IL-1 targeting agents in Schnitzler syndrome: a multicentre, real-world study from the international AIDA Network Schnitzler Registry.

IF 3.4 4区医学 Q2 RHEUMATOLOGY

Clinical and experimental rheumatology Pub Date : 2025-10-01 Epub Date: 2025-10-23 DOI:10.55563/clinexprheumatol/i9hsfr

Laura Calabrese, Alessandra Cartocci, Antonio Vitale, Eduardo Martín-Nares, Martina D'Onghia, Valeria Caggiano, Jiram Torres-Ruiz, Roberta Lopez, Karina Jahnz-Rozyk, Katarzyna Rybak, Micol Frassi, Franco Franceschini, Francesca Crisafulli, Abdurrahman Tufan, Hamit Kucuk, Aslihan Avanoglu Guler, Antonio Gidaro, Leyla La Cava, Francesca Della Casa, Ilaria Mormile, Elisa Cinotti, Giuseppe Lopalco, Jurgen Sota, Jessica Sbalchiero, Giacomo Emmi, Andreas Recke, Stefania Costi, Paolo Sfriso, Sara Monti, Ombretta Viapiana, Andrea Hinojosa-Azaola, Alberto Balistreri, Claudia Fabiani, Bruno Frediani, Pietro Rubegni, Ewa Wiesik-Szewczyk, Luca Cantarini

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引用次数: 0

Abstract

Objectives: Schnitzler syndrome (SchS) is a rare autoinflammatory disease characterised by a primary pathogenic involvement of interleukin (IL)-1. Therefore, IL-1 blockers are currently considered the optimal therapeutic option for SchS patients. However, while IL-1 blockers are first-line for SchS, long-term real-world evidence is limited by the rarity of the disease. We assessed the long-term effectiveness and safety of the IL-1 inhibitors anakinra and canakinumab used in SchS, also looking for variables capable of affecting global effectiveness and drug retention over time.

Methods: Data analysed in this study were drawn from the international AutoInflammatory Disease Alliance (AIDA) Registry dedicated to SchS.

Results: 28 SchS patients corresponding to 37 treatment lines were included in the study. Complete and partial responses occurred in 73.1% and 29.9% of anakinra-treated patients, and 66.8% and 33.3% with canakinumab. The overall anakinra and canakinumab drug retention rates at 12-, 36-, and 60-month follow-up were 85.6%, 81.7% and 64.7%, respectively; the probability of discontinuing IL-1 inhibitors at 12-, 36- and 60 months due to loss of effectiveness was 9.6%, 13.7% and 24.5%, respectively. The maximum IgG M-protein levels were found to be significantly higher in patients achieving partial response compared to those benefiting from complete response (p=0.032). Lymphadenopathy independently predicted anti-IL-1 discontinuation due to loss of effectiveness (HR 7.78, 95% CI: 1.27-47.9; p=0.027).

Conclusions: The present study confirms the high effectiveness of IL-1 inhibitors in controlling SchS, including the complete and partial response rates and the long-term survival. Elevated IgG M-protein levels and the presence of lymphadenopathy should be considered as potential indicators for identifying patients more likely to exhibit a partial response and a possible loss of treatment efficacy.

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施尼茨勒综合征中的IL-1靶向药物：来自国际AIDA网络施尼茨勒登记处的多中心真实世界研究

目的：施尼茨勒综合征（SchS）是一种罕见的自身炎症性疾病，其主要病原为白细胞介素(IL)-1。因此，IL-1阻滞剂目前被认为是SchS患者的最佳治疗选择。然而，尽管IL-1阻滞剂是治疗SchS的一线药物，但由于这种疾病的罕见性，长期的现实证据受到限制。我们评估了用于SchS的IL-1抑制剂anakinra和canakinumab的长期有效性和安全性，同时寻找能够影响全局有效性和药物保留的变量。方法：本研究分析的数据来自国际自身炎症疾病联盟（AIDA）专门用于SchS的登记处。结果：共纳入37条治疗线的28例SchS患者。anakinra治疗组的完全缓解和部分缓解分别为73.1%和29.9%，而canakinumab治疗组的完全缓解和部分缓解分别为66.8%和33.3%。在12个月、36个月和60个月的随访中，阿那单抗和卡那单抗的总体药物保留率分别为85.6%、81.7%和64.7%；IL-1抑制剂在12个月、36个月和60个月因疗效丧失而停用的概率分别为9.6%、13.7%和24.5%。获得部分缓解的患者的最大IgG m蛋白水平明显高于获得完全缓解的患者（p=0.032）。淋巴结病独立预测抗il -1药物因疗效丧失而停药（HR 7.78, 95% CI: 1.27-47.9; p=0.027）。结论：本研究证实了IL-1抑制剂在控制SchS方面的高有效性，包括完全缓解率和部分缓解率以及长期生存。IgG m蛋白水平升高和淋巴结病变的存在应被视为识别更可能表现出部分反应和可能失去治疗效果的患者的潜在指标。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Clinical and experimental rheumatology 医学-风湿病学

CiteScore

6.10

自引率

18.90%

发文量

377

审稿时长

3-6 weeks

期刊介绍： Clinical and Experimental Rheumatology is a bi-monthly international peer-reviewed journal which has been covering all clinical, experimental and translational aspects of musculoskeletal, arthritic and connective tissue diseases since 1983.