Ten Haar NM et al. Recommendations for the management of autoinflammatory diseases. Ann Rheum Dis 2015; 74: 1636–1644.

Authors: Š. Fingerhutová 1;  T. Dallos 2;  K. Bouchalova 3;  I. Brejchová 4;  J. Fráňová 5;  E. Jančová 6;  K. Jarošová 7;  K. Kobrová 8;  M. Macků 5;  H. Malcová 9;  L. Minxová 10;  D. Němcová 1;  M. Schüller 5;  E. Skalická 11;  V. Vargová 12;  P. Doležalová 1*
Authors‘ workplace: Klinika dětského a dorostového lékařství 1. LF UK a VFN v Praze, ČR 1;  Dětská klinika LF UK a FN Hradec Králové, ČR 10;  Dětská klinika LF UK a FN Plzeň, ČR 11;  Klinika detí a dorastu, LF UPJŠ a DFN v Košiciach, SR 12;  Detská klinika LF UK v Bratislave a NÚDCH, Bratislava, SR 2;  Dětská klinika, LF UP a FN Olomouc, ČR 3;  Nemocnice České Budějovice, ČR 4;  Pediatrická klinika LF MU a FN Br no, ČR 5;  Klinika nefrologie 1. LFUK a VFN v Praze, ČR 6;  Revmatologický ústav, Praha, ČR 7;  Dětská klinika Masarykovy nemocnice, Ústí nad Labem, ČR 8;  Oddělení revmatologie dětí a dospělých, Fakultní nemocnice v Motole, Praha, ČR 9
Published in: Čes. Revmatol., 26, 2018, No. 1, p. 8-22.
Category: Recommendation


This is a translation and adaptation of the original publication with Authors’ permission. 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 recom-mendations 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. Original translation was updated and modified according to the Czech and Slovak healthcare specificities.

Key words:
clinical recommendations, autoinflammatory diseases, periodic fever syndrome


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