Table 2

 Recommendation for use and definition of activity states in vasculitis

Activity stateDefinition
AAV, anti-neutrophil cytoplasmic antibody-associated vasculitis; BVAS, Birmingham Vasculitis Activity Score; BVAS/WG, Birmingham Vasculitis Activity Score for Wegener’s granulomatosis; CSS, Chug–Strauss syndrome; GC, glucocorticoid; WG, Wegener’s granulomatosis.
RemissionAbsence of disease activity attributable to active disease qualified by the need for ongoing stable maintenance immunosuppressive therapy. The term “active disease” is not restricted to vasculitis only, but also includes other inflammatory features like granulomatous inflammation in WG or tissue eosinophilia in CSS.
Response50% reduction of disease activity score and absence of new manifestations
RelapseRe-occurrence or new onset of disease attributable to active vasculitis
    MajorRe-occurrence or new onset of potentially organ- or life-threatening disease
    MinorRe-occurrence or new onset of disease which is neither potentially organ- nor life-threatening
Refractory diseaseUnchanged or increased disease activity in acute AAV after 4 weeks of treatment with standard therapy in acute AAV, or
Lack of response, defined as ⩽50% reduction in the disease activity score, after 6 weeks of treatment, or
Chronic, persistent disease defined as presence of at least one major or three minor items on the disease activity score list (eg, BVAS or BVAS/WG) after ⩾12 weeks of treatment
Low-activity disease statePersistence of minor symptoms (eg, arthalgia, myalgia) that respond to a modest increase of the GC dose and do not warrant an escalation of therapy beyond a modest dose increase in the current medication