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Case 1-Fluorescein angiography of the right eye and left eye shows hyperfluorescence of the drusen with drusen localized to the posterior pole (bottom left) and diseased retinal pigment epithelium (bottom right).
Source publication
Purpose: To assess the evolution of retinal findings in patients with membranoproliferative glomerulonephritis (MPGN) by funduscopy, intravenous fluorescein angiography and optical coherence tomography.
Observations: Three women and one man were followed for a period of 1.5–37 years. Four patients (8 eyes) had drusen detected at first fundus exam a...
Contexts in source publication
Context 1
... 1. This 53-year-old Caucasian woman with MPGN II was asymptomatic with best corrected visual acuity of 20/25 (right eye) and 20/20 (left eye). Funduscopy revealed bilateral diffuse drusen ( Fig. 1) involving uniquely the posterior pole ( Fig. 2) with retinal pigment epithelium (RPE) alterations in the right eye on fluorescein angiography (Fig. 2) and diffusely thickened Bruch's membrane (Fig. ...
Context 2
... 1. This 53-year-old Caucasian woman with MPGN II was asymptomatic with best corrected visual acuity of 20/25 (right eye) and 20/20 (left eye). Funduscopy revealed bilateral diffuse drusen ( Fig. 1) involving uniquely the posterior pole ( Fig. 2) with retinal pigment epithelium (RPE) alterations in the right eye on fluorescein angiography (Fig. 2) and diffusely thickened Bruch's membrane (Fig. ...
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Citations
... Extrarenal manifestations of the disease can involve the eye, and typically include 1) basal laminar (cuticular) drusen, 2) choroidal neovascularization, 3) retinochoroidal atrophy or 4) central serous chorioretinopathy (possibly secondary to renal transplantation and corticosteroid use) 2,3,4 . We report previously undescribed DDD-related ocular changes, including bilateral outer retinal columnar abnormalities (ORCA) and non-vasogenic cystoid macular edema (CME). ...
Purpose
To describe the occurrence of bilateral outer retinal columnar abnormalities, non-vasogenic cystoid macular edema, and drusen in the context of dense deposit disease
Methods
Case report.
Patient
An 18-year-old female with dense deposit disease was referred to our specialist center for diagnosis and management with findings consistent with bilateral non-vasogenic cystoid macular edema and drusen. She was followed up in our clinic for forty months and treated with acetazolamide and ketorolac drops.
Results
Baseline examination revealed bilateral visual acuity (VA) reduction, and macular elevation with peripapillary drusen on fundus biomicroscopy. Optical coherence tomography revealed bilateral hyporeflective cystoid central macula changes, microcystoid changes with increased central subfield thickness (>450 microns), and outer retinal columnar abnormalities (ORCAs). Fluorescein angiography showed no evidence of macular leakage. Electrodiagnostic testing was within normal limits. Over the course of follow-up, she received treatment with acetazolamide 250mg BD PO and ketorolac 0.5% eye drops, with a partial reduction in her edema and improvement in VA.
Conclusion
Dense deposit disease is a rare disease secondary to complement cascade dysregulation, associated with drusen. To the best of our knowledge, this is the first report of bilateral non-vasogenic cystoid macular edema and ORCA in a young female patient with dense deposit disease, confirmed with multimodal imaging.
... The association between lipodystrophy syndrome and MPGN-II is described in the literature, [24] with 22% of lipodystrophy patients developing this condition. [25] MPGN-II, also known as dense deposit disease, is a rare renal disorder with 2-3 cases per 1,000,000, arising from nephritic factor induced dysregulation of the alternate complement pathway and consequent low levels of serum C3. ...
... Patients with MPGN-II can present with early onset macular drusen from their second decade, which advances peripherally and is associated with thickening of the Bruch's membrane. [24] Interestingly, there is no correlation between the degree of retinal disease and severity of the renal disease. However, a correlation between the duration of renal disease and the likelihood of retinal disease developing has been described; all patients with MPGN-II in one study (n=23) demonstrated drusen after ≥16 months. ...
... [27] As such, it is recommended that patients presenting with this feature undergo screening for nephropathy. [24] Previous attempts at systemic treatment of MPGN with IV immunoglobulins, plasmapheresis and B-cell suppression have all been relatively unsuccessful. Nevertheless, recent studies on Eculizumab, a monoclonal antibody that prevents formation of the membrane attack complex by binding C5, and compstatin, a C3-targeted complement inhibitor have demonstrated promising results. ...
A 35-year-old woman with acquired partial lipodystrophy (PLD) and features of type 2 membranoproliferative glomerulonephritis (MPGN-II), presented with difficulty in her fine detailed vision over the past year. She had right amblyopia from a hypermetropic anisometropia with astigmatism, displaying a best-corrected visual acuity of 0.50 and 0.00 LogMAR, in the right and left eye, respectively. Funduscopy showed bilateral symmetrical drusenoid deposits most prominent in the temporal macula with clusters in the superior and inferior retina, outside the temporal vascular arcades. Multimodal retinal imaging was performed, which confirmed hyperautofluorescent drusen located between the retinal pigment epithelium and Bruch’s membrane. Electroretinography showed bilateral mild peripheral macular dysfunction, but normal central macular function on the pattern electroretinogram. Both PLD and macular drusen, are rare as distinct disease entities, but an association does exist and may be linked to MPGN-II.
... Complement-mediated C3 glomerulopathies (C3G), an uncommon cause of chronic nephritis involving predominantly children and young adults, with an estimated incidence of 1-2 cases per 1 million [3], show histopathological similarities to MN, such as IgG deposits along basement membranes [1,3]. The literature reports an association between C3G and ocular abnormalities such as drusen, RPE irregularities, CSC, and CNV [4,5]. This is probably due to histological similarities between kidney and choroid. ...
... This is probably due to histological similarities between kidney and choroid. Moreover, basal laminar drusen resemble glomerular deposits and their form and size variability may reflect the stage of the underlying renal disease [4]. This parallelism between renal and retinal findings may be valid also for patients affected by primary MN. ...
Patient: Male, 61-year-old
Final Diagnosis: Choroidal neovascularization
Symptoms: Decrease in the visual acuity • metamorphopsia
Medication:—
Clinical Procedure: —
Specialty: Nephrology • Ophthalmology
Objective
Rare co-existance of disease or pathology
Background
We describe the retinal findings in a patient affected by primary membranous nephropathy (MN).
Case Report
A 61-year-old man presented with a 3-month history of metamorphopsia and decreased visual acuity in both eyes. He was affected by nephrotic syndrome in primary MN and treated with systemic corticosteroids. Dilated fundus examination, optical coherence tomography, and fundus fluorescein angiography revealed the presence of peripapillary choroidal neovascularization (CNV) in the right eye and peripheral CNV in the left eye. A serous retinal detachment with gravitational tract was also observed in both eyes. The patient was treated with intravitreal bevacizumab in the right eye and oral corticosteroids were discontinued. Both eyes achieved a morphological and functional improvement.
Conclusions
We present the first case of primary MN associated with CNV, possibly secondary to central serous chorioretinopathy, successfully treated with intravitreal bevacizumab and discontinuation of oral corticosteroids.
... We performed genetic examination in patient P1 without findings related to other known macular dystrophies. Similar macular yellowish dots-like deposits have been previously reported in systemic disorders (20)(21)(22)(23)(24)(25) however general health was good in all our subjects. A similar nonprogressive retinal phenotype with drusenoid deposits that are present from childhood can be seen in North Carolina Macular Dystrophy, a dominantly inherited macular dystrophy that has been mapped to chromosome 6q16 (MCDR1 locus) (17,26). ...
Purpose: To describe the clinical features of 2 unrelated families affected with Benign Yellow Dot Maculopathy and to analyze anatomical and functional findings of this peculiar phenotype
Methods: Case series
Results: We retrospectively described 5 patients (3 males, 2 females) affected with Benign Yellow Dot Maculopathy. The mean age at referral was 50,8 years (range 34–69 yrs.). All patients were characterized by a good visual acuity (20/20 in both eyes) and by symmetric multiple yellow dots at the posterior pole in both eyes. In 3 patients (P1, P3, P4) the yellow dots were mainly located at the nasal side of the macula. The yellow dots appeared hyper-autofluorescent at the fundus autofluorescence (FAF) imaging. OCT examination revealed in 3 patients (P1, P3, P4) mild irregularities at the level of the retinal pigment epithelium (RPE) and at the interdigitation (IZ) and ellipsoid zone (EZ). OCT angiography (OCT-A), performed in 3 patients (P1, P4,P5), was normal. Adaptive Optics imaging (AO) showed a peculiar pattern of the cone mosaic: the yellow dots were detectable as hyper-reflective lesions at the macular region. In 2 patients (P1, P4) we reported a follow-up of 2 and 18 years respectively. Genetic examination performed on patient P1 did not reveal pathogenic variants for retinal dystrophies.
Conclusions: Our work confirmed the benign nature of this peculiar macular phenotype showing a normal macular function and a stable clinical picture during a long-term follow-up. Multimodal imaging allows a detailed detection and monitoring of Benign Yellow Dot Maculopathy.
... They can lead to visual impairment. 5,7,[36][37][38][39] We refer patients to an ophthalmologist to be screened for drusen by fluorescein angiography and optical coherence tomography. Even in the absence of symptoms, screening for drusen may be helpful for timely prevention or treatment and for the diagnosis of C3 glomerulopathy. ...
C3 glomerulopathy is a rare renal disease that has been distinguished as a renal disease for about 10 years. It is caused by an excessive activation of the alternative complement pathway in the circulation, which leads to deposition of complement factor C3 in glomeruli. It is diagnosed based on clinical presentation, histological patterns in a kidney biopsy and tests of the complement pathways. It can closely resemble immune complexmediated glomerulonephritis and postinfectious glomerulonephritis. Renal failure develops in up to half of all patients within 10 years after presentation. A curative treatment is not available. Treatment relies on renoprotective measures, occasional immunosuppressive medication and experimental novel complement inhibitors. Because the disease is rare, its care and cure are concentrated in centers of expertise. Here we provide an overview of the state-ofthe-art diagnosis and treatment of C3 glomerulopathy in a center of expertise in the Netherlands.
... They can lead to visual impairment. 5,7,[36][37][38][39] We refer patients to an ophthalmologist to be screened for drusen by fluorescein angiography and optical coherence tomography. Even in the absence of symptoms, screening for drusen may be helpful for timely prevention or treatment and for the diagnosis of C3 glomerulopathy. ...
The aim of this study was to evaluate the adequacy of single-frequency (sf-BIA) and multi-frequency bioelectrical impedance analyses (mf-BIA), in comparison with dual-energy x-ray absorptiometry (DXA), to evaluate body composition in maintenance haemodialysis (MHD) patients. Body composition of 27 adult MHD patients (9 f, 18 m), BMI 17.5-34.4 kg m(-2), was examined with DXA and BIA, with two different sf-BIA and 1 mf-BIA analysers. Biochemical markers of nutritional status and adequacy of dialytic treatment were also determined. Fat mass (FM) estimated by the different BIA analysers was found to be slightly but significantly higher than FM measured by DXA. In contrast, fat-free mass (FFM) obtained with BIA was found to be slightly but significantly lower than FFM DXA. No significant differences were found between LBM-DXA (that is FFM-DXA minus bone mass) and the different FFM BIA. The lowest mean prediction error versus DXA values was found with sf1BIA. In any case, a close correlation was found between all BIA values and DXA values, particularly for FFM. Furthermore, FFM and LBM results were significantly correlated with serum creatinine, which in MHD patients is an indicator of muscle mass. These results indicate that BIA can be used to evaluate body composition in MHD patients.
A 34-year-old female presented with complaints of decreased and distorted vision in the right eye (RE) for 3 months. She had hypertension and membranoproliferative glomerulonephritis (MPGN) type II for 5 years. Her best corrected visual acuity was 6/12, N24 and 6/6, N6 in the right and left eye, respectively. The diagnosis of bilateral MPGN type II-induced retinal drusen was made with active choroidal neovascularization (CNV) in the RE and confirmed by optical coherence tomography. The CNV was treated with antivascular endothelial growth factor injection. After the treatment, her visual acuity improved and metamorphopsia reduced. There have been a handful of case reports about retinal drusen with CNV secondary to MPGN type II across the globe. However, to the best of our knowledge, this is the first case report from India.
Subretinal autofluorescent deposits (SADs) may be found in the posterior pole, associated with very various conditions. These disorders usually present a typical pattern of autofluorescent lesions seen on short-wavelength fundus autofluorescence (FAF). We tdescribe SADs according to their putative pathophysiological origin, but also according to their clinical pattern, i.e. number, shape, and usual location. Five main putative pathophysiological origins of SADs were identified in disorders associated with an intrinsic impairment of phagocytosis and protein transportation, with excess of retinal pigment epithelium (RPE) phagocytic capacity, with direct or indirect RPE injury, and/or disorders associated with long-standing serous retinal detachment with mechanical separation between the RPE and the photoreceptor outer segments. However, clinically, they could be classified into eight subclasses of SADs, as observed on FAF as follows: single vitelliform macular lesion, multiple roundish or vitelliform lesions, multiple peripapillary lesions, flecked lesions, leopard-spot lesions, macular patterned lesions, patterned lesions located in the same area as the causal disorder, or non-patterned lesions. Thus, if multimodal imaging may be required to diagnose the cause of SADs, the proposed classification based on noninvasive, widely-available short-wavelength FAF could guide clinicians in making their diagnosis decision tree before considering the use of more invasive tools.
The complement system is part of the innate immune system activated by three distinct pathways: classical, lectin and alternative. It is also involved in retinal development and homoeostasis. Dense deposit disease is a rare renal disease associated with mutations in Complement factor H and overactivity of the alternative complement pathway. As well as glomerulonephritis, many affected individuals have retinal drusen and may be at risk of vision loss due to macular atrophy or choroidal neovascularisation. We discuss the reclassification of dense deposit disease as a type of C3 glomerulonephropathy, and hypothesise on the mechanisms of retinal abnormalities. Drusen have also been described in individuals with other types of glomerulonephritis involving abnormalities of the classical (membranoproliferative glomerulonephritis type 1) or lectin (IgA nephropathy, lupus nephritis) complement pathways. Although drusen are found in abnormalities of all three complement pathways, the age at onset, aetiology, and the threat to vision differs. This review describes drusen and other retinal abnormalities associated with the glomerulonephritides due to abnormal activation in each of the three complement activation pathways, and provides the first report of drusen occurring in a patient with the recently reclassified C3 glomerulonephritis with homozygous variant V62I in complement factor H. Optometric management of young patients presenting with retinal drusen is discussed, and complement-based therapies for visual loss are reviewed.
Purpose
To report our experience with a peculiar case of asynchronous bilateral retinal vascular occlusion in a patient suffering from membranoproliferative glomerulonephritis.
Case report
A 57-year-old dialysed male affected by membranoproliferative glomerulonephritis who underwent kidney transplantation complained of a sudden vision loss in his right eye (RE). His best-corrected visual acuity (BCVA) was 20/40 in RE and 20/20 in the left eye (LE); ophthalmological and fluorangiographic examinations revealed unilateral retinal obliterative vasculitis with panuveitis and apparent sparing of contralateral eye. About 6 months later the patient developed a branch retinal vein occlusion associated with a papillary neovascular membrane in LE. Corticosteroid therapy was administered and immunosuppressant dosage was increased with macular oedema reduction in both events.
Conclusion
We report a case of unilateral retinal obliterative vasculitis and subsequent contralateral retinal neovascularization and branch retinal vein occlusion in a patient affected by membranoproliferative glomerulonephritis.
