Autoimmune Inflammatory Eye Disease: Demystifying Clinical Presentations for the Internist.

PURPOSE OF REVIEW: Provide a framework for recognizing key symptoms and clinical findings in patients with autoimmune inflammatory eye disease. RECENT FINDINGS: The most common manifestations of autoimmune inflammatory eye disease are episcleritis, scleritis, uveitis (anterior, intermediate, posterior, and panuveitis), and keratoconjunctivitis sicca. Etiologies can be idiopathic or in association with a systemic autoimmune condition. Referral of patients who may have scleritis is critical for patients presenting with red eyes. Referral of patients who may have uveitis is critical for patients presenting often with floaters and vision complaints. Attention should also be directed to aspects of the history that might suggest a diagnosis of a systemic autoimmune condition, immunosuppression, drug-induced uveitis, or the possibility of a masquerade condition. Infectious etiologies should be ruled out in all cases. Patients with autoimmune inflammatory eye disease may present with ocular or systemic symptoms alone, or in combination. Collaboration with ophthalmologists and other relevant specialists is vital to optimal long-term medical care.

Primary Vitreoretinal Lymphoma Masquerading as Post-Operative Endophthalmitis in a Pregnant Patient with Longstanding Idiopathic Panuveitis.

PURPOSE: To report primary vitreoretinal lymphoma after surgical 0.59 mg fluocinolone acetonide implant (FAi) exchange in a patient treated with adalimumab for idiopathic bilateral panuveitis. METHODS: Retrospective case review. RESULTS: A 37-year-old woman with bilateral idiopathic panuveitis, who had favorable responses to previous FAi surgical implants, presented with right eye recurrent intraocular inflammation and cystoid macular edema that partially responded to systemic adalimumab. Her FAi was replaced, given her previous favorable response. She developed post-operative ocular inflammation transiently responsive to two serial vitreous taps and injections of intravitreal antimicrobials and then worsening inflammation and new layered flocculant material. Diagnostic vitrectomy showed a few atypical lymphocytes and cultures were negative. At post-diagnostic vitrectomy month 1, flocculant material recurred. Aqueous cytology and flow cytometry revealed large CD45 positive B-cells suspicious for lymphoma. Post-operatively, she revealed that she was pregnant. She was treated with 8 monthly intravitreal methotrexate injections and post-partum consolidation radiotherapy. Subsequent repeat cytology, flow cytometry, and corneal pathology revealed large B-cells that were CD20 positive, and next generation sequencing detected a dominant monoclonal B-cell population, diagnostic of PVRL. 19 months after FAi exchange, she developed an area of enhancement in the lateral aspect of the right frontal lobe on brain MRI, consistent with central nervous system involvement. CONCLUSION: We present a unique case of PVRL masquerading as post-operative endophthalmitis after FAi exchange in an eye with chronic panuveitis treated with adalimumab immunosuppressive therapy. We hypothesize that there may be a causal relationship between adalimumab and PVRL.

Quantification of Geographic Atrophy Using Spectral Domain OCT in Age-Related Macular Degeneration.

PURPOSE: To compare geographic atrophy (GA) area and enlargement rate measured with spectral domain OCT (SD-OCT) with fundus autofluorescence (FAF), color fundus photography (CFP), and infrared reflectance (IR) imaging. DESIGN: Retrospective, multicenter, natural history case series. PARTICIPANTS: A total of 70 eyes with GA from 48 participants were included. METHODS: Participants underwent SD-OCT, FAF, CFP, and IR imaging at baseline and 12 months in the study eye. Spectral domain OCT images were graded for GA area using 2 distinct criteria: (1) complete retinal pigment epithelium and outer retinal atrophy (cRORA) and (2) hypertransmission through Bruch's membrane. Areas were measured with SD-OCT using a custom-developed tool that allows for mapping areas of retinal layer loss on SD-OCT cross-sectional scans with co-registered IR images. Circularity index was calculated from area and perimeter. Spectral domain OCT images were also assessed for presence of reticular pseudodrusen, outer-retinal tubules, and hyporeflective wedge-shaped bands. Area of GA was measured in millimeters squared from FAF, CFP, and IR images. MAIN OUTCOME MEASURES: Geographic atrophy mean baseline area and enlargement rate measured with 2 SD-OCT criteria, FAF, CFP, and IR. RESULTS: At baseline, the mean GA area was 6.9 (standard deviation [SD], 4.7) mm2 using the SD-OCT cRORA criteria and 7.3 (SD, 4.7) mm2 using the SD-OCT hypertransmission criteria. The mean annual enlargement rate of GA was 1.6 (SD, 1.1) mm2 using the SD-OCT cRORA criteria and 1.5 (SD, 1.0) mm2 using the SD-OCT hypertransmission criteria. When comparing both SD-OCT grading criteria with FAF, CFP, and IR, there were no statistically significant differences in baseline area or annual enlargement rate of GA. Circularity index was identified as the risk factor for increased annual enlargement rate. CONCLUSIONS: Measuring GA using retinal layer morphology provides a novel means of obtaining area measurements. Area measurements tend to vary based on criteria used and are comparable to other imaging modalities.

Prevalence and Severity of Artifacts in Optical Coherence Tomographic Angiograms.

Importance: Artifacts can affect optical coherence tomographic angiography (OCTA) images and may be associated with misinterpretation of OCT scans in both clinical trials and clinical settings. Objectives: To identify the prevalence and type of artifacts in OCTA images associated with quantitative output and to analyze the role of proprietary quality indices in establishing image reliability. Design, Setting, and Participants: This cross-sectional study evaluated baseline OCTA images acquired in multicenter clinical trials and submitted to the Fundus Photograph Reading Center in Madison, Wisconsin, between January 1, 2016, and December 31, 2018. Images were captured using the 3 mm × 3 mm and/or 6 mm × 6 mm scan protocol with commercially available OCTA systems. Artifacts, including decentration, segmentation error, movement, blink, refraction shift, defocus, shadow, Z offset, tilt, and projection, were given a severity grade based on involvement of cross-sectional OCT and area of OCT grid affected. Main Outcomes and Measures: Prevalence and severity of OCTA artifacts and area under the receiver operating characteristic curve (AUC) of quality indices with image reliability. Results: A total of 406 OCTA images from 234 eyes were included in this study, of which 221 (54.4%) were 6 mm × 6 mm scans and 185 (45.6%) were 3 mm × 3 mm scans. At least 1 artifact was documented in 395 images (97.3%). Severe artifacts associated with the reliability of quantitative outputs were found in 217 images (53.5%). Shadow (26.9% [109 images]), defocus (20.9% [85 images]), and movement (16.0% [65 images]) were the 3 most prevalent artifacts. Prevalence of artifacts did not vary with the imaging system used or with the scan protocol; however, the type of artifacts varied. Commercially recommended quality index thresholds had an AUC of 0.80 to 0.83, sensitivity of 97% to 99%, and specificity of 37% to 41% for reliable images. Conclusions and Relevance: Findings from this study suggest that artifacts associated with quantitative outputs on commercially available OCTA devices are highly prevalent and that identifying common artifacts may require correlation with the angiogram and cross-sectional OCT scans. Knowledge of these artifacts and their implications for OCTA indices appears to be warranted for more accurate interpretation of OCTA images.