Kontzias Apostolos, Petryna Olga, Nakasato Priscila, Efthimiou Petros
Department of Internal Medicine, Division of Rheumatology, Allergy and Immunology, Stony Brook University Hospital, Stony Brook, NY, USA.
Department of Medicine, White Plains Hospital, White Plains, NY, USA.
Mediterr J Rheumatol. 2024 Mar 30;35(Suppl 1):45-57. doi: 10.31138/mjr.290323.dat. eCollection 2024 Mar.
We have summarised the existing evidence supporting the concept that systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD) are part of the same Still's disease spectrum.
A PubMed/Embase database search was conducted using specific search strings and free text words to screen for relevant articles. The search was limited to studies in humans, published up to June 2023, in English-language.
sJIA and AOSD are rare autoinflammatory disorders that have similar pathophysiological and clinical features. The clinical presentations of sJIA and AOSD are highly variable, with differential diagnoses that include a broad range of malignancies, infectious diseases, and autoimmune disorders, which contribute to delays in diagnosis. Several sets of classification exist to help diagnose patients in clinical practice; the International League of Associations for Rheumatology criteria for sJIA and the Yamaguchi and Fautrel criteria for AOSD are the most-used criteria. The therapeutic strategy for Still's disease aims to relieve signs and symptoms, prevent irreversible joint damage and potentially life-threatening complications, and avoid deleterious side effects of treatment. Recently, targeted therapies such as interleukin (IL)-1 and IL-6 inhibitors have become available for the treatment of sJIA and AOSD. While these biologics were originally largely reserved for patients in whom non-steroidal anti-inflammatory drugs, corticosteroids and conventional synthetic disease-modifying anti-rheumatic drugs had failed, they are increasingly used earlier in the treatment paradigm. Among IL-1 inhibitors, canakinumab is the only biologic approved in the US for the treatment of both sJIA and AOSD.
我们总结了现有证据，支持系统性幼年特发性关节炎（sJIA）和成人斯蒂尔病（AOSD）属于同一斯蒂尔病谱系这一概念。
使用特定检索词和自由文本词在PubMed/Embase数据库中进行检索，以筛选相关文章。检索仅限于截至2023年6月发表的英文人类研究。
sJIA和AOSD是罕见的自身炎症性疾病，具有相似的病理生理和临床特征。sJIA和AOSD的临床表现高度可变，鉴别诊断包括多种恶性肿瘤、传染病和自身免疫性疾病，这导致诊断延迟。临床实践中有几套分类方法可帮助诊断患者；sJIA的国际风湿病联盟标准和AOSD的山口和富特雷标准是最常用的标准。斯蒂尔病的治疗策略旨在缓解症状、预防不可逆的关节损伤和潜在的危及生命的并发症，并避免治疗的有害副作用。最近，白细胞介素（IL）-1和IL-6抑制剂等靶向治疗药物已可用于治疗sJIA和AOSD。虽然这些生物制剂最初主要用于非甾体抗炎药、皮质类固醇和传统合成改善病情抗风湿药治疗失败的患者，但它们在治疗模式中越来越早地被使用。在IL-1抑制剂中，卡那单抗是美国唯一获批用于治疗sJIA和AOSD的生物制剂。
