To demonstrate rapid improvement of recalcitrant cystoid macular edema (CME) and perivascular leakage, in a patient with non-paraneoplastic autoimmune retinopathy (npAIR) and autoimmune optic neuropathy (AON) after treatment with sarilumab, a human anti-interleukin-6 (IL-6) receptor antibody.
A 29-year-old woman was diagnosed with npAIR and AON and followed over 1.5 years. She had recalcitrant cystoid macular edema despite local corticosteroid and immunosuppressive therapy that included azathioprine and adalimumab. Subcutaneous Sarilumab, was initiated at a dose of 200mg every two weeks. CME significantly decreased after two injections and resolved after four injections with associated improvement in visual acuity as well as significant improvement in perivascular leakage on fluorescein angiography. There was sustained visual and anatomical improvement at 6 months along with mild improvement in electroretinogram responses. The patient tolerated the medication with no side effects.
Management of CME in npAIR is challenging and long-term immunosuppression is often employed with varying degrees of success. The improvement in refractory CME and perivascular leakage in this case supports the potential role of an IL-6 inhibitor to treat CME associated with npAIR suggesting the role.
We demonstrate rapid improvement of recalcitrant cystoid macular edema and perivascular leakage, with improvement in vision, after initiation of subcutaneous sarilumab, a human anti-interleukin-6 (IL-6) receptor antibody in a patient with non-paraneoplastic autoimmune retinopathy and autoimmune optic neuropathy, that was refractory to prior local therapy and systemic immunosuppression.