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Purpose: To report a case of bilateral occlusive retinal vasculitis in a patient with autoimmunity. Methods: A case was analyzed and a literature review performed. Results: A 55-year-old woman with autoimmune diagnoses of Isaacs syndrome and inclusion body myositis (IBM) reported decreased vision for 3 months. A fundus examination showed peripheral intraretinal hemorrhages in the right eye and an inferotemporal subhyaloid hemorrhage with adjacent intraretinal hemorrhages and preretinal fibrosis in the left eye. Fluorescein angiography showed temporal peripheral leakage and capillary dropout in both eyes, consistent with occlusive vasculitis. Scatter laser treatment to peripheral areas of retinal nonperfusion was followed by an intravitreal bevacizumab injection. Four months later, vision had stabilized at 20/15 in both eyes and the peripheral leakage had resolved. Conclusions: This patient developed retinal vasculitis associated with the rare autoimmune neuromuscular disorders of Isaacs syndrome and IBM. An extensive workup showed the most plausible mechanism for the vasculitis was autoimmunity with a history of previously elevated antibodies levels associated with the antiphospholipid syndrome.

Sector and pericentral are two rare, regional forms of retinitis pigmentosa (RP). While usually defined as stable or only very slowly progressing, the available literature to support this claim is limited. Additionally, few studies have analyzed the spectrum of disease within a particular genotype. We identified all cases (9 patients) with an autosomal dominant Rhodopsin variant previously associated with sector RP (RHO c.316G > A, p.Gly106Arg) at our institution. Clinical histories were reviewed, and testing included visual fields, multimodal imaging, and electroretinography. Patients demonstrated a broad phenotypic spectrum that spanned regional phenotypes from sector-like to pericentral RP, as well as generalized disease. We also present evidence of significant intrafamilial variability in regional phenotypes. Finally, we present the longest-reported follow-up for a patient with RHO-associated sector-like RP, showing progression from sectoral to pericentral disease over three decades. In the absence of comorbid macular disease, the long-term prognosis for central visual acuity is good. However, we found that significant progression of RHO p.Gly106Arg disease can occur over protracted periods, with impact on peripheral vision. Longitudinal widefield imaging and periodic ERG reassessment are likely to aid in monitoring disease progression.