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RMD Open. 2017 Jun 15;3(1):e000449. doi: 10.1136/rmdopen-2017-000449. eCollection 2017.

Validation of the EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis by disease content experts.

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Department of Rheumatology, Norfolk and Norwich University Hospital, Norwich, UK.
Norwich Medical School, Bob Champion Research and Education Building, Colney Lane, Norwich, UK.
Department of Rheumatology, Ipswich Hospital NHS Trust, Norwich Medical School, Norwich, UK.
Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands.
Vasculitis Research Unit, Department of Autoimmune Diseases, Hospital Clinic, University of Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.
Assistance Publique-Hôpitaux de Paris, Department of Pulmonology, Bichat-Claude Bernard University Hospital, Paris, France.
Immunologie-Zentrum Zürich, Zürich, Switzerland.
Vaskulits-Zentrum Süd, Klinik für Innere Medizin, Rheumatologie und Immunologie, Medius Klinik Kirchheim, Kirchheim-Teck, Germany.
Rheumazentrum Schleswig-Holstein Mitte, Kuhberg 5a-7, Neumünster, Germany.
Department of Otorhinolaryngology, Head and Neck Surgery, University of Kiel, Kiel, Germany.
Trinity Health Kidney Centre, Tallaght Hospital, Dublin, Ireland.
Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, Botnar Research Centre, University of Oxford, Oxford, UK.
Department of Internal Medicine, Hôpital Saint-Louis, Université Paris 7 René Diderot, Paris, France.
Division of Rheumatology and the Department of Biostatistics and Epidemiology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Vasculitis UK, West Bank House, Winster, Matlock, UK.
Department of Medical and Health Sciences Nephrology, Linköping University, Linköping, Sweden.
Department of Nephrology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic.
Department of Nephrology, Lund University, Skåne University Hospital, Lund and Malmö, Sweden.
Nephrology Unit, University Hospital of Parma, Parma, Italy.
Department of Ophthalmology, School of Medicine, Ankara University, Ankara, Turkey.
Lupus and Vasculitis Unit, Addenbrooke's Hospital, Cambridge, UK.


The European League Against Rheumatism recommendations for the management of antineutrophil cytoplasmic antibody-associated vasculitis have been recently published. Unique to recommendation development, they were also voted on by members of a learned society. This paper explores the wider validity of the recommendations among people who self-identify as clinicians caring for patients with vasculitis. In addition to the task force, a learned society (European Vasculitis Society-EUVAS) was invited, through online survey, to rate independently the strength of evidence of each recommendation to obtain an indication of the agreement among the final target audience and ultimate end-users of the recommendations. The survey took place in June 2015. Of the 158 EUVAS members surveyed, there were 88 responses (55.7%). There was a large degree of agreement in the voting patterns between EUVAS survey participants and task force members. Notable exceptions were lower grades for the recommendation of the use of rituximab for remission induction in patients with eosinophilic granulomatosis with polyangiitis and for methotrexate and mycophenolate mofetil as remission maintenance agents in patients with granulomatosis with polyangiitis/microscopic polyangiitis by EUVAS members. These results are encouraging and suggest that the voting patterns of the task force are representative of the wider vasculitis community. We recommend future recommendations adopt this approach for data/expert-based treatment guidelines, especially for multisystem diseases.


ANCA-associated vasculitis; Recommendations; Survey

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