Table 1

EULAR consensus definitions for disease activity states in AAV

Activity state	EULAR consensus definition
Active disease	Presence of typical signs, symptoms or other features (such as glomerulonephritis or pulmonary nodules) of active AAV
Remission	Absence of typical signs, symptoms, or other features of active AAV with or without immunosuppressive therapy
Sustained remission	Absence of typical signs, symptoms, or other features of active AAV over a defined time period with or without immunosuppressive therapy
Response	≥50% reduction of disease activity score and absence of new manifestations
Relapse	Recurrence of active AAV after a period of remission
Refractory	Unchanged or increased signs, symptoms or other features of active AAV after a period of standard induction therapy. Damage, infections, side effects of treatment or comorbidities as potential causes of the persistent or worsened disease manifestations need to be ruled out.