EULAR points to consider in the development of classifi cation and diagnostic criteria in systemic vasculitis

Objectives The systemic vasculitides are multiorgan diseases where early diagnosis and treatment can signifi cantly improve outcomes. Robust nomenclature reduces diagnostic delay. However, key aspects of current nomenclature are widely perceived to be out of date, these include disease defi nitions, classifi cation and diagnostic criteria. Therefore, the aim of the present work was to identify defi ciencies and provide contemporary points to consider for the development of future defi nitions and criteria in systemic vasculitis. Methods The expert panel identifi ed areas of concern within existing defi nitions/criteria. Consequently, a systematic literature review was undertaken looking to address these defi ciencies and produce ‘points to consider’ in accordance with standardised European League Against Rheumatism (EULAR) operating procedures. In the absence of evidence, expert consensus was used. Results There was unanimous consensus for re-evaluating existing defi nitions and developing new criteria. A total of 17 points to consider were proposed, covering 6 main areas: biopsy, laboratory testing, diagnostic radiology, nosology, defi nitions and research agenda. Suggestions to improve and expand current defi nitions were described including the incorporation of anti-neutrophil cytoplasm antibody and aetiological factors, where known. The importance of biopsy in diagnosis and exclusion of mimics was highlighted, while equally emphasising its problems. Thus, the role of alternative diagnostic tools such as MRI, ultrasound and surrogate markers were also discussed. Finally, structures to develop future criteria were considered. Conclusions Limitations in current classifi cation criteria and defi nitions for vasculitis have been identifi ed and suggestions provided for improvement. Additionally it is proposed that, in combination with the updated evidence, these should form the basis of future attempts to develop and validate revised criteria and defi nitions of vasculitis.