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EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis

. 2016 Sep ; 75 (9) : 1583-94. [epub] 20160623

Language English Country England, Great Britain Media print-electronic

Document type Journal Article, Practice Guideline

In this article, the 2009 European League Against Rheumatism (EULAR) recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have been updated. The 2009 recommendations were on the management of primary small and medium vessel vasculitis. The 2015 update has been developed by an international task force representing EULAR, the European Renal Association and the European Vasculitis Society (EUVAS). The recommendations are based upon evidence from systematic literature reviews, as well as expert opinion where appropriate. The evidence presented was discussed and summarised by the experts in the course of a consensus-finding and voting process. Levels of evidence and grades of recommendations were derived and levels of agreement (strengths of recommendations) determined. In addition to the voting by the task force members, the relevance of the recommendations was assessed by an online voting survey among members of EUVAS. Fifteen recommendations were developed, covering general aspects, such as attaining remission and the need for shared decision making between clinicians and patients. More specific items relate to starting immunosuppressive therapy in combination with glucocorticoids to induce remission, followed by a period of remission maintenance; for remission induction in life-threatening or organ-threatening AAV, cyclophosphamide and rituximab are considered to have similar efficacy; plasma exchange which is recommended, where licensed, in the setting of rapidly progressive renal failure or severe diffuse pulmonary haemorrhage. These recommendations are intended for use by healthcare professionals, doctors in specialist training, medical students, pharmaceutical industries and drug regulatory organisations.

Assistance Publique Hôpitaux de Paris Department of Pulmonology Bichat Claude Bernard University Hospital Paris France

Department of Internal Medicine Hôpital Saint Louis Université Paris 7 René Diderot Paris France

Department of Medical and Health Sciences Linköping University Linköping Sweden Department of Nephrology Linköping University Linköping Sweden

Department of Nephrology 1st School of Medicine Charles University Prague Czech Republic

Department of Nephrology Lund University Skåne University Hospital Lund and Malmö Sweden

Department of Ophthalmology School of Medicine Ankara University Ankara Turkey

Department of Otorhinolaryngology Head and Neck Surgery University of Kiel Kiel Germany

Department of Pathology Leiden University Medical Center Leiden The Netherlands

Department of Rheumatology Norfolk and Norwich University Hospital Norwich UK

Department of Rheumatology Norfolk and Norwich University Hospital Norwich UK Norwich Medical School University of East Anglia Norwich UK

Division of Rheumatology and the Department of Biostatistics and Epidemiology University of Pennsylvania Philadelphia Pennsylvania USA

Immunologie Zentrum Zürich Zürich Switzerland

Lupus and Vasculitis Unit Addenbrooke's Hospital Cambridge UK

Nephrology Unit University Hospital of Parma Parma Italy

Norwich Medical School University of East Anglia Norwich UK Department of Rheumatology Ipswich Hospital NHS Trust Ipswich Suffolk UK

Nuffield Department of Orthopaedics Rheumatology and Musculoskeletal Sciences Botnar Research Centre University of Oxford Oxford United Kingdom

Rheumazentrum Schleswig Holstein Mitte Neumünster Germany

Trinity Health Kidney Centre Tallaght Hospital Dublin Ireland

Vasculitis Research Unit Department of Autoimmune Diseases Hospital Clínic University of Barcelona Institut d'Investigacions Biomèdiques August Pi i Sunyer Barcelona Spain

Vasculitis UK West Bank House Winster Matlock UK

Vaskulits Zentrum Süd Klinik für Innere Medizin Rheumatologie und Immunologie Kreiskliniken Esslingen Kirchheim Teck Germany

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