Affiliation:
1. Center for Translational Immunology, University Medical Center Utrecht , Utrecht , The Netherlands
2. Department of pediatric rheumatology & immunology, University Medical Center Utrecht , Utrecht , The Netherlands
3. University of Utrecht , Utrecht , The Netherlands
Abstract
Summary
Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in children and comprises of multiple subtypes. The most relevant disease subtypes, grouped upon current insight in disease mechanisms, are nonsystemic (oligo- and polyarticular) JIA and systemic JIA (sJIA). In this review, we summarize some of the main proposed mechanisms of disease in both nonsystemic and sJIA and discuss how current therapeutic modalities target some of the pathogenic immune pathways. Chronic inflammation in nonsystemic JIA is the result of a complex interplay between effector and regulatory immune cell subsets, with adaptive immune cells, specifically T-cell subsets and antigen-presenting cells, in a central role. There is, however, also innate immune cell contribution. SJIA is nowadays recognized as an acquired chronic inflammatory disorder with striking autoinflammatory features in the first phase of the disease. Some sJIA patients develop a refractory disease course, with indications for involvement of adaptive immune pathways as well. Currently, therapeutic strategies are directed at suppressing effector mechanisms in both non-systemic and sJIA. These strategies are often not yet optimally tuned nor timed to the known active mechanisms of disease in individual patients in both non-systemic and sJIA. We discuss current treatment strategies in JIA, specifically the ‘Step-up’ and ‘Treat to Target approach’ and explore how increased insight into the biology of disease may translate into future more targeted strategies for this chronic inflammatory disease at relevant time points: preclinical disease, active disease, and clinically inactive disease.
Funder
Dutch Arthritis Foundation
Vrienden WKZ
Publisher
Oxford University Press (OUP)
Subject
Immunology,Immunology and Allergy
Reference113 articles.
1. Juvenile idiopathic arthritis;Prakken;Lancet,2011
2. International League of Associations for Rheumatology Classification of Juvenile Idiopathic Arthritis: Second Revision, Edmonton, 2001;Petty;J Rheumatol,2004
3. International League of Associations for Rheumatology Classification of Juvenile Idiopathic Arthritis: Second Revision, Edmonton, 2001;Petty;J Rheumatol,2004
4. Long-term outcomes and disease course of children with juvenile idiopathic arthritis in the ReACCh-Out cohort: a two-centre experience;Chhabra;Revmatologiia (Bulgaria),2020
5. Toward new classification criteria for juvenile idiopathic arthritis: first steps, Pediatric Rheumatology International Trials Organization International Consensus;Martini;J Rheumatol,2019