IgG4-related orbital inflammation
ICD-10 H05.1 · ICD-11 4A43.0/9A22

Treatment of IgG4-related orbital inflammation with severe visual loss and acute compressive optic neuropathy

Acute compressive optic neuropathy in the setting of IgG4-related orbital disease (IgG4-ROD) is a high-urgency presentation in which orbital infiltration places the optic nerve under direct mechanical pressure, making prompt management essential to preserve vision.

This protocol targets severe visual loss in IgG4-ROD with acute compressive optic neuropathy — a scenario in which disease activity has progressed to the point of significant, potentially irreversible optic nerve compromise.

Management in this setting is built around medical orbital decompression combined with adjunctive targeted therapies. The full regimen — including the specific agents and the complete treatment sequence — is available in the structured protocol below.

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References

DOI: 10.4103/tjo.tjo_12_17

Chen et al. described a successful medical orbital decompression with adjunctive rituximab and radiation therapy for severe visual loss in IgG4‑ROD with acute compressive optic neuropathy.

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