Wolfson Medical Center, Holon, Israel.
Tel Aviv University, Tel Aviv, Israel.
Arthritis Rheumatol. 2018 Jan;70(1):127-133. doi: 10.1002/art.40344. Epub 2017 Dec 7.
Familial Mediterranean fever (FMF) is an autoinflammatory disease manifested as recurrent serosal inflammation. An association between FMF and malignancy has not been evaluated. The aim of this study was to estimate cancer risk in a large cohort of FMF patients from a single institution.
The study cohort consisted of 8,534 FMF patients registered at the National FMF Center in Tel Hashomer, Israel. We linked the study cohort to the database of the Israel National Cancer Registry using the national identity number. Cancer incidence in FMF patients was determined and then stratified by age and sex. Standardized incidence ratios (SIRs) for cancers were calculated.
Among 8,534 FMF patients (4,400 men, 4,134 women), 350 developed cancer during the years 1970-2011. The overall cancer risk among patients with FMF was significantly lower than was expected in specific sex and ethnic groups of the Israeli population: for males of Jewish ethnicity, SIR 0.66 (95% confidence interval [95% CI] 0.55-0.77), P < 0.001; for females of Jewish ethnicity, SIR 0.75 (95% CI 0.64-0.86), P < 0.001; and for males of Arab ethnicity, SIR 0.34 (95% CI 0.07-0.99), P = 0.024.
FMF patients have a significantly lower incidence of cancer than the general population of Israel. This pattern was demonstrated in 2 ethnic populations: Jewish and Arab. We speculate that the lower cancer incidence could be attributed to a direct physiologic effect of FMF or to its treatment.
家族性地中海热（FMF）是一种以反复发作的浆膜炎为特征的自身炎症性疾病。FMF 与恶性肿瘤之间的关联尚未得到评估。本研究旨在评估来自单一机构的大型 FMF 患者队列的癌症风险。
研究队列由以色列特拉维夫哈索尔默国家 FMF 中心注册的 8534 名 FMF 患者组成。我们使用国家身份证号将研究队列与以色列国家癌症登记数据库相关联。确定 FMF 患者的癌症发病率，然后按年龄和性别进行分层。计算癌症的标准化发病比（SIR）。
在 8534 名 FMF 患者（4400 名男性，4134 名女性）中，350 名在 1970-2011 年期间发生了癌症。FMF 患者的总体癌症风险明显低于以色列特定性别和种族群体的预期：对于犹太裔男性，SIR 为 0.66（95%置信区间[95%CI]0.55-0.77），P<0.001；对于犹太裔女性，SIR 为 0.75（95%CI 0.64-0.86），P<0.001；对于阿拉伯裔男性，SIR 为 0.34（95%CI 0.07-0.99），P=0.024。
FMF 患者的癌症发病率明显低于以色列普通人群。这种模式在 2 个人群中得到了证明：犹太人和阿拉伯人。我们推测，较低的癌症发病率可能归因于 FMF 的直接生理效应或其治疗。
