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.

Anti-tumor necrosis factor-alpha treatment in pediatric patients with pars planitis: a single-center experience from Turkey.

AIM: To evaluate the effectiveness and safety of anti-tumor necrosis factor-alpha (anti-TNF-alpha) treatment (Adalimumab [ADA]) combined with immunomodulatory agents (IMAs) in the treatment of pars planitis (PP). METHODS: The patients with PP who were treated with anti-TNF-alpha agents for at least six months were qualified for the chart review. The outcome parameters were the steroid-free remission state, the best-corrected visual acuity (BCVA) and the central macular thickness (CMT) of the patients at the last visit. RESULTS: After a mean total follow-up time of 15.5 ± 5.8 months (8-24 months), all the cases were in steroid-free remission at the last visit. The mean BCVA increased, and the mean CMT decreased significantly at the last visit (p < 0.001, p < 0.001, respectively). CONCLUSION: ADA combined with IMAs offers effective and safe treatment modalities in the control of chronic intraocular inflammation in PP cases.

Clinical characteristics and treatment of pars planitis: an adalimumab experience.

PURPOSE: This study aims to investigate the clinical and demographic characteristics, treatment outcomes and complications of patients with pars planitis. METHODS: This retrospective study included patients diagnosed with pars planitis between 1998 and 2019 and followed for at least 6 months. Demographics, best-corrected visual acuity (BCVA), anterior segment and fundus examination findings, intraocular pressure (IOP) values at baseline and final examination, treatments used during the follow-up, surgeries and complications were noted from medical records of the patients. The percentage of patients given adalimumab (ADA), the reasons for treatment switch and response to ADA were investigated. RESULTS: One hundred fifteen eyes of 59 patients were included in the study. Forty-seven percent of patients were female. The median age of the patients was 10 (4-44) years. The median follow-up time was 33 (6-252) months. The median BCVA at admission was 0.20 (0.00-2.00) logMAR. The most common complications were cystoid macular oedema, cataract, epiretinal membrane and inferior peripheral retinoschisis. Prophylactic laser photocoagulation for peripheral retinoschisis was the most common surgical intervention, followed by cataract surgery and pars plana vitrectomy. Approximately 80% of patients received immunosuppressive and corticosteroid therapy for initial treatment. ADA was initiated in 23 patients (38.9%) due to refractory uveitis and adverse effects to the corticosteroid and helped control intraocular inflammation and decrease the use of systemic steroids/immunosuppressives in 22 of 23 (95%) of patients who received ADA. The median BCVA at final examination increased to 0.00 (0.00-2.00) logMAR. CONCLUSIONS: Pars planitis is a chronic, progressive and insidious disease with several ocular complications and requires early and aggressive treatment. ADA appeared to be effective especially in patients' refractory to conventional treatment.

Spectral domain optical coherence tomography findings of patients with pars planitis and risk factors affecting visual acuity.

PURPOSE: To evaluate spectral domain optical coherence tomography (SD-OCT) findings of 42 eyes with pars planitis and to identify risk factors affecting visual acuity. METHODS: Medical records and SD-OCT findings were retrospectively reviewed. RESULTS: Mean best-corrected visual acuity (BCVA) was 0.248 ± 0.3 on the logMAR scale at baseline. SD-OCT findings included epiretinal membrane (ERM) in 16 (38.1%) eyes, loss of normal foveal contour appearance in 8 (19.0%), ellipsoid zone (EZ) damage in 5 (11.9%), external limiting membrane (ELM) damage in 3 (7.1%), disruption of retinal pigment epithelium (RPE) in 2 (4.8%), and macular atrophy in 1 (2.4%). There was macular edema in 10 (23.8%) eyes [cystoid macular edema (CME) in 8 (19.0%), diffuse macular edema (DME) in 6 (14.3%), and serous retinal detachment in 2 (4.8%)]. The mean central macular thickness (CMT) was 272.1 ± 319.5 µm. Patients were followed up for a mean of 50.6 ± 36.7 months. Mean BCVA was 0.210 ± 0.3 at the final evaluation. SD-OCT findings included ERM in 28 (66.7%) eyes, EZ damage in 6 (14.3%), ELM damage in 3 (7.1%), disruption of RPE in 4 (9.5%), loss of normal foveal contour appearance in 12 (28.6%), and macular atrophy in 2 (4.8%). There was CME and/or DME in 6 (14.3%) eyes. The mean CMT was 238 ± 220.9 µm and was significantly lower than the baseline (p < 0.001). According to multivariate linear regression analysis, the presence of DME, and loss of normal foveal contour appearance at baseline were the independent factors associated with BCVA at the final examination (B = 0.726, p < 0.001; B = 0.766, p < 0.001, respectively). CONCLUSIONS: DME and loss of normal foveal contour appearance were more likely to have adverse effects on visual acuity.

Implications of pars planitis-associated cystoid macular edema on visual outcome and management in children.

PURPOSE: Pars planitis is a commonly observed type of pediatric uveitis. The aim of this study was to evaluate the implications of pars planitis-associated cystoid macular edema (CME) on visual outcome and treatment modalities. METHODS: A retrospective review of medical records in a single center with academic practice. RESULTS: Included were 33 children (mean age 8 years, 58 eyes). Eighteen eyes developed CME (31%): in 67% of them, CME was diagnosed at presentation and in 33%, it developed at a mean of 57 months after presentation. Anterior and posterior segment complications were more prevalent in eyes with CME. Papillitis was significantly associated with the development of CME (OR 12.4, 95% CI 2.3 to 65.6, p = 0.003). Patients with CME were 1.7 times more likely to be treated with systemic therapy. By the last follow-up, 50% of patients who never developed CME were without systemic therapy compared with 13% of patients who developed CME (p = 0.034). LogMAR visual acuity improvement between presentation and month 36 was 0.41 for eyes with CME compared with 0.14 for eyes that never developed CME (p = 0.009). CONCLUSION: Pars planitis-associated CME entailed higher prevalence of ocular complications, more frequent use of immunomodulatory therapy, and a lower rate of remission.

Progression of retinal pigmentation mimicking unilateral retinitis pigmentosa after bilateral pars planitis: a case report.

BACKGROUND: To report our findings in a young patient with unilateral retinitis pigmentosa (RP)-like appearance who developed pigmentary changes in his left retina after an episode of bilateral pars planitis. CASE PRESENTATION: A 17-year-old man presented with 6 months of blurry vision in both eyes. He was diagnosed with bilateral pars planitis. Progressive, intraretinal bone crepuscule pigmentation developed in his left retina during the following three months. An electroretinogram showed subnormal response only in the left eye, suggesting the diagnosis of unilateral pseudoRP. CONCLUSION: An inflammatory disease like pars planitis can accelerate the pigmentation of the retina and mimic a RP in young patients. Causes of pseudoRP may be considered, especially in those rare cases with unilateral affection.

Analysis of clinical features and visual outcomes of pars planitis.

PURPOSE: To evaluate the demographic characteristics, clinical features, treatment and outcomes of patients with pars planitis in a tertiary referral center in Turkey. METHODS: Medical records of patients with pars planitis were retrospectively reviewed. The data including demographic and ocular features and treatment outcomes were recorded. The distribution of clinical findings and complications were evaluated according to age and gender groups. The changes in final BCVA compared to the initial BCVA were noted. Statistical analysis was performed using SPSS software (Version 18.0, SPSS Inc., Chicago, USA). RESULTS: Twenty-seven patients (54 eyes) were included in this study. 16 patients were male (59.3%), and 11 were female (40.7%). Mean age at diagnosis was 12.84 ± 8.26 (range 4-36) years. Mean follow-up period was 61.3 ± 52.15 (range 9-172) months. Mean BCVA was 0.58 ± 0.36 (range 0.03-1.00) (0.40 ± 0.45 logMAR) at presentation, and 0.81 ± 0.28 (range 0.10-1.00) (0.14 ± 0.27 logMAR) at final visit (P = 0.001). Vitreous inflammation (100%), vitreous haze (92.6%), snowballs (74.1%), snowbanks (66.7%), anterior chamber cells (66.7%) and peripheral retinal vascular sheathing (48.1%) were the most common presentations. Ocular complications included vitreous condensation (51.9%), cystoid macular edema (22.2%), cataract (18.5%), inferior peripheral retinal detachment (11.1%), glaucoma (5.6%) and vitreous hemorrhage (3.7%). Treatments included topical, periocular, intravitreal and systemic corticosteroids, immunosuppressives, peripheral laser photocoagulation and pars plana vitrectomy when needed. CONCLUSIONS: Pars planitis is an idiopathic chronic intermediate uveitis mostly affecting children and adolescents. In spite of its chronic nature with high potential of causing ocular complications, adequate treatment and close follow-up lead to favorable visual outcomes.

Outcomes of cataract surgery with/without vitrectomy in patients with pars planitis and immunosuppressive therapy.

BACKGROUND: The purpose of this study was to evaluate the characteristics and outcomes of cataract surgery with/without vitrectomy in patients with pars planitis who received immunosuppressive therapy. METHODS: This was a retrospective case series, single-center study. Twenty-two patients with pars planitis who received immunosuppressive therapy were included, with a median age at presentation of 9.5 years, having had cataract surgery. The following data was collected: age at presentation and at cataract surgery, time of follow-up, best-corrected visual acuity (BCVA) before the surgery and at 1 week, 1 and 6 months after the procedure, immunosuppressive therapy, complications and causes for failed visual improvement. The variables associated with an improvement in visual acuity were evaluated. RESULTS: All patients had phacoemulsification with intraocular lens implantation. The most common immunosuppressive therapy used for the patients was methotrexate in nine patients (40.9%). The BCVA improved from a median of 20/400 to 20/100 after 6 months of follow-up (p = 0.0005); 14 patients (63.6%) improved two lines of vision or more. No significant risk factors were found for the association with improvement in visual acuity after the surgery. No improvement in visual acuity was attributed to posterior segment manifestations or amblyopia; the most common complication was posterior capsule opacification in 11 eyes (50%). The median follow-up after the surgery was 32 months. CONCLUSION: Phacoemulsification was the procedure for all the patients. Visual acuity improved in patients with pars planitis treated with immunosuppressive drugs who underwent cataract surgery, except for the patients with posterior segment complications or amblyopia.

A cross-sectional study of submacular thickening in intermediate uveitis and determination of treatment threshold.

BACKGROUND: The aim of this work is to refine understanding of anatomical and functional alterations in eyes with Intermediate Uveitis (IU), their natural history in mild cases not necessitating treatment and their response to treatment in severely affected eyes with macular edema. METHODS: 61 consecutive patients with IU presenting over a 6-year period were prospectively recruited into the study. Two subgroups of patients with IU were identified on the basis of the need or not for systemic cortico-steroid treatment. A group of healthy volunteers was identified for determining normal average central foveal thickness (CFT) values. Statistical comparisons were sought between patient sub-groups and with the group of normal volunteers for CFT and Best Corrected Visual Acuity (BCVA) at baseline and after 6 months. In a post hoc analysis, a cut-off value of CFT for systemic treatment initiation in IU was statistically identified and its sensitivity and specificity determined. RESULTS: A statistically significant difference in mean CFT at baseline was observed between patients under systemic treatment and untreated patients (p = 0.0005) as well as between untreated patients and healthy volunteers. (p < 0.001) After six months difference in CFT between the two patients subgroups was no longer significant (p = 0.699). BCVA was worse for patients under systemic treatment. No statistically significant difference could be identified between the subgroup of untreated patients and the group of healthy volunteers either at baseline or after 6 months. Correlation between LogMAR visual acuity and central retinal thickness at baseline was strong (r = 0.7436, p < 0.0001, Pearson's correlation coefficient). The cut-off value of CFT for initiating systemic treatment was determined at 215.5 µm in a post hoc analysis (sensitivity 62.5 %, specificity 96.4 %). CONCLUSIONS: Subclinical retinal thickening of mildly inflamed eyes with IU can occur though bearing no functional clinical significance and spontaneously resolving within 6 months. A cut-off CFT value for treatment of macular edema in IU, in the presence of other relevant morphological features on Optical Coherence Tomography, seems to emerge from post hoc analysis of collected data demonstrating strong specificity and moderate sensitivity.

Vitiligo in a Patient Receiving Adalimumab for Idiopathic Uveitis.

In recent years, adalimumab has been increasingly used in the chronic treatment of non-infectious uveitis. This case report aimed to describe a drug-induced adverse event in a 34-year-old man who presented with blurred vision and floaters in the right eye and was being treated for intermediate uveitis. The patient had started topical treatment with a diagnosis of uveitis at another center. Best corrected visual acuity at presentation was 0.8 (decimal) in the right eye and 1.0 in the left eye. On examination, the anterior chamber in the right eye was clear, with anterior vitreous cells and mild haze, and snow banking and vitreous opacities in the inferior periphery. Fluorescein angiography (FA) showed hyperfluorescence in the right disc and leakage in the inferior periphery. As the inflammation did not resolve with local treatment, systemic cyclosporine was administered, after which the patient exhibited vomiting and weakness. Cyclosporine was discontinued and adalimumab treatment was started. On examination 5 months later, bilateral vitreous cells and mild vitreous opacity were noted, and FA showed mild leakage in the inferior periphery bilaterally. In addition, a depigmented patchy vitiligo lesion was observed on the chin. Due to the persistence of intraocular inflammation and on the recommendation of the dermatology clinic, adalimumab treatment was continued and topical tacrolimus was started for the lesion. On examination 3 months later, the inflammatory findings had resolved and there was no progression of the vitiligo lesion. The patient's treatment was continued. Taken together with the previous literature findings, no pathology was found in the patient's systemic examination, suggesting that this lesion was a side effect of the treatment. Ophthalmologists should be alert for this side effect in patients receiving adalimumab.