Active GCA with Acute Visual Loss or Amaurosis Fugax — When Initial Treatment Has Not Achieved Sustained Remission

This protocol applies to patients with active giant cell arteritis presenting with acute visual loss or amaurosis fugax who did not achieve or maintain the goals of the prior treatment line.

Clinical Scenario

Active giant cell arteritis with acute visual loss or amaurosis fugax. The involvement of visual function characterises this presentation and underpins the clinical urgency of management decisions at every stage.

Prior Line — Why This Protocol Applies

The preceding treatment combined glucocorticoid tapering with adjunctive therapy — either tocilizumab or methotrexate. This protocol applies when that line failed to reach its target:

Sustained remission — absence of all clinical signs and symptoms of active giant cell arteritis with normal acute phase reactants, maintained for at least 6 months, with the ability to taper glucocorticoids to the target dose without relapse.

Next-Step Approach (Partial Overview)

Management at this stage distinguishes between major and minor relapses. A major relapse calls for reinstitution of glucocorticoid therapy, while a minor relapse involves an upward adjustment of glucocorticoid dosing. In both cases, initiation or modification of adjunctive therapy is considered — particularly when relapses are recurrent. The complete regimen, classification criteria, sequencing, and adjunctive therapy specifics are detailed in the full protocol.

Treatment Target Remission: absence of all clinical signs and symptoms attributable to active giant cell arteritis, and normalisation of ESR and CRP.

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References

DOI: 10.1136/annrheumdis-2019-215672

In patients with GCA with acute visual loss or amaurosis fugax, the administration of 0.25–1 g intravenous methylprednisolone for up to 3 days should be considered, because these high doses have both genomic and rapid non-genomic effects.

In case of major relapse (either with signs or symptoms of ischaemia or progressive vascular inflammation) we recommend reinstitution or dose escalation of GC therapy as recommended for new onset disease.

For minor relapses we recommend an increase in GC dose at least to the last effective dose.

we recommend an increase of the daily GCs dose either to the last effective dose or to 5–15 mg above this dose, the latter being common practice in many centres.

Initiation or modification of adjunctive therapy should be considered particularly after recurrent disease relapses.

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