbi, Giovanni Rosario Biasi, Riccardo Terribili, Piero Ruscitti, Emanuela Del Giudice, Samar Tharwat, Antonio Luca Brucato, Benson Ogunjimi, Andrea Hinojosa-Azaola, Alberto Balistreri, Claudia Fabiani, Bruno Frediani, Luca Cantarini

Research output: Contribution to journal › Article

Abstract

Objective: Inflammation has been associated with an increased risk for cancer development, while innate immune system activation could counteract the risk for malignancies. Familial Mediterranean fever (FMF) is a severe systemic inflammatory condition and also represents the archetype of innate immunity deregulation. Therefore, the aim of this study is to investigate the risk for cancer development in FMF. Methods: The risk ratio (RR) for malignancies was separately compared between FMF patients and fibromyalgia subjects, Still’s disease patients and Behçet’s disease patients. Clinical variables associated with cancer development in FMF patients were searched through binary logistic regression. Results: 580 FMF patients and 102 fibromyalgia subjects, 1012 Behçet’s disease patients and 497 Still’s disease patients were enrolled. The RR for the occurrence of malignant neoplasms was 0.26 (95% Confidence Interval [CI.] 0.10-0.73, p=0.006) in patients with FMF compared to fibromyalgia subjects; the RR for the occurrence of malignant cancer was 0.51 (95% CI. 0.23-1.16, p=0.10) in FMF compared to Still’s disease and 0.60 (95% CI. 0.29-1.28, p=0.18) in FMF compared to Behçet’s disease. At logistic regression, the risk of occurrence of malignant neoplasms in FMF patients was associated with the age at disease onset (b1 = 0.039, 95% CI. 0.001-0.071, p=0.02), the age at the diagnosis (b1 = 0.048, 95% CI. 0.039-0.085, p=0.006), the age at the enrolment (b1 = 0.05, 95% CI. 0.007-0.068, p=0.01), the number of attacks per year (b1 = 0.011, 95% CI. 0.001- 0.019, p=0.008), the use of biotechnological agents (b1 = 1.77, 95% CI. 0.43-3.19, p=0.009), the use of anti-IL-1 agents (b1 = 2.089, 95% CI. 0.7- 3.5, p=0.002). Conclusions: The risk for cancer is reduced in Caucasic FMF patients; however, when malignant neoplasms occur, this is more frequent in FMF cases suffering from a severe disease phenotype and presenting a colchicine-resistant disease.

Original language	English
Pages (from-to)	1-12
Number of pages	12
Journal	Frontiers in Immunology
Volume	15
DOIs	https://doi.org/10.3389/fimmu.2024.1397890
Publication status	Published - 2024

Keywords

Familial Mediterranean fever

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.3389/fimmu.2024.1397890

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Vitale, A., Caggiano, V., Tufan, A., Ragab, G., Batu, E. D., Portincasa, P., Aragona, E., Sota, J., Conti, G., De Paulis, A., Rigante, D., Olivieri, A. N., Şahin, A., La Torre, F., Lopalco, G., Cattalini, M., Maggio, M. C., Insalaco, A., Sfikakis, P. P., ... Cantarini, L. (2024). Risk for cancer development in familial Mediterranean fever and associated predisposing factors: an ambidirectional cohort study from the international AIDA Network registries. Frontiers in Immunology, 15, 1-12. https://doi.org/10.3389/fimmu.2024.1397890

@article{c570ee7238a04588806313b0cddeb080,

title = "Risk for cancer development in familial Mediterranean fever and associated predisposing factors: an ambidirectional cohort study from the international AIDA Network registries",

abstract = "Objective: Inflammation has been associated with an increased risk for cancer development, while innate immune system activation could counteract the risk for malignancies. Familial Mediterranean fever (FMF) is a severe systemic inflammatory condition and also represents the archetype of innate immunity deregulation. Therefore, the aim of this study is to investigate the risk for cancer development in FMF. Methods: The risk ratio (RR) for malignancies was separately compared between FMF patients and fibromyalgia subjects, Still{\textquoteright}s disease patients and Beh{\c c}et{\textquoteright}s disease patients. Clinical variables associated with cancer development in FMF patients were searched through binary logistic regression. Results: 580 FMF patients and 102 fibromyalgia subjects, 1012 Beh{\c c}et{\textquoteright}s disease patients and 497 Still{\textquoteright}s disease patients were enrolled. The RR for the occurrence of malignant neoplasms was 0.26 (95% Confidence Interval [CI.] 0.10-0.73, p=0.006) in patients with FMF compared to fibromyalgia subjects; the RR for the occurrence of malignant cancer was 0.51 (95% CI. 0.23-1.16, p=0.10) in FMF compared to Still{\textquoteright}s disease and 0.60 (95% CI. 0.29-1.28, p=0.18) in FMF compared to Beh{\c c}et{\textquoteright}s disease. At logistic regression, the risk of occurrence of malignant neoplasms in FMF patients was associated with the age at disease onset (b1 = 0.039, 95% CI. 0.001-0.071, p=0.02), the age at the diagnosis (b1 = 0.048, 95% CI. 0.039-0.085, p=0.006), the age at the enrolment (b1 = 0.05, 95% CI. 0.007-0.068, p=0.01), the number of attacks per year (b1 = 0.011, 95% CI. 0.001- 0.019, p=0.008), the use of biotechnological agents (b1 = 1.77, 95% CI. 0.43-3.19, p=0.009), the use of anti-IL-1 agents (b1 = 2.089, 95% CI. 0.7- 3.5, p=0.002). Conclusions: The risk for cancer is reduced in Caucasic FMF patients; however, when malignant neoplasms occur, this is more frequent in FMF cases suffering from a severe disease phenotype and presenting a colchicine-resistant disease.",

keywords = "Familial Mediterranean fever, Familial Mediterranean fever",

author = "Antonio Vitale and Valeria Caggiano and Abdurrahman Tufan and Gaafar Ragab and Batu, {Ezgi Deniz} and Piero Portincasa and Emma Aragona and Jurgen Sota and Giovanni Conti and {De Paulis}, Amato and Donato Rigante and Olivieri, {Alma Nunzia} and Ali {\c S}ahin and {La Torre}, Francesco and Giuseppe Lopalco and Marco Cattalini and Maggio, {Maria Cristina} and Antonella Insalaco and Sfikakis, {Petros P.} and Elena Verrecchia and Derya Yildirim and Hamit Kucuk and Kardas, {Riza Can} and Laymouna, {Ahmed Hatem} and Mahmoud Ghanema and Saad, {Moustafa Ali} and Seher Sener and {Ercan Emreol}, Hulya and Seza Ozen and Nour Jaber and Mohamad Khalil and {Di Ciaula}, Agostino and Carla Gaggiano and Giuseppe Malizia and Andrea Affronti and Serena Patroniti and Meri Romeo and Jessica Sbalchiero and {Della Casa}, Francesca and Ilaria Mormile and Sara Silvaroli and Gicchino, {Maria Francesca} and {\c C}elik, {Ne{\c s}e {\c C}abuk} and Maria Tarsia and Anastasios Karamanakos and Jos{\'e} Hern{\'a}ndez-Rodr{\'i}guez and Paola Parronchi and Daniela Opris-Belinski and Patrizia Barone and Andreas Recke and Stefania Costi and Paolo Sfriso and Giardini, {Henrique A. Mayrink} and Stefano Gentileschi and Ewa Wiesik-Szewczyk and Ibrahim Vasi and Roberta Loconte and Karina Jahnz-R{\'o}{\.z}yk and Eduardo Mart{\'i}n-Nares and Jiram Torres-Ruiz and Alberto Cauli and Alessandro Conforti and Giacomo Emmi and {Li Gobbi}, Francesca and Biasi, {Giovanni Rosario} and Riccardo Terribili and Piero Ruscitti and {Del Giudice}, Emanuela and Samar Tharwat and Brucato, {Antonio Luca} and Benson Ogunjimi and Andrea Hinojosa-Azaola and Alberto Balistreri and Claudia Fabiani and Bruno Frediani and Luca Cantarini",

year = "2024",

doi = "10.3389/fimmu.2024.1397890",

language = "English",

volume = "15",

pages = "1--12",

journal = "Frontiers in Immunology",

issn = "1664-3224",

publisher = "Lausanne : Frontiers Research Foundation, 2010-",

}

Vitale, A, Caggiano, V, Tufan, A, Ragab, G, Batu, ED, Portincasa, P, Aragona, E, Sota, J, Conti, G, De Paulis, A, Rigante, D, Olivieri, AN, Şahin, A, La Torre, F, Lopalco, G, Cattalini, M, Maggio, MC, Insalaco, A, Sfikakis, PP, Verrecchia, E, Yildirim, D, Kucuk, H, Kardas, RC, Laymouna, AH, Ghanema, M, Saad, MA, Sener, S, Ercan Emreol, H, Ozen, S, Jaber, N, Khalil, M, Di Ciaula, A, Gaggiano, C, Malizia, G, Affronti, A, Patroniti, S, Romeo, M, Sbalchiero, J, Della Casa, F, Mormile, I, Silvaroli, S, Gicchino, MF, Çelik, NÇ, Tarsia, M, Karamanakos, A, Hernández-Rodríguez, J, Parronchi, P, Opris-Belinski, D, Barone, P, Recke, A, Costi, S, Sfriso, P, Giardini, HAM, Gentileschi, S, Wiesik-Szewczyk, E, Vasi, I, Loconte, R, Jahnz-Różyk, K, Martín-Nares, E, Torres-Ruiz, J, Cauli, A, Conforti, A, Emmi, G, Li Gobbi, F, Biasi, GR, Terribili, R, Ruscitti, P, Del Giudice, E, Tharwat, S, Brucato, AL, Ogunjimi, B, Hinojosa-Azaola, A, Balistreri, A, Fabiani, C, Frediani, B & Cantarini, L 2024, 'Risk for cancer development in familial Mediterranean fever and associated predisposing factors: an ambidirectional cohort study from the international AIDA Network registries', Frontiers in Immunology, vol. 15, pp. 1-12. https://doi.org/10.3389/fimmu.2024.1397890

TY - JOUR

T1 - Risk for cancer development in familial Mediterranean fever and associated predisposing factors: an ambidirectional cohort study from the international AIDA Network registries

AU - Vitale, Antonio

AU - Caggiano, Valeria

AU - Tufan, Abdurrahman

AU - Ragab, Gaafar

AU - Batu, Ezgi Deniz

AU - Portincasa, Piero

AU - Aragona, Emma

AU - Sota, Jurgen

AU - Conti, Giovanni

AU - De Paulis, Amato

AU - Rigante, Donato

AU - Olivieri, Alma Nunzia

AU - Şahin, Ali

AU - La Torre, Francesco

AU - Lopalco, Giuseppe

AU - Cattalini, Marco

AU - Maggio, Maria Cristina

AU - Insalaco, Antonella

AU - Sfikakis, Petros P.

AU - Verrecchia, Elena

AU - Yildirim, Derya

AU - Kucuk, Hamit

AU - Kardas, Riza Can

AU - Laymouna, Ahmed Hatem

AU - Ghanema, Mahmoud

AU - Saad, Moustafa Ali

AU - Sener, Seher

AU - Ercan Emreol, Hulya

AU - Ozen, Seza

AU - Jaber, Nour

AU - Khalil, Mohamad

AU - Di Ciaula, Agostino

AU - Gaggiano, Carla

AU - Malizia, Giuseppe

AU - Affronti, Andrea

AU - Patroniti, Serena

AU - Romeo, Meri

AU - Sbalchiero, Jessica

AU - Della Casa, Francesca

AU - Mormile, Ilaria

AU - Silvaroli, Sara

AU - Gicchino, Maria Francesca

AU - Çelik, Neşe Çabuk

AU - Tarsia, Maria

AU - Karamanakos, Anastasios

AU - Hernández-Rodríguez, José

AU - Parronchi, Paola

AU - Opris-Belinski, Daniela

AU - Barone, Patrizia

AU - Recke, Andreas

AU - Costi, Stefania

AU - Sfriso, Paolo

AU - Giardini, Henrique A. Mayrink

AU - Gentileschi, Stefano

AU - Wiesik-Szewczyk, Ewa

AU - Vasi, Ibrahim

AU - Loconte, Roberta

AU - Jahnz-Różyk, Karina

AU - Martín-Nares, Eduardo

AU - Torres-Ruiz, Jiram

AU - Cauli, Alberto

AU - Conforti, Alessandro

AU - Emmi, Giacomo

AU - Li Gobbi, Francesca

AU - Biasi, Giovanni Rosario

AU - Terribili, Riccardo

AU - Ruscitti, Piero

AU - Del Giudice, Emanuela

AU - Tharwat, Samar

AU - Brucato, Antonio Luca

AU - Ogunjimi, Benson

AU - Hinojosa-Azaola, Andrea

AU - Balistreri, Alberto

AU - Fabiani, Claudia

AU - Frediani, Bruno

AU - Cantarini, Luca

PY - 2024

Y1 - 2024

N2 - Objective: Inflammation has been associated with an increased risk for cancer development, while innate immune system activation could counteract the risk for malignancies. Familial Mediterranean fever (FMF) is a severe systemic inflammatory condition and also represents the archetype of innate immunity deregulation. Therefore, the aim of this study is to investigate the risk for cancer development in FMF. Methods: The risk ratio (RR) for malignancies was separately compared between FMF patients and fibromyalgia subjects, Still’s disease patients and Behçet’s disease patients. Clinical variables associated with cancer development in FMF patients were searched through binary logistic regression. Results: 580 FMF patients and 102 fibromyalgia subjects, 1012 Behçet’s disease patients and 497 Still’s disease patients were enrolled. The RR for the occurrence of malignant neoplasms was 0.26 (95% Confidence Interval [CI.] 0.10-0.73, p=0.006) in patients with FMF compared to fibromyalgia subjects; the RR for the occurrence of malignant cancer was 0.51 (95% CI. 0.23-1.16, p=0.10) in FMF compared to Still’s disease and 0.60 (95% CI. 0.29-1.28, p=0.18) in FMF compared to Behçet’s disease. At logistic regression, the risk of occurrence of malignant neoplasms in FMF patients was associated with the age at disease onset (b1 = 0.039, 95% CI. 0.001-0.071, p=0.02), the age at the diagnosis (b1 = 0.048, 95% CI. 0.039-0.085, p=0.006), the age at the enrolment (b1 = 0.05, 95% CI. 0.007-0.068, p=0.01), the number of attacks per year (b1 = 0.011, 95% CI. 0.001- 0.019, p=0.008), the use of biotechnological agents (b1 = 1.77, 95% CI. 0.43-3.19, p=0.009), the use of anti-IL-1 agents (b1 = 2.089, 95% CI. 0.7- 3.5, p=0.002). Conclusions: The risk for cancer is reduced in Caucasic FMF patients; however, when malignant neoplasms occur, this is more frequent in FMF cases suffering from a severe disease phenotype and presenting a colchicine-resistant disease.

AB - Objective: Inflammation has been associated with an increased risk for cancer development, while innate immune system activation could counteract the risk for malignancies. Familial Mediterranean fever (FMF) is a severe systemic inflammatory condition and also represents the archetype of innate immunity deregulation. Therefore, the aim of this study is to investigate the risk for cancer development in FMF. Methods: The risk ratio (RR) for malignancies was separately compared between FMF patients and fibromyalgia subjects, Still’s disease patients and Behçet’s disease patients. Clinical variables associated with cancer development in FMF patients were searched through binary logistic regression. Results: 580 FMF patients and 102 fibromyalgia subjects, 1012 Behçet’s disease patients and 497 Still’s disease patients were enrolled. The RR for the occurrence of malignant neoplasms was 0.26 (95% Confidence Interval [CI.] 0.10-0.73, p=0.006) in patients with FMF compared to fibromyalgia subjects; the RR for the occurrence of malignant cancer was 0.51 (95% CI. 0.23-1.16, p=0.10) in FMF compared to Still’s disease and 0.60 (95% CI. 0.29-1.28, p=0.18) in FMF compared to Behçet’s disease. At logistic regression, the risk of occurrence of malignant neoplasms in FMF patients was associated with the age at disease onset (b1 = 0.039, 95% CI. 0.001-0.071, p=0.02), the age at the diagnosis (b1 = 0.048, 95% CI. 0.039-0.085, p=0.006), the age at the enrolment (b1 = 0.05, 95% CI. 0.007-0.068, p=0.01), the number of attacks per year (b1 = 0.011, 95% CI. 0.001- 0.019, p=0.008), the use of biotechnological agents (b1 = 1.77, 95% CI. 0.43-3.19, p=0.009), the use of anti-IL-1 agents (b1 = 2.089, 95% CI. 0.7- 3.5, p=0.002). Conclusions: The risk for cancer is reduced in Caucasic FMF patients; however, when malignant neoplasms occur, this is more frequent in FMF cases suffering from a severe disease phenotype and presenting a colchicine-resistant disease.

KW - Familial Mediterranean fever

UR - http://hdl.handle.net/10807/278696

U2 - 10.3389/fimmu.2024.1397890

DO - 10.3389/fimmu.2024.1397890

M3 - Article

SN - 1664-3224

VL - 15

SP - 1

EP - 12

JO - Frontiers in Immunology

JF - Frontiers in Immunology

ER -

Risk for cancer development in familial Mediterranean fever and associated predisposing factors: an ambidirectional cohort study from the international AIDA Network registries

Abstract

Keywords

UN SDGs

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