Acute Posterior Multifocal Placoid Pigment Epitheliopathy Research Articles

ACUTE POSTERIOR MULTIFOCAL PLACOID PIGMENT EPITHELIOPATHY 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 in elderly individuals. APMPPE or PPM eyes in patients above age 55 years with macular retinal pigment epithelium 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, optical coherence tomography (OCT), and OCT angiography (OCTA) was performed in all eyes for diagnosis and to monitor for macular neovascularization. Eight elderly male patients presented with vision loss and were all initially diagnosed with non-neovascular or neovascular age-related macular degeneration. With the aid of multimodal retinal imaging, a final diagnosis of either APMPPE or PPM was rendered. With FA and indocyanine green angiography, choroidal hypoperfusion was detected in all but one eye. With OCT, the angular sign of Henle fiber layer hyperreflectivity was identified in >50% of eyes. With OCTA, inner choroidal flow deficits were detected in all eyes. Macular neovascularization requiring anti-vascular endothelial growth factor injection therapy complicated three of eight cases. Both APMPPE and PPM may develop in elderly individuals and may masquerade as age-related macular degeneration on presentation. Multimodal imaging including FA, indocyanine green angiography, and OCTA are important diagnostic modalities to assess for inner choroidal hypoperfusion to arrive at an accurate diagnosis and to detect macular neovascularization, which frequently complicates APMPPE and PPM. In these patients, serial anti-vascular endothelial growth factor intravitreal injections are essential in treating macular neovascularization 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.

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.

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.

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.

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.

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.

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.

Long-term outcomes of small-incision cataract surgery in patients with uveitis.

To evaluate the long-term outcomes of manual small-incision cataract surgery (MSICS) in eyes with uveitis. Patients who underwent MSICS for uveitic cataract from 2009 to 2019 were retrospectively evaluated. Visually significant cataract and presence of less than five cells per high-power field in the anterior chamber for a minimum of 3 months were the prerequisites for surgery. Patients with follow-up less than 9 months were excluded. After exclusion, 283 eyes of 264 patients were evaluated. The mean age of patients was 44.3 ± 11.3 years. The mean follow-up duration was 22 ± 11.5 months. The mean surgical time was 11.2 ± 3.2 min. One hundred and seventy-two eyes (60.8%) had anterior uveitis, 78 (27.5%) had posterior uveitis, and 33 (11.7%) had panuveitis. At the final follow-up, 253 eyes (88.4%) had corrected distance visual acuity (CDVA) better than 0.6 log of minimum angle of resolution (LogMAR) unit. The final endothelial cell counts were significantly (analysis of variance [ANOVA], P = 0.001) lower in eyes with human leukocyte antigen (HLA)-B27-associated uveitis and in eyes with idiopathic anterior uveitis. Patients on systemic corticosteroids had significantly better (P = 0.031) final visual acuity than those without preoperative corticosteroids. Recurrent uveitis (43.8%), Posterior capsule opacification (PCO) (19.4%), glaucoma (8.5%), cystoid macular edema (CME; 13.5%), and Epiretinal membrane (ERM) (5.6%) were the frequent complications. A significantly worse (ANOVA, P = 0.001) visual prognosis was seen in patients with Vogt-Koyanagi-Harada disease (VKH), sarcoidosis, acute posterior multifocal placoid pigment epitheliopathy (APMPPE), and serpiginous choroiditis. MSICS is safe in most cataracts due to uveitis and results in improvement in CDVA at 9 months. Posterior capsule opacification, macular edema, persistent uveitis, etiology of uveitis, and use of preoperative steroids significantly influenced the visual outcome.

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.

Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE): A Comprehensive Approach and Case Series: Systemic Corticosteroid Therapy Is Necessary in a Large Proportion of Cases.

Background and objectives: Acute posterior multifocal pigment epitheliopathy/acute multifocal ischaemic choriocapillaritis (APMPPE/AMIC) is part of the group of choriocapillaritis entities. The aim of this article was to report a series of patients with emphasis on the clinical presentation and treatment paradigms. Materials and Methods: Retrospective case series study performed in the Centre for Ophthalmic Specialised care (COS), Lausanne, Switzerland, on patients diagnosed from 2000 to 2021 with APMPPE/AMIC. Procedures performed at presentation and upon follow-up (when available) included best corrected visual acuity (BCVA), routine ocular examination, laser flare photometry (LFP) microperimetry (when available) and visual field testing. Imaging investigations included spectral domain optical coherence tomography (SD-OCT)/enhanced depth imaging OCT (EDI-OCT), OCT angiography (OCT-A) as well as fluorescein and indocyanine green angiography (FA, ICGA). The presence or not of prodromal systemic viral-like symptoms was noted. The localisation of lesions whether foveal or extrafoveal, divided the patients into 2 groups (foveal, peri-or parafoveal). Exclusion criteria were patients diagnosed with APMPPE/AMIC and a positive QuantiFERON test and/or VDRL-TPHA tests. Results: Nineteen (35 eyes) of 1664 new patients (1.14%) were diagnosed with APMPPE/AMIC and included in our study. 13 (68%) were male and 6 (32%) were female. The mean age was 33.1 ± 9.2 years. 16 (84%) patients mentioned a viral prodromal episode or other systemic symptoms, and 3 (16%) did not mention any episode before the onset of ocular symptoms. 15 (39%) out of 38 eyes had foveal localisation of the lesions, 20 (52.6%) had peri- or para-foveal localisations and 3 eyes were normal [3 unilateral cases (15%)]. Mean BCVA at presentation was 0.83 ± 0.24 for the whole group. It was 0.58 ± 0.28 for the group with foveal lesions, increasing to 0.97 ± 0.13 at last follow-up (p = 0.0028). For the group with extrafoveal lesions mean BCVA at presentation was 0.94 ± 0.18, improving to 1.18± 0.10 at last follow-up (p = 0.0039). 13 (68%) patients received prednisone treatment, of whom 2 (10%) received additionally at least one immunosuppressive agent, 4 (20%) patients received no treatment and in 2 patients the information was unavailable. All patients in the foveal lesion group received corticosteroid treatment except one who evolved to bilateral macular atrophy. Conclusions: APMPPE/AMIC is a primary choriocapillaritis. Although it is thought that the disease is self-limited, treatment is necessary in most cases, especially when lesions are located in the fovea.

A rare case of unilateral acute posterior multifocal placoid pigment epitheliopathy with features of Vogt-Koyanagi-Harada disease

The purpose of this study is to report a rare case of unilateral acute posterior multifocal placoid pigment epitheliopathy (APMPPE) with features of Vogt-Koyanagi-Harada (VKH) disease. Both the diseases have their own unique presentations and can lead to vision loss in the affected eye. Overlapping features of both diseases in the same eye is rare. A 28-year-old healthy female presented with loss of vision in her right eye for 10 days duration. Visual acuity in the affected eye was 20/200, N36. Fundus examination revealed numerous creamy yellow lesions overlying the posterior pole with subretinal fluid. Further investigations including fundus fluorescein angiography and optical coherence tomography were suggestive of overlapping features of both APMPPE and VKH disease. Baseline laboratory investigations and markers for Sarcoidosis, Tuberculosis, and Syphilis were normal. The patient was treated with tapering doses of oral steroids. One month following initiation of treatment, the vision had improved to 20/20, N6 with complete resolution of subretinal fluid in the right eye. The overlapping clinical and imaging features suggest that both diseases may be a part of a common inflammatory process that secondarily damages the outer retinal structures.

Mechanisms, Pathophysiology and Current Immunomodulatory/Immunosuppressive Therapy of Non-Infectious and/or Immune-Mediated Choroiditis.

Non-infectious choroiditis comprises immune-mediated diseases resulting from diverse pathophysiological mechanisms. These conditions are sub-divided into two main groups, (1) diseases of the choriocapillaris and (2) diseases of the choroidal stroma. The purpose of this study is to expose the pathophysiology of the most common diseases of both these groups and recommend the optimal immunomodulatory/immunosuppressive therapy of each analyzed condition based on literature data and data from our own centers. Material and Methods: Narrative review. In the group of choriocapillaritis entities or primary inflammatory choriocapillaropathies (PICCPs) including multiple evanescent white dot syndrome (MEWDS), acute posterior multifocal placoid pigment epitheliopathy (APMPPE), idiopathic multifocal choroiditis (MFC) and serpiginous choroiditis (SC), as well as secondary choriocapillaritides including acute syphilitic posterior multifocal placoid chorioretinitis (ASPMPC) and tuberculosis-related SC (TB-SC), were analyzed. In the group of stromal choroidites, HLA-A29 birdshot retinochoroiditis (BRC) and Vogt-Koyanagi-Harada (VKH) disease were included. For each entity a literature search, in the PubMed database, on treatment was performed and analyzed and the therapeutic attitudes of our own centers were presented. Management of immune-mediated choroiditis implies vigorous immunosuppressive therapy given in a prompt and prolonged fashion in most of these entities.

Diagnosis and Treatment of Primary Inflammatory Choriocapillaropathies (PICCPs): A Comprehensive Overview.

Purpose: Primary inflammatory choriocapillaropathies (PICCPs) belong to a group of intraocular inflammatory diseases with the common characteristic of inflammatory choriocapillaris hypo- or non-perfusion as the main clinicopathological mechanism. The purpose of our article is to describe clinical characteristics and multimodal imaging, that can help the diagnosis and treatment of PICCPs. Methods: Narrative review with multimodal imaging analysis. Results: Choriocapillaris non-perfusion can affect the end-choriocappilaries, at the benign end of the PICCP spectrum (MEWDS), to larger choriocapillaris vessels or precapillary vessels at the origin of more severe forms such as acute posterior multifocal placoid pigment epitheliopathy (APMPPE), idiopathic multifocal choroiditis (MFC) and Serpiginous Choroiditis (SC). Diagnosis is mostly based on multimodal imaging and especially on indocyanine green angiography (ICGA), fundus autofluorescence (FAF) and spectral-domain optical coherence tomography (SD-OCT)/OCT-angiography (OCT-A). ICGA shows the typical pattern of patchy lobular hypofluorescence reflecting hypo- or non-perfusion of the choriocapillaris that can also take the aspect of geographic areas in the more severe forms. Treatment depends on the severity of the disease and goes from observation in MEWDS and some mild cases of APMPPE, to oral corticosteroid and/or immunomodulator agents in the more severe conditions of APMPPE and MFC and SC cases. Close multimodal monitoring is crucial in order to introduce or adjust treatment. Conclusion: PICCPs are resulting from one common clinicopathological mechanism, inflammatory choriocapillaris hypo- or non-perfusion. ICGA findings are essential for the diagnosis and follow-up of PICCPs, but non-invasive methods such as FAF and SD-OCT/OCT-A also have their role especially in follow-up of the diseases. Treatment should be individualized according to the pathology and the evolution of lesions.