目的:由于复发性多软骨炎(RP)的罕见性,迄今为止尚未进行随机临床试验,治疗仍是经验性的.我们进行了系统的文献综述,以评估RP中使用的主要常规免疫抑制剂和生物疗法的疗效。
方法:我们搜索了MEDLINE中没有语言限制的原创文章。美国风湿病学会(ACR)和欧洲风湿病协会联盟(EULAR)的摘要也被考虑纳入。选择报告常规免疫抑制剂和生物疗法在成人RP患者中的疗效的观察性研究和临床试验,并计算每种治疗的汇总反应率。
结果:在确定的304篇文章和摘要中,31进行了全文审查,11人被包括在内。这项研究共涉及177名患者,暴露于总共247行处理。研究的主要治疗方法(按品系数)是:TNF抑制剂(TNFi),n=92;甲氨蝶呤(MTX),n=38;托珠单抗(TCZ),n=26;阿纳金拉(ANA),n=21;利妥昔单抗(RTX),n=16;abatacept(ABT),n=14;环磷酰胺(CYC),n=14;硫唑嘌呤(AZA),n=13。所有研究的汇总缓解率为:ABT的72%[95%CI:42-95],TCZ的66%[95%CI:49-82],TNFi的64%[95%CI:53-74],MTX为56%[95%CI:37-73],ANA的47%[95%CI:26-68],RTX的43%[95%CI:20-68]。基于更有限的数据,AZA和CYC的反应率范围为38%至100%和25%至100%,分别。
结论:在本关于复发性多软骨炎治疗的现有证据的系统综述中,ABT,TCZ和TNFi是与最佳结果相关的药物。ABT疗效必须根据接受治疗的患者数量少来解释。虽然MTX的疗效略低,它是数据最可靠的药物之一。
OBJECTIVE: Due to the rarity of relapsing polychondritis (RP), no randomised clinical trial has been conducted to date and treatment remains empirical. We performed a systematic literature
review to assess the efficacy of the main conventional immunosuppressants and biotherapies used in RP.
METHODS: We searched MEDLINE for original articles without language restriction. Abstracts from American College of Rheumatology (ACR) and European Alliance of Associations for Rheumatology (EULAR) were also considered for inclusion. Observational studies and clinical trials reporting on the efficacy of conventional immunosuppressants and biotherapies in adult patients with RP were selected and pooled response rates for each treatment were computed.
RESULTS: Of 304 articles and abstracts identified, 31 underwent full-text
review, and 11 were included. The studies involved a total of 177 patients, exposed to a total of 247 lines of treatments. The main treatments studied (by number of lines) were: TNF inhibitors (TNFi), n=92; methotrexate (MTX), n=38; tocilizumab (TCZ), n=26; anakinra (ANA), n=21; rituximab (RTX), n=16; abatacept (ABT), n=14; cyclophosphamide (CYC), n=14; azathioprine (AZA), n=13. The pooled response rates across studies were: 72% [95% CI: 42-95] for ABT, 66% [95% CI: 49-82] for TCZ, 64% [95% CI: 53-74] for TNFi, 56% [95% CI: 37-73] for MTX, 47% [95% CI: 26-68] for ANA, 43% [95% CI: 20-68] for RTX. Based on more limited data, response rates for AZA and CYC ranged from 38 to 100% and from 25 to 100%, respectively.
CONCLUSIONS: In this systematic
review of available evidence regarding the treatment of relapsing polychondritis, ABT, TCZ and TNFi were the drugs associated with the best outcomes. ABT efficacy must be interpreted in light of the small number of patients treated. While MTX had slightly less efficacy, it is one of the drugs for which data are the most robust.