Refractory optic perineuritis related to granulomatosis with polyangiitis treated with intensive immunosuppressive therapy combined with plasma exchange

Abstract

Optic perineuritis (OPN), which is an inflammatory disorder affecting the optic nerve sheath, is one of the rare complications in granulomatosis with polyangiitis (GPA). Although several groups have reported that immunosuppressive therapies are generally effective against GPA-associated OPN, so far, there is little information as to other options for refractory cases. Here we demonstrate a case of GPA-associated OPN, which is refractory to potent immunosuppressive therapy including high-dose glucocorticoid, intravenous cyclophosphamide and rituximab therapy, and effective application of therapeutic plasma exchange. We also report here that CSF IL-6 levels may serve as a new biomarker for GPA-associated OPN.

Keywords:

• Granulomatosis with polyangiitis
• optic perineuritis
• interleukin-6
• rituximab
• plasma exchange

Acknowledgements

We thank Mr. Tom Kiper for his review of the manuscript.

Conflict of interest

None

Patient consent

We obtained written informed consent from this patient to publish this case report.