Atopic Dermatitis Associated Retinal Detachment and Retinal Vasculitis: A Case Report.

Atopic dermatitis is usually associated with various ocular complications. We report a 21-year-old Chinese male who presented to our ophthalmology clinic with bilateral retinal detachment and cataracts. The patient had a clear medical history of atopic dermatitis, which had been diagnosed eight years earlier and had been treated with loratadine and pimecrolimus. Cataract surgery was performed for both eyes, combined with scleral buckling for the right eye and pars plana vitrectomy for the left eye. During postoperative follow-up, fundus fluorescein angiography showed retinal vasculitis in both eyes and macular edema in the left eye, which coincided with an exacerbation of atopic dermatitis. Macular edema improved after four months of regular dupilumab treatment in the dermatology department. The ocular condition remained stable three years postoperatively.

Rituximab, Intravitreal Bevacizumab and Laser Photocoagulation for Treatment of Macrophage Activation Syndrome and Retinal Vasculitis in Lupus: A Case Report.

Systemic lupus erythematosus (SLE) most commonly manifests as mild to moderate disease with severe manifestations such as diffuse alveolar hemorrhage, central nervous system vasculitis, macrophage activation syndrome (MAS) or retinal vasculitis (RV) with visual disturbances occurring in a significantly smaller proportion of patients, most of whom have a poor outcome. Macrophage activation syndrome and RV are insufficiently early and rarely recognized presentations of lupus-consequently there are still no treatment recommendations. Here we present the course of diagnosis and treatment of a patient with an SLE flare that resulted in both life-threatening disease (MAS) and vision-threatening disease (RV). The patient was successfully treated with systemic immunosuppressives, a high dose of glucocorticoids and rituximab (RTX), in parallel with intraocular therapy, intravitreal bevacizumab (BEV) and laser photocoagulation.

MERLIN: Phase 3a, Multicenter, Randomized, Double-Masked Trial of Brolucizumab in Participants with Neovascular Age-Related Macular Degeneration and Persistent Retinal Fluid.

PURPOSE: To assess the 52-week efficacy and safety of brolucizumab 6 mg administered every 4 weeks compared with aflibercept 2 mg dosed every 4 weeks in eyes with neovascular age-related macular degeneration (nAMD) and persistent retinal fluid. DESIGN: Multicenter, randomized, double-masked phase 3a study. PARTICIPANTS: Participants with recalcitrant nAMD (persistent residual retinal fluid despite previous frequent anti-vascular endothelial growth factor treatment). METHODS: Eyes were randomized (2:1) to intravitreal brolucizumab 6 mg or aflibercept 2 mg every 4 weeks up to and including week 100. MAIN OUTCOME MEASURES: The primary end point was analysis of noninferiority in mean best-corrected visual acuity (BCVA) change from baseline to week 52 (margin, 4 letters). Other key end points included change in central subfield thickness (CST) from baseline to week 52, fluid-free status (no intraretinal fluid and no subretinal fluid), and safety. RESULTS: At week 52, brolucizumab was noninferior to aflibercept in BCVA change from baseline (least squares mean difference, -0.6 Early Treatment Diabetic Retinopathy Study letters; 95% confidence interval [CI], -2.1 to 0.9; P < 0.001). A total of 4.8% and 1.7% of participants reported a 15-letter or more BCVA loss from baseline at week 52 in the brolucizumab and aflibercept groups, respectively. In eyes treated with brolucizumab compared with those treated with aflibercept, the CST was reduced significantly (P < 0.001), and a significantly greater proportion of eyes were fluid free at week 52 (40.4% brolucizumab vs. 19.0% aflibercept; 95% CI, 13.9-29.0; P < 0.001). Incidence of intraocular inflammation (IOI), including retinal vasculitis and retinal vascular occlusion, were 9.3% (0.8% and 2.0%) for brolucizumab versus 4.5% (0% and 0%) for aflibercept, respectively. CONCLUSIONS: Visual acuity outcomes in previously treated participants with nAMD and persistent retinal fluid receiving brolucizumab 6 mg dosed every 4 weeks were noninferior to aflibercept 2 mg dosed every 4 weeks, with superior anatomic outcomes. However, incidences of IOI, including retinal vasculitis and retinal vascular occlusion, also were higher, leading to study termination.

ISOLATED RETINAL VASCULITIS: Prognostic Factors and Expanding the Role of Immunosuppressive Treatment in Retinal Vasculitis Associated With Positive QuantiFERON-TB Gold Test.

PURPOSE: To identify prognostic factors for poor visual outcomes in patients with isolated retinal vasculitis and to elucidate the outcome of immunosuppressive treatment without the use of antituberculosis drugs for patients with retinal vasculitis associated with a positive QuantiFERON-TB Gold In-Tube (QFT) test. METHODS: A retrospective chart review was performed of patients presenting with retinal vasculitis. After the diagnosis of active retinal vasculitis had been confirmed by fluorescein angiography and other possible causes of retinal vasculitis had been excluded, patients were categorized into two groups by their QFT result. Potential associated factors between the poor and good visual outcome groups were statistically analyzed by the chi-square test and logistic regression model with generalized estimating equations. RESULTS: Seventy-three eyes (48 patients) were enrolled in this study. After univariate analysis, multivariate logistic regression analysis was performed and revealed that logMAR visual acuity at the initial visit ( P = 0.01) and outer retinal disruption ( P = 0.03) were the two factors significantly associated with poor visual outcomes. Systemic corticosteroids were administered without the use of antituberculosis drugs to all 16 cases of presumed tuberculous retinal vasculitis associated with positive QFT (26 eyes), 10 (63%) of whom were given nonsteroidal immunosuppressive drugs and achieved inflammatory control and treatment success. CONCLUSION: Risk factors leading to poor visual outcome in patients with isolated retinal vasculitis have been identified. Immunosuppressive treatment without antituberculosis drugs seems to be a promising regimen for selected patients with presumed tuberculous retinal vasculitis under vigilant care.

Swept-source optical coherence tomography angiography of retinal occlusive vasculitis following brolucizumab administration: a case report.

BACKGROUND: We present a case of retinal occlusive vasculitis following brolucizumab administration and the first report of optical coherence tomography angiography (OCTA) findings after treatment. CASE PRESENTATION: A 71-year-old man complained of vision loss in the left eye 6 weeks after brolucizumab injection. His visual acuity was counting fingers, and examination revealed 1 + anterior chamber cells with 2 + vitreous cells. Fundus examination demonstrated vitreous haze, retinal whitening, and vascular sheathing. Fluorescein angiography revealed filling defects in the retinal arteries and veins, and OCTA showed extensive capillary nonperfusion. Under the diagnosis of brolucizumab-associated intraocular inflammation (IOI) and retinal occlusive vasculitis, topical, sub-Tenon, and systemic corticosteroids were administered. After the treatment, visual acuity improved to 20/200, and OCTA revealed gradual improvement in capillary dropout; however, with the limited improvement of reperfusion in the perifoveal areas. CONCLUSIONS: Prompt evaluation and intensive corticosteroid treatments are required for brolucizumab-associated IOI. OCTA imaging provides detailed information on microvascular changes in the retinal vascular plexuses in brolucizumab-associated retinal occlusive vasculitis.

Cooperation with rheumatologists on intensive systemic treatment for psoriatic arthritis-related panuveitis with retinal vasculitis: a case report.

BACKGROUND: Patients with psoriatic arthritis (PsA) may develop uveitis, a potentially serious ocular complication. PsA-related uveitis may result in significant morbidity and even vision loss if underdiagnosed or under-treated. We presented a case with long-standing recurrent uveitis and retinal vasculitis successfully managed by fortified systemic immunomodulators for systemic PsA. CASE PRESENTATION: A 47-year-old woman was referred under the impression of acute anterior uveitis in her right eye in recent one month. Ocular examinations showed panuveitis in both eyes with intense vitreous opacity in her right eye. Fundus fluorescence angiography revealed retinal vasculitis in both eyes. Systemic surveys excluded the possibility of infection but showed an elevated inflammation marker. With intensive immunosuppressive treatment, inflammation resolved and the vision improved. CONCLUSION: Our case highlights not only the importance of intensified systemic therapy in treating PsA-related uveitis but the importance of multidisciplinary collaboration. Recurrent uveitis may be an indicator of disease activity prior to other inflammatory markers.

Use of Ultra-Widefield Fluorescein Angiography to Guide the Treatment to Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis-Case Report and Literature Review.

PURPOSE: To review the clinical features, diagnosis, and treatment of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) and to report a case with the use of ultra-widefield fluorescein angiography (UWFA) for confirming the precise staging of IRVAN and aid in early treatment. The patient improved after being treated with intravitreal aflibercept injection. RESULTS: A 26-year-old female complained of progressive blurred vision OD for one week. Her BCVA was 0.6 OD and 1.0 OS. Fundus examination showed vitritis, retinal hemorrhage, and vasculitis over bilateral eyes. Fluorescein angiography (FA) with a 55 degree of view revealed aneurysmal dilations of the peripapillary arteriole, peripapillary focal leakage, venous leakage, and capillary nonperfusion area. Stage 2 IRVAN was impressed OU. Oral prednisolone was administered. After four months, she experienced decreased visual acuity OS. Optical coherence tomography (OCT) revealed subretinal and intraretinal fluid with hyperreflective material. One posterior subtenon triamcinolone and one intravitreal aflibercept injection were performed OS, and macular edema subsided. A 105-degree ultra-widefield fluorescein angiography (UWFA) showed multiple peripheral background hypofluorescence areas corresponding to capillary nonperfusion. Retinal neovascularization (NV) was found OS, which had not been revealed by the previous 55-degree FA. Stage 3 IRVAN was made OS and panretinal laser photocoagulation (PRP) was performed. Oral prednisone and cyclosporine were prescribed. Her vision improved to 1.0 OU. CONCLUSION: UWFA provides visualization of peripheral retinal pathology and for precise staging. It also had direct implications in the follow-up and treatment strategy.

[OCULAR MANIFESTATIONS IN BEHÇET DISEASE].

INTRODUCTION: Ocular involvement in Behçet disease occurs is most patients and can be the presenting organ. Intra-ocular inflammation (uveitis) related to Behçet disease is mainly a panuveitis including an occlusive retinal vasculitis. Recurrent inflammation can result in the development of ocular complications, including macular edema and retinal neovascularization, with up to a quarter of eyes developing vision loss. Diagnosis is based on clinical ophthalmic findings and ocular imaging including retinal fluorescein angiography and optical coherence tomography. Early diagnosis and treatment may prevent the development of ocular complications and vision loss. Treatment is based primarily on systemic corticosteroids and 2nd-line immunosuppressive agents, particularly anti-tumor necrosis factor α agents. Extensive treatment, prevention of relapses and complications can result in long term stable vision.

Collaborative Ocular Tuberculosis Study Consensus Guidelines on the Management of Tubercular Uveitis-Report 2: Guidelines for Initiating Antitubercular Therapy in Anterior Uveitis, Intermediate Uveitis, Panuveitis, and Retinal Vasculitis.

TOPIC: The Collaborative Ocular Tuberculosis Study (COTS), supported by the International Ocular Inflammation Society, International Uveitis Study Group, and Foster Ocular Immunological Society, set up an international, expert-led consensus project to develop evidence- and experience-based guidelines for the management of tubercular uveitis (TBU). CLINICAL RELEVANCE: The absence of international agreement on the use of antitubercular therapy (ATT) in patients with TBU contributes to a significant heterogeneity in the approach to the management of this condition. METHODS: Consensus statements for the initiation of ATT in TBU were generated using a 2-step modified Delphi technique. In Delphi step 1, a smart web-based survey based on background evidence from published literature was prepared to collect the opinion of 81 international experts on the use of ATT in different clinical scenarios. The survey included 324 questions related to tubercular anterior uveitis (TAU), tubercular intermediate uveitis (TIU), tubercular panuveitis (TPU), and tubercular retinal vasculitis (TRV) administered by the experts, after which the COTS group met in November 2019 for a systematic and critical discussion of the statements in accordance with the second round of the modified Delphi process. RESULTS: Forty-four consensus statements on the initiation of ATT in TAU, TIU, TPU, and TRV were obtained, based on ocular phenotypes suggestive of TBU and corroborative evidence of tuberculosis, provided by several combinations of immunologic and radiologic test results. Experts agreed on initiating ATT in recurrent TAU, TIU, TPU, and active TRV depending on the TB endemicity. In the presence of positive results for any 1 of the immunologic tests along with radiologic features suggestive of past evidence of tuberculosis infection. In patients with a first episode of TAU, consensus to initiate ATT was reached only if both immunologic and radiologic test results were positive. DISCUSSION: The COTS consensus guidelines were generated based on the evidence from published literature, specialists' opinions, and logic construction to address the initiation of ATT in TBU. The guidelines also should inform public policy by adding specific types of TBU to the list of conditions that should be treated as tuberculosis.

CLINICAL PREDICTORS OF TUBERCULAR RETINAL VASCULITIS IN A HIGH-ENDEMIC COUNTRY.

PURPOSE: To determine clinical signs suggestive of tubercular etiology in retinal vasculitis. METHODS: A retrospective comparative study of patients who presented with retinal vasculitis at three tertiary care centers in India. All patients underwent detailed clinical evaluation and tailored laboratory investigations for etiological diagnosis. Tubercular etiology was diagnosed on basis on the presence of retinal periphlebitis in association with ancillary evidence of systemic tuberculosis and exclusion of nontuberculosis entities. Patients with tubercular (Group A) and nontubercular (Group B) etiology were compared for demographic characteristics, supportive diagnostic evidence, and specific ocular signs. Statistical analysis was performed at 5% confidence levels. RESULTS: Of the 114 patients diagnosed with retinal vasculitis, Group A had 69 patients (100 eyes) and Group B had 45 patients (75 eyes). Active or healed subvascular lesions (P ≤ 0.0001), focal vascular tortuosity (P ≤ 0.0001), and occlusive vasculitis (P = 0.002) were significantly more common in Group A patients than in Group B patients. All three were independent predictors of tubercular etiology on multivariate regression analysis. CONCLUSION: The presence of healed or active subvascular lesions, focal vascular tortuosity, and occlusive vasculitis could be predictive of tubercular etiology in retinal vasculitis.

[Rétinol frosted angiitis]. / Le cas clinique du mois. Angéite givrée rétinienne.

Frosted angiitis is a rare form of retinal vasculitis in which venous development gives it an appearance of perivascular frost. It is most often of idiopathic origin but can also be found in the context of infectious (most often viral) or inflammatory pathologies (lupus erythematosus, sarcoidosis or granulomatosis with polyangiitis). As with most vasculitis, frosty angiitis can be complicated by occlusion of the central retinal vein. Close monitoring is necessary as well as rapid treatment based on high-dose systemic corticosteroid therapy as well as intraocular injections of anti-VEGF compounds if necessary.

L'angéite givrée est une forme rare de vascularite rétinienne dont l'engainement veineux lui confère un aspect de givre périvasculaire. Elle est le plus souvent d'origine idiopathique, mais peut également se rencontrer dans le cadre de pathologies infectieuses (virales, le plus souvent ) ou inflammatoires (lupus érythémateux, sarcoïdose ou encore granulomatose avec polyangéite). Comme pour la plupart des vascularites, l'angéite givrée peut se compliquer d'une occlusion de la veine centrale de la rétine. Un suivi rapproché est nécessaire ainsi qu'un traitement rapide basé sur une corticothérapie systémique à haute dose ainsi que des injections intraoculaires d'anti-VEGF si nécessaire.

SLE presenting as DAH and relapsing as refractory retinitis.

Dear Editor, A 17-year-old boy, diagnosed with Systemic Lupus Erythematosus (SLE), presented to ophthalmology department with gradual painless diminution of vision in both eyes (right more than left). He had already received 6 pulses of cyclophosphamide and steroids at monthly intervals one year back for diffuse alveolar hemorrhage (DAH) and was on maintenance oral 40 mg prednisolone and 3 grams mycophenolate mofetil (MMF). There was no history of oliguria, skin rash, joint pain, oral ulcers, photosensitivity or any neurological deficit in this presentation. There was no proteinuria, hematuria or worsening of renal function.

Haemorrhagic occlusive retinal vasculitis caused by intravitreal vancomycin.

Intraocular Vancomycin is used as a treatment as well as prophylaxis of endophthalmitis, but in rare instances it can cause serious ischaemic vasculitis. The most salient features of the disease include painless visual loss after cataract surgery, mild to moderate inflammation in the anterior chamber and peripheral retinal involvement with patchy haemorrhages and ischaemic vasculitis. We present case reports of two such patients who were identified with ischaemic vasculitis when they were given intravitreal Vancomycin for treatment of suspected endophthalmitis after complicated phacoemulsification surgery. Both developed profound visual loss with typical signs of haemorrhagic occlusive retinal vasculitis (HORV). 25 gauge 3 ports parsplana (25G 3 PPV) vitrectomy was performed on both the patients and dropped lenticular matter were removed from the vitreous cavity. Topical antibiotics and steroids were given. There was no significant visual improvement in both the cases which illustrates the toxic potential of intraocular use of Vancomycin.

THE COLLABORATIVE OCULAR TUBERCULOSIS STUDY (COTS)-1: A Multinational Review of 251 Patients With Tubercular Retinal Vasculitis.

PURPOSE: Tubercular retinal vasculitis (TRV) is a heterogeneous disease that can be difficult to manage because of nonspecific presentation and limitations of confirmatory tests for tuberculosis. This is a big data analysis on phenotypes and treatment outcomes for TRV. METHODS: Multicentre retrospective study of patients with TRV between January 2004 and December 2014 and a minimum follow-up of 1 year. RESULTS: Two hundred and fifty-one patients with TRV with a mean age of 38.9 ± 14.4 years (range, 9-86 years) were included. The patients were predominantly males (n = 167/251; 66.5%) of Asian ethnicity (n = 174/246; 70.7%), and geographical origin (n = 137/251; 54.6%). Most patients had features of occlusive type of RV (n = 113/185; 61.1%) except Caucasians (n = 11; 28.2%). There was no significant difference in treatment failure whether patients received antitubercular therapy (ATT) (P = 0.29), although treatment failure was less frequent in patients who received ATT (13.6%; n = 31/228) compared with those who did not (21.7%, n = 5/23). Less treatment failures were observed in patients with occlusive type RV who received ATT; however, this was not significant on survival analysis (P = 0.09). Treatment with ATT was associated with higher failure rates in patients of Hispanic and African American race and those with TRV associated with panuveitis (compared with posterior uveitis). CONCLUSION: In this multinational study of TRV, there was no significant therapeutic effect of ATT. However, a definitive conclusion about the role of ATT could not be made because of a few patients who did not receive ATT. Because this is a retrospective study with a limited 1-year follow-up, the effect of ATT may have been overestimated (or underestimated) in the duration of follow-up.

Resolution of arterial aneurysms in idiopathic retinal vasculitis, aneurysms and neuroretinitis: a case report and review of literature.

PURPOSE: To report a case of resolution of retinal arterial aneurysms in a patient of idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) treated with oral steroids. METHODS: This study contains case report and review of literature. RESULTS: A 16-year-old girl with stage 2 IRVAN was treated with oral steroids alone. Fluorescein angiography confirmed the presence of aneurysms and absence of neovascularization in both eyes. The aneurysms resolved gradually over 4-month follow-up. CONCLUSIONS: This case demonstrates previously unreported reversibility of arterial aneurysms with steroid therapy alone in early stages of IRVAN.

[IDIOPATHIC RETINAL VASCULITIS - THE INTERPLAY BETWEEN A CHRONIC IDIOPATHIC OCULAR DISEASE, ITS THERAPY AND THE PATIENT].

INTRODUCTION: This is an article on a six year follow-up of a patient diagnosed with idiopathic retinal vasculitis. Her medical history, symptoms and findings are presented in detail, related to the diagnostic investigations and the resulting diagnosis. Patient follow-up was marked with repeated attempts to utilize steroid sparing strategies including antimetabolites such as Methotrexate and mycophenolate Mofetyl with only limited success. Biologic agent (anti TNF), Adalimumab, was also not successful. We discuss the difficulties experienced by the patient and her response to our inability to completely control her symptoms. On another level, we relate to our own difficulties to assess her response to therapy given her preserved vision on the one hand and her apparent uncontrolled retinal vascular leakage. The patient's ability to function in daily life reduces her willingness to endure therapy-related adverse events.

Early treatment of tuberculous uveitis improves visual outcome: a 10-year cohort study.

PURPOSE: Diagnosis of tuberculous uveitis (TBU) is often challenging and is usually made after excluding other causes of uveitis. We analysed the characteristics of TBU and variables associated with visual outcome. METHODS: A retrospective, observational analysis was performed in patients with presumptive TBU who were started on specific TB treatment between January 2006 and June 2016. Demographic, clinical, radiological, analytical and ophthalmic examination variables were studied. After completing TB treatment, a follow-up of at least 9 months was performed. A univariate and logistic regression analysis was applied to identify the variables associated with visual acuity and recurrences of uveitis. RESULTS: Forty affected eyes of 24 individuals were identified; 79% of patients were diagnosed during the last 3 years of the study period. Median delay from onset of symptoms to diagnosis was 12 weeks. Loss of visual acuity was the most frequent symptom (87.5%). Posterior uveitis was the most frequent localization (72.9%); 19 patients (79.2%) presented at least one of the Gupta signs predictive of TBU, but there were no confirmed diagnoses. OUTCOME: There was improvement in visual acuity in 74.4% of the eyes, but a complete response was achieved only in 56.4%. There was recurrence in two patients. The initiation of treatment ≥ 24 weeks after onset of symptoms was significantly associated with no improvement (p = 0.026). CONCLUSION: TBU can cause permanent damage to visual acuity, particularly in patients with delayed diagnosis. A prompt initiation of systemic TB treatment is essential to improve visual prognosis.

Review of people with retinal vasculitis and positive QuantiFERON®-TB Gold test in an area nonendemic for tuberculosis.

PURPOSE: To assess the frequency of a positive QuantiFERON®-TB Gold test (quantiferon) among patients with retinal vasculitis in an area nonendemic for tuberculosis (TB); to review clinical characteristics and management of affected individuals. METHODS: Consecutive patients with retinal vasculitis and a positive quantiferon were retrospectively analyzed. Demographics, clinical data, laboratory, imaging findings, and therapy were evaluated. RESULTS: Among 49 patients with retinal vasculitis, 12 (24%) had a positive quantiferon. Median age was 37 years, there were five female patients. Five individuals (42%) had previously lived in a country endemic for TB. Retinal vasculitis was occlusive in six patients (50%). On chest imaging, pulmonary tuberculosis was suspected in one patient (8.3%). Treatment modalities included full antitubercular treatment (n = 1), isoniazid prophylaxis (n = 6), systemic corticosteroids (n = 8), and laser treatment (n = 5). After a median follow-up of 27.5 months, inflammation was inactive (n = 6) or recurrently present (n = 6). No patient lost ≥ 2 lines of best-corrected visual acuity during follow-up. CONCLUSIONS: The quantiferon test is recommended in the evaluation of people with retinal vasculitis. Interpretation of a positive result can be challenging in a country nonendemic for TB. The majority of patients with quantiferon-positive retinal vasculitis were found to have latent TB.

Steroid-responsive painful ophthalmoplegia: Tolosa-Hunt syndrome, Eales disease, or both?

Introduction Tolosa-Hunt syndrome (THS) is one of the most common 'benign' causes of painful ophthalmoplegia. Diagnosis is based on clinical and imaging findings and the exclusion of other causes because there is no specific biomarker for the syndrome. Eales disease, an idiopathic inflammatory venous disease that primarily affects the eye, can also affect the central (as stroke or myelitis) and peripheral nervous system. Case report We report the case of a 32-year-old woman with a subacute left ophthalmoplegia and evidence of a gadolinium-enhanced lesion suggesting an inflammatory granuloma that resolved within 48 hours after treatment with steroids. A diagnosis of THS was considered at this time. On a follow-up ophthalmological examination, a diagnosis of Eales disease with involvement of the left eye was made. The patient was treated successfully. Conclusion Eales disease could be a cause of painful ophthalmoplegia and may mimic THS. Long-term follow-up of patients diagnosed with THS may be necessary to exclude other diagnoses.