Is Still's Disease an Autoinflammatory Syndrome?

Systemic juvenile idiopathic arthritis (sJIA), formerly called Still's disease, is officially classified as a subset of juvenile idiopathic arthritis (JIA). Beside arthritis, it is characterized by prominent systemic features and a marked inflammatory response. Even if it is still included in t...

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Main Authors: Linda Rossi-Semerano, Isabelle Koné-Paut
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:International Journal of Inflammation
Online Access:http://dx.doi.org/10.1155/2012/480373
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author Linda Rossi-Semerano
Isabelle Koné-Paut
author_facet Linda Rossi-Semerano
Isabelle Koné-Paut
author_sort Linda Rossi-Semerano
collection DOAJ
description Systemic juvenile idiopathic arthritis (sJIA), formerly called Still's disease, is officially classified as a subset of juvenile idiopathic arthritis (JIA). Beside arthritis, it is characterized by prominent systemic features and a marked inflammatory response. Even if it is still included in the group of juvenile arthritides, sJIA is set apart from all the other forms of JIA. This disorder has markedly distinct clinical and laboratory features suggesting a different pathogenesis. sJIA does not show any association with HLA genes or with autoantibodies and is characterised by an uncontrolled activation of phagocytes with hypersecretion of IL-1 and IL-6. Based on clinical and laboratory features, as well as on new acquisitions on the pathogenesis, it seems evident that sJIA is an autoinflammatory disease related to abnormality in innate immune system. The new insights on the pathogenesis of sJIA have therefore dramatically changed the approach to treatment, with the development of targeted treatments (anti-IL-1 and anti-IL-6 agents) more effective and safer than earlier medications.
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spelling doaj-art-1d8b324dd61e4331b89a5f2f22c7fca42025-08-20T03:54:37ZengWileyInternational Journal of Inflammation2090-80402042-00992012-01-01201210.1155/2012/480373480373Is Still's Disease an Autoinflammatory Syndrome?Linda Rossi-Semerano0Isabelle Koné-Paut1Department of Paediatrics and Paediatric Rheumatology, Bicêtre Hospital, National Reference Centre for Autoinflammatory Diseases, 78 rue du Général Leclerc, Le Kremlin Bicêtre, 94270 Paris, FranceDepartment of Paediatrics and Paediatric Rheumatology, Bicêtre Hospital, National Reference Centre for Autoinflammatory Diseases, 78 rue du Général Leclerc, Le Kremlin Bicêtre, 94270 Paris, FranceSystemic juvenile idiopathic arthritis (sJIA), formerly called Still's disease, is officially classified as a subset of juvenile idiopathic arthritis (JIA). Beside arthritis, it is characterized by prominent systemic features and a marked inflammatory response. Even if it is still included in the group of juvenile arthritides, sJIA is set apart from all the other forms of JIA. This disorder has markedly distinct clinical and laboratory features suggesting a different pathogenesis. sJIA does not show any association with HLA genes or with autoantibodies and is characterised by an uncontrolled activation of phagocytes with hypersecretion of IL-1 and IL-6. Based on clinical and laboratory features, as well as on new acquisitions on the pathogenesis, it seems evident that sJIA is an autoinflammatory disease related to abnormality in innate immune system. The new insights on the pathogenesis of sJIA have therefore dramatically changed the approach to treatment, with the development of targeted treatments (anti-IL-1 and anti-IL-6 agents) more effective and safer than earlier medications.http://dx.doi.org/10.1155/2012/480373
spellingShingle Linda Rossi-Semerano
Isabelle Koné-Paut
Is Still's Disease an Autoinflammatory Syndrome?
International Journal of Inflammation
title Is Still's Disease an Autoinflammatory Syndrome?
title_full Is Still's Disease an Autoinflammatory Syndrome?
title_fullStr Is Still's Disease an Autoinflammatory Syndrome?
title_full_unstemmed Is Still's Disease an Autoinflammatory Syndrome?
title_short Is Still's Disease an Autoinflammatory Syndrome?
title_sort is still s disease an autoinflammatory syndrome
url http://dx.doi.org/10.1155/2012/480373
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