Cas Lek Cesk. 2022 Spring;161(1):3-10.
Monogenic periodic fever syndromes are heterogeneous group of autoinflammatory diseases including distinct syndromes, such as cryopyrin-associated periodic syndrome (CAPS), tumor necrosis factor alpha receptor-associated periodic syndrome (TRAPS), mevalonate kinase deficiency/hyper IgD syndrome (MKD/HIDS), and familial Mediterranean fever (FMF). Individual diseases differ in pathogenesis, clinical manifestations, and severity. However, cytokines from the interleukin 1 (IL-1) family play a key role in all of them. Inhibition of these cytokines, especially IL-1, thus plays a crucial role in their treatment. At present, we have a wide range of drugs that differ in structure, mechanism of action, efficacy, and spectrum of side effects. The most available are anakinra, canakinumab and rilonacept. Moreover, several clinical trials are currently underway with other very promising drugs, such as gevokizumab, tadekinig alfa or tranilast. In the following review, we provide a new perspective on the efficacy and safety of IL-1 inhibitors that have provided the novel results coming from recently published clinical trials.
单基因周期性发热综合征是一组异质性的自身炎症性疾病，包括不同的综合征，如 Cryopyrin 相关周期性综合征 (CAPS)、肿瘤坏死因子受体相关周期性综合征 (TRAPS)、甲羟戊酸激酶缺乏/高 IgD 综合征 (MKD/HIDS) 和家族性地中海热 (FMF)。 每种疾病在发病机制、临床表现和严重程度上都有所不同。 然而，白细胞介素 1 (IL-1) 家族的细胞因子在所有这些疾病中都起着关键作用。 抑制这些细胞因子，尤其是 IL-1，因此在它们的治疗中起着至关重要的作用。 目前，我们有多种结构、作用机制、疗效和副作用谱不同的药物。 最常用的是阿那白滞素、卡那单抗和利纳西普。 此外，目前正在进行几项临床试验，研究其他非常有前途的药物，如 gevokizumab、tadekinig alfa 或曲尼司特。 在以下综述中，我们提供了关于白细胞介素 1 抑制剂的疗效和安全性的新视角，这些抑制剂提供了最近发表的临床试验的新结果。
