自身炎性疾病(AID)是表现为无菌性炎症发作的罕见疾病。这些涉及多个器官,可引起急性器官损伤和严重的长期影响,比如淀粉样变.针对一些AID建立了疾病特异性抗炎治疗策略。然而,他们的临床过程通常包括复发,不受控制的条件。因此,需要新的治疗方法。Janus激酶抑制剂(JAKi)阻断AID发病机理的关键细胞因子,可能是一种潜在的选择。
■根据PRISMA指南对文献进行了系统回顾。三个数据库(MEDLINE,搜索Embase和Cochrane中央对照试验登记册),以获取有关将JAKI用于AID的出版物。从所包括的出版物中提取数据并进行叙述性合成。定义并应用了定义治疗反应的标准。
■我们报告了38篇出版物的数据,共有101例患者描述了JAKi在AID中的作用。关于I型干扰素病的数据,成人发作的静止病(AOSD),系统性幼年特发性关节炎(sJIA),家族性地中海热(FMF),并确定了Behçet综合征(BS)。在总共52例I型干扰素病患者中,7名患者(7/52,13.5%)达到完全缓解,大多数(35/52,67.3%)显示部分反应,少数(10/52,19.2%)显示无治疗反应.对于AOSD,11例(11/26,42.3%)患者均获得完全或部分缓解.两名sJIA患者达到完全反应(2/4,50%),在两例(2/4,50%)中报告了部分反应。一半的FMF患者显示完全反应,另一半有部分反应(3/6,50.0%)。在BS患者中,大多数达到部分应答(8/13,61.5%)。5例患者对治疗无反应(5/13,38.5%)。总的来说,最常见的AE是上呼吸道感染(17),肺炎(10),BK病毒病毒血症(10)和病毒血症(4),带状疱疹感染(5),病毒性胃肠炎(2)和其他感染(4)。
■本系统综述的结果表明,JAKi在某些AID中可以是有益的。AE的风险,尤其是病毒感染,应该考虑。为了准确评估JAKI的风险收益比,应进行临床试验。
UNASSIGNED: Autoinflammatory diseases (AID) are rare diseases presenting with episodes of sterile inflammation. These involve multiple organs and can cause both acute organ damage and serious long-term effects, like amyloidosis. Disease-specific anti-inflammatory therapeutic strategies are established for some AID. However, their clinical course frequently includes relapsing, uncontrolled conditions. Therefore, new therapeutic approaches are needed. Janus Kinase inhibitors (JAKi) block key cytokines of AID pathogenesis and can be a potential option.
UNASSIGNED: A systematic
review of the literature in accordance with the PRISMA guidelines was conducted. Three databases (MEDLINE, Embase and Cochrane Central Register of Controlled Trials) were searched for publications regarding the use of JAKi for AID. Data from the included publications was extracted and a narrative synthesis was performed. Criteria for defining treatment response were defined and applied.
UNASSIGNED: We report data from 38 publications with a total of 101 patients describing the effects of JAKi in AID. Data on Type I Interferonopathies, Adult-Onset Still\'s Disease (AOSD), Systemic Juvenile Idiopathic Arthritis (sJIA), Familial Mediterranean Fever (FMF), and Behçet\'s Syndrome (BS) was identified. From a total of 52 patients with type I interferonopathies, in seven patients (7/52, 13.5%) a complete response was achieved, most (35/52, 67.3%) showed a partial response and a minority (10/52, 19.2%) showed no treatment response. For AOSD, a complete or a partial response was achieved by eleven (11/26, 42.3%) patients each. Two sJIA patients achieved complete response (2/4, 50%) and in two cases (2/4, 50%) a partial response was reported. Half of FMF patients showed a complete response and the other half had a partial one (3/6, 50.0%). Amongst BS patients most achieved a partial response (8/13, 61.5%). Five patients showed no response to therapy (5/13, 38.5%). Overall, the most frequent AEs were upper respiratory tract infections (17), pneumonia (10), BK virus viremia (10) and viruria (4), herpes zoster infection (5), viral gastroenteritis (2) and other infections (4).
UNASSIGNED: The results from this systematic
review show that JAKi can be beneficial in certain AID. The risk of AEs, especially viral infections, should be considered. To accurately assess the risk benefit ratio of JAKi for AID, clinical trials should be conducted.