Tocilizumab for Cystoid Macular Edema Secondary to Immune Recovery Uveitis in a Patient with Contraindications to Long-Term Systemic Corticosteroid

Abstract

Purpose: To report a case of cystoid macular edema (CME) secondary to immune recovery uveitis (IRU) in a patient with previous history of cytomegalovirus (CMV) retinitis and leukemia, which was successfully treated with tocilizumab (TCZ), an interleukin-6 (IL-6) receptor antagonist. Method: The clinical records of the case were reviewed, focusing on demographics, image findings, and clinical course. Results: A 17-year-old female with a past medical history of T-cell acute lymphoblastic leukemia (T-ALL) undergoing chemotherapy for two years presented with active CMV retinitis. She was successfully treated with intravitreal foscarnet injections and systemic ganciclovir. After 5 months of systemic valganciclovir maintenance and following cessation of chemotherapy, the patient developed bilateral CME and vasculitis, and was diagnosed with IRU. CME management was challenging due to a history of bilateral avascular necrosis of the femoral head resulting from prolonged systemic corticosteroid use. Two cycles of monthly TCZ infusions were administered at the dosage of 8mg/kg. Subsequently, the CME and retinal vasculitis resolved significantly without any evidence of inflammation in the anterior chamber and vitreous. Conclusion: The index case report demonstrated the safety and efficacy of the IL-6 receptor antagonist TCZ in treating CME associated with IRU in a non-HIV CMV retinitis patient.

Keywords: Cystoid macular edema; Cytomegalovirus retinitis; IL-6; Immune reconstitution inflammatory syndrome; Immune recovery uveitis; Tocilizumab.

Figure 1

Multimodal retinal imaging of active CMV retinitis of the right and left eye. Wide-angle fundus photography shows active necrotizing retinitis with hemorrhage and vascular sheathing of the right eye (A) and the left eye (B). Wide-angle fundus autofluorescence (FAF) shows hypo-autofluorescence with a surrounding halo of hyper-autofluorescence in the central, hyper-autofluorescence areas in the peripheral retina of the right eye (C) and left eye (D). Late-phase fluorescein angiography (FA) shows leakage on the peripheral retina of the right (E) and left eye (F). Spectral-domain optical coherence tomography (SD-OCT) of the right eye shows intra- and sub-retinal fluid, temporal retinal thinning, hyperreflectivity, and disorganization (G) and SD-OCT of the left eye shows temporal retinal thinning, hyperreflectivity, and disorganization (H).

Figure 2

Multimodal retinal imaging of immune recovery uveitis (IRU) of the right and left eye. Wide-angle fundus photography of the right eye (A) and the left eye (B) are unremarkable except for a residual scar in the superior nasal site of the left eye. Wide angle FAF of the right eye (C) and left eye (D) show hypo-autofluorescence in the central and hypo-autofluorescence areas associated with stippled hyper-autofluorescence areas in the peripheral retina corresponding with the scar from the previous lesions. Late-phase FA of the right (E) and left eye (F) show optic disc and peripheral peri-vascular leakage (yellow arrows) and staining of the retinal scars (blue arrows). SD-OCT of the right eye (G) shows temporal atrophy and intra-retinal fluid in the macula. SD-OCT of the left eye (H) shows cystoid macular edema.

Figure 3

SD-OCT of the right and left eye. Before immune recovery uveitis (IRU) treatment, SD-OCT shows intraretinal fluid in the right eye (A) and CME in the left eye (B). After one cycle of IVMP, SD-OCT shows intraretinal resolved in the right eye (C) and decreased CME in the left eye (D). After two cycles of tocilizumab (TCZ) infusion, SD-OCT shows completely resolved CME in right eye (E) and left eye (F).

Figure 4

Timeline of the patient’s treatment course. Abbreviations: CME, cystoid macular edema; CMV, cytomegalovirus; IRU, immune recovery uveitis; IVMP, intravenous pulse methylprednisolone; T-ALL, T-cell acute lymphoblastic leukemia.