Piero Ruscitti , Luca Cantarini , Francesco Ciccia , Fabrizio Conti , Lorenzo Dagna , Florenzo Iannone , Carlomaurizio Montecucco , Pistone Giovanni , Paolo Sfriso , Roberto Giacomelli
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引用次数: 0
Abstract
Still's disease is an inflammatory syndrome affecting patients across all ages, previously known as systemic juvenile idiopathic arthritis (sJIA) in children and adult-onset Still's disease (AOSD) in adults. Multiple lines of evidence reported overlapping clinical features between sJIA and AOSD, commonly manifesting with daily fever, arthritis, evanescent salmon-coloured skin rash. The concomitant various degree of multiorgan involvement may increase the heterogeneity of the patient clinical picture. In active patients, a typical hyperferritinemia is recognized in association with increases of erythrocyte sedimentation rate and C reactive protein. Concerning pathogenesis, also in this case, similar mechanisms are reported in sJIA and AOSD involving both innate and adaptive arms of the immune systems; thus, Still's disease is peculiarly codified at the cross-road of autoinflammatory and autoimmune disorders. Furthermore, life-threatening complications burden the disease course in challenging the management of these patients, mainly macrophage activation syndrome, and worsening the prognosis. Concerning the treatment, glucocorticoids (GCs), conventional synthetic disease-modifying anti rheumatic drugs (csDMARDs) and biologic DMARDs (bDMARDs), mainly IL-1 inhibitors, are administered to treat these patients. Usually, bDMARDs are considered in case of failure of GCs or GC-dependence. However, in some circumstances, bDMARDs may be administered as first-line modifying therapy without GCs, thus avoiding GC predictable side effects and optimizing the long-term outcome.
In this work, we aimed to synthetize the recent available literature considering the clinical management of patients with Still's disease, reviewing features about early diagnosis, optimal treatment algorithm, clinical therapeutic targets, treatment of complications, and patient monitoring in the follow-up.
期刊介绍:
Autoimmunity Reviews is a publication that features up-to-date, structured reviews on various topics in the field of autoimmunity. These reviews are written by renowned experts and include demonstrative illustrations and tables. Each article will have a clear "take-home" message for readers.
The selection of articles is primarily done by the Editors-in-Chief, based on recommendations from the international Editorial Board. The topics covered in the articles span all areas of autoimmunology, aiming to bridge the gap between basic and clinical sciences.
In terms of content, the contributions in basic sciences delve into the pathophysiology and mechanisms of autoimmune disorders, as well as genomics and proteomics. On the other hand, clinical contributions focus on diseases related to autoimmunity, novel therapies, and clinical associations.
Autoimmunity Reviews is internationally recognized, and its articles are indexed and abstracted in prestigious databases such as PubMed/Medline, Science Citation Index Expanded, Biosciences Information Services, and Chemical Abstracts.