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Stabilization of Visual Field With Intravitreal Triamcinolone in Cancer-Associated Retinopathy: A Case Report and Review of the Literature
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The purpose of this study is to report the use of intravitreal triamcinolone for treatment of cancer-associated retinopathy (CAR) refractory to systemic therapy.
This was a retrospective chart review study.
A 67-year-old man presented with cancer-associated retinopathy with antibodies against a 46-kDa retinal protein, alpha enolase. There was disease progression despite therapy with mycophenolate and intravenous immunoglobulin. Serial intravitreal injections of triamcinolone resulted in restoration of photoreceptor anatomy on optical coherence tomography and visual improvement. The patient's vision was preserved at 20/40 OD and 20/32 OS until his death from lung cancer 31 months after CAR diagnosis.
Intravitreal triamcinolone may be beneficial for maintenance of vision in patients with CAR.
To report the results of treating autoimmune retinopathy (AIR) with immunosuppression therapy.
Retrospective review of 30 consecutive patients with AIR followed for 3 to 89 months (median, 17 months) who were treated with immunosuppression (systemic or local). Subgroups were cancer-associated retinopathy (CAR), nonparaneoplastic AIR (npAIR), and npAIR with cystoid macular edema (npAIR/CME). Outcome measures were improvement of Snellen visual acuity by at least 2 lines, expansion of the visual field area by more than 25%, and resolution of CME.
Overall, 21 of 30 patients (70%) showed improvement. All 6 CAR patients, 7 of 13 (54%) with npAIR, and 8 of 11 (73%) with npAIR/CME showed improvement. Five of 21 patients (24%) had improvement in visual acuity, 15 of 21 (71%) had expansion of visual field area, and 6 of 11 (55%) had resolution of CME. Twenty-six of 30 patients exhibited diffuse retinal atrophy without pigment deposits. An autoimmune family history was common in all the groups: npAIR, 69% (9 of 13); npAIR/CME, 64% (7 of 11); and CAR, 50% (3 of 6).
Long-term treatment with immunosuppression resulted in clinical improvement in all subgroups of AIR. The most responsive subgroup was CAR; the least was npAIR. These results challenge the commonly held belief that AIR is untreatable.
To assess the cytotoxic T lymphocyte antigen 4 (CTLA4) pathway in the recoverin peptide (R64; AYAQHVFRSF) mouse model of cancer-associated retinopathy (CAR) and to assess the protective effects of subconjunctival triamcinalone injections in this model.
To study the role of the CTLA4 pathway on the R64-induced mouse model of CAR, BALB/c mice were immunized with R64. The mice were further intraperitoneally treated with anti-CTLA4 antibody to get stronger immunoreaction. The development of CAR was evaluated by electroretinogram (ERG) examinations 21 days after treatment. A cytotoxicity assay was employed to detect induction of R64-specific cytotoxic T lymphocytes (CTLs). Immunoblotting to assess the development of anti-recoverin antibody and a T cell proliferation assay to determine the activity of lymphocytes against R64 were examined in two experimental groups, anti-CTLA4 antibody treated and untreated mice.To study the protective effect of subconjunctival triamcinalone in this model, mice immunized with R64 peptide and anti-CTLA4 antibody were either treated with 50 mg/kg/body weight of triamcinalone or phosphate buffered saline (PBS). These mice were assayed using ERG and histological examination 35 days after the first R64 immunization.
When mice were challenged with R64 peptide and anti-CTLA4 antibody, R64 peptide-specific CTLs were induced and decreased b-wave amplitudes were observed in ERG. Conversely, no CAR symptoms were detected in mice not treated with anti-CTLA4 antibody. Anti-CTLA4 antibody treatment did not give any significant differences in T cell proliferation and humoral reaction against recoverin. Subconjunctival triamcinalone treated mice show a trend toward improved survival of outer nuclear layer cell bodies, but did not show significant improvement of ERG amplitudes compared to the untreated mice.
Inhibition of the CTLA4 pathway is essential for the development of recoverin-induced murine CAR, suggesting that strengthening negative T cell signaling through CTLA4 may lessen the retinal degenerations in CAR-affected subjects. The positive effects of attenuation of the CTLA4 pathway must be weighed against a potential negative effect on survival since this pathway may also provide natural immunotherapy against the underlying malignancy. Subconjunctival injections of triamcinalone may have beneficial effects on the integrity of the outer nuclear layer (ONL) of the retina in the CAR model, although there was no significant effect on the ERG recordings.
We write to report a rare case of unilateral cancer-associated retinopathy previously undocumented in the literature. Cancer-associated retinopathy is an uncommon paraneoplastic syndrome. It is characterised by retinal antigen autoantibodies causing rod and cone dysfunction and abnormal electroretinography findings with consequent progressive visual loss.Our patient, known to have a primary cervical intraepithelial neoplasia, presented with unilateral blurred vision as well as a disturbance in colour and night vision. Electroretinography findings of reduced a and b waves in the right eye, together with a fundoscopic appearance of a mottled retinal pigment epithelium, attenuated blood vessels and optic disc pallor were consistent with unilateral cancer-associated retinopathy. Posterior subtenon injections of triamcinolone were administered to control active disease. With periocular steroid injections, at 4 years, our patient's visual acuity remained relatively stable and her condition persisted strictly unilaterally. Cancer-associated retinopathy may be the first presenting sign of an underlying malignancy or may indicate its recurrence. Moreover, in patients with a diagnosed gynaecological malignancy, visual symptoms could reflect cancer-associated retinopathy. In our patient visual symptoms came secondary to the diagnosis of cancer.
© The Author(s) 2015.
Paraneoplastic syndromes involving the visual system are a heterogeneous group of disorders occurring in the setting of systemic malignancy. Timely recognition of one of these entities can facilitate early detection and treatment of an unsuspected, underlying malignancy, sometimes months before it would have otherwise presented, and gives the patient an increased chance at survival. We outline the clinical features, pathogenesis, and treatment strategies for the retinal- and optic nerve-based paraneoplastic syndromes: cancer-associated retinopathy; melanoma-associated retinopathy; paraneoplastic vitelliform maculopathy; bilateral diffuse uveal melanocytic proliferation; paraneoplastic optic neuropathy; and polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome. Distinguishing these disorders from their non-paraneoplastic counterparts (e.g., autoimmune-related retinopathy and optic neuropathy, and acute zonal occult outer retinopathy) and determining appropriate systemic evaluation for the responsible tumor can be challenging. In addition, we discuss the utility and interpretation of autoantibody testing.
Cancer associated and related autoimmune retinopathies
- R K Maturi
Maturi RK, et al. Cancer associated and related autoimmune retinopathies. Medscape 2016.