Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.
Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy.
Front Immunol. 2024 May 10;15:1397890. doi: 10.3389/fimmu.2024.1397890. eCollection 2024.
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, =0.10) in FMF compared to Still's disease and 0.60 (95% CI. 0.29-1.28, =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, =0.02), the age at the diagnosis (β1 = 0.048, 95% CI. 0.039-0.085, =0.006), the age at the enrolment (β1 = 0.05, 95% CI. 0.007-0.068, =0.01), the number of attacks per year (β1 = 0.011, 95% CI. 0.001- 0.019, =0.008), the use of biotechnological agents (β1 = 1.77, 95% CI. 0.43-3.19, =0.009), the use of anti-IL-1 agents (β1 = 2.089, 95% CI. 0.7-3.5, =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.
炎症与癌症发展风险增加有关，而先天免疫系统的激活可能会对抗恶性肿瘤的风险。家族性地中海热（FMF）是一种严重的系统性炎症性疾病，也是先天免疫失调的典型代表。因此，本研究旨在研究 FMF 患者癌症发展的风险。
通过二元逻辑回归，分别比较 FMF 患者和纤维肌痛患者、Still 病患者和 Behcet 病患者之间恶性肿瘤的风险比（RR）。
纳入 580 例 FMF 患者和 102 例纤维肌痛患者、1012 例 Behcet 病患者和 497 例 Still 病患者。与纤维肌痛患者相比，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 Still 病相比，FMF 患者发生恶性癌症的 RR 为 0.51（95%CI.0.23-1.16，=0.10），与 Behcet 病相比，FMF 患者发生恶性癌症的 RR 为 0.60（95%CI.0.29-1.28，=0.18）。在逻辑回归中，FMF 患者恶性肿瘤发生的风险与发病年龄（β1=0.039，95%CI.0.001-0.071，=0.02）、诊断年龄（β1=0.048，95%CI.0.039-0.085，=0.006）、纳入年龄（β1=0.05，95%CI.0.007-0.068，=0.01）、每年发作次数（β1=0.011，95%CI.0.001-0.019，=0.008）、使用生物技术药物（β1=1.77，95%CI.0.43-3.19，=0.009）、使用抗 IL-1 药物（β1=2.089，95%CI.0.7-3.5，=0.002）相关。
白种人 FMF 患者的癌症风险降低；然而，当发生恶性肿瘤时，在患有严重疾病表型且存在秋水仙碱耐药性疾病的 FMF 病例中更为常见。
