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PW03-034 – How to classify autoinflammatory diseases?

Authors :
Katia Stankovic
Véronique Hentgen
Serge Amselem
I Jéru
Olivier Steichen
Gilles Grateau
CHU Tenon [AP-HP]
Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)
Service de Pédiatrie
Centre Hospitalier de Versailles André Mignot (CHV)
Physiopathologie des maladies génétiques d'expression pédiatrique
Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC)
BMC, Ed.
Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)
Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)
Source :
Pediatric Rheumatology, Pediatric Rheumatology, BioMed Central, 2013, 11 (Suppl 1), pp.A260, Pediatric Rheumatology, 2013, 11 (Suppl 1), pp.A260, Pediatric Rheumatology Online Journal, Pediatric Rheumatology, May 2013, Lausanne, Switzerland. pp.A260, ⟨10.1186/1546-0096-11-S1-A26⟩
Publisher :
Springer Nature

Abstract

International audience; IntroductionDefinitions and classifications of autoinflammatory diseases have been multiple. Their succession highlightsthe advances in our understanding of the innate immune system, especially the role of interleukin 1b and theinflammasome. However, these definitions and classifications face a number of structure and content issues.ObjectivesTo propose a novel definition of autoinflammatory diseases and to challenge the global classification of inflammatory diseases.MethodsWe appeal to the desirable characteristics of classification systems (exhaustiveness, disjointness, naturalness, usefulness) and to a critical analysis of the notion of continuum.ResultsWe propose a clinically-oriented definition: “autoinflammatory diseases are diseases with clinical signs of inflam-mation, associated with elevated acute phase reactants and due to a dysfunction in the innate immune system,genetically determined or triggered by an endogenous factor”. It is hard to find natural properties able to underlie a useful classification of autoinflammatory diseases, and inflammatory diseases as a whole, into disjoint and exhaustive categories. The notion of continuum is therefore appealing. However, a single continuum from purely autoinflammatory to purely autoimmune diseases oversimplifies, and even distorts, reality. How to locate, for instance, the disease caused by a deletion in PLCG2 (the gene encoding phospholipase Cg2) that associatesautoinflammatory symptoms to both common variable immunodeficiency and autoimmune features? Herewe have an overactivation of both the innate and the adaptive immune system, associated with a deficiency ofthe adaptive immune system. More than one dimension is needed to properly represent the immunological dysfunctions underlying inflammatory diseases. Furthermore, a classification of inflammatorydiseases should also make sense of the clinical, pathological and biological phenotypes.ConclusionTo be adequate and useful, a definition of autoinflammatory diseases and a classification of inflammatory diseases must take the multiple facets of reality into account, including clinical features. This can be done within a continuum only if it is multidimensional.

Details

Language :
English
ISSN :
15460096
Volume :
11
Issue :
Suppl 1
Database :
OpenAIRE
Journal :
Pediatric Rheumatology
Accession number :
edsair.doi.dedup.....d1bbc64ce3717c46488433cbc07aa8d4
Full Text :
https://doi.org/10.1186/1546-0096-11-s1-a260