RT Journal Article
SR Electronic
T1 Recommendations for the management of autoinflammatory diseases
JF Annals of the Rheumatic Diseases
JO Ann Rheum Dis
FD BMJ Publishing Group Ltd and European League Against Rheumatism
SP 1636
OP 1644
DO 10.1136/annrheumdis-2015-207546
VO 74
IS 9
A1 Nienke M ter Haar
A1 Marlen Oswald
A1 Jerold Jeyaratnam
A1 Jordi Anton
A1 Karyl S Barron
A1 Paul A Brogan
A1 Luca Cantarini
A1 Caroline Galeotti
A1 Gilles Grateau
A1 Veronique Hentgen
A1 Michael Hofer
A1 Tilmann Kallinich
A1 Isabelle Kone-Paut
A1 Helen J Lachmann
A1 Huri Ozdogan
A1 Seza Ozen
A1 Ricardo Russo
A1 Anna Simon
A1 Yosef Uziel
A1 Carine Wouters
A1 Brian M Feldman
A1 Sebastiaan J Vastert
A1 Nico M Wulffraat
A1 Susanne M Benseler
A1 Joost Frenkel
A1 Marco Gattorno
A1 Jasmin B Kuemmerle-Deschner
YR 2015
UL http://ard.bmj.com/content/74/9/1636.abstract
AB Autoinflammatory diseases are characterised by fever and systemic inflammation, with potentially serious complications. Owing to the rarity of these diseases, evidence-based guidelines are lacking. In 2012, the European project Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate regimens for the management of children and young adults with rheumatic diseases, facilitating the clinical practice of paediatricians and (paediatric) rheumatologists. One of the aims of SHARE was to provide evidence-based recommendations for the management of the autoinflammatory diseases cryopyrin-associated periodic syndromes (CAPS), tumour necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS) and mevalonate kinase deficiency (MKD). These recommendations were developed using the European League Against Rheumatism standard operating procedure. An expert committee of paediatric and adult rheumatologists was convened. Recommendations derived from the systematic literature review were evaluated by an online survey and subsequently discussed at a consensus meeting using Nominal Group Technique. Recommendations were accepted if more than 80% agreement was reached. In total, four overarching principles, 20 recommendations on therapy and 14 recommendations on monitoring were accepted with ≥80% agreement among the experts. Topics included (but were not limited to) validated disease activity scores, therapy and items to assess in monitoring of a patient. By developing these recommendations, we aim to optimise the management of patients with CAPS, TRAPS and MKD.