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.

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.

Uveítis intermedia como presentación inicial de un linfoma primario del sistema nervioso central / Uveitis intermedia as the initial presentation of a primary lymphoma of the central nervous system

El linfoma primario del sistema nervioso central (LPSNC) es una de las neoplasias cerebrales más infrecuentes, representando el 3% de los tumores primarios en dicha localización. Sumado a su baja prevalencia, las manifestaciones clínicas de esta patología son poco específicas, por lo que es común el retraso diagnóstico de esta entidad. El compromiso intraocular relacionado al LPSNC ocurre sólo en un 15% de los casos, siendo aún más inusual que dicha topografía se presente como debut en esta enfermedad. Presentamos el caso clínico de una paciente con uveítis intermedia como primera manifestación clínica de esta neoplasia (AU)

Primary central nervous system lymphoma is one of the most infrequent brain tumours, accounting for 3% of primary central nervous system neoplasms. In addition to its low prevalence, clinical presentation is usually nonspecific, leading to diagnostic delay. Intraocular involvement occurs in 15% of cases, and disease onset in this location is even rarer. We present a case of a patient with intermediate uveitis as the first clinical manifestation of this neoplasm (AU)

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.

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.

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.

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.

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.

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.

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.

Intermediate uveitis in children and young adults: differences in clinical course, associations and visual outcome.

AIM: Intermediate uveitis (IU) is considered to carry a considerable risk for complications and visual loss. This study compares juvenile and adult onset IU with regard to visual loss and complications. METHODS: Single-centre retrospective analysis of 110 consecutive children with onset of IU at the age of ≤16 years compared with 107 consecutive adult patients (17-35 years). All patients were followed for ≥1 year (mean 2.5±2.3 years). RESULTS: Average age at first uveitis diagnosis in children (65 boys; 59%) was 9.7±3.17 years and in adults was 24.3±4.92 years (36 men; 34%; p=0.003). Best-corrected visual acuity (BCVA) in both groups was similar at first and last presentation. Compared with initial presentation, BCVA improved in 31% of children and 21% of adults during the follow-up period, while it worsened in 9% and 8.7%, respectively. Lyme disease in children (8%) and multiple sclerosis in adults (17%) were the most frequent systemic associations. Poor visual acuity at first presentation, snowbanks and vitreoretinal traction were associated with a higher risk for poor BCVA at last presentation. In children, cataract formation (OR 7.6; 95% CI 1.7 to 33.8) and macular oedema (13.6; 1.6 to 113.5) and in adults posterior synechiae (7.3; 1.8 to 30.2), cataract formation (19.02; 2.31 to 156.56), macular epiretinal membrane (5.1; 1.02 to 25.28) and retinal detachment (8.4; 1.4 to 51.2) were associated with poor BCVA during follow-up. Uveitis course was not worse if associated systemic disease was present. CONCLUSIONS: IU with onset in childhood and early adulthood showed a similar visual course at intermediate follow-up. Associated diseases and complication patterns differed between children and young adults.

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.