Clinical characteristics of intermediate uveitis in adults according to criteria of the SUN working group.

PURPOSE: To analyse the clinical characteristics of adult patients with pars planitis (PP-IU), non-pars planitis (NPP-IU) and multiple sclerosis-associated intermediate uveitis (MS-IU) and distinguish between groups. METHODS: Seventy-three adult patients with intermediate uveitis (IU) reviewed retrospectively and divided as PP-IU, NPP-IU and MS-IU according to 'The standardization of uveitis nomenclature working group classification criteria.' Demographic and clinical characteristics, OCT and fluorescein angiography (FA) findings, complications and treatments were recorded. RESULTS: A total of 134 eyes of 73 patients were included, and 42 of the patients were classified as PP-IU, 12 as NPP-IU, and 19 as MS-IU. If a patient presenting with blurred vision, or tent-shaped vitreous band/snowballs/snowbank on examination, or vascular leakage on FA and accompanying neurological symptoms, the frequency of demyelinating plaque detection on cranial MRI and the risk of MS-IU increased. Mean BCVA was increased from 0.22 ± 0.30 logMAR to 0.19 ± 0.31 logMAR (p = 0.021). Gender, initial BCVA, snowbank formation, disc oedema and periphlebitis on examination, and disc leakage/occlusion on FA were found predictive of decreased BCVA at final visit (p < 0.05). CONCLUSIONS: The clinical features of these three groups are similar, some features that can guide the differential diagnosis. It may be recommended to periodically evaluate "suspicious" patients with MRI for MS.

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.

[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?

Intermediate Uveitis in Retinitis Pigmentosa Associated with a Novel Homozygous Splice Site Mutation in PRPF8.

The manifestation of intermediate uveitis (IU) in patients with retinitis pigmentosa (RP) is uncommon and poses diagnostic and management challenges. In this case, we describe the clinical features and management outcomes in an RP patient with a novel homozygous splice site mutation in PRPF8. A 21-year-old male presented with unilateral decrease of vision in the right eye for 1 week. Retinal dystrophy features were present in the left eye. After 2 weeks of topical steroid therapy, near-total resolution of IU was achieved and vision improved to 20/30. Signs of (RP) were present bilaterally, with the right eye more affected than the left. Genetic testing indicated a novel homozygous c. 3061-6_3061-3del mutation in the PRPF8 gene. IU in young patients with RP can be effectively treated with a short course of topical steroids, sparing the need for systemic immunosuppressives. After the improvement in IU, the right eye showed more advanced RP changes.

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.

Classification Criteria for Multiple Sclerosis-Associated Intermediate Uveitis.

PURPOSE: The purpose of this study was to determine classification criteria for multiple sclerosis-associated intermediate uveitis. DESIGN: Machine learning of cases with multiple sclerosis-associated intermediate uveitis and 4 other intermediate uveitides. METHODS: Cases of intermediate uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on the diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used in the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the intermediate uveitides. The resulting criteria were evaluated in the validation set. RESULTS: A total of 589 cases of intermediate uveitides, including 112 cases of multiple sclerosis-associated intermediate uveitis, were evaluated by machine learning. The overall accuracy for intermediate uveitides was 99.8% in the training set and 99.3% in the validation set (95% confidence interval: 96.1-99.9). Key criteria for multiple sclerosis-associated intermediate uveitis included unilateral or bilateral intermediate uveitis and multiple sclerosis diagnosed by the McDonald criteria. Key exclusions included syphilis and sarcoidosis. The misclassification rates for multiple sclerosis-associated intermediate uveitis were 0 % in the training set and 0% in the validation set. CONCLUSIONS: The criteria for multiple sclerosis-associated intermediate uveitis had a low misclassification rate and appeared to perform sufficiently well enough for use in clinical and translational research.

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.

Prognosis of Ocular Tuberculosis Following Long-Term Antitubercular Therapy.

Purpose: This study presents clinical features and prognosis after long-term (12-18 months) antitubercular therapy (ATT) in patients with ocular tuberculosis (OTB) in East China, an endemic area of tuberculosis. Methods: This retrospective study reviewed data from OTB patients treated at the Eye and ENT Hospital of Fudan University from 2008 to 2018. All the patients completed a minimum follow-up of 6 months after the cessation of ATT. Results: Sixty-six patients with OTB were studied. The ocular manifestations included retinal vasculitis (51.6%), choroiditis (24.2%), panuveitis (23.2%), intermediate uveitis (7.4%), scleritis (5.3%), anterior uveitis (2.1%), and optic neuropathy (1%). Except for two patients (ATT for 6 months), all other patients (64/66, 96.97%) received ATT for at least 12 months (6 patients for 12 months, 30 patients for 15 months, and 28 patients for 18 months). Treatment in conjunction with oral corticosteroids was used in 48 patients (72.7%). The average initial best-corrected visual acuity (BCVA) was 0.8 ± 0.64 (LogMAR), which improved to 0.31 ± 0.35 (LogMAR) at the last follow-up (P < 0.05). The final BCVA was significantly associated with the initial BCVA and the duration of clinical symptoms. A complete remission of uveitis was achieved in 97% of the patients. Conclusions: This study observed a favorable prognosis with long-term ATT regimens. Patients with better baseline visual acuity and a shorter duration of clinical symptoms before diagnosis had a better prognosis.

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.

INTERMEDIATE UVEITIS ASSOCIATED WITH PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS, AND CERVICAL ADENITIS SYNDROME.

BACKGROUND/PURPOSE: To report two novel cases of intermediate uveitis associated with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis syndrome. METHODS: Observational case reports and review of the literature. RESULTS: Both patients in this report had an established diagnosis of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis syndrome before the onset of ocular inflammation. Infectious and noninfectious systemic conditions known to be associated with intermediate uveitis were excluded. Intermediate uveitis was confirmed clinically in both patients by the presence of vitritis, snowballs, and peripheral snowbanks in the region of the pars plana. Both cases had a course characterized by recurrent inflammation; in which systemic steroid treatment, and in one case, immunomodulatory therapy was necessary. CONCLUSION: Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis syndrome is an auto-inflammatory fever disorder in childhood. Although other auto-inflammatory disorders such as, Blau syndrome, Muckle-Wells syndrome, and Behcets disease have been associated with various forms of uveitis, Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis has never been reported to be associated with any type of ocular inflammation. We describe for the first time, two cases of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis syndrome presenting with intermediate uveitis.

Anterior and Intermediate Uveitis Following Yellow Fever Vaccination with Fractional Dose: Case Reports.

Purpose: To describe two cases of anterior and intermediate uveitis following yellow fever vaccination with fractional dose. Methods: Case report. Results: Case 1: A 35 year-old healthy woman presented with unilateral anterior uveitis 10 days after the yellow fever vaccination. Testing excluded infectious and rheumatic diseases and the episode was fully recovered after a short course of topical treatment. Case 2: A 21 year-old previously healthy woman presented with blurred vision in the left eye (OS) 14 days after the yellow fever vaccination. The ophthalmic examination of the OS revealed intermediated uveitis. Testing excluded infectious and neurological diseases. After six weeks of treatment with oral prednisone, the ocular inflammation had resolved. Conclusion: Physicians should be aware of the possibility of eye inflammation following the yellow fever vaccination.

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.

Chikungunya Virus Infection Manifesting as Intermediate Uveitis.

PURPOSE: To describe a case of intermediate uveitis caused by chikungunya virus infection in the Western Hemisphere. METHODS: Case report of a patient diagnosed with chikungunya infection presenting with blurry vision and floaters. RESULTS: Exam revealed a unilateral intermediate uveitis, with an extensive work-up positive for chikungunya virus immunoglobulin M and G titers. The patient responded to oral corticosteroids with signs and symptoms resolving over the course of 3 months' treatment. CONCLUSIONS: While anterior uveitis and retinitis are the most common ocular manifestations of chikungunya infection, we report here a case of chikungunya infection presenting as an intermediate uveitis, responding well to oral corticosteroids. This case demonstrates the varied presentation of chikungunya-related uveitis and highlights its consideration in the differential diagnoses of those who have had preceding systemic viral symptoms and uveitis.

Intermediate and posterior uveitis.

Sight-threatening intraocular inflammation affecting the posterior segment of the eye may be predominantly located in the peripheral retina and vitreous (intermediate uveitis) or postequatorially where it manifests as inflammation of the retina, retinal vessels and/or optic nerve with cellular infiltration of the choroid and retina and edema particularly at the macula. Involvement of the macula is the main cause for visual loss. Experimental models of posterior uveitis have revealed much concerning the mechanisms of inflammatory cell damage to the retina, implicating CD4 T cells, effector macrophages and pro-inflammatory cytokines. In particular, transgenic and gene deletion models of inflammation have allowed an understanding of how immune privilege in the posterior segment of the eye is disrupted. Importantly, this has led to the development of new treatments with novel immunosuppressants and 'biologics' and the promise of cell-based therapies which may allow customized therapies tailored to the individual's inflammatory profile.

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.

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.