Bilateral Posterior Uveitis Research Articles

Visual acuity and visual field as a function of disease duration in patients with birdshot chorioretinitis

BackgroundBirdshot chorioretinitis (BSCR) is a chronic bilateral posterior uveitis, which can affect central as well as peripheral vision. The aim of this study was to assess how visual acuity and...

Immune recovery uveitis in Whipple’s disease: an unusual ocular presentation

PurposeTo describe an unusual case of Whipple’s disease (WD) complicated by uveitis, and subsequent paradoxical worsening after effective antibiotic treatment targeting Tropheryma whipplei (TW).MethodsCase report.ResultsA 53-year-old male presented with bilateral knee arthritis, weight loss, chronic low-grade fever, and cognitive disorders. He was under treatment with tumor necrosis factor α inhibitors (TNFi) for seronegative spondyloarthritis. Given this unusual clinical presentation, further investigations were performed and revealed blood, saliva, stool, synovial fluid and cerebrospinal fluid positivity for TW, confirming the diagnosis of systemic WD. Ophthalmologic examination revealed bilateral posterior uveitis and an aqueous humor sample confirmed the presence of intraocular TW. TNFi were stopped, and the patient was subsequently treated with adequate antibiotics (ceftriaxone, followed by doxycycline and hydroxychloroquine), and subconjunctival corticosteroid injections. After a transient improvement of the ocular symptoms, he presented a recurrence of posterior segment inflammation, leading to repeated PCR testing for TW which were negative. Therefore, paradoxical worsening of the inflammation in the context of immune recovery uveitis (IRU) was thought to be the culprit. The patient was treated with systemic corticosteroid therapy, allowing for rapid improvement of the ocular findings.ConclusionsThis case underlines the possibility of IRU complicating WD. Ophthalmologists, rheumatologists, and internists should be aware of this rare complication, particularly in the context of previous immunosuppressive therapy.

The Influence of Time on the Epidemiology and Clinical Manifestations of Behçet's Disease in Brazil.

Modifications in the severity and clinical expression of Behçet's disease (BD) have been described in some areas that are considered endemic for the disease. This study aims to evaluate the chronological changes in epidemiology and clinical characteristics of BD patients in a referral center in Brazil, which is considered a non-endemic area for the disease. A descriptive and cross-sectional study involving BD patients divided into two groups: group 1 patients were diagnosed and followed between 1988 and 2010, and group 2 were diagnosed and followed between 2011 and 2022. No significant differences were found regarding gender and age at onset of symptoms between groups. We found a significant decrease in the frequency of bilateral ocular involvement, posterior uveitis, and retinal vasculitis. The demographic dates of this group of Brazilian BD patients remained similar over the last decade. Our study supports the notion that BD is becoming lighter in some regions. BD is a severe blinding disorder, and we found a lower frequency of ocular involvement over time. These findings may be attributed to a higher level of education of patients and a growing awareness of the disease. Newer immunomodulating and biologic agents may offer an improved prognosis in patients with BD with severe manifestations.

Retraction of cystoid macular edema from the fovea after intravitreal anti-VEGF therapy for birdshot chorioretinopathy.

To report the effect of anti-vascular endothelial growth factor inhibitor (anti-VEGF) on fovea-involving cystoid macular edema in a patient with Birdshot chorioretinopathy. A 42-year-old male patient presented to our hospital with bilateral posterior uveitis with retinal vasculitis, cystoid macular edema and optic disc edem a. He was diagnosed with birdshot chorioretinopathy based on clinical appearance and tissue type HLA-A29. The patient underwent vitrectomy in the right eye without any change in visual acuity. Retinal leakage was reduced by oral prednisolone, which could not be tapered below 50 mg per day without relapse. Oral prednisolone, topical dexamethasone and subtenonal kenalog were associated with intraocular pressure rise in both eyes. Hence, his uveitis responded to steroids, but there was no detectable effect of any steroid-sparing immunomodulatory drugs. The patient had been on oral prednisolone 50 mg for five years when it was decided to attempt intravitreal VEGF inhibitor injection therapy. The anti-VEGF therapy diminished cystoid macular edema in the fovea and improved the visual acuity. Here we report for the first time the long-term outcomes of anti-VEGF injections on fovea-involving cystoid macular edema in Birdshot chorioretinopathy to keep steroid at the minimal possible doses and preserve a satisfying visual acuity level.

A case report of autosomal recessive bestrophinopathy

The patient is a 40-year-old male who presented to the ophthalmology clinic due to easy visual fatigue for the past 3 months. Two months ago, the patient was misdiagnosed with "bilateral posterior uveitis", but the diagnosis was ruled out after ineffective treatment with corticosteroids. During the current visit, fundus examination revealed yellow-white material exudation below the macular center in both eyes. Considering the results of the ophthalmic examination and the genetic testing of the patient and his son, the patient was diagnosed with autosomal recessive bestrophinopathy.

AN UNUSUAL PRESENTATION OF CNS CANDIDIASIS

AN UNUSUAL PRESENTATION OF CNS CANDIDIASIS

Ciclosporin A in bilateral auto-immune chronic posterior uveitis associated with macular oedema: a Long-term Observational Safety and Efficacy Study.

A non-interventional, longitudinal, retrospective follow-up study to assess CsA-induced nephrotoxicity (IN) and its reversibility after withdrawal in patients exhibiting a bilateral chronic posterior uveitis (CPU) associated with cystoid macular oedema (CMO) in at least one eye. Data from medical records between 1986 and 2013. Primary outcome was the renal tolerance during and after CsA treatment assessed by plasma creatinine concentration and glomerular filtration rate (GFR) estimated by Chronic Kidney Disease Epidemiology (CKD-Epi) formula. Secondary outcomes were CsA through concentration, occurrence of cancers and ophthalmologic efficacy assessed by three parameters including CMO, vitreous inflammation, and best-corrected visual acuity BVCA changes. One hundred forty-three patients were followed for renal tolerance. Underlying diseases were Birdshot retinochoroiditis (n = 67), Behçet disease (n = 9), probable sarcoidosis (n = 23), sympathetic ophthalmia (n = 3), idiopathic (n = 41). After CsA discontinuation in 115 patients (mean treatment duration of 5.9 ± 3.8 years) mean plasma creatinine concentration was 82.2 ± 14.2 µmol/L versus 82.1 ± 14.1 µmol/L at baseline, mean GFR was 79.4 ± 13.9 mL/min versus 82.5 ± 14.3 mL/min at baseline, with no significant difference (respectively p = 0.91 and p = 0.09). Blood pressure did not significantly change during follow-up. CMO was completely resorbed in at least one eye, in 70.8% patients (n = 72) at 6 months, in 71.4% patients (n = 49) at 10 years and in 54.2% patients (n = 24) at 20 years. BCVA did not statistically change over time. Early and long-term monitoring of renal tolerance and dual adjustment of CsA doses in inflammatory stages of CPU were associated with reversible CsA IN. CsA could be effective in the treatment of CMO in CPU patients.

Unilateral Retinal Arteritis and Macroaneurysm in Sarcoidosis

ABSTRACT Background Sarcoidosis, an idiopathic systemic disorder characterized by noncaseating granulomas, is often associated with granulomatous uveitis. Anterior segment involvement can present with mutton-fat keratic precipitates, anterior chamber cell, and iris nodules. Sarcoid associated posterior uveitis may present with vitritis, retinal vasculitis, and choroidal lesions. Case summary Sarcoid-associated retinal vasculitis is classically thought of as predominantly involving veins, but in this case report we describe a 76-year-old Caucasian woman presenting with bilateral posterior uveitis, unilateral optic nerve head granuloma, and retinal arteritis as the first manifestation of ocular involvement in systemic sarcoidosis. Conclusion This case describes the uncommon first manifestation of ocular involvement in systemic sarcoidosis presenting with unilateral retinal arteritis, macroaneurysms and optic nerve head granuloma.

Long-Term Outcomes of Behçet's Syndrome-Related Uveitis: A Monocentric Italian Experience.

Objectives To examine demographic and clinical characteristics and long-term visual outcome in a cohort of Italian patients affected by Behçet's uveitis (BU). Materials and Methods Retrospective chart review of 47 patients with BU attending our unit between January 2018 and December 2019. Ophthalmologic manifestations, best-corrected visual acuity (BCVA), fluoroangiography and optical coherence tomography findings, and ocular complications were recorded. Predictive factors of a poor visual outcome and long-term complications were also investigated. Results Forty-seven patients (23 males and 24 females) for a total of 84 eyes were enrolled. Uveitis was bilateral in 37 (78.7%) patients with panuveitis being the most frequent anatomical pattern (40 out of 84 eyes), whereas 27 eyes presented a posterior uveitis. Isolated anterior uveitis was detected in 16 eyes. A significant improvement of median BCVA between baseline and last follow-up values was detected (p = 0.042). A higher risk of poor visual prognosis was observed in patients with uveitis duration greater than 15 years (p = 0.019). A significant resolution of retinal vasculitis was detected between baseline and last follow-up evaluation (p < 0.0001) whereas the mean ± SD macular thickness did not decrease significantly between baseline (376.00 ± 97.45 μm) and last follow-up evaluation (338.08 ± 55.81 μm). Forty-two eyes developed 57 complications during the disease course. Cataract was the most frequent (n = 12), followed by epiretinal membranes (n = 11) and cystoid macular edema (n = 6). The following variables were identified as predictors of long-term complications: human leukocyte antigen- (HLA-) B51 (p = 0.006), panuveitis (p = 0.037), and a uveitis duration of more than 15 years (p = 0.049). Conclusions In Italian patients, BU typically arises in the third decade and predominantly manifests as a bilateral posterior uveitis or panuveitis. Its duration is associated with a poor visual prognosis. Uveitis duration, the presence of HLA-B51, and panuveitis are predictors of long-term structural complications, thus representing main drivers in the treatment decision-making.

Birdshot chorioretinopathy: Importance of early diagnosis and therapeutic individualization

Introduction: Birdshot chorioretinopathy (BCR) is a bilateral and recurrent posterior uveitis with an uncertain natural history and visual prognosis. Currently, there is no consensus on the best treatment option and its total duration. Clinical Cases: We present a series of three clinical cases of patients with BCR diagnosed in different stages of the disease, which demonstrates the clinical spectrum of this pathology and their treatment with different immunomodulatory drugs. Conclusion: As shown in our series of cases, the early use of immunomodulatory drugs is recommended with a close monitoring and individualized treatment to achieve the best anatomical and functional results.

A sarcoidosis-lymphoma syndrome revealed by hypopituitarism.

SummaryA 26-year-old woman presented with persistent headache and tiredness. Biological investigations disclosed a moderate inflammatory syndrome, low PTH-hypercalcemia and complete anterior hypopituitarism. A magnetic resonance imaging (MRI) of the pituitary gland was performed and revealed a symmetric enlargement with a heterogeneous signal. Ophthalmological examination showed an asymptomatic bilateral anterior and posterior uveitis, and a diagnosis of pituitary sarcoidosis was suspected. As the localization of lymphadenopathies on the fused whole-body FDG-PET/computerized tomography (CT) was not evoking a sarcoidosis in first instance, an excisional biopsy of a left supraclavicular adenopathy was performed showing classic nodular sclerosis Hodgkin’s lymphoma (HL). A diagnostic transsphenoidal biopsy of the pituitary gland was proposed for accurate staging of the HL and surprisingly revealed typical granulomatous inflammation secondary to sarcoidosis, leading to the diagnosis of a sarcoidosis–lymphoma syndrome. The co-existence of these diseases constitutes a diagnostic challenge and we emphasize the necessity of exact staging of disease in order to prescribe adequate treatment.Learning points:The possibility of a sarcoidosis–lymphoma syndrome, although rare, should be kept in mind during evaluation for lymphadenopathies.In the case of such association, lymphoma usually occurs after sarcoidosis. However, sarcoidosis and lymphoma can be detected simultaneously and development of sarcoidosis in a patient with previous lymphoma has also been reported.An accurate diagnosis of the disease and the respective organ involvements, including biopsy, is necessary in order to prescribe adequate treatment.

Uveitis Presented as a Case of Ocular Syphilis – A Rare Presentation

Ocular syphilis is one of the clinical presentation or complication of syphilis. Uveitis is the most common ocular diagnosis of the condition but any component of the eyes can be affected. Serology test that include both treponemal and non-treponemal antibody detections play an important role for supporting the diagnosis. This condition is considered as part of neurosyphilis and the recommended treatment follows the treatment for neurosyphilis.&#x0D; We reported a 47-year-old man presented with chronic episode of blurring of vision associated with floaters involving both eyes. Both eyes showed abnormal visual acuity with involvement of optic disc on the left eye. Serology tests for non-treponemal antibody and treponemal antibody test were positive. He was diagnosed as a case of bilateral posterior uveitis with left eye neuroretinitis. He was successfully treated with intravenous aqueous crystalline penicillin 3 million unit every four hour daily for 14 days.&#x0D; J MEDICINE JUL 2019; 20 (1) : 52-54

ZIKA virus infection causes persistent chorioretinal lesions

Zika-infected patients can have eye involvement ranging from mild conjunctivitis to severe chorioretinal lesions, however the possible long-term sequelae of infection and timeline to recovery remain unknown. Here we describe the partial recovery of chorioretinal lesions in an immunocompetent patient diagnosed with bilateral posterior uveitis associated with Zika infection and show that some lesions resolved with focal atrophy evident as pigmentary changes on funduscopy. To better understand the progression of the lesions and correlate the changes in fundus imaging with local viral load, immune responses, and retinal damage, we developed a symptomatic mouse model of ocular Zika virus infection. Imaging of the fundus revealed multiple hypopigmentary patches indicative of chorioretinal degeneration as well as thinning of the retina that mirror the lesions in patients. Microscopically, the virus primarily infected the optic nerve, retinal ganglion cells, and inner nuclear layer cells, showing thinning of the outer plexiform layer. During acute infection, the eyes showed retinal layer disorganization, retinitis, vitritis, and focal choroiditis, with mild cellular infiltration and increased expression of tumor necrosis factor, interferon-γ, granzyme B, and perforin. Focal areas of gliosis and retinal degeneration persisted 60 dpi. The model recapitulates features of ZIKA infections in patients and should help elucidate the mechanisms underlying the damage to the eyes and aid in the development of effective therapeutics.

Bilateral Posterior Uveitis with Retinitis Revealing Primary Sjögren’s Syndrome

Introduction: Ocular manifestations are frequent during primary Sjögren's syndrome (PSS) and largely dominated by the signs of xerophthalmia. The extra-glandular ocular involvement is, on the contrary, exceptional and unusual. We are reporting an original case of bilateral posterior uveitis with retinitis inaugural of PSS. Case report: A 48-year-old Tunisian woman, with no notable pathological history, was explored for progressive decline in visual acuity, bilateral visual blur, and moderate eye pain. The examination noted a visual acuity at 6/10 on the left and 5/10 on the right, without redness of the eyes or irritative signs. The anterior segment of both eyes was normal. Ophthalmologic exam (slit lamp, fundus, and retinal angiography) revealed bilateral, non-granulomatous posterior uveitis associated with retinitis and retinal vasculitis. Further investigations concluded to PSS and treatment with intravenous pulse methylprednisolone followed by oral prednisone was introduced. The evolution was rapidly favorable and ophthalmological exam with eye fundus was totally normal at two months. Conclusion: PSS remains an exceptional and unusual cause of uveitis. It is therefore appropriate to evoke it in front of any uveitis that does not prove itself, especially if recidivating.

Adalimumab as an alternative treatment in Serpiginous Choroiditis

PurposeSerpiginous Choroiditis is a type of chronic bilateral posterior uveitis, with a geographical pattern that begins in the juxtapapillary zone and spreads in an intermittent and centrifugal manner. The purpose is to describe an alternative treatment for SC based on Adalimumab monotherapy.MethodsA female patient with decreased visual acuity in the last 6 months of the left eye and characteristic lesions in the ocular fundus of SC with juxtapapillary and paramacular involvement. The disease continued its course despite treatment with Prednisone at high doses, and affected the contralateral eye with small foci. Disease control was achieved by triple therapy of Prednisone, Azatriopine and Cyclosporin but the patient started with side effects derived from the treatment. Due to the immunological basis and supported on the literature, treatment with Adalimumab and decreasing doses of Prednisone was instituted.ResultsAfter 8 months of follow up, the patient presents a good control of the disease, besides the improvement of the secondary effects derived from the previous treatment.ConclusionsAdalimumab may be a good alternative in cases of CS that are not controlled despite the maximum treatment with triple therapy, or in which the treatment can not be continued due to side effects or poor compliance.

Electroretinogram and visual field changes in a case of birdshot chorioretinopathy

This case report explores the role of the visual field perimetry and electroretinogram (ERG) in cases of non-infectious uveitis in the assessment and monitoring of retinal function and response to treatment. A 59-year-old Caucasian female presenting with bilateral posterior uveitis newly diagnosed as birdshot chorioretinopathy (BSCR) presenting with bilateral decrease in visual acuity and cystoid macular edema, as well as a paracentral scotoma in the right eye. The diagnosis and follow-up of the case was done using visual field perimetry, optical coherence tomography (OCT), and ERG. Baseline ERG showed a marked decrease in the amplitudes of the scotopic and photopic responses and a delay of peak times mainly in the right eye. Mycophenolate mofetil at 2g/day and oral prednisolone at 1g/kg/day were administered with gradual tapering of the corticosteroids. After 5months, there was a noticeable improvement in the visual acuity, macular edema in OCT, and an obvious increase in the amplitudes of the ERG associated with a decrease in peak times, particularly in the 30Hz photopic 3.0 Flicker of the right eye. This case report highlights the importance of the peak time and wave amplitudes of the 30Hz photopic DA 3.0 Flicker as being a sensitive parameter in the diagnosis and follow-up of BSCR.

Efficacy of Anti-IL6-Receptor Tocilizumab in Refractory Cystoid Macular Edema of Birdshot Retinochoroidopathy Report of Two Cases and Literature Review

ABSTRACTPurpose: To evaluate tocilizumab (TCZ) efficacy in severe and refractory birdshot chorioretinopathy (BSCR).Methods: Assessment of BSCR patients refractory to conventional immunosuppressive and anti-TNF-α drugs who underwent TCZ therapy.Results: Two HLA-A29 positive patients (man/37 years and woman/38 years; four affected eyes) with BSCR were studied. They had a chronic bilateral posterior uveitis. Patient 1 had been treated with intraocular and oral corticosteroids, cyclosporine A, and infliximab whereas Patient 2 received intravenous methylprednisolone pulses, cyclosporine A, azathioprine, and adalimumab. At TCZ onset they had macular edema (four eyes); visual acuity (VA) impairment (four eyes); vitritis (one eye); and diffuse angiographic signs of vasculitis (periphlebitis) (two eyes). Improvement of VA and OCT was observed following TCZ therapy in both patients. After a follow-up of 18 months (Patient 1) and 10 months (Patient 2), respectively, a corticosteroid sparing effect without any adverse effects was achieved in both cases.Conclusions: TCZ was effective in two patients with BSCR refractory to anti-TNF-α agents.

Syphilitic placoid chorioretinitis. A case-report

Clinical caseWe report the case of a 45-year-old woman, with unremarkable past medical history, who presented with acute visual loss in her left eye due to bilateral posterior uveitis. After the screening, she was diagnosed with acute syphilitic placoid chorioretinitis and was treated with intravenous penicillin. DiscussionClinical manifestations of ocular syphilis are extremely heterogeneous and may mimic several aetiologies. Anti-treponema treatment usually induces a quick and positive response in affected patients. Prompt and proper diagnosis of these patients is crucial, although anatomical and functional damage may persist.

Coriorretinitis placoide sifilítica. Caso clínico

Clinical caseWe report the case of a 45-year-old woman, with unremarkable past medical history, who presented with acute visual loss in her left eye due to bilateral posterior uveitis. After the screening, she was diagnosed with acute syphilitic placoid chorioretinitis and was treated with intravenous penicillin. DiscussionClinical manifestations of ocular syphilis are extremely heterogeneous and may mimic several aetiologies. Anti-treponema treatment usually induces a quick and positive response in affected patients. Prompt and proper diagnosis of these patients is crucial, although anatomical and functional damage may persist.

Efficacy and tolerability of bilateral sustained-release dexamethasone intravitreal implants for the treatment of noninfectious posterior uveitis and macular edema secondary to retinal vein occlusion.

PurposeTo report our experience with bilateral placement of dexamethasone 0.7 mg (DEX) sustained-release intravitreal implant in the management of noninfectious posterior uveitis or macular edema secondary to retinal vein occlusion.MethodsA retrospective chart review of patients with bilateral noninfectious posterior uveitis and macular edema secondary to retinal vein occlusion who were treated with DEX intravitreal implant was performed. Ocular side effects such as intraocular pressure (IOP), cataract, and tolerability of bilateral injections was reviewed.ResultsTwenty-two eyes of eleven patients treated with a total of 32 DEX implants were included. Ten of eleven patients received bilateral implants due to active noninfectious uveitis while the other demonstrated macular edema in both eyes following separate central retinal vein occlusions. Among the patients with bilateral uveitis, the mean interval between DEX implant in the initial eye and the subsequent DEX in the fellow eye was 15.6 days (range 2–71 days). Seven of the ten patients received the second implant in the fellow eye within 8 days of the initial implantation. None of the patients had bilateral implantations on the same day. Seven eyes required reimplantation for recurrence of inflammation (mean interval between first and repeat implantation was 6.00±2.39 months). Following single or, in the case of the aforementioned seven eyes, repeat DEX implantation, all 20 uveitic eyes demonstrated clinical and/or angiographic evidence of decreased inflammation in the form of reduction in vitreous cells on slit lamp ophthalmoscopy, macular edema on ophthalmoscopy, or optical coherence tomography and/or disc and vascular leakage on fluorescein angiography. The mean follow-up for all eyes after initial implantation was 23.57 months (range 1–48 months). IOP was significantly higher (P=0.028) at 6 months (16.62 mmHg ±5.97) but not (P=0.82) at most recent follow-up (14.9±3.37 mmHg) when compared with baseline (14.68±3.02 mmHg). Four eyes (18.2%) required initiation of IOP-lowering medications. During the follow-up period, no eyes underwent filtration or cataract extraction. No serious ocular adverse effects were noted during the follow-up period.ConclusionIn patients with bilateral noninfectious posterior uveitis and macular edema secondary to vein occlusion, bilateral injection of DEX intravitreal implant was well tolerated and had an acceptable safety profile.