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Sarwar Zahid, Melisa Nika, Amani Al-Tarouti, Kari E Branham, John R Heckenlively, Thiran Jayasundera; Distinct Patterns of Fundus Autofluorescence in Paraneoplastic and Non-Paraneoplastic Forms of Autoimmune Retinopathy Responding to Systemic Immunosuppressive Therapy. Invest. Ophthalmol. Vis. Sci. 2014;55(13):2505.
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To describe distinct patterns of fundus autofluorescence (FAF) in patients with paraneoplastic (pAIR) and non-paraneoplastic autoimmune retinopathy (npAIR) who demonstrate a clinical response to systemic immunosuppression.
A retrospective study was conducted of 35 patients with AIR, who met the following diagnostic criteria: 1) presented with sudden adult-onset rapidly progressive visual disturbance; 2) exhibited abnormal full-field ERG (ffERG) parameters; 3) anti-retinal antibody positivity, including anti-recoverin. Clinical data focused on FAF findings in patients who exhibited objective clinical improvement after immunosuppressive treatment as detected by a 25% improvement in quantified Goldmann visual fields (GVF) or visual acuity (VA) during follow-up.
Twenty-four patients with npAIR and 11 patients with pAIR exhibited improvement after immunosuppressive treatment during follow-up. Sixteen patients had FAF imaging available. Fourteen of these patients exhibited a ring of parafoveal hyperfluorescence; 2 of these patients had hyperfluorescence outside the vascular arcades as well. Two patients exhibited central foveal hyperfluorescence, while 4 patients exhibited peripheral hypofluorescence outside and along the vascular arcades suggestive of retinal pigment epithelium (RPE) atrophy. Three patients exhibited peripapillary atrophy with surrounding hyperfluorescence. FAF findings were symmetric in each eye irrespective of interocular differences in GVF areas or VA in each patient.
Patients with suspected AIR that exhibit improved visual function with treatment constitute an ideal group in which to investigate the presence of FAF signatures in this heterogeneous disease. We confirm that FAF changes seen in retinal dystrophies, such as concentric rings of parafoveal hyperfluorescence and central foveal hyperfluorescence, are also present in patients with AIR.
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