Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2020;58(6):436-443.
doi: 10.5114/reum.2020.102010. Epub 2020 Dec 23.

Clinical variability of the systemic juvenile idiopathic arthritis course: literature review based on case series

Oksana Boyarchuk  1 , Tetiana Kovalchuk  1 , Nataliya Kovalchuk  1 , Oksana Chubata  2
Affiliations
Review

Clinical variability of the systemic juvenile idiopathic arthritis course: literature review based on case series

Oksana Boyarchuk et al. Reumatologia. 2020.

Abstract

Systemic juvenile idiopathic arthritis (sJIA) is a heterogeneous category of arthritis that frequently leads to disability and severe complications. The clinical cause of sJIA is very variable, which results in difficulties of disease recognition. In this literature review based on case series we outline the main challenges in diagnostic of sJIA and macrophage activation syndrome (MAS). Using the 2016 criteria for MAS diagnostic allowed to consider MAS in the diagnostically challenging cases, that confirms their sensitivity in pediatric patients. The reviewed literature showed last updates for the improvement of classification, diagnostic of sJIA and its complication. The modification of JIA criteria, initiated by Paediatric Rheumatology International Trials Organisation, will allow to improve detection and treatment of JIA. The presentation of this clinical cases and the discussion may be useful for understanding the disease cause and will help to differentiate sJIA and MAS from other disorders, and to improve treatment outcomes.

Keywords: diagnosis; macrophage activation syndrome; systemic juvenile idiopathic arthritis.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflict of interest.

References

    1. Cassidy JT, Petty RE. Chronic arthritis in childhood. In: Cassidy JT, Petty RE, Laxer RM, Lindsley CB, editors. Textbook of Pediatric Rheumatology. Philadelphia: Saunders Elesvier; 2005. pp. 206–300.
    1. Martini A, Ravelli A, Avcin T, et al. Toward new classification criteria for juvenile idiopathic arthritis: first steps, Pediatric Rheumatology International Trials Organization international consensus. J Rheumatol. 2019;46:190–197. doi: 10.3899/jrheum.180168. - DOI - PubMed
    1. Vandenhaute J, Wouters CH, Matthys P. Natural killer cells in systemic autoinflammatory diseases: a focus on systemic juvenile idiopathic arthritis and macrophage activation syndrome. Front Immunol. 2020;10:3089. doi: 10.3389/fimmu.2019.03089. - DOI - PMC - PubMed
    1. Petty RE, Southwood TR, Manners P, et al. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton 2001. J Rheumatol. 2004;31:390–392. - PubMed
    1. Woo P. Systemic juvenile idiopathic arthritis: diagnosis, management, and outcome. Nat Clin Pract Rheumatol. 2006;2:28–34. doi: 10.1038/ncprheum0084. - DOI - PubMed