炎症与癌症发生风险增加有关，而先天免疫系统的激活可以抵消恶性肿瘤的风险。家族性地中海热（FMF）是一种严重的全身炎症性疾病，也代表先天免疫失调的原型。因此，本研究的目的是调查FMF患癌症的风险。分别比较FMF患者与纤维肌痛受试者、斯蒂尔病患者和白塞病患者的恶性肿瘤风险比（RR）。通过二元 Logistic 回归搜索与 FMF 患者癌症发展相关的临床变量。纳入了 580 名 FMF 患者和 102 名纤维肌痛受试者、1012 名白塞病患者和 497 名斯蒂尔病患者。与纤维肌痛受试者相比，FMF 患者发生恶性肿瘤的 RR 为 0.26（95% 置信区间 [CI.] 0.10-0.73，p=0.006）；与斯蒂尔病相比，FMF 中发生恶性肿瘤的 RR 为 0.51 (95% CI. 0.23-1.16, p=0.10)，与白塞病相比，FMF 中发生恶性肿瘤的 RR 为 0.60 (95% CI. 0.29-1.28, p=0.18) 。在logistic回归中，FMF患者发生恶性肿瘤的风险与发病年龄（β1 = 0.039，95% CI.0.001-0.071，p=0.02）、诊断时年龄（β1 = 0.048， 95% CI. 0.039-0.085，p=0.006）、入组年龄（β1 = 0.05，95% CI. 0.007-0.068，p=0.01）、每年发作次数（β1 = 0.011，95% CI） 0.001- 0.019，p=0.008），使用生物技术药物（β1 = 1.77，95% CI。0.43-3.19，p=0.009），使用抗 IL-1 药物（β1 = 2.089，95% CI） . 0.7-3.5, p=0.002)。高加索 FMF 患者患癌症的风险降低；然而，当发生恶性肿瘤时，这种情况在患有严重疾病表型并呈现秋水仙碱耐药性疾病的 FMF 病例中更为常见。版权所有 © 2024 Vitale, Caggiano, Tufan, Ragab, Batu, Portincasa, Aragona, Sota, Conti, De保利斯、里甘特、奥利维埃里、萨辛、拉托雷、洛帕尔科、卡塔利尼、马吉奥、因萨拉科、斯菲卡基斯、韦雷基亚、耶尔德勒姆、库库克、卡尔达斯、莱穆纳、加内马、萨阿德、塞内尔、埃坎·埃姆雷奥尔、奥森、贾比尔、哈利勒、迪西亚乌拉、加贾诺, Malizia, Affronti, 帕特罗尼蒂, 罗密欧, 斯巴尔基耶罗, 德拉卡萨, 莫米尔, 西尔瓦罗利, 吉奇诺, 切利克, 塔西亚, 卡拉马纳科斯, 埃尔南德斯-罗德里格斯, 帕龙奇, 奥普里斯-贝林斯基, 巴罗内, 雷克, 科斯蒂, 斯弗里索, 贾迪尼, 真蒂莱斯基, 维西克-斯泽夫奇克、瓦西、洛孔特、扬兹-罗日克、马丁-纳雷斯、托雷斯-鲁伊斯、考利、孔福尔蒂、埃米、李·戈比、比亚西、特里比利、鲁西蒂、德尔朱迪斯、塔尔瓦特、布鲁卡托、奥贡吉米、伊诺霍萨-阿佐拉、巴里斯特雷、法比亚尼、弗雷迪亚尼和坎塔里尼。

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.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.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 (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).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.Copyright © 2024 Vitale, Caggiano, Tufan, Ragab, Batu, Portincasa, Aragona, Sota, Conti, De Paulis, Rigante, Olivieri, Şahin, La Torre, Lopalco, Cattalini, Maggio, Insalaco, Sfikakis, Verrecchia, Yildirim, Kucuk, Kardas, Laymouna, Ghanema, Saad, Sener, Ercan Emreol, Ozen, Jaber, Khalil, Di Ciaula, Gaggiano, Malizia, Affronti, Patroniti, Romeo, Sbalchiero, Della Casa, Mormile, Silvaroli, Gicchino, Çelik, Tarsia, Karamanakos, Hernández-Rodríguez, Parronchi, Opris-Belinski, Barone, Recke, Costi, Sfriso, Giardini, Gentileschi, Wiesik-Szewczyk, Vasi, Loconte, Jahnz-Różyk, Martín-Nares, Torres-Ruiz, Cauli, Conforti, Emmi, Li Gobbi, Biasi, Terribili, Ruscitti, Del Giudice, Tharwat, Brucato, Ogunjimi, Hinojosa-Azaola, Balistreri, Fabiani, Frediani and Cantarini.