Table 4 Important definitions for GCA diseasea
Disease activity state | Definition |
|---|---|
Suspected GCA | Clinical manifestations suggestive of GCA and not explained by other conditions |
Active Disease | 1. The presence of typical signs or symptoms of active GCA, plus: 2. At least one of the following: - Current activity confirmed by imaging or temporal artery biopsy - GCA associated ischaemic complications - Persistently elevated inflammatory markers (after exclusion of other causes) |
Severe Disease | Vasculitis with life-or organ-threatening manifestations (e.g., vision loss, cerebrovascular ischemia, cardiac ischemia, limb ischemia, tongue ischemia) |
Non-severe disease | Vasculitis without life-or organ-threatening manifestations (e.g., constitutional symptoms, headache, jaw claudication, symptoms of polymyalgia rheumatica) |
Relapse | Recurrence of active disease following a period of remission |
Major Relapse | Recurrence of active disease with either of the following: a. Clinical features of ischaemiaa (including jaw claudication, visual symptoms, visual loss attributable to GCA, scalp necrosis, stroke, limb claudication) b. Evidence of active aortic inflammation resulting in progressive aortic or large vessel dilatation, stenosis or dissection aCranial Ischemia: Visual and neurologic involvement including amaurosis fugax, vision loss, and stroke |
Minor Relapse | Recurrence of active disease, not fulfilling the criteria for a major relapse |
Refractory | Persistent active disease (inability to induce remission) despite the use of an appropriate course of immunosuppressive therapy |
Remission | -Absence of clinical signs or symptoms attributed to active GCA, on or off immunosuppressive therapy -Normal ESR and CRP -No evidence of progressive narrowing or dilatation of blood vessels |
Sustained remission | 1. Remission for at least 6 months 2. Achievement of the individual target GC dose |
Glucocorticoid-free remission | Sustained remission Discontinued GC therapy (but could still be receiving other immunosuppressive therapy) |
Clinical monitoring | Assessing for clinical signs and symptoms of active disease, obtaining 4 extremity blood pressures, and obtaining clinical laboratory results, including inflammation marker levels |