Graves’ orbitopathy
ICD-10 H06.2 · ICD-11 5A02.0/9A20.00

Moderate-to-Severe Active Thyroid Eye Disease: Management When Intravenous Glucocorticoid Therapy Has Not Achieved Disease Control

This protocol applies to patients with active, moderate-to-severe Graves’ orbitopathy in whom disease activity is the dominant clinical concern — without significant proptosis or diplopia — and where initial intravenous glucocorticoid (IVGC) therapy has not produced adequate disease control.

Clinical scenario

Active moderate-to-severe thyroid eye disease with disease activity as the prominent feature, in the absence of significant proptosis or diplopia. The eye disease carries sufficient impact on daily life to justify the risks of systemic intervention.

Escalation trigger — when this protocol applies

First-line management for this presentation is intravenous glucocorticoid (IVGC) therapy. This next-line protocol is indicated when IVGC has not produced an improvement in clinical activity score (CAS) of more than 2 points, or has not achieved disease inactivation (CAS ≤2) at 12 weeks — and for patients who were unresponsive, only partially responsive, or intolerant to IVGC.

Next-line approach (partial)

For patients unresponsive, partially responsive, or intolerant to intravenous glucocorticoid therapy, the approach involves systemic agents — including certain biologic therapies — with additional options that depend on the nature and degree of prior glucocorticoid response.

Goal: disease inactivation — CAS <3
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References
doi: 10.1089/thy.2022.0251

Patients without sight-threatening disease whose eye disease has sufficient impact on daily life to justify the risks of medical or surgical intervention.

IVGC therapy is a preferred treatment for active moderate-to-severe TED when disease activity is the prominent feature in the absence of either significant proptosis (see Section 2.1. for definition) or diplopia.

RTX and tocilizumab (TCZ) may be considered for TED inactivation in glucocorticoid (GC)-resistant patients with active moderate-to-severe TED.

Other options, based on anecdotal evidence, are an additional course of IVGC (in patients with previous partial response, aiming not to exceed 8 g of methylprednisolone), or RT (see section 7.2).

At 24 weeks, disease inactivation (CAS <3) occurred in significantly more RTX-treated patients than in IVMP-treated patients (100% vs. 69%).

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