Table 2

EULAR consensus definitions for disease activity states in GCA and other types of LVV

Activity stateEULAR consensus definition
Active disease1. The presence of typical signs or symptoms of active LVV (table 4).
2. At least one of the following:
  1. Current activity on imaging or biopsy.

  2. Ischaemic complications attributed to LVV.

  3. Persistently elevated inflammatory markers (after other causes have been excluded).

FlareWe do not recommend use of this term
RelapseWe recommend use of the terms major relapse or minor relapse as defined below
Major relapseRecurrence of active disease with either of the following:
  1. Clinical features of ischaemia* (including jaw claudication, visual symptoms, visual loss attributable to GCA, scalp necrosis, stroke, limb claudication).

  2. Evidence of active aortic inflammation resulting in progressive aortic or large vessel dilatation, stenosis or dissection.

Minor relapseRecurrence of active disease, not fulfilling the criteria for a major relapse
Refractor’yInability to induce remission (with evidence of reactivation of disease, as defined above in ‘Active disease’) despite the use of standard care therapy
RemissionAbsence of all clinical signs and symptoms attributable to active LVV and normalisation of ESR and CRP; in addition, for patients with extracranial disease there should be no evidence of progressive vessel narrowing or dilatation (frequency of repeat imaging to be decided on an individual basis)
Sustained remission
  1. Remission for at least 6 months.

  2. Achievement of the individual target GC dose.

Glucocorticoid-free remissionSustained remission
Discontinued GC therapy (but could still be receiving other immunosuppressive therapy)
  • *Some symptoms listed are typical only for GCA and may require further diagnostic work-up if present in other types of LVV.

  • GC, glucocorticoid; GCA, giant cell arteritis; LVV, large vessel vasculitis.