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 ter Haar, Nienke M
A1 Oswald, Marlen
A1 Jeyaratnam, Jerold
A1 Anton, Jordi
A1 Barron, Karyl S
A1 Brogan, Paul A
A1 Cantarini, Luca
A1 Galeotti, Caroline
A1 Grateau, Gilles
A1 Hentgen, Veronique
A1 Hofer, Michael
A1 Kallinich, Tilmann
A1 Kone-Paut, Isabelle
A1 Lachmann, Helen J
A1 Ozdogan, Huri
A1 Ozen, Seza
A1 Russo, Ricardo
A1 Simon, Anna
A1 Uziel, Yosef
A1 Wouters, Carine
A1 Feldman, Brian M
A1 Vastert, Sebastiaan J
A1 Wulffraat, Nico M
A1 Benseler, Susanne M
A1 Frenkel, Joost
A1 Gattorno, Marco
A1 Kuemmerle-Deschner, Jasmin B
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.