title: Recommendations for the management of autoinflammatory diseases.
abstract: : 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.
authors.affiliation: Laboratory for Translational Immunology, University Medical Center Utrecht, Utrecht, Netherlands Department of Paediatric Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Nienke M
authors.initials: NM
authors.lastname: ter Haar
authors.affiliation: Division of Paediatric Rheumatology, University Hospital Tuebingen, Tuebingen, Germany.
authors.forename: Marlen
authors.initials: M
authors.lastname: Oswald
authors.affiliation: Department of Paediatrics, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Jerold
authors.initials: J
authors.lastname: Jeyaratnam
authors.affiliation: Paediatric Rheumatology Unit, Hospital Sant Joan de Déu, University of Barcelona, Barcelona, Spain.
authors.forename: Jordi
authors.initials: J
authors.lastname: Anton
authors.affiliation: Division of Intramural Research, National Institute of Health, Bethesda, USA.
authors.forename: Karyl S
authors.initials: KS
authors.lastname: Barron
authors.affiliation: Department of Rheumatology, UCL Institute of Child Health, London, UK.
authors.forename: Paul A
authors.initials: PA
authors.lastname: Brogan
authors.affiliation: Rheumatology Unit, Policlinico Le Scotte, University of Siena, Siena, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Cantarini
authors.affiliation: Department of Pediatric Rheumatology, Reference Centre for Autoinflammatory Disorders CEREMAI, Bicêtre Hospital, Paris, France.
authors.forename: Caroline
authors.initials: C
authors.lastname: Galeotti
authors.affiliation: Department of Internal Medicine, APHP, Hôpital Tenon, University Pierre-et-Marie-Curie, Paris, France.
authors.forename: Gilles
authors.initials: G
authors.lastname: Grateau
authors.affiliation: French Reference Centre for Auto-Inflammatory Diseases in Children, Centre Hospitalier de Versailles, Le Chesnay Cedex, France.
authors.forename: Veronique
authors.initials: V
authors.lastname: Hentgen
authors.affiliation: Department of Paediatrics, University of Lausanne, Lausanne, Switzerland.
authors.forename: Michael
authors.initials: M
authors.lastname: Hofer
authors.affiliation: Department of Paediatric Pneumology and Immunology, Charité University Medicine, Berlin, Germany.
authors.forename: Tilmann
authors.initials: T
authors.lastname: Kallinich
authors.affiliation: Department of Paediatric Rheumatology, Reference Centre for Autoinflammatory Disorders CEREMAI, Bicêtre Hospital, University of Paris SUD, Paris, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Kone-Paut
authors.affiliation: National Amyloidosis Centre, University College London Medical School, London, UK.
authors.forename: Helen J
authors.initials: HJ
authors.lastname: Lachmann
authors.affiliation: Division of Rheumatology, Cerrahpasa Ic Hastaliklari Klinigi, University Istanbul, Istanbul, Turkey.
authors.forename: Huri
authors.initials: H
authors.lastname: Ozdogan
authors.affiliation: Department of Paediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
authors.forename: Seza
authors.initials: S
authors.lastname: Ozen
authors.affiliation: Service of Immunology and Rheumatology, Hospital de Pediatría Garrahan, Buenos Aires, Argentina.
authors.forename: Ricardo
authors.initials: R
authors.lastname: Russo
authors.affiliation: Department of Internal Medicine, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: Anna
authors.initials: A
authors.lastname: Simon
authors.affiliation: Department of Paediatrics, Meir Medical Center, Tel-Aviv University, Tel-Aviv, Israel.
authors.forename: Yosef
authors.initials: Y
authors.lastname: Uziel
authors.affiliation: Department of Microbiology and Immunology, University Hospital Leuven, Leuven, Belgium.
authors.forename: Carine
authors.initials: C
authors.lastname: Wouters
authors.affiliation: Division of Rheumatology, The Hospital for Sick Children, Toronto, Canada.
authors.forename: Brian M
authors.initials: BM
authors.lastname: Feldman
authors.affiliation: Department of Paediatric Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Sebastiaan J
authors.initials: SJ
authors.lastname: Vastert
authors.affiliation: Department of Paediatric Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Nico M
authors.initials: NM
authors.lastname: Wulffraat
authors.affiliation: Department of Paediatrics, Rheumatology, Alberta Children's Hospital, University of Calgary, Calgary, Canada.
authors.forename: Susanne M
authors.initials: SM
authors.lastname: Benseler
authors.affiliation: Department of Paediatrics, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Joost
authors.initials: J
authors.lastname: Frenkel
authors.affiliation: Division of Paediatrics II, G. Gaslini Institute, Genoa, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Gattorno
authors.affiliation: Division of Paediatric Rheumatology, University Hospital Tuebingen, Tuebingen, Germany.
authors.forename: Jasmin B
authors.initials: JB
authors.lastname: Kuemmerle-Deschner
mesh_terms: D032921:Consensus; D056587:Cryopyrin-Associated Periodic Syndromes / Q000628:therapy*; D005334:Fever; D056660:Hereditary Autoinflammatory Diseases / Q000628:therapy*; D006801:Humans; D054078:Mevalonate Kinase Deficiency / Q000628:therapy*; D017410:Practice Guidelines as Topic
keywords: Fever Syndromes; Multidisciplinary team-care; Treatment