Unmet needs and research gaps in Still's disease across ages: proceedings from a pediatric and adult joint expert panel.

IF 2.8 3区 医学 Q1 PEDIATRICS
Claudia Bracaglia, Francesca Minoia, Sebastiaan J Vastert, Christoph Kessel, Lorenzo Dagna, Angelo Ravelli, Fabrizio De Benedetti
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引用次数: 0

Abstract

Background: Still's disease (SD), including systemic juvenile idiopathic arthritis (sJIA) and adult-onset SD (AOSD), is an inflammatory condition typically characterized by daily fever, arthritis, and skin rash together with neutrophilic leukocytosis, thrombocytosis, and increased acute phase reactants. The reported differences between sJIA and AOSD appear to reflect variations along an inflammatory spectrum influenced by age, rather than differences in the underlying pathology.

Methods: In February 2023, an expert meeting, including pediatric and adult rheumatologists, was held in Rome, Italy, with the aim of defining more precise and timely strategies for disease management. The following four topics were discussed: (1) early recognition and diagnosis of SD; (2) pathogenetic pathways and possible biomarkers for diagnosis and response; (3) refractory disease and risk factors, and (4) treatment of SD and its complications.

Results: The development of improved diagnostic criteria and validation of biomarkers are important steps towards achieving early diagnosis, although several biomarkers remain to be universally validated and available for clinical practice. Additionally, awareness of important complications of SD, including macrophage activation syndrome and lung disease, is crucial for improving patient outcomes, alongside an improved understanding of risk factors for the development of refractory disease. While interleukin (IL)-1 and IL-6 inhibitors have improved the treatment landscape of SD, harmonizing the therapeutic approach across centers and countries, together with developing treatment strategies for refractory patients, still represents a challenge.

Conclusions: Here, we summarize the results of discussions among experts, supplemented by relevant literature, and highlight unmet needs in the diagnosis and management of SD.

不同年龄斯蒂尔氏病未满足的需求和研究差距:来自儿科和成人联合专家小组的会议记录。
背景:斯蒂尔氏病(SD),包括全身性幼年特发性关节炎(sJIA)和成人发病SD (AOSD),是一种炎症性疾病,典型特征为每日发热、关节炎和皮疹,同时伴有中性粒细胞增多、血小板增多和急性期反应物增加。报道的sJIA和AOSD之间的差异似乎反映了受年龄影响的炎症谱的变化,而不是潜在病理的差异。方法:2023年2月,在意大利罗马举行了一次专家会议,包括儿科和成人风湿病学家,目的是确定更精确和及时的疾病管理策略。讨论了以下四个主题:(1)SD的早期识别和诊断;(2)发病途径和可能用于诊断和反应的生物标志物;(3)难治性疾病和危险因素;(4)SD及其并发症的治疗。结果:改进诊断标准和验证生物标志物的发展是实现早期诊断的重要步骤,尽管一些生物标志物仍有待普遍验证并可用于临床实践。此外,意识到SD的重要并发症,包括巨噬细胞激活综合征和肺部疾病,对于改善患者的预后至关重要,同时也提高了对难治性疾病发展的危险因素的理解。虽然白细胞介素(IL)-1和IL-6抑制剂改善了SD的治疗前景,但协调不同中心和国家的治疗方法,以及为难治性患者制定治疗策略,仍然是一个挑战。结论:在此,我们总结了专家讨论的结果,并辅以相关文献,突出了SD诊断和管理中尚未满足的需求。
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
Pediatric Rheumatology
Pediatric Rheumatology PEDIATRICS-RHEUMATOLOGY
CiteScore
4.10
自引率
8.00%
发文量
95
审稿时长
>12 weeks
期刊介绍: Pediatric Rheumatology is an open access, peer-reviewed, online journal encompassing all aspects of clinical and basic research related to pediatric rheumatology and allied subjects. The journal’s scope of diseases and syndromes include musculoskeletal pain syndromes, rheumatic fever and post-streptococcal syndromes, juvenile idiopathic arthritis, systemic lupus erythematosus, juvenile dermatomyositis, local and systemic scleroderma, Kawasaki disease, Henoch-Schonlein purpura and other vasculitides, sarcoidosis, inherited musculoskeletal syndromes, autoinflammatory syndromes, and others.
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