About the complexity of adult onset Still’s disease… and advances still required for its management

BMC Medicine

Commentary

Alain Le Quellec¹  Philippe Guilpain² 

[1] Montpellier 1 University, Medical School, F-34967, Montpellier, France;Department of Internal Medicine – Multiorganic Diseases, University of Montpellier, Local Referral Center for Auto-Immune Diseases, Saint-Eloi Hospital, F-34295, Montpellier, France;Montpellier 1 University, Medical School, F-34967, Montpellier, France;Department of Internal Medicine – Multiorganic Diseases, University of Montpellier, Local Referral Center for Auto-Immune Diseases, Saint-Eloi Hospital, F-34295, Montpellier, France;Inserm, U 1183, IRMB, Saint-Eloi Hospital, F-34295, Montpellier, France;

关键词: Adult onset Still’s disease;    Macrophage activation syndrome;    Score;    Diagnosis;    Prognosis;    Therapy;    Cytokine;    Inflammasome;   

DOI  :  10.1186/s12916-016-0769-1

 received in 2016-12-09, accepted in 2016-12-09,  发布年份 2017

来源: Springer

【 摘 要 】

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.

【 授权许可】

CC BY   
© The Author(s). 2017