ARPE – acute retinal pigment epitheliitis as a rare inflammatory disorder of the retina

Revisiting acute retinal pigment epitheliitis (Krill disease)

Purpose:To describe a presumed case of acute retinal pigment epitheliitis (ARPE) which presented as an angular sign of Henle fiber layer hyperreflectivity (ASHH).Methods:We report a 27-year-old male with ARPE and ASHH suggestive of Henle fiber layer ischemia.Results:A 27-year-old male patient came with complaints of black spot in the left eye. There was a yellow lesion seen inferotemporal to the fovea, and multimodal imaging was suggestive of ARPE with ASHH. ARPE is an acute disorder affecting the ellipsoid and interdigitation zone which is seen on optical coherence tomography as ASHH sign, suggesting inflammatory insult to the photoreceptors. He was treated with oral steroids which led to the resolution of the lesion.Conclusion:ARPE may result in the inflammatory insult of photoreceptors.

We present a rare case of acute retinal pigment epitheliitis (ARPE) following vaccination. An 18-year-old Japanese man visited our hospital with a 5-day history of a central scotoma in the right eye. He had received the second dose of coronavirus disease 2019 vaccination (BNT162b2 mRNA, Pfizer-BioNTech) 1 month prior, following which he developed a low-grade fever of 37.3–37.5°C for 2 days accompanied by joint pain. Although he had received influenza vaccination 5 days prior to this presentation, no systemic symptoms other than injection site pain were observed. Blood test results were unremarkable. Ophthalmological examination revealed a decimal best-corrected visual acuity (BCVA) of 0.8 and 1.2 in the right and left eyes, respectively. Intraocular pressure was 15 mm Hg in both eyes. Intraocular inflammation was not observed. Fundus examination revealed a localized lesion of pigment stippling associated with yellowish hypopigmentation in the fovea. Fluorescein angiography revealed slight transmission hyperfluorescence without leakage. Optical coherence tomography (OCT) revealed disruption of the external limiting membrane (ELM), ellipsoid zone (EZ), and interdigitation zone (IZ). We diagnosed the patient with ARPE in the right eye. The patient was followed up without treatment. Five weeks after onset, the central scotoma in the right eye disappeared, and patient’s BCVA in the right eye improved to 1.5. OCT showed improvement in ELM and EZ continuity in the right eye, but IZ remained disruptive. Although the exact pathophysiology of the association between ARPE and these vaccinations is unclear, ARPE may develop after the vaccination.

Objective To evaluate efficacy and safety of anti-VEGF treatment for acute retinal pigment epitheliitis (ARPE) . Methods In this cases control study,15 cases (15eyes) of ARPE from October 2013 to February 2015 were studied. Vision acuity, fundus photographs, FFA and OCT were examined. The patients were divided into: anti-VEGF group and control group, and followed up 2 months. Results The difference in BCVA of two groups at baseline were not significant(P=0.06). BCVA of treatment group was significantly increased than that is the control group after 2 months followed up (P=0.001). Followed up 2 months, 8 cases in therapy group showed the impaired IS/OS and RPE layer were restored, however, the impaired IS/OS or RPE layer in control group were not completely restored. FFA also showed hyperfluorescein around macular center. Conclusion OCT and FFA repealed that initial lesion in ARPE located at the area between IS/OS and PRE inner layer. In acute stage, vision decreased associated with transmission of FFA, as well as disruption of IS/OS and PRE inner layer. Anti-VEGF therapy could improve visual acuity of ARPE quickly. Key words: Retinal pigment epitheliitis; anti-VEGF; Ranibizumab

To report new findings revealed by spectral-domain optical coherence tomography in a patient with bilateral acute retinal pigment epitheliitis. A patient presented with bilateral central scotoma and clinical and angiographic findings consistent with acute retinal pigment epitheliitis. Spectral-domain optical coherence tomography was performed at presentation and throughout follow-up. Early spectral-domain optical coherence tomography showed hyperreflectivity throughout the full thickness of the foveola, suggesting a full-thickness foveal inflammatory disorder. Later images provide insight into the natural course of acute retinal pigment epitheliitis and eventually showed a small outer retinal defect similar to that reported in some patients with macular microhole or foveal red spot. Acute retinal pigment epitheliitis is a macular inflammatory disorder that can involve the full thickness of the fovea and underlying retinal pigment epithelium and may result in a small defect in the outer retina similar to that reported in some patients with macular microhole or foveal red spot.

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. 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. 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.

Acute retinal pigment epitheliitis (ARPE) is an idiopathic, self-limitinginflammatory retinal disorder that particularly affects healthy youngindividuals. The characteristic fundoscopic appearance of the acute retinalpigment epitheliitis includes a fine pigment stippling surrounded by ayellow-white hypopigmented halos in the macula. Although the exact pathogenesisof the disease remains unknown, some reports have suggested a relationshipbetween a viral infection and acute retinal pigment epitheliitis. Acute retinalpigment epitheliitis is a rare disorder, and only single case reports or caseseries are found in the literature. The clinical and demographic characteristicsof patients with this disease are not fully understood because of its rarity. Inthis study, we searched the literature to collect clinical and demographicfeatures of the reported cases. We detail the characteristics of acute retinalpigment epitheliitis were pointed and discuss the pathogenesis of thedisease.

Spectral domain optical coherence tomography findings in acute retinal pigment epitheliitis

Épithélite rétinienne aiguë : apport de la tomographie par cohérence optique en Spectral Domain

To determine the specific location of the initial lesion in acute retinal pigment epitheliitis. Four patients diagnosed with acute retinal pigment epitheliitis were studied. Fundus photographs, fluorescein angiography and indocyanine green angiography, and spectral-domain optical coherence tomography findings were reviewed. Four healthy young patients presented with acute onset of unilateral decreased vision. Ophthalmoscopy showed macular pigment mottling with surrounding yellow hypopigmented areas at the level of the retinal pigment epithelium (RPE). Fluorescein angiography revealed transmission hyperfluorescence. Early-phase and midphase indocyanine green angiography images showed a patchy macular hyperfluorescence. At late phase of indocyanine green angiography, a hyperfluorescent halo with a cockadelike appearance of the macular area was observed. Spectral-domain optical coherence tomography showed a disruption of the photoreceptors' inner segment and outer segment interface associated with a wider disruption of the RPE inner band. These disrupted lines were replaced by a dome-shaped highly reflective lesion involving the RPE inner layer, the photoreceptors' inner segment and outer segment layers, and, in two cases, the outer nuclear layer. With time, indocyanine green angiography showed resolution of the observed lesions. Spectral-domain optical coherence tomography showed restored and continuous inner segment and outer segment layers and RPE inner band. Spectral-domain optical coherence tomography findings suggest that the initial lesion in acute retinal pigment epitheliitis is located at the junction between the photoreceptor outer segments and the apical side of the RPE cells. Indocyanine green angiography and spectral-domain optical coherence tomography show that the RPE appears to be more widely involved than the neurosensory retina.

Acute retinal pigment epitheliitis (ARPE) is a rare and transient macular disorder that was first described by Krill and Deutman in 1972. Later, Gerardo Ledesma-Gil and Spaide described a novel disease named acute fovealitis in 2022. According to them, this entity was different from ARPE on the basis of distinct features found in high-resolution optical coherence tomography (OCT) imaging. Angular sign of Henle fiber layer hyperreflectivity (ASHH) is a newly described terminology that is caused by an acute insult to photoreceptor compartments. In this article, we report a case that has both the ophthalmoscopic and OCT features of acute fovealitis as described in the literature, with the presence of ASHH in the acute stage, which resolves on its own rapidly along with the symptoms without any intervention.

Because of some authors presumptions that acute retinal pigment epitheliitis is not a distinct clinical entity but the early stage of other fundus diseases, the results of long-term observations of 5 patients with this disease are reported. The results of 6- to 7-year observations of these patients have shown that in none of them the fundus lesions developed into other retinal diseases. This indicates that acute retinal pigment epitheliitis is a distinct clinical entity.

Background and Clinical Significance: Acute retinal pigment epitheliitis is a retinal disorder considered as part of a larger group named idiopathic choroidopathies. Little gray round macular lesions at the retinal pigment epithelium can be found, which are self-limited, resolving within 6–12 weeks. It can decrease best corrected visual acuity (BCVA), but visual quality has not been studied yet. Case Presentation: A 17-year-old Caucasian boy who came to our ophthalmology department and presented with acute retinal pigment epitheliitis in his right eye (OD). BCVA under mesopic lighting was 0.18 logMAR in his OD and −0.18 in his OS. With a neutral density filter, it was 0.52 and 0.04, respectively. Contrast sensitivity was assessed with the CSV-1000E test, but OD outcomes were worse only in the case of mesopic lighting. Chromatic discrimination was assessed with the Farnsworth–Munsell 100 test and revealed marked impairment of both red-green and yellow-blue axes. No central scotoma was detected on a 10.2 visual field, nor was any halo perception detected with the Halometer test. Conclusions: BCVA under low illumination and color perception in the yellow-blue axis may be affected in patients with acute retinal pigment epitheliitis to a greater extent than previously described. Contrast sensitivity may also be altered, but to a lesser extent.

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Acute retinal pigment epitheliitis (Krill’s disease) is a rare idiopathic self-limiting inflammatory macular disease. The article presents the results of multimodal dynamic imaging in 2 patients with acute retinal pigment epitheliitis, including, for the first time ever, the results of optical coherence tomography-angiography, suggesting the pathogenetic significance of changes in perfusion in the choriocapillaris for the development of this disease.