Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress 2023 EHA Congress 2023 ASCO Annual Meeting
Clinical Collections
Advances in Alzheimer’s

Keynote webinar | Spotlight on sleep in brain health

LIVE: Thursday 2nd May 2024, 18:00-19:30 (CEST)

Quality sleep is essential for health. But what happens to our brains when sleep patterns are disturbed? Join our experts to explore the interplay between sleep disruption and neurological diseases, and the questions that you need to be asking your patients to help you prevent the harmful effects of sleep deprivation.
ADVANCED SEARCH
Log in
Top
BMC Medicine
Published in: BMC Medicine 1/2017

Open Access 01-12-2017 | Commentary

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

Authors: Philippe Guilpain, Alain Le Quellec

Published in: BMC Medicine | Issue 1/2017

Login to get access

Abstract

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.
Literature
1.
Maria ATJ, Le Quellec A, Jorgensen C, Touitou I, Rivière S, Guilpain P. Adult onset Still’s disease (AOSD) in the era of biologic therapies: dichotomous view for cytokine and clinical expressions. Autoimmun Rev. 2014;13:1149–59.CrossRefPubMed
2.
Ruscitti P, Cipriani P, Masedu F, Iacono D, Ciccia F, Liakouli V, Guggino G, Carubbi F, Berardicurti O, Di Benedetto P, Valenti M, Triolo G, Valentini G, Giacomelli R. Adult onset Still’s disease: evaluation of prognostic tools and validation of the systemic score, by analysis of 100 cases from three centers. BMC Med. 2016;14:194.CrossRefPubMedPubMedCentral
3.
Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol. 1992;19:424–30.PubMed
4.
Fautrel B, Zing E, Golmard J-L, Le Moel G, Bissery A, Rioux C, et al. Proposal for a new set of classification criteria for adult-onset still disease. Medicine (Baltimore). 2002;81:194–200.CrossRef
5.
Pouchot J, Sampalis JS, Beaudet F, Carette S, Décary F, Salusinsky-Sternbach M, et al. Adult Still’s disease: manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore). 1991;70:118–36.CrossRef
6.
Reginato AJ, Schumacher HR, Baker DG, O’Connor CR, Ferreiros J. Adult onset Still’s disease: experience in 23 patients and literature review with emphasis on organ failure. Semin Arthritis Rheum. 1987;17:39–57.CrossRefPubMed
7.
Sampalis JS, Esdaile JM, Medsger TA, Partridge AJ, Yeadon C, Senécal JL, et al. A controlled study of the long-term prognosis of adult Still’s disease. Am J Med. 1995;98:384–8.CrossRefPubMed
8.
Arlet J-B, Le THD, Marinho A, Amoura Z, Wechsler B, Papo T, et al. Reactive haemophagocytic syndrome in adult-onset Still’s disease: a report of six patients and a review of the literature. Ann Rheum Dis. 2006;65:1596–601.CrossRefPubMedPubMedCentral
9.
Cush JJ, Medsger TA, Christy WC, Herbert DC, Cooperstein LA. Adult-onset Still’s disease. Clinical course and outcome. Arthritis Rheum. 1987;30:186–94.CrossRefPubMed
10.
Wouters JM, van de Putte LB. Adult-onset Still’s disease; clinical and laboratory features, treatment and progress of 45 cases. Q J Med. 1986;61:1055–65.PubMed
11.
Medsger TA, Christy WC. Carpal arthritis with ankylosis in late onset Still’s disease. Arthritis Rheum. 1976;19:232–42.CrossRefPubMed
12.
Kong X-D, Xu D, Zhang W, Zhao Y, Zeng X, Zhang F. Clinical features and prognosis in adult-onset Still’s disease: a study of 104 cases. Clin Rheumatol. 2010;29:1015–9.CrossRefPubMed
13.
Zeng T, Zou Y-Q, Wu M-F, Yang C-D. Clinical features and prognosis of adult-onset still’s disease: 61 cases from China. J Rheumatol. 2009;36:1026–31.CrossRefPubMed
14.
Lee S-W, Park Y-B, Song J-S, Lee S-K. The mid-range of the adjusted level of ferritin can predict the chronic course in patients with adult onset Still’s disease. J Rheumatol. 2009;36:156–62.PubMed
15.
Bae C-B, Jung J-Y, Kim H-A, Suh C-H. Reactive hemophagocytic syndrome in adult-onset Still disease: clinical features, predictive factors, and prognosis in 21 patients. Medicine (Baltimore). 2015;94:e451.CrossRef
16.
Fukaya S, Yasuda S, Hashimoto T, Oku K, Kataoka H, Horita T, et al. Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: analysis of 30 cases. Rheumatology. 2008;47:1686–91.CrossRefPubMed
17.
Kumakura S, Murakawa Y. Clinical characteristics and treatment outcomes of autoimmune-associated hemophagocytic syndrome in adults. Arthritis Rheumatol. 2014;66:2297–307.CrossRefPubMedPubMedCentral
Metadata
Title
About the complexity of adult onset Still’s disease… and advances still required for its management
Authors
Philippe Guilpain
Alain Le Quellec
Publication date
01-12-2017
Publisher
BioMed Central
Published in
BMC Medicine / Issue 1/2017
Electronic ISSN: 1741-7015
DOI
https://doi.org/10.1186/s12916-016-0769-1

Other articles of this Issue 1/2017

BMC Medicine 1/2017 Go to the issue

Research article

Association between plasma phospholipid saturated fatty acids and metabolic markers of lipid, hepatic, inflammation and glycaemic pathways in eight European countries: a cross-sectional analysis in the EPIC-InterAct study

Research article

Methylome analysis of extreme chemoresponsive patients identifies novel markers of platinum sensitivity in high-grade serous ovarian cancer

Research article

The contribution of risk factors to socioeconomic inequalities in multimorbidity across the lifecourse: a longitudinal analysis of the Twenty-07 cohort

Commentary

Micronutrient powders to combat anemia in young children: does it work?

Research article

Borrelia infection and risk of celiac disease

Research article

The risk of type 2 oral polio vaccine use in post-cessation outbreak response