Combination Treatment with Rituximab and Bortezomib in a Patient with Non-Paraneoplastic Autoimmune Retinopathy

ABSTRACT

Purpose

To describe outcomes of combined rituximab and bortezomib treatment for non-paraneoplastic autoimmune retinopathy.

Case

A 37-year-old female developed photopsias and reduced vision. Electroretinography, optical coherence tomography, and positive serum anti-retinal antibodies were consistent with autoimmune retinopathy. A negative malignancy work-up specified her non-paraneoplastic presentation. Given absence of response to periocular steroids, azathioprine, and methotrexate, a combination of rituximab and bortezomib was initiated as fifth-line therapy.

Results

There was no significant improvement in the patient’s symptoms or visual function following treatment. The full field electroretinogram amplitudes were reduced with progressive outer retinal degeneration evident on optical coherence tomography. Post-treatment anti-retinal antibody testing demonstrated the persistence of antibodies and revealed additional antibodies not previously detected.

Conclusion

Combined rituximab and bortezomib treatment did not result in significant clinical improvement and there was evidence of disease progression. Further prospective studies are required to assess the efficacy of immunotherapy in patients with autoimmune retinopathy.

Keywords:

• Anti-retinal antibodies
• autoimmune retinopathy
• bortezomib
• non-paraneoplastic autoimmune retinopathy
• rituximab

Acknowledgments

There was no funding or grant support for this study. Bortezomib was donated by Janssen.

Disclosure of Interest

Benson, MD: no disclosures; Plemel, DJA: no disclosures; Yacyshyn, E: no disclosures; Sandhu, I: received Honoria from Janssen, Takeda; MacDonald, IM: no disclosures; Baker, CF: no disclosures. All authors attest that they meet the current ICMJE criteria for authorship.

Patient Consent

Written consent was obtained from the patient for the publication of this case report, including medical record details, fundus photos, and electrophysiologic and ancillary testing.