Clinical course of HTLV-1 infection associated intermediate uveitis.

OBJECTIVE: To describe the clinical features at presentation, delivered treatment and follow-up of a case series of human T-cell lymphotropic virus type 1 (HTLV-1) associated intermediate uveitis. PATIENTS AND METHODS: Retrospective, descriptive and longitudinal study of patients with HTLV-1 associated intermediate uveitis treated at a reference ophthalmology facility in Lima, Peru, during the years 2012 to 2018. RESULTS: A total of 18 patients (28 eyes) were included, the average age at presentation was 57.3 years, 66.6% were women, and the average follow-up time was 1,280 days. The most frequent symptoms were blurred or diminished vision (78.6%) and floaters (57.1%). Best corrected visual acuity was 20/40 or better in 53.6%. The mean initial intraocular pressure was 14.95 mmHg. Keratic precipitates were observed in 50% of eyes, 17.9% were of the stellate type. The most frequent treatment was periocular corticosteroid injections (53.6%). Complications such as epimacular membrane (50%), cataract (21.4%) and glaucoma (7.1%) occurred. At the end of follow-up, only 2 eyes lost one line of vision; the final best corrected visual acuity was 20/40 or better in 85.7%, and 20/70 or better in 96.4%. Patients with both eyes affected increased from 33% at presentation to 55.5%. The course of the disease was chronic in 60.7%. CONCLUSION: HTLV-1 associated intermediate uveitis mainly occurred in patients in the second half of life, developing a chronic course and with good visual prognosis.

Intermediate Uveitis Associated with Tattooing of Eyebrows as a Manifestation of Systemic Sarcoidosis: Report of Two Cases.

Permanent make-up tattooing as a cosmetic procedure is gaining popularity especially among women. Although it is considered a relatively safe intervention, the ink used can rarely be a trigger of sarcoidosis. Uveitis can occur as part of this inflammatory process. In this study, we describe two ladies presented with tattoo-associated uveitis as the first manifestations of systemic sarcoidosis. They developed intermediate uveitis shortly after skin inflammation several months after permanent make-up tattooing of eyebrows. Lung involvement, high ACE levels, and negative PPD were present. Skin granuloma formation was diffuse over the area of tattoo in one patient and localized in the other one. This is the first report of uveitis following make-up tattoo.

Intermediate Uveitis as the Initial and Only Presentation of Syphilis

We report a patient with unilateral syphilitic intermediate uveitis without dermatological, neurological, or any systemic involvement. He presented to our clinic with complaints of eye floaters and worsening visual acuity in the left eye. He had intermediate uveitis and cystoid macular edema in that eye and both venereal disease research laboratory and microhemagglutination assay for Treponema pallidum serological tests were confirmatory for syphilis. Ocular manifestations of syphilis have variable presentations, and it should be considered when diagnosing unexplained ocular inflammatory diseases, even if the patient's recent history and systemic evaluation are not compatible.

Intermediate uveitis in a child with phosphatase and tensin homolog gene mutation and Bannayan-Riley-Ruvalcaba syndrome.

Bannayan-Riley-Ruvalcaba syndrome (BRRS) is a congenital disorder characterised by macrocephaly, multiple hamartomas, lipomas, and pigmented macules of the glans penis. Intermediate uveitis is characterised by chronic inflammatory cells aggregates on the pars plana (snowbanks) and within the vitreous cavity (snowballs). We describe what we believe to be the first case of intermediate uveitis associated with BRRS. Early examination under anaesthesia should be considered in the management of young children diagnosed with this syndrome in order to provide appropriate ocular evaluation, treatment and follow-up. Further research is needed to establish a better understanding of the ophthalmic manifestations of this syndrome.

The Role of Dexamethasone Implant in the Management of Tubercular Uveitis.

PURPOSE: To study the safety and efficacy of intravitreal injection of dexamethasone implant in the management of posterior segment involvement in tubercular uveitis (TBU). METHODS: In this study, retrospective analysis of safety and efficacy of intravitreal injection of dexamethasone implant for various indications such as cystoid macular edema (CME), vitritis, or paradoxical worsening in TB-related intermediate uveitis, retinal vasculitis, and multifocal serpiginoid choroiditis (MSC) was performed. RESULTS: The study included 17 patients (19 eyes; 7 males). IOP increased from 14.88 ± 2.68 mm Hg to 16.4 ± 5.82 mm Hg (p = 0.18) at 3 months. BCVA improved from 0.37 ± 0.35 to 0.27 ± 0.21 at 3 months (p = 0.03). CME resolved at 3 months and two patients with paradoxical worsening showed resolution within 1 month. CONCLUSION: Intravitreal dexamethasone implant is safe and efficacious as an adjunct to ATT in reducing the central macular thickness, vitritis, and progression of choroiditis lesions in paradoxical worsening of MSC.

Effects of Fuchs uveitis syndrome on the ultrastructure of the anterior lens epithelium: A transmission electron microscopic study.

PURPOSE: The purpose of the study was to investigate the electron microscopic findings of the lens epithelial cells (LECs) in patients with Fuchs uveitis syndrome (FUS) who suffered from cataracts and to compare those with age-matched controls. METHODS: This study was a prospective, comparative case series. The anterior lens capsules (ALC: basement membrane and associated LECs) were taken from 12 eyes of 12 cases of FUS and ten eyes of ten control patients. The ALCs were obtained from cataract surgery and prepared for transmission electron microscopy (TEM). RESULTS: There were no statistically significant differences regarding the age or gender between the FUS group and the control group (P > 0.05). In the TEM examinations of the ALCs, all of the FUS cases revealed similar significant ultrastructural changes when compared to the control patients. In the FUS group, the LECs showed homogeneous thickening and irregularity which included some small vacuoles in different areas of the epithelial tissue. Moreover, in some areas of the LECs, widespread, oval-shaped, pigment clusters were detected. Conversely, in the control group, the LECs and all of their elements were in normal ultrastructural patterns, with the exception of some small intraepithelial vacuoles which were fewer and smaller than those in the FUS group. CONCLUSION: Ultrastructural analysis of the ALC of the patients with FUS disclosed some significant alterations which may be related to the summation of oxidative stress, intraocular inflammation, and iris atrophy.

Intermediate uveitis: pattern of etiology, complications, treatment and outcome in a tertiary academic center.

BACKGROUND: Patients with intermediate uveitis (IU) represent a heterogenous group characterized by a wide spectrum of etiologies and regional differences. Aim of the study was to analyze the characteristics of patients with IU examined in an academic center in Germany. METHODS: We conducted a retrospective analysis of the clinical records of all patients with intermediate uveitis referred to the Eye Center, University of Freiburg from 2007 to 2014. Diagnosis followed the Standardization in Uveitis Nomenclature (SUN) criteria. Data analysis included: etiology of IU, demographics, complications, treatment and visual acuity. RESULTS: We identified 159 patients with intermediate uveitis during that period. Mean age at diagnosis was 35 years. Most are female (64%), and the mean duration of IU was 6.1 years (range 1 month - 35 years). Etiology of IU was idiopathic in 59%. Multiple sclerosis (MS) (20%) and sarcoidosis (10%) were frequent systemic causes of IU. Other etiologies including infectious diseases (tuberculosis, borreliosis) or immune-mediated conditions (eg, after vaccination) were present in 11%. The pattern of complications included macular edema (CME) (36%), cataract (24%), secondary glaucoma (7%), and epiretinal membrane formation (19%). Periphlebitis and optic neuritis were more frequent in conjunction with MS. Treatment comprised local and systemic steroids, immunosuppressive agents, biologics, and surgery. Best corrected visual acuity was better than 20/25 in 60% of the eyes after more than 10 years of follow-up. CONCLUSIONS: In our German academic center, most IU cases were idiopathic or associated with MS or sarcoidosis. In contrast to other countries, infectious cases were rare. Patients' overall visual prognosis is favorable even when the duration of IU has been long and and despite numerous complications.

Anterior and Intermediate Uveitis with Retinal Vasculitis: An Unusual Manifestation of Post-Streptococcal Uveitis Syndrome.

PURPOSE: To report a case of bilateral panuveitis with vasculitis, an unusual manifestation of post-streptococcal uveitis syndrome (PSU). METHODS: An 8-year-old patient consulted for bilateral red eye following acute tonsillitis. Exploration revealed bilateral anterior uveitis, vitritis, macular edema, and Frosted Branch Angiitis. Given a clinical suspicion of PSU, blood and serology tests were performed to rule out other causes of vasculitis and retinitis. RESULTS: Serologies came back negative except for highly elevated antistreptolysin-O titers. Topical and oral corticosteroids normalized the patient's visual acuity and clinical findings within a few weeks. A subsequent anterior-only recurrence was successfully resolved with topical treatment. CONCLUSIONS: Although PSU most commonly manifests as anterior uveitis, it may present with involvement of the posterior pole. To achieve a correct diagnosis, clinical suspicion and assessment of antistreptolysin-O titers as well as ruling out other conditions with similar clinical features are the mainstay approaches to diagnosis. Prognosis is generally good.

Tumor necrosis factor polymorphisms associated with tumor necrosis factor production influence the risk of idiopathic intermediate uveitis.

PURPOSE: Idiopathic intermediate uveitis (IIU) is a potentially sight-threatening inflammatory disorder with well-defined anatomic diagnostic criteria. It is often associated with multiple sclerosis, and both conditions are linked to HLA-DRB1*15. Previously, we have shown that non-infectious uveitis (NIU) is associated with interleukin 10 (IL10) polymorphisms, IL10-2849A (rs6703630), IL10+434T (rs2222202), and IL10+504G (rs3024490), while a LTA+252AA/TNFA-238GG haplotype (rs909253/rs361525) is protective. In this study, we determined whether patients with IIU have a similar genetic profile as patients with NIU or multiple sclerosis. METHODS: Twelve polymorphisms were genotyped, spanning the tumor necrosis factor (TNF) and IL10 genomic regions, in 44 patients with IIU and 92 population controls from the UK and the Republic of Ireland. RESULTS: IIU was strongly associated with the TNFA-308A and TNFA-238A polymorphisms. We found the combination of TNFA-308 and -238 loci was more strongly associated with IIU than any other loci across the major histocompatibility complex, including HLA-DRB1. CONCLUSIONS: TNF polymorphisms, associated with increased TNF production, are highly associated with IIU. These results offer the potential to ascribe therapeutic response and risk (i.e., the influence of HLA-DRB1*15 status and TNFR1 polymorphism) to anti-TNF therapy in IIU.

Intermediate uveitis, an ophthalmological manifestation of systemic disease.

PURPOSE: Intermediate uveitis is frequently indicative associated with systemic disease. In addition to the initial evaluation of the patient with intermediate uveitis, we sought to determine the role of longitudinal follow-up in improving the diagnosis of systemic disease associated with intermediate uveitis. METHOD: Retrospective analysis of a cohort of 51 patients with intermediate uveitis followed for 5 to 13 years. RESULTS: Upon initial evaluation, an underlying disease associated with the intermediate uveitis was found in nine out of the 51 patients. Among the remaining patients, after at least 5 years of follow-up, eight new associated diagnoses were revealed (primarily inflammatory diseases and cancers). CONCLUSION: These results suggest that the initial work-up of the patient with intermediate uveitis is not sufficiently sensitive and that careful follow-up of these patients considerably improves the diagnosis of associated disease.

The impact of macular edema on visual function in intermediate, posterior, and panuveitis.

PURPOSE: To evaluate the impact of macular edema on visual acuity and visual field sensitivity in uveitis. DESIGN: This study utilized baseline data from the Multicenter Uveitis Steroid Treatment (MUST) Trial, a randomized, parallel treatment clinical trial comparing alternative treatments for intermediate, posterior and panuveitis. PATIENTS & METHODS: 255 patients (481 eyes with uveitis) recruited at 23 subspecialty centers. Visual acuity, optical coherence tomography and Humphrey 24-2 visual field testing. RESULTS: Macular edema was associated with impaired visual acuity (p < 0.01). Different phenotypes of macular edema were associated with different degrees of visual impairment: cystoid changes without retinal thickening were associated with moderately impaired visual acuity (-5 ETDRS letters), but visual acuity was worse in eyes with retinal thickening (-13 letters) and with both cysts and thickening (-19 letters). Uveitis sufficient to satisfy the study's inclusion criteria was associated with impaired visual field sensitivity, but eyes with macular edema had even worse visual field sensitivity (p < 0.01). CONCLUSIONS: The observation that macular edema substantially reduces visual function suggests macular edema itself is an important endpoint to study in the treatment of uveitis. As uveitis and macular edema both impair visual field sensitivity as measured by Humphrey 24-2 perimetry, both should be considered when evaluating patients with uveitis and raised intraocular pressure for glaucoma.

[Uveitis: diagnostic approach]. / Aproximación diagnóstica a las uveítis.

A 32 year-old woman was referred from the Ophthalmology Department to rule out a possible systemic disease. Her only past medical history of relevance was a tuberculosis contact during childhood. She complained of floaters and progressive blurring of vision in both eyes for some months, as well as arthralgia and cough. Her visual acuity was 0.3 in the right eye and 0.4 in the left eye. Biomicroscopy showed bilateral anterior granulomatous uveitis (1+ cells). Funduscopy showed bilateral vitritis 3+, snow banking and peripheral phlebitis. Fluorescein angiography did not show central vasculitis, and optical coherence tomography showed bilateral cystoid macular oedema. Fundus autofluorescence was normal. How would you initially assess this patient in order to decide which systemic examination should be performed, bearing in mind the ophthalmological manifestations?

Clinical characteristics of intermediate uveitis in Tunisian patients.

To analyze the pattern of intermediate uveitis (IU) in a referral center in Tunisia, North Africa. A retrospective, descriptive study of 87 consecutive patients (145 eyes) examined at the Department of Ophthalmology of Monastir (Tunisia) from January 1996 to August 2008. All patients underwent an extensive ocular and systemic history, a complete ophthalmic examination, and fluorescein angiography. Standard diagnostic criteria of IU were employed. The mean follow-up period was 43.2 months (range, 4-65 months). The mean age at diagnosis of IU was 29.1 years (range, 5-54 years). The male-to-female ratio was 1:1.6. Both eyes were affected in 58 patients (66.7%). The best-corrected visual acuity at onset of uveitis ranged from light perception to 20/20 (mean, 20/32). The most frequent ocular findings were vitritis (100%), snowballs (53.8%), and retinal vasculitis (28.3%). Concurrent anterior uveitis was noted in 38.6% of affected eyes. Intermediate uveitis was most commonly idiopathic (86.2%). Systemic associations were found in 13.8%; sarcoidosis (9.2%) and multiple sclerosis (2.3%) were the most common systemic diseases. The ocular complications that developed during the follow-up period included cystoid macular edema (39.3%), cataract (31.7%), inflammatory optic disc involvement (26.2%), and glaucoma or ocular hypertension (9%). One hundred-twelve affected eyes (77.2%) had a final visual acuity more than or equal to 20/40. Factors of poor visual outcome were initial VA less than 20/40 (P = 0.00011), CME (P = 0.0016), and vitritis more than 3+ cells (P = 0.023). In a hospital population in Tunisia, intermediate uveitis started frequently at the 3rd decade. This type of uveitis was most commonly idiopathic. Cystoid macular edema and cataract were the most frequent complications. Visual prognosis appeared to be fairly good.

Persistent intermediate uveitis associated with latent manifestation of facial large B-cell non-Hodgkin lymphoma.

PURPOSE: To report a case of intraocular non-Hodgkin lymphoma (NHL) manifesting as persistent intermediate uveitis, facial mass, and Bell palsy. DESIGN: Retrospective, interventional case report. METHODS: A 51-year-old patient with persistent intermediate uveitis for 4 years is diagnosed with intraocular NHL after developing Bell palsy and a facial mass. Initial diagnostic vitrectomy was inconclusive. RESULTS: Subsequent treatment with cyclophosphamide, adriamycin, vincristine, prednisone, and rituximab resulted in regressed facial mass and improved visual acuity. CONCLUSION: NHL can manifest as latent Bell palsy and facial mass in addition to the more common symptoms of uveitis. Intraocular NHL should be suspected in any patient of any age with persistent uveitis.

[Uveitis masquerade syndrome as a presenting form of diffuse retinoblastoma]. / Síndrome Mascarada por retinoblastoma difuso.

CLINIC REPORT: A 3-year-old boy presented with an intermediate uveitis. Complete ophthalmic exam, ocular ultrasonography, magnetic resonance imaging and computerized tomography of the orbit were inconclusive. Determination of the aqueous humor/serum rate of Lactate dehydrogenase (LDH) was the key for the diagnosis of a diffuse retinoblastoma. DISCUSSION: A masquerade syndrome is the initial presentation in 1-3% of retinoblastomas. Aqueous humor punction is contraindicated in patients with retinoblastoma but it might be the only way to achieve a correct diagnosis in these difficult and very unusual cases: enzymatic assays such as LDH offer a good sensitivity and specificity for the diagnosis of these patients.

Anterior and intermediate uveitis cases referred to a tertiary centre in Alberta.

BACKGROUND: We investigated the characteristics and causes of various uveitis subtypes in patients presenting to the Regional Eye Centre at the Royal Alexandra Hospital, University of Alberta, Edmonton, Alta., and estimated the incidence of anterior uveitis in northern Alberta. METHODS: A retrospective study was conducted of all patients presenting with uveitis to a single, full-time ophthalmologist at the Regional Eye Centre from September 2004 to June 2005. Uveitis was classified according to onset, severity, anatomical subtype, etiology, recurrence rate, and response to treatment. Statistical analysis was used to compare patients referred by ophthalmologists with those referred by non-ophthalmologists. RESULTS: Two hundred and nine eyes of 171 patients were included in the study. Ophthalmologist referrals consisted of 67.4% anterior, 14.0% intermediate, and 18.6% panuveitis, and non-ophthalmological referrals were 92.8% anterior, 5.4% intermediate, and 1.8% panuveitis. Referrals from ophthalmologists were significantly more likely to be chronic, recurrent, and (or) less responsive to treatment than referrals from other sources. INTERPRETATION: Referral bias strongly affects the proportions of uveitis subtypes seen. Human leukocyte antigen-B27-associated diseases (especially ankylosing spondylitis), sarcoidosis, and herpes infections should be considered among the most likely causes of uveitis to be diagnosed in this patient population.

Interferon as a treatment for uveitis associated with multiple sclerosis.

AIM: In addition to optic neuritis (ON), multiple sclerosis (MS) may also involve the eye with a typically bilateral intermediate uveitis. The aim of this pilot study was to evaluate the efficacy of type I interferons (IFN) for the treatment of MS associated uveitis. METHODS: In this non-randomised, retrospective observational case series 13 patients (eight female, five male) with proved MS and associated uveitis from five uveitis centres who were treated with interferon beta1a were included. Visual acuity (VA), cell count in the aqueous humour and vitreous, as well as the presence of cystoid macula oedema (CMO) were observed. RESULTS: All except one patient had a bilateral form of intermediate uveitis (total of 24 eyes). Seven patients had documented CMO before IFN treatment (n = 13 eyes). Median duration of treatment was 24.6 months (range 7.9-78.7). VA improved in 17 eyes (comparing VA before therapy and at last follow up); while 10 eyes (36%) improved >or=3 Snellen lines. Aqueous cell count improved by 1.2 (SD 1.1) grades in all eyes. Vitreous cell count improved by 1.7 (1.4) in all eyes. Only two patients still had minimal CMO on last follow up angiographically. CMO resolved after or during IFN treatment in nine eyes. CONCLUSIONS: IFN has been shown to have beneficial effects in patients with MS and/or ON. As shown in the models of experimental allergic encephalomyelitis (EAE) and uveitis, the neurological and ophthalmological manifestations seem to share similar pathogenic mechanisms. Treatment of MS associated uveitis with IFN appears to have beneficial effects on VA, intraocular inflammation activity, and the presence of CMO.

Uveítes associadas a doenças reumáticas / a

Uveíte é uma inflamação do trato uveal ocular. Pode estar associada à doenças reumáticas em até 40 por cento dos casos, sendo a uveíte anterior e a pan-uveíte agudas as formas de apresentação mais encontradas. A uveíte anterior aguda (UAA) pode estar associada com a positividade do HLA-B27, freqüente nas espondiloartropatias soronegativas e na artrite reumatóide juvenil (ARJ) pauciarticular tipo II. Pode ser encontrada também na sarcoidose e na doença de Behçet. A uveíte anterior crônica é muito menos freqüente que a UAA e comumente ocorre em associação com a ARJ pauciarticular tipo I e com a presença de anticorpos antinucleares. A uveíte posterior pode ocorrer no lúpus eritematoso sistêmico (LES) e a pan-uveíte pode ser encontrada principalmente na sarcoidose

Intermediate uveitis.

The cause of intermediate uveitis remains unknown. It is important to rule out other causes of the condition before initiating therapy. Many patients presenting with a mild form of the disease and who have good visual acuity may not require treatment. Those with decreased vision because of inflammation or cystoid macular edema may require periocular injections or systemic administration of corticosteroids (Fig. 2). Those who develop recalcitrant disease and those who experience severe side effects from the steroid therapy may require other immunosuppressive agents. Close monitoring of systemic side effects is required. Laser photocoagulation or cryotherapy of the peripheral retina is useful in patients who develop neovascularization of the vitreous base, in those who are not responsive to periocular injections, and in those who develop severe side effects from corticosteroids. This should be considered before starting systemic immunosuppressive agents. Pars plana vitrectomy with or without cryotherapy or laser photocoagulation is indicated in patients with marked vitreous debris, cystoid macular edema, and in those who develop a vitreous hemorrhage.