Treatment of Cancer Associated Retinopathy: A Systematic Literature Review.

TOPIC: Systematic literature review of treatment efficacy of previously used protocols in treating patients with proven cancer-associated retinopathy (CAR).

CLINICAL RELEVANCE: There is no universally accepted treatment algorithm for CAR and visual prognosis in very poor. We describe a patient with CAR with dramatic improvement in vision after treatment with high doses of corticosteroids followed by plasma exchange (PLEX) and present results of systematic literature review of treatment efficacy of previously used protocols in treating patients with proven CAR.

METHODS: We describe a 70-year-old man with CAR who demonstrated dramatic improvement in vision after treatment with high doses of systemic corticosteroids followed by 7 sessions of PLEX. We then report results of a systematic review of all previously published English literature discussing visual outcomes of various treatment regiments used for patients with antibody-proven CAR.

RESULTS: The index patient is the first case of CAR with sustained significant improvement in vision after treatment with high doses of corticosteroids followed by PLEX. The systematic review identified 28 antibody-proven cases of CAR, 26 of these were treated with steroids which resulted in varying degree of improvement in visual acuity in 58% (15/26). The time from symptoms onset to initiation of treatment and the dose of steroids did not influence visual outcome. Three patients were also treated with PLEX in addition to steroids and 2/3 demonstrated improvement in vision, however, there was no difference in visual outcome in patients treated with steroids only vs those treated with steroids + PLEX.

CONCLUSION: Treatment with steroids and/or steroids + PLEX results in some improvement of visual acuity in 60% of patients. Removal of anti-recoverin antibodies with PLEX can arrest the immune attack on the photoreceptors and potentially improve visual function thus it should be considered in addition to steroids. Further studies with larger cohorts are needed in order to establish a treatment protocol and further determine the effectiveness of the different approaches.