PDF(Pubmed)
Abstract：
Familial Mediterranean fever (FMF), the most frequent monogenic autoinflammatory disease, is manifested with recurrent and chronic inflammation and amyloid A (AA) amyloidosis, driven by overproduction of interleukin 1 (IL-1) through an activated pyrin inflammasome. Consequently, non-responsiveness to colchicine, the cornerstone of FMF treatment, is nowadays addressed by IL-1- blockers. Each of the two IL-1 blockers currently used in FMF, anakinra and canakinumab, has its own merits for FMF care. Here we focus on anakinra, a recombinant form of the naturally occurring IL-1 receptor antagonist, and explore the literature by using PubMed regarding the utility of anakinra in certain conditions of FMF. Occasionally we enrich published data with our own experience. To facilitate insights to anakinra role, the paper briefs some clinical, genetic, pathogenetic, and management aspects of FMF. The clinical settings of FMF covered in this review include colchicine resistance, AA amyloidosis, renal transplantation, protracted febrile myalgia, on- demand use, leg pain, arthritis, temporary suspension of colchicine, pediatric patients, and pregnancy and lactation. In many of these instances, either because of safety concerns or a necessity for only transient and short-term use, anakinra, due to its short half-life, is the preferred IL-1 blocker.
摘要：
家族性地中海热（FMF），最常见的单基因自身炎症性疾病，表现为复发性和慢性炎症和淀粉样蛋白A（AA）淀粉样变性，由通过激活的pyrin炎性体的白细胞介素1（IL-1）的过量产生驱动。因此,对秋水仙碱无反应，FMF治疗的基石，现在由IL-1-阻断剂解决。目前FMF中使用的两种IL-1阻断剂中的每一种，anakinra和canakinumab,FMF护理有其自身的优点。这里我们关注阿纳金拉，天然存在的IL-1受体拮抗剂的重组形式，并通过使用PubMed探索有关anakinra在FMF的某些条件下的效用的文献。有时，我们会根据自己的经验丰富已发布的数据。为了促进对阿纳金拉角色的洞察，这篇论文简要介绍了一些临床，遗传,致病,和FMF的管理方面。本综述涵盖的FMF的临床设置包括秋水仙碱耐药性，AA淀粉样变性,肾移植,持续性高热肌痛，按需使用，腿部疼痛,关节炎,暂时使用秋水仙碱,儿科患者,怀孕和哺乳。在许多情况下，出于安全考虑或只需要暂时和短期使用，anakinra,由于其半衰期短，是优选的IL-1阻断剂。
