Guilpain Philippe, Le Quellec Alain
Montpellier 1 University, Medical School, Montpellier, F-34967, France.
Department of Internal Medicine - Multiorganic Diseases, University of Montpellier, Local Referral Center for Auto-Immune Diseases, Saint-Eloi Hospital, Montpellier, F-34295, France.
BMC Med. 2017 Jan 6;15(1):5. doi: 10.1186/s12916-016-0769-1.
Adult onset Still's disease (AOSD) is a rare inflammatory disorder that remains poorly understood. Its pathophysiology is yet to be completely elucidated, but is known to consist mainly on a cytokine cascade, responsible for the systemic manifestations. AOSD diagnosis is usually difficult and delayed, with physicians having to rule out several other conditions, including cancer or infectious diseases. Prognosis is heterogeneous and difficult to establish, ranging from benign outcome to chronic destructive polyarthritis and/or life-threatening events. In addition, treatment remains to be codified, especially considering the development of new drugs. In this commentary, we attempt to elucidate the complexity of AOSD and to highlight the need of working on prognostic tools for this disorder. We also discuss the numerous advances that would be useful for patients in the daily management of this disease.Please see related article: http://bmcmedicine.biomedcentral.com/articles/ 10.1186/s12916-016-0738-8 .
成人斯蒂尔病(AOSD)是一种罕见的炎症性疾病,目前人们对其了解甚少。其病理生理学尚未完全阐明,但已知主要由细胞因子级联反应组成,该反应导致全身症状。AOSD的诊断通常困难且延迟,医生必须排除其他几种疾病,包括癌症或传染病。预后因人而异且难以确定,范围从良性结果到慢性破坏性多关节炎和/或危及生命的事件。此外,治疗方法仍有待规范,尤其是考虑到新药的研发。在这篇评论中,我们试图阐明AOSD的复杂性,并强调针对这种疾病开发预后工具的必要性。我们还讨论了众多对患者日常管理该病有用的进展。请参阅相关文章:http://bmcmedicine.biomedcentral.com/articles/ 10.1186/s12916-016-0738-8 。