OR7-004 – Validation of AIDAI score

M Piram,N Ruperto,I Koné-Paut,M Gattorno

doi:10.1186/1546-0096-11-s1-a105

Abstract

With the increasing potential for targeted therapies in autoinflammatory diseases, there is the need for validated and standardized assessment tools which can be used to evaluate the level of disease activity and response to therapy. An international collaboration, initiated by Assistance Publique-Hopitaux de Paris (APHP) in association with the Paediatric Rheumatology International Trials Organization (PRINTO at http://www.printo.it) and supported by the EUROFEVER and EUROTRAPS networks, has previously designed the content and the preliminary scoring of an Auto-Inflammatory Disease Activity Index (AIDAI).

Highlights

With the increasing potential for targeted therapies in autoinflammatory diseases, there is the need for validated and standardized assessment tools which can be used to evaluate the level of disease activity and response to therapy.An international collaboration, initiated by Assistance Publique-Hôpitaux de Paris (APHP) in association with the Paediatric Rheumatology International Trials Organization (PRINTO at www.printo.it) and supported by the EUROFEVER and EUROTRAPS networks, has previously designed the content and the preliminary scoring of an Auto-Inflammatory Disease Activity Index (AIDAI)
Consensus was achieved for 98/106 (92%) cases (39 familial Mediterranean fever (FMF), 35 cryopyrin-associated periodic syndromes (CAPS), 14 tumor necrosis factor receptor-associated periodic syndrome (TRAPS) and 10 mevalonate kinase deficiency (MKD)) with 26 patients declared as having inactive disease and 72 active disease
An AIDAI cut-off score ≥ 9 discriminated active versus inactive patients, with sensitivity/specificity/accuracy of 89%/92%/90% respectively and an area under the curve of 98% (95%CI=96%>100%)

Summary

Introduction

With the increasing potential for targeted therapies in autoinflammatory diseases, there is the need for validated and standardized assessment tools which can be used to evaluate the level of disease activity and response to therapy.An international collaboration, initiated by Assistance Publique-Hôpitaux de Paris (APHP) in association with the Paediatric Rheumatology International Trials Organization (PRINTO at www.printo.it) and supported by the EUROFEVER and EUROTRAPS networks, has previously designed the content and the preliminary scoring of an Auto-Inflammatory Disease Activity Index (AIDAI)

Objectives

Methods

Results

Conclusion