Significance of clinical-immunological patterns and diagnostic yield of biopsies in microscopic polyangiitis and granulomatosis with polyangiitis.

BACKGROUND: Microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) are the two major antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). OBJECTIVES: To characterize a homogenous AAV cohort and to assess the impact of clinicopathological profiles and ANCA serotypes on clinical presentation and prognosis. Clinical differences in GPA patients according to ANCA serotype and the diagnostic yield for vasculitis of biopsies in different territories were also investigated. RESULTS: This retrospective study (2000-2021) included 152 patients with AAV (77 MPA/75 GPA). MPA patients (96.1% myeloperoxidase [MPO]-ANCA and 2.6% proteinase 3 [PR3]-ANCA) presented more often with weight loss, myalgia, renal involvement, interstitial lung disease (ILD), cutaneous purpura, and peripheral nerve involvement. Patients with GPA (44% PR3-ANCA, 33.3% MPO, and 22.7% negative/atypical ANCA) presented more commonly with ear, nose, and throat and eye/orbital manifestations, more relapses, and higher survival than patients with MPA. GPA was the only independent risk factor for relapse. Poor survival predictors were older age at diagnosis and peripheral nerve involvement. ANCA serotypes differentiated clinical features in a lesser degree than clinical phenotypes. A mean of 1.5 biopsies were performed in 93.4% of patients in different territories. Overall, vasculitis was identified in 80.3% (97.3% in MPA and 61.8% in GPA) of patients. CONCLUSIONS: The identification of GPA presentations associated with MPO-ANCA and awareness of risk factors for relapse and mortality are important to guide proper therapeutic strategies in AAV patients. Biopsies of different affected territories should be pursued in difficult-to-diagnose patients based on their significant diagnostic yield.

Orbital/ocular inflammatory involvement in VEXAS syndrome: Data from the international AIDA network VEXAS registry.

VEXAS syndrome is a recently described monogenic autoinflammatory disease capable of manifesting itself with a wide array of organs and tissues involvement. Orbital/ocular inflammatory manifestations are frequently described in VEXAS patients. The objective of this study is to further describe orbital/ocular conditions in VEXAS syndrome while investigating potential associations with other disease manifestations. In the present study, twenty-seven out of 59 (45.8 %) VEXAS patients showed an inflammatory orbital/ocular involvement during their clinical history. The most frequent orbital/ocular affections were represented by periorbital edema in 8 (13.6 %) cases, episcleritis in 5 (8.5 %) patients, scleritis in 5 (8.5 %) cases, uveitis in 4 (6.8 %) cases, conjunctivitis in 4 (6.8 %) cases, blepharitis in 3 (5.1 %) cases, orbital myositis in 2 (3.4 %) cases. A diagnosis of systemic immune-mediated disease was observed in 15 (55.6 %) cases, with relapsing polychondritis diagnosed in 12 patients. A significant association was observed between relapsing polychondritis and orbital/ocular involvement in VEXAS syndrome (Relative Risk: 2.37, 95 % C.I. 1.03-5.46, p = 0.048). Six deaths were observed in the whole cohort of patients after a median disease duration of 1.2 (IQR=5.35) years, 5 (83.3 %) of which showed orbital/ocular inflammatory involvement. In conclusion, this study confirms that orbital/ocular inflammatory involvement is a common finding in VEXAS patients, especially when relapsing polychondritis is diagnosed. This makes ophthalmologists a key figure in the diagnostic process of VEXAS syndrome. The high frequency of deaths observed in this study seems to suggest that patients with orbital/ocular involvement may require increased attention and more careful follow-up.

Effectiveness and Safety of Biosimilars in Pediatric Non-infectious Uveitis: Real-Life Data from the International AIDA Network Uveitis Registry.

INTRODUCTION: Since many biological drug patents have expired, biosimilar agents (BIOs) have been developed; however, there are still some reservations in their use, especially in childhood. The aim of the current study is to evaluate the efficacy and safety of tumor necrosis factor (TNF) inhibitors BIOs as treatment for pediatric non-infectious uveitis (NIU). METHODS: Data from pediatric patients with NIU treated with TNF inhibitors BIOs were drawn from the international AutoInflammatory Disease Alliance (AIDA) registries dedicated to uveitis and Behçet's disease. The effectiveness and safety of BIOs were assessed in terms of frequency of relapses, risk for developing ocular flares, best-corrected visual acuity (BCVA), glucocorticoids (GCs)-sparing effect, drug survival, frequency of ocular complications, and adverse drug event (AE). RESULTS: Forty-seven patients (77 affected eyes) were enrolled. The BIOs employed were adalimumab (ADA) (89.4%), etanercept (ETA) (5.3%), and infliximab (IFX) (5.3%). The number of relapses 12 months prior to BIOs and at last follow-up was 282.14 and 52.43 per 100 patients/year. The relative risk of developing ocular flares before BIOs introduction compared to the period following the start of BIOs was 4.49 (95% confidence interval [CI] 3.38-5.98, p = 0.004). The number needed to treat (NNT) for ocular flares was 3.53. Median BCVA was maintained during the whole BIOs treatment (p = 0.92). A significant GCs-sparing effect was observed throughout the treatment period (p = 0.002). The estimated drug retention rate (DRR) at 12-, 24-, and 36-month follow-up were 92.7, 83.3, and 70.8%, respectively. The risk rate for developing structural ocular complications was 89.9/100 patients/year before starting BIOs and 12.7/100 patients/year during BIOs treatment, with a risk ratio of new ocular complications without BIOs of 7.1 (CI 3.4-14.9, p = 0.0003). Three minor AEs were reported. CONCLUSIONS: TNF inhibitors BIOs are effective in reducing the number of ocular uveitis relapses, preserving visual acuity, allowing a significant GCs-sparing effect, and preventing structural ocular complications. TRIAL REGISTRATION: ClinicalTrials.gov ID NCT05200715.

Ocular involvement in adult and paediatric patients with monogenic autoinflammatory diseases: a Spanish multicentre retrospective study.

OBJECTIVES: Ophthalmologic involvement in monogenic autoinflammatory diseases has been explored mainly in paediatric patients. The aim of this study is to characterise ophthalmologic manifestations, therapeutic management and visual outcomes in a Spanish (UVESAI) cohort of adult/paediatric patients with monogenic autoinflammatory diseases. METHODS: Multicentre and retrospective study of patients with monogenic autoinflammatory diseases and ocular involvement. Eye manifestations, structural complications, treatments used and visual outcomes were analysed, and compared with previous studies. RESULTS: Forty-six patients (44/2 adults/children; 21/25 adult/paediatric-onset) with monogenic autoinflammatory diseases [cryopyrin associated periodic syndromes (n=13/28.3%), mainly Muckle-Wells syndrome (MWS) (n=11/24%); familial Mediterranean fever (FMF) (n=12/26%); TNF receptor-associated periodic syndrome (TRAPS); (n=9/20%); Blau syndrome (n=8/17%); hyperimmunoglobulin D syndrome (HIDS) (n=2/4.3%), deficiency of adenosine deaminase-2 and NLRC4-Autoinflammatory disease] (one each) were included. Conjunctivitis (n=26/56.5%) and uveitis (n=23/50%) were the most frequent ocular manifestations. Twelve (26.1%) patients developed structural complications, being cataracts (n=11/24%) and posterior synechiae (n=10/22%) the most frequent. Conjunctivitis predominated in TRAPS, FMF, MWS and HIDS (mainly in adults), and uveitis, in Blau syndrome. Seven (8%) eyes (all with uveitis) presented with impaired visual acuity. Local and systemic treatment led to good visual outcomes in most patients. Compared with previous studies mainly including paediatric patients, less severe ocular involvement was observed in our adult/paediatric cohort. CONCLUSIONS: Conjunctivitis was the most common ocular manifestation in our TRAPS, FMF, MWS and HIDS patients, and uveitis predominated in Blau syndrome. Severe eye complications and poor visual prognosis were associated with uveitis. Adults with monogenic autoinflammatory diseases seem to exhibit a less severe ophthalmologic presentation than paediatric patients.

Intravitreal fluocinolone acetonide 0.19 mg (ILUVIEN®) in patients with non-infectious uveitis: real-world effectiveness and safety outcomes at 12 months.

PURPOSE: This study assessed the effectiveness of the 0.19-mg fluocinolone acetonide (FAc) implant by multimodal measurements in patients with non-infectious uveitis (NIU) in a real-world setting in Spain. METHODS: A prospective study of patients who had NIU including uveitic macular oedema (UME) with ≥ 12 months follow-up was done. Exclusion criteria include infectious uveitis and uncontrolled glaucoma or ocular hypertension requiring more than 2 medications. Effectiveness was assessed using a multicomponent outcome measure that included nine outcomes. Effectiveness was defined as all components being met at every timepoint. Secondary outcome measures were onset or progression of glaucoma and investigator-reported adverse events. RESULTS: Twenty-six eyes from 22 patients were included, with 96.2% having an indication including UME. During the 12-month study, the FAc implant was effective in 15 (57.7%) eyes, reaching effectiveness as soon as 2 weeks post-implantation. Mean best-corrected visual acuity and mean central macular thickness (CMT) were significantly improved vs. baseline at all timepoints (all comparisons p < 0.01). During the 12-month study, inflammation markers (anterior chamber cells and vitreous haze) had also significantly declined. Factors predicting effectiveness at month 12 were systemic corticosteroid dose pre-FAc, higher immunomodulatory therapy (IMT) load at baseline and thicker retinal nerve fibre layer (RNFL) at baseline (all p < 0.05). Factors predicting failure were male gender, thinner RNFL at baseline and treatment ineffectiveness at 1 month (all p < 0.05). In parallel, corticosteroid and IMT use also declined significantly. No significant increase in IOP was detected. CONCLUSION: The FAc implant is safe and effective at treating NIU over 12 months in a real-world setting in Spain.

Intravitreal fluocinolone acetonide implant for the treatment of persistent post-surgical cystoid macular edema in vitrectomized eyes.

Cystoid macular edema (CME) is the most common cause of decreased visual acuity after both vitrectomy and cataract surgery. Various strategies have been used for its treatment, such as intravitreal corticosteroids. The intravitreal fluocinolone acetonide implant (Iluvien®) is approved for the treatment of persisting diabetic macular edema and for the prevention of recurrence of non-infectious uveitis affecting the posterior segment. There are very few reports about its off-label use for post-surgical CME. We present four clinical cases of post-surgical CME (three following vitrectomy and one following cataract surgery in a vitrectomized eye 2 years ago). All of them had been previously treated with an average of four injections of intravitreal dexamethasone implant (Ozurdex®), with repeated recurrence of CME. After treatment with Iluvien, three cases showed improvement of both visual acuity and macular anatomy, with resolution of the macular edema. One patient required additional treatment with Ozurdex during follow-up, further improving CME. Two of the cases required topical pressure lowering treatment, and none required filtering surgery. Iluvien could be an effective therapeutic option for persistent non-diabetic macular edema after vitrectomy or cataract surgery refractory to other intravitreal therapies, with the benefit of being able to provide longer recurrence-free periods.

CHARACTERIZING COVID-19-RELATED RETINAL VASCULAR OCCLUSIONS: A Case Series and Review of the Literature.

PURPOSE: To describe clinical and ophthalmologic features and outcomes of patients with coronavirus disease-19 with retinal vascular occlusions. METHODS: Retrospective multicenter case series and PubMed review of cases reported from March 2020 to September 2021. Outcome measures are as follows: type of occlusion, treatments, best-corrected visual acuity, and central macular thickness on optical coherence tomography. RESULTS: Thirty-nine patients were identified. Fifteen patients with a median age of 39 (30-67) years were included in the multicenter study. Vascular occlusions included central retinal vein occlusion (12 eyes), branch retinal vein occlusion (4 eyes), and central retinal artery occlusion (2 eyes). Three cases were bilateral. Baseline best-corrected visual acuity was 20/45 (no light perception-20/20). Baseline central macular thickness was 348.64 (±83) µm. Nine eyes received anti-vascular endothelial growth factor agents, dexamethasone intravitreal implant, or both. Final best-corrected visual acuity was 20/25 (no light perception-20/20), and central macular thickness was 273.7 ± 68 µm (follow-up of 19.6 ± 6 weeks). Among the 24 cases from the literature review, retinal vein occlusion was the predominant lesion. Clinical characteristics and outcomes were similar to those found in our series. CONCLUSION: Coronavirus disease-19-associated retinal vascular occlusions tend to occur in individuals younger than 60 years. Retinal vein occlusion is the most frequent occlusive event, and outcomes are favorable in most cases.

Simultaneous pars plana vitrectomy, panretinal photocoagulation, cryotherapy, and Ahmed valve implantation for neovascular glaucoma.

AIM: To describe and evaluate the efficacy of Ahmed glaucoma valve implantation (AGV) combined with pars plana vitrectomy (PPV) in a single surgical act for the treatment of advanced neovascular glaucoma (NVG). METHODS: Retrospective observational case series included 51 eyes from 50 patients with severe NVG treated with PPV, AGV, and panretinal photocoagulation and/or cryotherapy in a single surgical act during a 13-year period (2005-2018). Preoperative, intraoperative and postoperative data at day 1 and months 1, 3, 6, 21, and 24 were systematically collected. Definition of surgical success was stablished at IOP between 6 and 21 mm Hg with or without topical treatment. RESULTS: Main indications for surgery were NVG secondary to proliferative diabetic retinopathy (39.2%) and central retinal vein occlusion (37.3%). Mean (±SD) preoperative IOP was 42.0±11.2 mm Hg decreasing to 15.5±7.1 mm Hg at 12mo and 15.8±9.1 mm Hg at 24mo of follow up. Cumulative incidence of success of IOP control was 76.0% at first postoperative month, reaching 88.3% at 6mo. Prevalence of successful IOP control at long term was 74.4% at 12mo and 71.4% at 24mo. Eye evisceration for unsuccessful NVG management was required in 1 case (2.0%). CONCLUSION: Combination of AGV implantation and PPV in a single act may be a suitable option for severe forms of NVG in a case-by-case basis for effective IOP control and a complete panretinal photocoagulation.

New Potential Weapons for Refractory Scleritis in the Era of Targeted Therapy.

OBJECTIVE: To assess the efficacy of biologic drugs, beyond tumor necrosis factor- (TNF-) α inhibitors, in the management of noninfectious refractory scleritis, either idiopathic or associated with systemic immune-mediated disorders. Patients and Methods. This is a retrospective study assessing the efficacy of several biologic agents (rituximab, anakinra, tocilizumab, and abatacept) and the small molecule tofacitinib in the treatment of scleritis through assessment of scleral inflammation and relapses, as well as treatment impact on best-corrected visual acuity (BCVA) and safety profile. RESULTS: Fourteen patients (19 eyes) were enrolled in the study. Scleritis inflammatory grading significantly improved from baseline to 3 months (p = 0.002) and from baseline to the last follow-up visit (p = 0.002). Scleritis relapses significantly decreased between the 12 months preceding and following biologic therapy (p = 0.007). No differences regarding BCVA were observed (p = 0.67). Regarding adverse events, only one patient developed pneumonia and septic shock under rituximab treatment. CONCLUSIONS: Our results, though limited to a low number of patients, highlight the effectiveness of different biologic therapies in the treatment of noninfectious refractory scleritis, showing to control scleral inflammation and allowing a significant reduction in the number of relapses.

Effectiveness of TNF-α blockade in the treatment of refractory non-infectious scleritis: a multicentre study.

OBJECTIVES: To evaluate the efficacy of tumour necrosis factor (TNF)-α inhibitors in refractory non-infectious scleritis. METHODS: We carried out a retrospective study assessing the efficacy of TNF-α inhibitors in the treatment of scleritis, scleritis relapses, glucocorticoid (GC)-sparing effect, impact on best-corrected visual acuity (BCVA) and safety profile. RESULTS: Nineteen patients (28 eyes) were eligible for analysis. Scleritis inflammatory grading significantly improved from baseline to the last follow-up (median ± IQR 2±4 and 0±0 respectively, p=0.0006). Scleritis relapses significantly decreased between the 12 months preceding and following biologic therapy (p=0.001). Mean GC dosage decreased from baseline (19.00±13.56 mg) to the last follow-up (7.59±5.56 mg) (p=0.003). No significant differences regarding BCVA were observed. Two AEs were recorded (1 severe urticaria and 1 case of pneumonia and paradoxical psoriasis). CONCLUSIONS: TNF-α inhibitors are effective in the treatment of scleritis while allowing a GC-sparing effect and preserving BCVA.

Botulinum toxin for treating unilateral apraxia of eyelid opening in a patient with congenital myotonia / Tratamento da apraxia de abertura palpebral unilateral em portador de miotonia congênita com botulinum toxin

ABSTRACT A 37-year-old female presented with severe apraxia of lid opening (ALO) affecting the right upper lid associated with Becker congenital myotonia (MC). The patient had a history of right upper lid ptosis for 25 years that was exacerbated over the previous month with severe incapacity to open her right eye. No other associated neurological or ophthalmic symptoms were observed. The patient was treated with botulinum toxin (BoNT-A) injection into the pretarsal and lateral canthus region of the orbicularis oculi of the affected eyelid. Treatment with BoNT-A is an effective method of managing ALO in Becker MC. This is the first case of unilateral ALO in the course of Becker MC that was successfully treated with injections of botulinum toxin.

RESUMO Trata-se de uma mulher de 37 anos apresentando grave apraxia de abertura da pálpebra (AAP) superior direita associada com miotomia congênita de Becker (MC). A paciente há 25 anos apresentava ptose palpebral a direita e há um mês desenvolveu incapacidade de abertura do olho direito. Não havia associação com outro sintoma neurológico ou oftalmológico. A paciente recebeu injeção de botulinum toxin (BoNT-A) no músculo orbicular a direita, na região pretarsal e no canto lateral. A BoNT-A foi efetiva para o tratamento da AAP associada com miotomia congênita de Becker.

An old disease in an atypical place.

A 72-year-old man presented with signs on uveítis. Biopsy of an iris lesion established the diagnosis of uveal MALT lymphoma with presumed choroidal involvement.

Experiences with rituximab for the treatment of autoimmune diseases with ocular involvement.

OBJECTIVE: To report the efficacy of rituximab (RTX) in the treatment of ocular or orbital inflammation accompanying autoimmune diseases refractory to previous standard immunosuppressive therapy. METHODS: We reviewed medical records of 9 consecutive patients with noninfectious ocular or orbital inflammation treated with RTX. RESULTS: Over a mean followup of 42 months, 7 patients were in clinical remission, 1 had partial response to treatment, and 1 did not respond. Best corrected visual acuity improved ≥ 1 line in 4 patients, was stable in another 4 patients, and worsened in 1. Concomitant immunosuppressive therapy was tapered in 6 cases. Systemic corticosteroids were tapered or kept below 7.5 mg a day in 5 patients 1 year after the first RTX cycle. CONCLUSION: RTX therapy, in patients who are refractory to standard immunosuppressive therapy, was effective and showed a beneficial response to treatment including induction of clinical remission of inflammation in most patients.

Tocilizumab treatment for refractory uveitis-related cystoid macular edema.

BACKGROUND: This retrospective study investigated the efficacy of tocilizumab (TCZ), a fully humanized antibody that binds both to soluble and membrane bound IL-6 receptors, for the treatment of uveitis-related cystoid macular edema (CME) refractory to immunomodulatory therapy. METHODS: Five refractory patients with uveitis-related CME who received TCZ between January and August 2012 were included. All patients received 8 mg/kg TCZ at 4-week intervals. Data regarding patient demographics, use of immunosuppressive drugs, biologic agents or intravitreal therapies prior to TCZ infusions were collected. Main outcome measure was central foveal thickness (CFT) measured by optical coherence tomography at 6 months. Secondary outcome measures were degree of anterior and posterior chamber inflammation (Standardization of Uveitis Nomenclature Working Group criteria) and visual acuity (logarithm of the minimum angle of resolution [log-MAR]) at month 6. Adverse events (AEs) related to TCZ therapy were also assessed. RESULTS: Eight eyes from five patients (all females) were included. Mean age was 49.4 years (range, 30-68). Mean follow-up was 8.4 months (range, 6-12). Before TCZ, all patients received and failed conventional immunosuppressive therapy and had received at least another biologic agent. Uveitis diagnoses were Birdshot chorioretinopathy (n = 3), juvenile idiopathic arthritis (JIA)-associated uveitis (n = 1), and idiopathic panuveitis (n = 1). Mean evolution of CME was 13.4 years (range, 2-30). Mean baseline CFT (95% confidence interval) was 602 ± 236 µm at baseline, 386 ± 113 µm at month 1 (p = 0.006), 323 ± 103 µm at month 3 (p = 0.026), and 294.5 ± 94.5 µm at month 6 (p = 0.014). Median best-corrected visual acuity (BCVA) improved from 0.66 ± 0.57 at baseline to 0.47 ± 0.62 at month 6 (p = 0.035). After 6 months, an improvement of ≥ 2 lines of BCVA was observed in 50% of eyes (p = 0.028) remained stable in 25% and worsened in none of the patients. Sustained uveitis remission was achieved in all patients. No AEs were reported. CONCLUSIONS: These data suggest that TCZ is effective for treating CME in otherwise treatment-refractory cases of uveitis.

Cytokine profiling reveals decreased serum levels of CCL2 in active ocular toxoplasmosis.

BACKGROUND: Toxoplasma gondii infection is an important cause of ocular disease. Although parasite-mediated host cell lysis is probably the principal cause of tissue destruction in immunodeficiency states, hypersensitivity and inflammatory responses may underlie severe disease in otherwise immunocompetent individuals. The purpose of the current investigation was to study the cytokine profiles in serum from patients with ocular toxoplasmosis and to compare them with those obtained from healthy control subjects. METHODS: Using a multiplex assay, we determined the serum concentration of granulocyte colony-stimulating factor (GCSF), interferon γ (IFNγ), interleukin (IL)-1ß, IL-2, IL-6, IL-8, IL-10, chemokine (C-C motif) ligand 2 (CCL2) and tumour necrosis factor α (TNFα) in patients with inactive ocular toxoplasmosis (n=48), active ocular toxoplasmosis (n=21), and an age-matched and sex-matched healthy control group (n=25). In a subgroup of 17 patients with active disease, a second serum sample was obtained when the disease was inactive. Cytokine profiles were correlated with disease activity, severity and visual outcome. RESULTS: Levels of CCL2 were significantly reduced in patients with active ocular toxoplasmosis compared to the control group (564 ± 42 pg/mL vs 455 ± 35 pg/mL, p<0.05). Moreover, CCL2 levels were significantly lower during active ocular toxoplasmosis compared to inactive disease (569 ± 32 pg/mL vs 433 ± 32 pg/mL, p<0.01). GCSF and TNFα were elevated in patients with toxoplasmosis with poor visual outcome. No significant correlations were found with specific cytokine profiles and disease severity. CONCLUSIONS: Decreased serum levels of CCL2 may be associated with active ocular toxoplasmosis and could therefore serve as a marker of disease activity.

Clinical pattern of toxoplasmic retinochoroiditis in a Spanish referral center.

BACKGROUND/AIMS: To analyze the clinical pattern of ocular toxoplasmosis in a referral center in Spain. METHODS: The medical records of consecutive patients with ocular toxoplasmosis admitted from a single referral center for uveitis in Barcelona (Spain) were retrospectively analyzed between January 2005 and January 2011. RESULTS: One hundred and thirteen eyes from 113 patients (74 Spanish and 39 South American) with active ocular toxoplasmosis were analyzed with a 12-month follow-up. Final BCVA ≤ 20/200 was found in 30 eyes (26.5%). The most frequent complications were macular edema (16.8%) and epiretinal membrane (11.5%). Anterior chamber cell scores of ≥ 2+ (p = 0.003), vitreous cell scores of ≥ 2+ (p = 0.001), and the presence of cataracts (p = 0.047) or serous retinal detachment (p = 0.008) were more common among the South American than Spanish cohort. Active macular lesions (p < 0.001) with an initial BCVA ≤ 20/200 (p < 0.001) and advanced age (p = 0.019) were predictors for final BCVA ≤ 20/200, whereas female gender (p = 0.021) and an initial BCVA ≤20/200 (p = 0.045) were predictors for ocular complications. Moreover, a BCVA ≤ 20/200 (p < 0.001) and a vitreous cell score of ≥ 2+ (p = 0.045) at the initial examination were predictors of an eventual need for ocular surgery. CONCLUSION: The clinical features of ocular toxoplasmosis in Spanish patients differ from those of South American patients. In general, active macular lesions with an initial BCVA ≤ 20/200 and advanced age were shown to be predictors for final BCVA ≤ 20/200 in our patient cohort.

Dexamethasone intravitreal implant for treatment of uveitic persistent cystoid macular edema in vitrectomized patients.

PURPOSE: To evaluate the safety and efficacy of Ozurdex (dexamethasone intravitreal implant) 0.7 mg in the treatment of uveitic macular edema in vitrectomized eyes. METHODS: Data from 13 patients (17 eyes) with persistent uveitic cystoid macular edema and a history of pars plana vitrectomy in the study eyes that were treated with intravitreal injection of 0.7-mg dexamethasone implant were reviewed retrospectively. Main outcome measures were changes in central retinal thickness measured by optical coherence tomography and changes in best-corrected visual acuity. RESULTS: The median age of patients was 61 years (range, 19-81 years). The median duration of uveitic macular edema was 12 months (range, 2-72 months). The mean baseline central retinal thickness (95% confidence interval) was 461.6 µm (403.8-519.4), decreased to 277.2 µm (244.6-309.8) at 4 weeks (P < 0.01), remained low at 349.9 µm (281.8-418.0) at 3 months (P = 0.01), and then reached 394.1 µm (328.3-459.8) at 6 months (P = 0.14). After 3 months, there was a median improvement of 2 lines of best-corrected visual acuity, with 52.9% of eyes gaining 2 lines or more (P < 0.01). At 6 months, there were 5 eyes that maintained the 2 lines gain and none had lost >1 line from baseline (P = 0.03). In 8 eyes (47.1%), reinjection of the implant was performed at a mean of 6.5 months. Ocular hypertension (47.1%), hypotony (11.8%), anterior chamber displacement of the implant (5.9%), and glaucoma, which required filtration surgery (5.9%), were the most common adverse events. Mean follow-up was 9.6 months (range, 6-17 months). CONCLUSION: In this small case series of eyes with limited follow-up, treatment with dexamethasone intravitreal implant injection for uveitic macular edema in vitrectomized eyes was associated with favorable visual outcomes and had an acceptable safety profile.

Pars plana vitrectomy for vitreoretinal complications of Behçet uveitis.

Purpose. To evaluate the role of pars plana vitrectomy (PPV) for the treatment of vitreoretinal complications of ocular Behçet disease (BD).
Methods. Retrospective review of the medical records of all patients who underwent PPV for vitreoretinal complications of Behçet uveitis in the Ophthalmology Department of the Hospital Clinic of Barcelona, Barcelona, Spain, from January 1994 until December 2011. Preoperative and postoperative best-corrected visual acuities (BCVA), ocular findings, treatments, surgical indications, and outcomes were evaluated in all patients.
Results. Twelve patients (14 eyes) were included in the study. Indications for PPV were the following: vitreous hemorrhage in 4 eyes (28.5%), persistent vitreous opacities in 3 eyes (21.4%), rhegmatogenous retinal detachment in 2 eyes (14.3%), macular hole in 2 eyes (14.3%), persistent cystoid macular edema in 2 eyes (14.3%), and combined tractional and rhegmatogenous retinal detachment in 1 eye (7.1%). At last examination (median follow-up 105 months, range 6-209 months), BCVA increased 3 Snellen lines or more in 6 eyes (42.9%). Immunosuppressive treatment could be reduced in 5 patients (41.6%). No serious ocular inflammatory episodes presented during the follow-up in those operated patients. 
Conclusions. In this retrospective series with long-term follow-up, PPV can treat secondary vitreoretinal complications of Behçet uveitis, restoring or stabilizing vision without major adverse events.

Behçet disease-associated uveitis successfully treated with golimumab.

Over the past decade, the off-label use of biologic agents such as TNF-α antagonists, including infliximab and adalimumab, has improved the treatment armamentarium for refractory immune-mediated uveitis, with particular success in Behçet disease-associated uveitis. Golimumab is a novel fully human anti-TNF-α monoclonal antibody that has been approved for the treatment of rheumatoid arthritis, psoriatic arthritis, and ankylosing spondylitis, with very promising results. Herein, the authors present the use of GLM in a case of Behçet uveitis refractory to other TNF-α blockers. There are only two reports in the literature about the use of GLM in uveitis, describing four patients with JIA-associated uveitis and a case of idiopathic retinal vasculitis. To the authors' knowledge, this is the first report about the use of GLM in Behçet uveitis.