Use of Rituximab After Orbital Decompression Surgery in Two Grave's Ophthalmopathy Patients Progressing to Optic Neuropathy.

Benping Zhang,Bei Peng,Gang Yuan,Weijie Xu,Yaling Li

doi:10.3389/fendo.2020.583565

Abstract

BackgroundWhile orbital decompression can alleviate optic nerve compression and prevent further vision loss in dysthyroid optic neuropathy (DON), it cannot relieve inflammatory symptoms. Very high doses of intravenous glucocorticoids (GCs) are the first-line therapy for DON; however, the effective rate is only 40% and might be much lower in patients who fail high-dose GC pulse therapy and progressed to DON. The results of two case series studies indicated that rituximab treatment had a much better curative effect compared to very high doses of intravenous GCs, but some patients required urgent orbital decompression after rituximab injection because rituximab might lead to the release of cytokines, aggravated intraorbital edema, and further vision loss.MethodsWe retrospectively studied the therapeutic process of two Grave’s ophthalmopathy (GO) patients complicated with DON who failed high-dose GC pulse therapy and underwent orbital decompression. Both patients received single-dose (500 mg) rituximab treatment.ResultsDuring more than 2 years of follow-up, rituximab treatment exhibited significant improvement in inflammatory symptoms, as manifested by a substantial decrease in Clinical Activity Score (CAS); meanwhile, the vision of both patients improved significantly and their diplopia was relieved.ConclusionsThe results of this study were consistent with those of two previous case series studies indicating the significant and lasting effect of rituximab treatment on DON, especially for patients with GC resistance or recurrence after GC therapy. Orbital decompression before rituximab treatment might reduce the incidence of rapid vision loss and urgent orbital decompression surgery caused by aggravated orbital edema after rituximab injection; however, the necessity for preventive decompression surgery requires further study.

Highlights

Grave’s disease (GD) is an autoimmune disease involving the thyroid, skin, and orbit, with an incidence in the adult population of 1–2% [1]
The vision of both patients improved significantly, and their diplopia was relieved after rituximab treatment
Total dose of MPS before orbital decompression Duration of MPS use before orbital decompression Orbital decompression Immunosuppressive treatments after orbital decompression RTX therapy RTX therapy Reason for RTX therapy RTX dose Immunosuppressive treatments after RTX

Summary

Introduction

Grave’s disease (GD) is an autoimmune disease involving the thyroid, skin, and orbit, with an incidence in the adult population of 1–2% [1]. For some patients, the signs and symptoms persist or aggravate gradually, which require specific treatment besides smoking cessation and anti-thyroid drug administration [4]. The pathogenesis of GO is incompletely understood, immunological cross-reactivity between the thyroid and orbital antigens may play a key role, and disorders of inflammatory cytokines, thyrotropin receptor autoantibodies, and immunoglobulins targeting the insulin-like growth factor 1 receptor may be correlated with GO [2, 5]. The results of two case series studies indicated that rituximab treatment had a much better curative effect compared to very high doses of intravenous GCs, but some patients required urgent orbital decompression after rituximab injection because rituximab might lead to the release of cytokines, aggravated intraorbital edema, and further vision loss

Results

Discussion

Conclusion