Tofacitinib for Refractory Uveitis and Scleritis in Children: A Case Series.

PURPOSE: This study analyzes the efficacy and safety of tofacitinib in pediatric patients presenting with treatment-resistant uveitis and scleritis. METHOD: Retrospective Chart Review. RESULT: Nine children diagnosed with uveitis and one with scleritis received oral tofacitinib treatment. The median age of these patients was 9 years, with bilateral involvement observed in nine of them. Juvenile idiopathic arthritis was the most identifiable cause of uveitis, with anterior uveitis (50%) being the most frequent subtype of inflammation among these children. The median duration of immunosuppressive treatment before switching to tofacitinib was 18 (16-49) months. Remission of uveitis was achieved in all but two children, who experienced recurrence - manifesting as anterior uveitis. The median duration of follow-up in these children after tofacitinib treatment was 277.5 (183-549) days. At the end of follow-up, topical steroids could be withdrawn in six children, and two children were on topical steroids once a day. None of the children developed any systemic side-effect during the follow-up period. The mean BCVA at presentation was 0.62 ± 0.55, which improved to a mean of 0.27 ± 0.325 at the final follow-up (p = 0.0014). CONCLUSION: Treatment of pediatric uveitis with tofacitinib can be a valuable second-line treatment option and useful alternative in low- and middle-income countries.

The Collaborative Ocular Tuberculosis Study (COTS)-1: A Multinational Review of 165 Patients with Tubercular Anterior Uveitis.

Purpose: The Collaborative Ocular Tuberculosis Study (COTS) Group sought to address the diagnostic uncertainty through retrospective cohort analysis of treatment regimens and therapeutic outcomes for patients with tubercular Anterior Uveitis (TAU) across international centers.Methods: Multicentre retrospective analysis of patients diagnosed with TAU between January 2004 to December 2014 that had a minimum follow-up of 1 year.Results: One hundred and sixty-five patients were included. One hundred and seven subjects received antitubercular therapy (ATT) (n = 107/165; 64.9%) with all the patients receiving topical steroid therapy. Treatment failure was noted in 17 patients (n = 17/165; 10.3%), more frequently described in patients that received ATT (n = 13/107, 12.2%), than those that did not receive ATT (n = 4/58, 6.9%).Conclusion: In this retrospective study, addition of ATT did not have any statistically significant impact on outcome in patients with TAU.

Twenty-four Month Outcomes in the Collaborative Ocular Tuberculosis Study (COTS)-1: Defining the "Cure" in Ocular Tuberculosis.

Purpose: To report the clinical findings, anatomical features, and treatment outcomes in subjects with ocular tuberculosis (OTB) at 24 months in the Collaborative Ocular Tuberculosis Study (COTS)-1.Methods: Of the 945 subjects included in COTS-1, those who completed a 24-month follow-up after completion of treatment were included. The main outcome measure was a number of patients with treatment failure (TF).Results: 228 subjects (120 males; mean age of 42.82 ± 14.73 years) were included. Most common phenotype of uveitis was posterior (n = 81; 35.53%), and panuveitis (n = 76; 33.33%). Fifty-two patients (22.81%) had TF. On univariable analysis, odds of high TF was observed with bilaterality (OR: 3.46, p = .003), vitreous haze (OR: 2.14, p = .018), and use of immunosuppressive therapies (OR: 5.45, p = .003). However, only bilaterality was significant in the multiple regression model (OR: 2.84; p = .02).Conclusions: Majority of subjects (>75%) achieved cure in the COTS-1 at 24-month follow-up. The concept of "cure" may be a valuable clinical endpoint in trials for OTB.

The Collaborative Ocular Tuberculosis Study (COTS)-1: A Multinational Description of the Spectrum of Choroidal Involvement in 245 Patients with Tubercular Uveitis.

Purpose: To contribute a global description of the spectrum of choroidal involvement in tubercular uveitis (TBU).Methods: Retrospective cohort study of TBU patients with choroidal involvement from 25 centers between January 2004 and December 2014. Medical records of patients with a minimum follow-up of 1 year were reviewed.Results: 245 patients were included. The phenotypic variations included serpiginous-like choroiditis (SLC) (46%), tuberculoma (13.5%), multifocal choroiditis (MFC) (9.4%), ampiginous choroiditis (9%), among others. 219 patients were treated with anti-tubercular therapy (ATT) (n = 219/245, 89.38%), 229 patients with steroids (n = 229/245, 93.47%) and 28 patients with immunosuppressive agents (n = 28/245, 11.42%). Treatment failure was noted in 38 patients (n = 38/245, 15.5%). Patients with SLC and ampiginous choroiditis appeared to have superior outcomes on survival analysis (p = 0.06).Conclusion: This study provides a comprehensive description of choroidal involvement in TBU. Patients with SLC and ampiginous choroiditis may have better clinical outcomes.

Tubercular Uveitis: Nuggets from Collaborative Ocular Tuberculosis Study (COTS)-1.

Background: Tuberculosis (TB) is a major infection that can affect the eye as first and sole presentation without features of systemic disease. Controversy exists regarding diagnosis and management of tubercular uveitis (TBU), further compounded by regional variations in disease expression. Purpose: Collaborative Ocular Tuberculosis Study (COTS)-1 aims to address knowledge deficits through collaboration amongst uveitis specialists across the globe by sharing the data of patients with TBU presented at participating centers from January 2004 to December 2014. Methods: Data collection was facilitated by a novel method of real-time encrypted web-based data entry allowing regular updates as new data and recommendations become available. Results: Information on clinical features, investigation findings, management, and treatment outcomes were reviewed to get an idea about real world scenario. Conclusion: The current review aims to focus on methodology and briefing of published reports from COTS group in COTS-1 study to highlight key messages from this large data.

Paradoxical reactions in ocular tuberculosis.

Paradoxical reactions following initiation of anti-tubercular therapy have been documented most often in extrapulmonary tuberculosis. A combination of factors such as delayed hypersensitivity, decreased suppressor mechanisms, and an increased response to mycobacterial antigens mediated by the host's immune system have been implicated in the development of these reactions. Similar worsening in patients with ocular tuberculosis while on treatment has been described. It is therefore important for the clinician to be aware of this occurrence, as prompt recognition and timely institution of corticosteroids and immunosuppressants can lead to restoration of vision. In these patients, an alteration or discontinuation of anti-tubercular therapy may not be indicated.

Treatment outcomes of zone 1 retinopathy of prematurity: A study from a tertiary eye care center in South India.

PURPOSE: The main purpose is to study the treatment outcomes of zone 1 retinopathy of prematurity (ROP). MATERIALS AND METHODS: A retrospective analysis was done of infants diagnosed with zone 1 ROP with any stage with or without plus disease who were treated with either laser photocoagulation and/or intravitreal injection of antivascular endothelial growth factor (anti-VEGF) agents and/or underwent surgery according to their stage at presentation. The retinal outcome at the final visit was analyzed. A favorable outcome was characterized by an attached retina at the posterior pole with regression of ROP (regression of plus disease as well as new vessels) while an unfavorable outcome was detached retina at posterior pole in spite of treatment. RESULTS: Seventy-eight eyes of 39 infants presented with zone 1 ROP in various stages with plus disease in 50% cases. About 60 eyes underwent treatment. Forty eyes (66.6%) had an attached retina at the final follow-up. Thirty-three eyes (55%) underwent monotherapy with 14 eyes (23.3%) showing regression of ROP with laser alone. Nineteen (31.6%) eyes were treated only by surgery. Of these, a favorable outcome was seen in four eyes (44.4%) with Stage 4 disease and three eyes (30%) with Stage 5 disease. None of the eyes received anti-VEGF as monotherapy. A combination of two or more modalities was required in the remaining 27 eyes (45%). Six eyes (10%) needed anti-VEGF injections in addition to laser and six eyes needed surgery in addition to laser to achieve a favorable outcome. Six eyes (10%) required surgery in addition to both laser and anti-VEGF therapy, and one eye (1.6%) required surgery in addition to anti-VEGF therapy for a favorable final outcome. Among the eyes undergoing treatment, 66.6% had a favorable outcome with 92.9% of eyes in Stage 3, 59% in Stage 4, and 33% in Stage 5 showing regression of disease and attached retina. CONCLUSION: In spite of the aggressive nature of zone 1 ROP, favorable outcome is possible as was seen in 66.6% of our cases. A multipronged approach using a combination of laser, intravitreal anti-VEGF agents with or without surgery may be necessary for the management of these eyes.

The Collaborative Ocular Tuberculosis Study (COTS)-1 Report 3: Polymerase Chain Reaction in the Diagnosis and Management of Tubercular Uveitis: Global Trends.

Purpose: To analyze the role of polymerase chain reaction (PCR) of ocular fluids in management of tubercular (TB) anterior, intermediate, posterior, and panuveitis. Methods: In Collaborative Ocular Tuberculosis Study (COTS)-1 (25 centers, n = 962), patients with TB-related uveitis were included. 59 patients undergoing PCR of intraocular fluids (18 females; 53 Asian Indians) were included. Results: 59 (6.13%) of COTS-1 underwent PCR analysis. PCR was positive for Mycobacterium TB in 33 patients (23 males; all Asian Indians). 26 patients were PCR negative (18 males). Eight patients with negative PCR had systemic TB. Anti-TB therapy was given in 18 negative and 31 PCR cases. At 1-year follow-up, five patients with positive PCR (15.15%) and three with negative PCR (11.54%) had persistence/worsening of inflammation. Conclusions: Data from COTS-1 suggest that PCR is not commonly done for diagnosing intraocular TB and positive/negative results may not influence management or treatment outcomes in the real world scenario.

Oguchi's disease with Mizuo-Nakamura phenomenon in a seven-year-old boy.

A seven-year-old boy presented with difficulty in night vision of five years duration. Vision was 20/20 OU. Fundus examination revealed a golden sheen over the posterior pole in both eyes which disappeared after 45 minutes of dark adaptation, suggestive of Mizuo-Nakamura phenomenon. Clinical findings were suggestive of Oguchi's disease. ERG was confirmatory.

Antiretinal antibody- proven autoimmune retinopathy.

A young female presented with bilateral subacute onset of progressive decrease in night vision and reduced peripheral field of vision. The short duration and rapid progression of symptoms along with the lack of family history of night blindness prompted a diagnosis of autoimmune retinopathy (AIR). Fundus fluorescein angiography, optical coherence tomography, visual fields, and electroretinogram were suggestive of AIR. A differential diagnosis of retinitis pigmentosa (RP) was also made. Antiretinal autoantibodies were detected in the blood sample. Treatment was with oral steroids and subsequently oral immunosuppressive agents. Visual acuity was maintained, fundus examination reverted to normal, and investigations repeated at every visit were stable with improvement in visual fields. Our case suggests that AIR, if diagnosed early and treated appropriately, may have a good outcome and should be considered in patients with an atypical presentation of RP.