This article is part of the supplement: Proceedings of 18th Pediatric Rheumatology European Society (PReS) Congress

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Therapy of autoinflammatory diseases: results from an international registry

NM ter Haar12*, J Frenkel12, P Woo2, L Cantarini2, H Lachmann2, A Insalaco2, M Hofer2, Y Uziel2, S Ozen2, S Nielsen2, A Naselli2, C Modesto2, SM Al-Mayouf2, I Kone-Paut2, I Nikishina2, N Iagaru2, L Obici2, E Papadopoulou-Alataki2, D Rigante2, C Boros2, A Martini2, N Ruperto2 and M Gattorno2

Author Affiliations

1 Department of Pediatrics University Medical Center Utrecht, Netherlands

2 For the PRINTO and Eurofever Project

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Pediatric Rheumatology 2011, 9(Suppl 1):P18  doi:10.1186/1546-0096-9-S1-P18

The electronic version of this article is the complete one and can be found online at:

Published:14 September 2011

© 2011 ter Haar et al; licensee BioMed Central Ltd.

This is an open access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


The evidence for therapy in autoinflammatory diseases is limited. There are few randomized controlled trials or disease registries.


To evaluate the response to treatment of autoinflammatory diseases based on data of an international registry.


A web-based registry collecting baseline and clinical information of autoinflammatory diseases was located at the member area of the PRINTO website ( Participating hospitals included pediatric rheumatology centers of the PRINTO network and adult centers with a specific interest in autoinflammatory diseases. Data was collected on Blau’s syndrome, Behçet’s disease, CAPS, CRMO, DIRA, FMF, MKD, NLRP12-mediated periodic fever, PAPA, PFAPA TRAPS and undefined periodic fevers. In total, 704 patients were included in this retrospective study on therapy.


NSAIDs and steroids were beneficial in most diseases. Anakinra induced a complete response in 70% of 40 treated CAPS patients, 82% of 28 TRAPS patients and in both DIRA patients. Furthermore, it was beneficial in 80% of 10 MKD patients. Etanercept was completely effective in all 6 Behçet patients, 34% of 32 TRAPS patients and 13% of 8 MKD patients and partially effective in 53% of TRAPS and 38% of MKD patients. Colchicine was beneficial in approximately 95% of 131 FMF and 18 Behçet patients, although the complete response rate was just 56% and 22%, respectively. For PFAPA syndrome, corticosteroids aborted the attacks in 78% of 113 patients.


The results were compared to the literature. These combined findings could serve as a base for therapeutic guidelines and identify candidate drugs for future therapeutic trials.