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Abstract Background Acute Retinal Pigment Epitheliitis (ARPE, Krill’s disease) is a rare inflammatory retinal disorder commonly affecting young adults. It often presents unilaterally with central vision disruption, and typically resolves with vision restoration within 6 to 12 weeks. The pathogenesis of ARPE remains a subject of ongoing debate. Adaptive Optics Flood Illumination Ophthalmoscopy (AO-FIO) imaging has emerged as a valuable tool capable of detecting early cone photoreceptor changes and recovery. This case study presents two patients with ARPE, with longitudinal follow-up using multimodal imaging, including optical coherence tomography (OCT) and AO-FIO. Case presentations A 30-year-old male presented with sudden vision loss in both eyes. The best corrected visual acuity (BCVA) was 20/33 and 20/40 Snellen in the right and left eye, respectively. OCT showed interruption of the ellipsoid zone (EZ) band and outer nuclear layer (ONL) in both eyes; AO-FIO imaging revealed a foveal lesion and diminished parafoveal cone density in both eyes compared to two age-matched controls. After 6 months, BCVA was restored to 20/20, and OCT showed recovery of the ONL and EZ. On AO-FIO, the foveal lesion was still present and the parafoveal cone density increased but remained reduced even up to 15 months after onset when compared to the controls. The second patient, a 30-year-old woman, presented with a unilateral drop in vision to 20/63 Snellen. OCT showed discontinuation of the EZ and hyperreflectivity within the ONL and retinal pigment epithelium in the affected eye. The unaffected eye showed no abnormalities. After 3 months, the BCVA improved to 20/16 Snellen and OCT showed recovery of the EZ. AO-FIO was conducted 9 months after onset and revealed reduced parafoveal cone density in the affected and non-affected eye compared to the controls while OCT still showed recovery of all retinal layers. Conclusions ARPE is a self-limiting disease with recovery of BCVA and OCT retinal layers within 6 months. However, our 2 cases showed that parafoveal cone density recovered during follow-up but did not reach levels observed in controls. AO-FIO is an imaging modality that enhances sensitivity in measurements and can therefore be used as a complementary tool for follow-up.

Background: Acute Retinal Pigment Epitheliitis (ARPE, Krill's disease) is a rare inflammatory retinal disorder commonly affecting young adults. It often presents unilaterally with central vision disruption, and typically resolves with vision restoration within 6 to 12 weeks. The pathogenesis of ARPE remains a subject of ongoing debate. Adaptive Optics Flood Illumination Ophthalmoscopy (AO-FIO) imaging has emerged as a valuable tool capable of detecting early cone photoreceptor changes and recovery. This case study presents two patients with ARPE, with longitudinal follow-up using multimodal imaging, including optical coherence tomography (OCT) and AO-FIO. Case presentations: A 30-year-old male presented with sudden vision loss in both eyes. The best corrected visual acuity (BCVA) was 20/33 and 20/40 Snellen in the right and left eye, respectively. OCT showed interruption of the ellipsoid zone (EZ) band and outer nuclear layer (ONL) in both eyes; AO-FIO imaging revealed a foveal lesion and diminished parafoveal cone density in both eyes compared to two age-matched controls. After 6 months, BCVA was restored to 20/20, and OCT showed recovery of the ONL and EZ. On AO-FIO, the foveal lesion was still present and the parafoveal cone density increased but remained reduced even up to 15 months after onset when compared to the controls. The second patient, a 30-year-old woman, presented with a unilateral drop in vision to 20/63 Snellen. OCT showed discontinuation of the EZ and hyperreflectivity within the ONL and retinal pigment epithelium in the affected eye. The unaffected eye showed no abnormalities. After 3 months, the BCVA improved to 20/16 Snellen and OCT showed recovery of the EZ. AO-FIO was conducted 9 months after onset and revealed reduced parafoveal cone density in the affected and non-affected eye compared to the controls while OCT still showed recovery of all retinal layers. Conclusions: ARPE is a self-limiting disease with recovery of BCVA and OCT retinal layers within 6 months. However, our 2 cases showed that parafoveal cone density recovered during follow-up but did not reach levels observed in controls. AO-FIO is an imaging modality that enhances sensitivity in measurements and can therefore be used as a complementary tool for follow-up. [ABSTRACT FROM AUTHOR]

We reevaluate acute retinal pigment epitheliitis (ARPE) first described by Krill and Deutman in 1972, integrating a meticulous literature review with advanced multimodal imaging analyses. Our review included 98 eyes from 86 published cases diagnosed with ARPE. We scrutinized ARPE's clinical presentations, variability, and imaging characteristics, revealing that a large majority (90 %) of cases previously diagnosed as ARPE align more closely with other retinal disorders based on modern diagnostic criteria and imaging techniques. Only a small fraction (5 eyes) did not fit into any known categories, casting doubt on ARPE's distinct existence. This underscores the critical role of multimodal imaging in redefining our understanding of macular diseases and challenges the historical classification of ARPE as a unique clinical entity. [ABSTRACT FROM AUTHOR]

Abstract Purpose: To report a case of a young female who presented with scotoma in the right eye for few days. Case Report: Krill's disease or acute retinal pigment epithelitis (ARPE) is a self-limiting retinal disease with no specific treatment. Typical clinical and imaging features helped us to diagnose her with ARPE. Intravenous methylprednisolone (IVMP), which gives a rapid anti-inflammatory response, was advised. An SD-OCT scan post-injection showed a reduction in hyperreflectivity and height of lesion at day 3 and near total resolution by day 5. Conclusion: This case suggests rapid resolution of ARPE with the use of IVMP.

Purpose: To report a case of a young female who presented with scotoma in the right eye for few days. Case Report: Krill's disease or acute retinal pigment epithelitis (ARPE) is a self-limiting retinal disease with no specific treatment. Typical clinical and imaging features helped us to diagnose her with ARPE. Intravenous methylprednisolone (IVMP), which gives a rapid anti-inflammatory response, was advised. An SD-OCT scan post-injection showed a reduction in hyperreflectivity and height of lesion at day 3 and near total resolution by day 5. Conclusion: This case suggests rapid resolution of ARPE with the use of IVMP. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate if high resolution adaptive optics confocal scanning laser ophthalmoscopy (AO-SLO) can be used as an adjunct complementary diagnostic tool to spectral domain optical coherent tomography (SD-OCT) in characterizing three macular diseases: rod-cone dystrophy, acute retinal pigment epitheliitis (Krill’s disease), and occult macular dystrophy. Methods: As part of a complete clinical examination, each patient was subjected to color fundus pictures, multimodal imaging scans with Heidelberg SpectralisTM and high resolution retinal images with a custom built adaptive optics scanning laser ophthalmolscope (AO-SLO). The registered AO-SLO images were averaged to improve the signal to noise ratio and used to generate larger photoreceptor mosaics. Results: AO-SLO mosaics for all three conditions showed distinct, characteristic disruptions of the photoreceptors in areas that corresponded to the abnormalities observed on fundus photography and SD-OCT scans. Conclusions: AO-SLO defined fine structural changes associated with retinal pathology at the photoreceptor level that could not be achieved using standard diagnostic methods. A combination of adaptive optics scanning laser ophthalmoscopy (AO-SLO) and SD-OCT provided views of the retina with enhanced lateral and axial resolution. High-resolution, ultra-structural details of the retina may provide additional insights into the disease etiology, progression and management of patients with vision threatening macular diseases.