Posterior Multifocal Placoid Pigment Epitheliopathy Research Articles

Application of Ophthalmic Electrophysiology in Inflammatory Disorders of Retina and Optic Nerve.

This review covers the utility of electrophysiological studies relevant to inflammatory diseases of the retina in conditions such as acute posterior multifocal placoid pigment epitheliopathy, acute zonal occult outer retinopathy, Adamantiades-Behçet disease, autoimmune retinopathy and neuro-retinopathy, birdshot chorioretinopathy, multiple evanescent white dot syndrome, and Vogt-Koyanagi-Harada disease. Electrophysiological studies can help with the diagnosis, prognostication, evaluation of treatment effects, and follow-up for these conditions.

APMPPE and Placoid Variant Diseases Masquerading as Age-Related Macular Degeneration in the Elderly: A Case Series.

To report eight cases of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) or persistent placoid maculopathy (PPM) initially masquerading as age-related macular degeneration (AMD) in elderly individuals. APMPPE or PPM eyes in patients above age 55 years with macular RPE disruption including drusenoid lesions on macular examination and/or with multimodal imaging were included. At least one method of multimodal imaging including fluorescein angiography (FA), indocyanine green angiography (ICGA), optical coherence tomography (OCT) and OCT angiography (OCTA) was performed in all eyes for diagnosis and to monitor for macular neovascularization (MNV). Eight elderly male patients presented with vision loss and were all initially diagnosed with non-neovascular or neovascular AMD. With the aid of multimodal retinal imaging, a final diagnosis of either APMPPE or PPM was rendered. With FA and ICGA, choroidal hypoperfusion was detected in all but one eye. With OCT, the angular sign of Henle fiber layer hyperreflectivity (ASHH) was identified in >50% of eyes. With OCTA, inner choroidal flow deficits were detected in all eyes. MNV requiring anti-VEGF injection therapy complicated 3 of 8 cases. Both APMPPE and PPM may develop in elderly individuals and may masquerade as AMD on presentation. Multimodal imaging including FA, ICGA, and OCTA are important diagnostic modalities to assess for inner choroidal hypoperfusion to arrive at an accurate diagnosis, and to detect MNV which frequently complicates APMPPE and PPM. In these patients, serial anti-VEGF intravitreal injections are essential in treating MNV and in preventing significant vision loss.

The Spectrum of Relentless Placoid and Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE): Multimodal Imaging Analysis.

To showcase the spectrum between APMPPE and relentless placoid utilizing ultra-widefield imaging findings of a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) progressing to relentless placoid chorioretinitis (RPC). A 23-year-old Caucasian female presented with worsening vision in both eyes. Clinical examination and multimodal imaging modalities including fundus photos, fundus autofluorescence, fluorescein angiography (FA), indocyanine green angiography (ICGA) and spectral-domain optical coherence tomography and angiography were utilized to diagnose APMPPE and clinically follow the patient. Clinical examination of the patient initially revealed posterior lesions consistent with APMPPE but subsequent multimodal images including ultra-widefield FA and ICGA revealed newer, more peripheral lesions more typical of RPC. When compared to standard multimodal imaging, ultra-widefield imaging is an effective tool to delineate nuances between APMPPE and RPC through identification of peripheral lesions, which may be of clinical importance when determining management and therapeutics for patients.

Unilateral acute posterior multifocal placoid pigment epitheliopathy (APMPPE) with delayed contralateral eye involvement

BackgroundAcute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare presumed inflammatory chorioretinopathy characterized by creamy, yellow-white placoid lesions at the level of the retinal pigment epithelium (RPE). Unilateral cases often have fellow eye involvement within days to a few weeks. This report details a rare case of delayed contralateral APMPPE, in which unilateral lesion resolution was followed by contralateral eye involvement 31 months later.Case presentationA 38-year-old woman presented with three days of blurry vision and photopsias in the right eye (OD). She endorsed a viral GI illness one month prior. Visual acuity was 20/25 -2 OD and 20/20 -1 in the left eye (OS). Examination revealed creamy, yellow-white placoid lesions in the posterior pole. Fluorescein angiography (FA) was notable for early hypofluorescence and late hyperfluorescence of the lesions, consistent with APMPPE. MRI and MRA brain were negative for cerebral vasculitis. She was treated with oral prednisone with complete resolution of her symptoms, vision, and lesion regression. She then presented 31 months later, with blurry vision OS and similar new creamy, yellow-white placoid lesions in the posterior pole OS. She endorsed receiving an influenza vaccine one month prior. FA again was notable for early hypofluorescence. She was diagnosed with APMPPE, this time involving the left eye, and was once again started on oral steroids with complete resolution. She denied any neurologic symptoms.ConclusionsAPMPPE is an inflammatory vasculitis of the choroid, leading to hypoperfusion and ischemic injury of the RPE with subsequent lesion formation. APMPPE may be preceded by a viral prodrome or vaccination, both of which were seen in this case. Choroidal inflammation seen in APMPPE is therefore thought to stem from immune-mediated processes. Unilateral cases often have fellow eye involvement within days to a few weeks. Single eye involvement with delayed contralateral presentation, as seen in our patient, is rare. This case demonstrates that lesion resolution in one eye can be followed by contralateral eye involvement up to 31 months later, highlighting the importance of routine ophthalmic monitoring for patients with unilateral APMPPE.

Acute posterior multifocal placoid pigment epitheliopathy following inactivated SARS-CoV-2 vaccination: A case report

A 25-year-old woman presented with redness and pain in both eyes after the first dose of the inactivated vaccine Corona Vac (Sinovac Biotech Ltd., Beijing, China). She had non granulomatous anterior segment inflammation and multiple creamy placoid lesions within the posterior pole and midperipheral retina bilaterally. Fluorescein angiography (FA) revealed early hypofluorescent and late hyperfluorescent lesions, while indocyanine green angiography (ICGA) demonstrated hypofluorescent lesions throughout all phases. A diagnosis of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) was made, and the patient was started on a course of oral prednisolone that was tapered and the inflammation was completely resolved.

A case of syphilitic chorioretinitis masquerading as acute posterior multifocal placoid pigment epitheliopathy

Ocular involvement can be the first and only manifestation of syphilis, and it may mimic any sign of posterior segment involvement. We report a 22-year-old man with syphilitic multifocal chorioretinitis who had clinical and imaging findings with acute posterior multifocal placoid pigment epitheliopathy (APMPPE) features in both eyes without any systemic and neurological symptoms. Systemic antibiotic and corticosteroid treatment led to regression in multifocal chorioretinitis lesions and improvement in visual acuity. Syphilis may present as APMPPE. Syphilis tests should be kept in mind and carefully evaluated in the differential diagnosis of patients presenting with intraocular inflammation of unknown origin.

Ocular Inflammation Post-Vaccination.

The association between vaccines and ocular disorders has attracted significant attention in scientific research. Numerous mainstream vaccines are associated with a range of uveitis types, including anterior, intermediate, and posterior uveitis. Additionally, they are associated with distinct ocular diseases such as multifocal choroiditis, Vogt-Koyanagi-Harada (VKH) disease, acute posterior multifocal placoid pigment epitheliopathy (APMPPE), and multiple evanescent white dot syndrome (MEWDS). These ocular conditions are often transient, with a vast majority of patients experiencing improvement after steroid intervention. To date, numerous cases of vaccine-induced uveitis have been reported. This study analyzed the correlation between antiviral vaccines, including the hepatitis B virus (HBV), human papillomavirus (HPV), measles-mumps-rubella (MMR), varicella zoster virus (VZV), and influenza vaccines, and different manifestations of uveitis. This is the first comprehensive study to offer a detailed analysis of uveitis types induced by antiviral vaccines. Through an extensive database search, we found a particularly strong link between influenza vaccines, followed by VZV and HPV vaccines. While anterior uveitis is common, conditions such as APMPPE, MEWDS, and VKH are particularly notable and merit careful consideration in clinical practice. Corticosteroid treatment was effective; however, half of the observed patients did not achieve full recovery, indicating potentially prolonged effects of the vaccine.

Blue-Light Fundus Autofluorescence (BAF), an Essential Modality for the Evaluation of Inflammatory Diseases of the Photoreceptors: An Imaging Narrative.

Our purpose is to describe blue-light fundus autofluorescence (BAF) features of inflammatory diseases of the outer retina characterised by photoreceptor damage. BAF from patients diagnosed with secondary and primary inflammatory photoreceptor damage were retrospectively analyzed and compared to other imaging modalities including fluorescein angiography (FA), indocyanine green angiography (ICGA), and spectral domain optical coherence tomography (SD-OCT). Multiple evanescent white dot syndrome (MEWDS), idiopathic multifocal choroiditis (MFC), acute posterior multifocal placoid pigment epitheliopathy (APMPPE), serpiginous choroiditis (SC), and acute syphilitic posterior placoid chorioretinitis (ASPPC), all cases corresponding to secondary photoreceptor diseases caused by inflammatory choriocapillaris nonperfusion, were included and compared to primary photoreceptor disease entities, including acute zonal occult outer retinopathy (AZOOR) and cancer-associated retinopathy (CAR). Both groups showed increased BAFs of variable intensity. In severe cases of APMPPE and ASPPC, BAF also showed hypoautofluorescent areas. In group 1 (secondary diseases) BAF hyperautofluorescent areas were associated with colocalized ICGA hypofluorescent areas, indicating choriocapillaris nonperfusion; whereas in group 2 (primary diseases), no ICGA signs were detected. The associated colocalized areas of hypofluorescence on ICGA in the first group, which were absent in the second group, were crucial to allow the differentiation between primary (photoreceptoritis) and secondary (choriocapillaritis) photoreceptor diseases. BAF patterns in inflammatory diseases of the outer retina can give relevant information on the photoreceptor and RPE involvement, with ICGA being crucial to detect concurring choriocapillaris damage and differentiating the two pathologies.

Острая задняя мультифокальная плакоидная пигментная эпителиопатия

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is more common in people of young working age. It manifests itself in the appearance of light foci on the retina, leading to a decrease in visual acuity. The presented clinical cases demonstrate an interdisciplinary approach in the diagnosis and treatment of this disease. In some cases, the disease may occur without reducing visual acuity and be undiagnosed. In the acute stage of APMPPE, the use of anti-inflammatory therapy, including systemic steroids, gives a good clinical effect. Keywords: acute posterior multifocal placoid pigment epitheliopathy, gray-white foci, diagnosis, anti-inflammatory therapy

Clinical Outcomes of Observed and Treated Acute Posterior Multifocal Placoid Pigment Epitheliopathy and Relentless Placoid Chorioretinitis

ABSTRACT Purpose To assess the efficacy of treatment on acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and relentless placoid chorioretinopathy (RPC). Methods Cases were identified from three UK uveitis centers. Retrospective analysis of visual acuity recovery; OCT structural outcomes; and retinal lesion quantification in observed and treated cases of APMPPE/RPC. Results There were nine APMPPE and three RPC cases. Out of 12 patients, six were female. Median age: 26.5 years (range, 20–57 years). Four cases (six eyes) were observed, and eight cases (15 eyes) received corticosteroids ± immunosuppression. 4/4 observed and 6/10 treated foveal involving eyes regained 0.00 LogMAR vision. Observed lesions achieved more favorable anatomical outcomes. New lesions post-presentation developed in 1/6 (16%) observed eye versus 10/15 (66%) treated eyes. In three cases, a delayed, rebound lesion occurrence was observed post-high-dose corticosteroids. Conclusions While subject to potential treatment bias, in this small case series, natural history alone appears non-inferior to corticosteroid treatment.

An typical case of Acute Posterior Multifocal placoid pigment Epitheliopathy with intraretinal fluid

An typical case of Acute Posterior Multifocal placoid pigment Epitheliopathy with intraretinal fluid

OCT Angiography in Noninfectious Uveitis: A Description of Five Cases and Clinical Applications.

Optical coherence tomography angiography (OCTA) is a noninvasive imaging modality used to analyze the retinochoroidal vasculature and detect vascular flow. The resulting images can be segmented to view each vascular plexus individually. While fluorescein angiography is still the gold standard for the diagnosis of posterior uveitis, it has limitations, and can be replaced by OCTA in some cases. This case series describes five patients with posterior noninfectious uveitis and their description by OCTA. Cases included lupus retinopathy (n = 1) for which OCTA showed ischemic maculopathy as areas of flow deficit at the superficial and deep capillary plexus; choroidal granulomas (n = 1) with a non-detectable flow signal in the choroid; active punctate inner choroiditis and multifocal choroiditis (n = 1) with OCTA that showed active inflammatory chorioretinal lesions as non-detectable flow signals in choriocapillaris and choroid; dense type 2 inflammatory secondary neovascularization (n = 1) associated with active choroiditis; and acute posterior multifocal placoid pigment epitheliopathy (APMPPE) (n = 1) without flow abnormalities at the superficial and deep retinal plexuses but non-detectable flow at the levels of the choriocapillaris and choroid. Ophthalmologists can use OCTA to identify inflammatory changes in retinal and choroidal vasculature, aiding in the diagnosis, management, and monitoring of posterior uveitis.

Relentless placoid chorioretinitis: A review of four cases in pediatric and young adult patients with a discussion of therapeutic strategies.

Relentless placoid chorioretinitis (RPC) is a rare, bilateral disease of the retinal pigment epithelium. The clinical course is prolonged and relapsing. No standard treatment has been established to date. The purpose of this case series is to report four cases of RPC in pediatric and young adult patients in which varying treatments were used, comparing them to previously published cases. A literature review was conducted to investigate currently published presentations and treatment options for RPC. A multicenter retrospective chart review was also performed on four consecutive patients. These patients were diagnosed with RPC because of new chorioretinitis lesions continuing to appear without or despite therapy for 5-36 months (2 patients), with a clinical course prolonged and relapsing, or because of the atypical location of the multiple lesions (>50) extending from the posterior pole to the equator and mid-peripheral retina (all four patients), which were not consistent with other entities like acute posterior multifocal placoid pigment epitheliopathy and serpiginous choroiditis. All four cases of RPC received oral or IV steroids acutely, and three of these patients were transitioned to a steroid-sparing agent and biologic therapy: anti-TNF alpha or anti-IL-6. Quiescence of the chorioretinitis lesions was obtained after 7 months, 1 month, and 36 months; however, the latter had issues with treatment adherence. Mycophenolate mofetil was insufficient to control the disease in one patient, but tocilizumab and infliximab thereafter were effective after cessation of adalimumab due to side effects. Adalimumab when started the first month after the presentation was effective in controlling the disease in one patient. After the failure of interferon-alpha-2a, one patient displayed long-term control with infliximab. One patient did not require a steroid-sparing agent after oral prednisone taper as there was no evidence of progression or recurrence. This case series adds to the current knowledge regarding potential treatments for RPC, specifically the use of anti-TNF-alpha treatment and anti-IL-6 tocilizumab. In this case study, relapses of RPC were found among patients on mycophenolate mofetil and interferon-alpha-2a, and one case did not relapse on oral steroids without a steroid-sparing agent. Our findings suggest that adalimumab, infliximab, and tocilizumab may be useful medications to obtain quiescence of RPC.

Multimodal imaging for the management of Lyme-associated uveitis: A case report from an Italian tertiary center.

Lyme borreliosis may present with different systemic manifestations and also the ocular involvement may be difficult to diagnose because of its multifaceted presentation. Considering the growing incidence of Lyme disease in European countries, ophthalmologist should be trained to distinguish ocular borreliosis. Several clinical presentations have been previously described, including uveitis, unilateral or bilateral chorioretinitis, keratitis, episcleritis, papillitis and ischemic optic neuropathy, retinal vasculitis and acute posterior multifocal placoid pigment epitheliopathy (APMPPE); however our case report showed a rare presentation with the presence of unilateral uveitis with vitreitis and multiple, patchy, yellowish lesions, in association with retinal vasculitis. This clinical picture was to be considered in differential diagnosis with ocular Toxoplasmosis. The appropriate management of this patient was made possible by the combination of multimodal imaging and appropriate laboratory tests, representing the optimal process in the diagnostic and therapeutic pathway in high-risk patients for ocular Lyme disease.

Acute posterior multifocal placoid pigment epitheliopathy: clinical presentation and risk of stroke and transient ischaemic attack

Background/aimsAcute posterior multifocal placoid pigment epitheliopathy is a rare but important disease that can be associated with life-threatening complications due to cerebral vasculitis. The primary objective was to determine the...

Patterns of structural and functional changes in the retina and choroid in acute posterior multifocal placoid pigment epitheliopathy

The study analyzes the patterns of pathological changes in the retina and choroid in acute posterior multifocal placoid pigmented epitheliopathy (APMPPE). The results of the examination of two patients with bilateral APMPPE were analyzed retrospectively. The examination had included visometry, tonometry, static perimetry, autofluorescence, optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA). The analysis revealed signs of the inflammatory nature of the choriocapillary vascular wall lesion with the development of their local obstruction and, consequently, local ischemia of the retinal pigment epithelium (RPE) and the outer layers of the retina in the acute period. At the same time, partial destruction of the ellipsoid zone, uneven hyperreflectivity of the RPE with local areas of its elevation and impaired connection with photoreceptors were revealed. The subsequent restoration of choriocapillaris perfusion was accompanied by significant restoration of the functional and structural state of the RPE over a larger area, as well as partial restoration of the ellipsoid zone of the retina. Two years after the onset of the disease, separate areas of defects of the RPE and the outer retina were observed, coinciding in localization with zones of severe circulatory deficiency at the level of choriocapillaris in the acute stage. According to the results of multimodal imaging, choriocapillaritis is the initial link in the pathogenesis of APMPPE. The change in RPE and the outer retina is secondary to the local ischemic zones due to choriocapillaris nonperfusion. The nature of RPE change over a larger area manifests as a functional structural disorder, with the potential for recovery in case of choriocapillaris reperfusion.

A multicenter study of ocular inflammation after COVID-19 vaccination.

To report the characteristics of a case series of ocular inflammatory events following COVID-19 vaccination in Japan. Retrospective multicenter study METHODS: In this retrospective multicenter survey, a questionnaire was sent to 16 Japanese hospitals that had uveitis specialty clinics. Information on patients who developed ocular inflammatory events within 14 days of COVID-19 vaccination between February 2021 and December 2021 was collected. Thirty-seven patients were diagnosed with ocular inflammatory events following COVID-19 vaccination. The mean age was 53.4 ± 16.4 years (range, 26-86 years), and the mean time to onset after vaccination was 6.3 ± 4.2 days (range, 1-14 days). Vogt-Koyanagi-Harada disease (VKH) was the most common event (n = 17 patients, 46%), followed by anterior uveitis (n = 6), infectious uveitis (n = 3), acute zonal occult outer retinopathy (AZOOR) (n = 2), sarcoidosis-associated uveitis (n = 1), acute posterior multifocal placoid pigment epitheliopathy (APMPPE) (n = 1), optic neuritis (n = 1), multiple evanescent white dot syndrome (MEWDS) (n = 1), Posner-Schlossman syndrome (n = 1), and unclassified uveitis (n = 4). Twenty-eight cases occurred after BNT162b2 vaccination (Pfizer-BioNTech) and 8 after mRNA-1273 vaccination (Moderna), whilst 1 patient had no information about vaccine type. COVID-19 vaccination can be related to various types of ocular inflammatory events. When we encounter patients with ocular inflammatory disease, we should consider that it may be an adverse effect of COVID-19 vaccination.

Multimodal imaging of the course of retinal changes in acute posterior multifocal placoid pigment epitheliopathy with bilateral retinal detachment: a case report

BackgroundTo report a rare case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) with a combination of serous retinal detachment, papilledema, and retinal vasculitis.Case presentationA 19-year-old male complained of floaters in both eyes with decreased vision for 4 days. The best corrected visual acuity of the right eye and the left eye were 1.1 and 0.9 (logMAR), respectively. In both eyes, inflammatory cells can be seen suspended within the vitreous, multiple yellow/white lesions can be seen near the macula, and retinal neuroepithelial detachment. Swelling of the optic disc with blurring of the disc margins, in the left eye. Optical coherence tomography (OCT): showed retinal detachment in both eyes. The patient received oral prednisone treatment. 1 week later, OCT showed absorption of subretinal fluid in the macula of both eyes his binocular vision improved to 0.1 (logMAR). During the subsequent 28-month follow-up, fundus fluorescein angiography and OCT revealed extensive and progressive pigment epithelial atrophy in both eyes, and abnormal retinal vascular perfusion in the right eye due to persistent retinal vasculitis. Although the patient's binocular visual acuity remained at 0.1 (logMAR).ConclusionsIn the present case of APMPPE with a combination of serous retinal detachment, papilledema, and retinal vasculitis, through the multimodal imaging, further confirming that the lesions were located in the choroid, while the pigment epithelial lesions were secondary changes.

Spectrally resolved autofluorescence imaging in posterior uveitis

Clinical discrimination of posterior uveitis entities remains a challenge. This exploratory, cross-sectional study investigated the green (GEFC) and red emission fluorescent components (REFC) of retinal and choroidal lesions in posterior uveitis to facilitate discrimination of the different entities. Eyes were imaged by color fundus photography, spectrally resolved fundus autofluorescence (Color-FAF) and optical coherence tomography. Retinal/choroidal lesions’ intensities of GEFC (500–560 nm) and REFC (560–700 nm) were determined, and intensity-normalized Color-FAF images were compared for birdshot chorioretinopathy, ocular sarcoidosis, acute posterior multifocal placoid pigment epitheliopathy (APMPPE), and punctate inner choroidopathy (PIC). Multivariable regression analyses were performed to reveal possible confounders. 76 eyes of 45 patients were included with a total of 845 lesions. Mean GEFC/REFC ratios were 0.82 ± 0.10, 0.92 ± 0.11, 0.86 ± 0.10, and 1.09 ± 0.19 for birdshot chorioretinopathy, sarcoidosis, APMPPE, and PIC lesions, respectively, and were significantly different in repeated measures ANOVA (p < 0.0001). Non-pigmented retinal/choroidal lesions, macular neovascularizations, and fundus areas of choroidal thinning featured predominantly GEFC, and pigmented retinal lesions predominantly REFC. Color-FAF imaging revealed involvement of both, short- and long-wavelength emission fluorophores in posterior uveitis. The GEFC/REFC ratio of retinal and choroidal lesions was significantly different between distinct subgroups. Hence, this novel imaging biomarker could aid diagnosis and differentiation of posterior uveitis entities.

Reconstitution of the Choriocapillaris in Acute Posterior Multifocal Placoid Pigment Epitheliopathy.

To describe findings derived from extensions of optical coherence tomography, including volume rendering and frame averaged OCT angiography (OCT-A), in a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE). Case report of a patient with APMPPE imaged during the acute and convalescent stages. A 20-year-old male presented with an acute change in vision. He had multiple yellow-white placoid lesions at the level of the retinal pigment epithelium (RPE) in the macula and nasal to the optic nerve in both eyes. Within 2 weeks, his visual acuity worsened to 20/80 and 20/400 in the right and left eyes, respectively. SD-OCT showed focal defects in the ellipsoid and RPE layers. Volume rendering OCT-A showed inflammatory cells in the outer nuclear layer (ONL) above the APMPPE lesion. Frame averaged OCT-A revealed significant loss of capillary flow signal within capillary segments of the choriocapillaris. Ten weeks after presentation, there was resolution of the placoid changes, discontinuance of the inflammatory infiltrate in the ONL, and significant reconstitution of flow in the choriocapillaris. The visual acuity was 20/20 in both eyes. Novel volume rendered and frame averaged OCT-A images in a patient with APMPPE allowed detection of inflammatory cell infiltrate in the ONL as well as reversible capillary segment non-perfusion of associated APMPPE lesions. The findings suggest short term insults to choriocapillaris function may be reversible and can be tracked with modern imaging techniques.