1. Risk for cancer development in familial Mediterranean fever and associated predisposing factors: an ambidirectional cohort study from the international AIDA Network registries
- Author
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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, and Luca Cantarini
- Subjects
autoinflammatory diseases ,FMF ,tumor ,neoplasm ,rare diseases ,treatment ,Immunologic diseases. Allergy ,RC581-607 - Abstract
ObjectiveInflammation 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.MethodsThe 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.Results580 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 (β1 = 0.039, 95% CI. 0.001-0.071, p=0.02), the age at the diagnosis (β1 = 0.048, 95% CI. 0.039-0.085, p=0.006), the age at the enrolment (β1 = 0.05, 95% CI. 0.007-0.068, p=0.01), the number of attacks per year (β1 = 0.011, 95% CI. 0.001- 0.019, p=0.008), the use of biotechnological agents (β1 = 1.77, 95% CI. 0.43-3.19, p=0.009), the use of anti-IL-1 agents (β1 = 2.089, 95% CI. 0.7-3.5, p=0.002).ConclusionsThe 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.
- Published
- 2024
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