Epidemiology of Intermediate Uveitis.

Intermediate uveitis is defined as inflammation of the vitreous and pars planitis. Etiology can be infectious, associated with a systemic disease, neoplastic or idiopathic. Pars planitis is the term used for idiopathic intermediate uveitis that presents with snowballs and snowbanks. While relatively rare, intermediate uveitis is present globally and typically affects adult females. Awareness of the presentation and a dilated fundus examination in patients presenting with floaters is essential to the diagnosis. PCR testing has enhanced the ability to diagnose infectious and neoplastic conditions that masquerade as intermediate uveitis. A structured review of systems and focused imaging, and laboratory testing will assist with early diagnosis and initiation of treatment.

Intravitreal steroid implants in the management of noninfectious intermediate and posterior uveitis.

ABSTRACT: The management of intermediate and posterior uveitis poses a significant challenge of achieving adequate drug concentrations in the posterior segment over the chronic nature of the disease. Systemic agents seldom reach effective drug levels, and even with low maintenance or tapering doses, it is hard to avoid systemic toxicity. The use of intravitreal and periocular injections is often unable to prevent recurrences due to their short half-life. Since the emergence of intravitreal implants (Vitrasert, Retisert), it has become possible to circumvent these therapeutic challenges. A detailed review in the PubMed index yielded 155 articles, of which 22 were analyzed based on exclusion criteria. A recent shift from surgically sutured to minimally invasive injectable implants mainly indicated for noninfectious uveitis is evident from the literature. This review article also provides insights into dexamethasone (Ozurdex) and recent fluocinolone acetonide (Yutiq, Iluvien) implants with particular emphasis on their improved safety and efficacy. Dexamethasone implants favor the therapeutic goal of prevention of recurrences, whereas the use of fluocinolone implants helps to attain better visual outcomes due to their longer duration of action. Thus, the review provides recent literature supporting the role and indication of sustained release intravitreal implants in the management of noninfectious intermediate and posterior uveitis.

Ocular Syphilis - Clinical Features and Outcome in HIV Positive and HIV Negative Patients from a Tertiary Eye Center from India - A Comparative Study.

PURPOSE: To study and compare the clinical characteristics and outcome of ocular syphilis between HIV positive and HIV negative patients. METHODS: Retrospective hospital-based case series from a tertiary eye care hospital in India. Patients with uveitis and positive syphilis serology were included. Demographics, clinical features, investigations, imaging and treatment modalities were noted. RESULTS: Hundred and five (105) eyes of 66 patients were analyzed. Males were predominantly affected (n = 57/66, 86.4%). Secondary syphilis was the most common stage of presentation (n = 48/66, 72.7%). Two groups were identified: HIV positive (HIVP) patients (n = 39/66, 59%) and HIV negative (HIVN) patients (n = 27/66, 41%). 12/39 (30.8%) patients were newly diagnosed with HIV at the time of ocular presentation. Panuveitis was the most common presenting feature in both groups (n = 66/105 eyes, 62.8%). Diffuse necrotizing retinitis was more common in HIV patients (HIVP - 15 Vs HIVN - 5 eyes). Ocular co-infections were more common in HIV patients, ocular tuberculosis, the commonest in both groups. Intravenous penicillin and titrated dose of systemic steroids were the mainstay of treatment. Improvement in mean logMAR was noted from 1.415 to 0.828 with p-value < 0.001. At final follow-up, 71.8% patients showed visual improvement. Complete resolution of ocular inflammation was noted in 95.5% patients. CONCLUSION: Ocular syphilis poses a diagnostic challenge considering the varied presentations and clinical course both in immunocompromised and immunocompetent groups. Clinical presentations are not always classical. High index of suspicion with supportive laboratory investigations and with characteristic OCT features helps diagnosis. All uveitis patients, especially with those suspected with infectious etiology, need to be tested for syphilis serology to prevent vision loss in this resurgent disease.

Efficacy of adalimumab in noninfectious pediatric uveitis: Analysis of 29 eyes from a tertiary eye care center in India.

PURPOSE: To describe the treatment outcomes of adalimumab in noninfectious pediatric uveitis. METHODS: The electronic medical records of children with noninfectious uveitis were reviewed retrospectively. The visual improvement and ocular inflammation were assessed according to the Standardization of Uveitis Nomenclature criteria before and after treatment with adalimumab. RESULTS: Twenty-nine eyes of 16 patients were included. The mean age of children was 9.4 ± 3.8 years. There were eight (50%) males and eight (50%) females. Juvenile idiopathic arthritis was the most common cause of uveitis in these children (N = 10, 62.5%), followed by Behçet's disease (N = 3, 18.75%) and Vogt-Koyanagi-Harada disease (N = 2, 12.5%). One child (6.25%) was diagnosed with Crohn's disease. Nine (56.3%) children were previously treated with methotrexate, one each with mycophenolate mofetil (MMF) (6.3%), azathioprine (6.3%), and tofacitinib (6.3%), and one child received MMF followed by azathioprine and cyclosporine before shifting to adalimumab. Three (18.8%) children did not receive prior immunosuppressive therapy. The average number of adalimumab injections given was 20.3 ± 12.0 at an interval of 2 weeks. The mean follow-up duration was 22.2 ± 16.6 months. Disease inactivity was achieved in 28 (96.6%) eyes at last visit ( P < 0.001). The best-corrected visual acuity (BCVA) improved from 0.48 ± 0.6 logMAR (logarithm of minimum of resolution) units at baseline to 0.20 ± 0.2 logMAR units at last visit ( P = 0.018). Disease remission was achieved in 23 (79.3%) eyes at the third month ( P = 0.001). CONCLUSION: Adalimumab is effective in the treatment of pediatric noninfectious uveitis by achieving disease inactivity, reducing the recurrence rate and improving BCVA. The drug is safe and well tolerated.

Sphingomonas paucimobilis: Navigating from Recurrent Hypopyon Anterior Uveitis to Endogenous Endophthalmitis: A Case Report.

PURPOSE: To report a case of endogenous endophthalmitis caused by Sphingomonas paucimobilis in a young male. MATERIALS AND METHODS: A retrospective case report. RESULTS: A 25-year-old male presented with reduced vision in the right eye and recurrent past episodes of hypopyon uveitis. The right eye had vision of counting fingers close to the face with cells, flare, and hypopyon in the anterior chamber with vitritis and exudates in the fundus. Blood investigations for tuberculosis, syphilis, toxoplasma, sarcoidosis, RA, ANA, HLA B27, and HLA B29 were negative. Anterior chamber tap investigations for herpes simplex viruses, varicella-zoster virus, cytomegalovirus, and toxoplasma, as well as Mycobacterium tuberculosis, yielded negative results. Ultrasound B-scan revealed a moderate number of low-reflective dot echoes in the vitreous, along with a few membranous echoes suggestive of vitritis. Blood culture and urine culture were negative. Since there was progressive deterioration, diagnostic and therapeutic vitrectomy was done with intravitreal antibiotics. The culture of the vitreous sample grew Sphingomonas paucimobilis. In the post-operative period, the patient developed retinal detachment, and re-surgery was done with a lensectomy, and the vision improved to 6/18 with contact lenses in the follow-up. CONCLUSION: This case report describes the distinct occurrence of endogenous endophthalmitis in an immunocompetent young male, which was previously reported only in peripartum cases. The clinical course is characterized by masquerading symptoms and recurrent episodes, despite the organism being of low virulence.

Surgically induced necrotizing scleritis (SINS): Is it a standalone condition or a variant of necrotizing scleritis?

PURPOSE: To report the clinical pattern of surgically induced necrotizing scleritis (SINS) in a tertiary eye care center in Southern India. METHODS: Retrospective analysis of all SINS cases visiting the uveitis clinic of a tertiary eye institute between January 2009 and April 2019. RESULTS: In total, 15 patients with a median age of 65 (IQR: 52-70) years were included in the study. Male (53%) predominance was noted, and SINS was unilateral (100%) in all cases. Most (87%) of the patients developed SINS after a single surgical procedure, with a median onset period of 251 (IQR: 127-1095) days. None of these patients had any evidence of systemic association. Ocular hypertension (n = 3, 20%), and cataract (n = 5, 33%) were the most common complications. When compared with a cohort of patients with idiopathic necrotizing scleritis, the index study did not find any statistically significant difference between SINS and idiopathic scleritis. CONCLUSION: SINS is idiopathic necrotizing scleritis rather than an independent entity of scleritis.

Masquerade syndrome: A review of uveitic imposters.

Masquerade syndromes in uveitis are complex clinical conditions where non-inflammatory diseases mimic uveitic manifestations, often leading to diagnostic and therapeutic challenges. This review delves into the diverse spectrum of masquerade syndromes, categorizing them into neoplastic and non-neoplastic entities. We explore the prevalence of primary intraocular lymphoma, leukaemia, retinoblastoma, and other malignancies, as well as conditions like retinitis pigmentosa and endophthalmitis that can present as uveitis. Through detailed analysis of symptoms, diagnostic methods, and treatment approaches, the review emphasizes the importance of considering masquerade syndromes in differential diagnoses to prevent mismanagement. The synthesis of current knowledge aims to enhance clinicians' ability to discern these complex presentations, advocating for a multidisciplinary approach to diagnosis and care, thereby improving patient outcomes in cases of uveitic masquerade syndromes.

An Analysis of the clinical and investigative profile of uveitis in the elderly: First case series from India.

PURPOSE: To evaluate the spectrum of uveitis occurring after 60 years of age in elderly patients who presented to a tertiary care eye center in India. METHODS: Retrospective study of patients who visited a tertiary eye care institute between January 2010 and July 2020. RESULT: Eighty-seven patients developed uveitis after 60 years, with only 44.8% having sufficient follow-up documentation and were included in the final analysis. The median age of these patients was 64 (IQR: 62-70) years, and 69% of them were male. Among the identifiable causes of uveitis, infectious uveitis (36%) was the most common and noninfectious uveitis was noted in 23% of patients. The most common subtype of uveitis was anterior uveitis (52%), followed by intermediate uveitis (32%), panuveitis (11%), and posterior uveitis (7%). Tuberculosis (28%) was the most common cause in our cohort, followed by HLA B27 (10%), sarcoid (8%), and Vogt-Koyanagi-Harada disease (5%). In 41% of patients, a definitive diagnosis of uveitis could not be achieved, and the anterior uveitis group had the highest number of undifferentiated uveitis cases. There were no undifferentiated cases of uveitis in the posterior and panuveitis category. The median follow-up period of these patients was 52 (15-91) months and 66% of eyes had recurrence. A statistically significant improvement in vision was seen in anterior uveitis and panuveitis groups, whereas the median visual acuity of the intermediate uveitis group remained stable throughout the follow-up period. CONCLUSION: Uveitis in the elderly can have a higher recurrence rate; however, the lack of follow-up in these groups of patients is a major challenge.

Evolution of research in diagnosis and management of uveitis over four decades in India.

Uveitis and its complications are more common in the developing world, in which the condition occurs in up to 714 per 100,000 in the population and accounts for up to 25% of all blindness. In India, the ophthalmic sub speciality of uveitis greatly evolved in the last four decades. In the early decades most of the studies were epidemiological studies. In recent years, more research has been published due to tremendous advancements in clinical diagnosis, laboratory investigations and ancillary test and treatment modalities. In this review article, we did a medline search with key words 'uveitis' and 'India', and selectively incorporated articles showing the evolution of this sub-speciality in India.

Retinal Vasoproliferative Tumours in Uveitis.

PURPOSE: To describe a cohort of patients with retinal vasoproliferative tumours (RVPT) seen in the uveitis clinic of a single tertiary eye care institute in India. METHODS: Retrospective Chart Review. RESULTS: In our study of 17 patients with 18 eyes affected by RVPTs, 94.1% showed unilateral involvement, one patient had bilateral RVPTs, and another had two RVPTs in the same eye. The mean age was 36.3 ± 15.1 years (range: 12-63 years), with a male majority (76.4%). The most common location was inferotemporal quadrant (44.4%). The most common associated uveitic condition was intermediate uveitis (50%). Patients were treated with oral corticosteroid (in 94.1%) and immunosuppressive (35.2%) in addition to transconjunctival cryotherapy (in 52.9%) or laser photocoagulation (in 41.1%). Regression of the RVPT was achieved in all cases except one. Visual acuity at time of detection of RVPT ranged from 6/6 to HM. No significant change was noted in the mean best corrected visual acuity pre and post treatment in the study group. CONCLUSION: RVPTs, rare peripheral retinal lesions often associated with intermediate uveitis, require early diagnosis with targeted therapy to prevent vision loss and achieve favorable long-term outcomes with rare recurrences.

Antiviral treatment for acute retinal necrosis: A systematic review and meta-analysis.

Acute retinal necrosis is a progressive intraocular inflammatory syndrome characterized by diffuse necrotizing retinitis that can lead to a poor visual outcome, mainly from retinal detachment. The antiviral treatment approach for acute retinal necrosis varies as there are no established guidelines. We summarize the outcomes of acute retinal necrosis with available antiviral treatments. Electronic searches were conducted in PubMed/MEDLINE, EMBASE, Scopus, and Google Scholar for interventional and observational studies. Meta-analysis was performed to evaluate the pooled proportion of the predefined selected outcomes. This study was registered in PROSPERO (CRD42022320987). Thirty-four studies with a total of 963 participants and 1,090 eyes were included in the final analysis. The estimated varicella-zoster virus and herpes simplex virus polymerase chain reaction-positive cases were 63% (95% CI: 55-71%) and 35% (95% CI: 28-42%), respectively. The 3 main antiviral treatment approaches identified were oral antivirals alone, intravenous antivirals alone, and a combination of systemic (oral or intravenous) and intravitreal antivirals. The overall pooled estimated proportions of visual acuity improvement, recurrence, and retinal detachment were 37% (95% CI: 27-47%), 14% (95% CI: 8-21%), and 43% (95% CI: 38-50%), respectively. Patients treated with systemic and intravitreal antivirals showed a trend towards better visual outcomes than those treated with systemic antivirals (oral or intravenous) alone, even though this analysis was not statistically significant (test for subgroup differences P = 0.83).

Systemic lupus erythematosus (SLE) associated uveitis in India - A case series.

PURPOSE: To report the uveitic manifestations of patients with systemic lupus erythematosus (SLE). METHODS: This was a retrospective analysis of all SLE cases with ocular manifestations seen by a single ophthalmologist between 2015 and December 2021. RESULTS: In total, seven patients with a median age of 40 (range 18-50) years were included in the study. Female (85.7%) predominance was noted. Ocular findings were bilateral in 71% (five patients) of cases. Majority (10 eyes, 83%) of the patients had retinal vasculitis as the common finding. Antinuclear antibodies were positive in all the patients. The vision improved in two (16.6%) eyes, was stable in eight (66%) eyes, and worsened in one (8%) eye. All the patients were treated with oral steroids along with immunosuppressive agents. CONCLUSION: Though SLE is rare cause of uveitis, it can be associated with significant ocular morbidity. Hence, early diagnosis and treatment can salvage vision in many cases.

Clinical spectrum and clinicopathological correlation of pediatric orbital tumors: 20 years' experience from a tertiary eye care center.

PURPOSE: To study the epidemiological profile of various pediatric orbital tumors and determine their clinicopathological correlation over 20 years. METHODS: A retrospective analysis of all pediatric patients with orbital tumors from the medical records between 1995 and 2015 was done. Tumors were categorized based on their cellular origin. Demographic details, clinical and histopathological diagnoses, frequency, and clinicopathological correlation were studied. RESULTS: A total of 265 pediatric patients with orbital tumors were analyzed, of which 145 (54.7%) patients were males and 120 (45.3%) were females. The mean age was 7 years (median: 6 years; range: 10 days-16 years). The distribution of lesions in each group was as follows: vasculogenic, 76 (28.7%); lipogenic and myxoid, 66 (25%); myogenic, 31 (11.7%); optic nerve and meningeal, 22 (8.3%); lymphoid and lymphoproliferative, 18 (6.8%); secondary orbital tumors, 14 (5.2%); histiocytic, 9 (3.4%); miscellaneous, 9 (3.4%), lacrimal gland, 8 (3%); mesenchymal with neural differentiation, 6 (2%); osseous, 4 (1.5%), and fibrocystic, 2 (0.8%). The most common benign and malignant pediatric orbital tumors were dermoid cysts (65, 24.5%) and rhabdomyosarcoma (31, 11.7%). A statistically significant ( P < 0.0001) clinicopathological correlation was observed in 208 (83.2%) out of 250 biopsy-proven cases. CONCLUSION: Pediatric orbital neoplasms include a broad spectrum of benign and malignant lesions. Vasculogenic tumors constitute the majority of them, followed by lipogenic and myxoid tumors. A thorough knowledge of the incidence, clinical profile, imaging features, and histopathology of specific orbital tumors aids in accurate diagnosis and their successful management.

Clinical association and visual morbidity in uveitis with systemic diseases: An analysis from a tertiary ophthalmic center.

AIM: The aim of this study was to identify the incidence, etiology, most common presentations, complications, and the causes of visual loss in posterior uveitis (PU). MATERIALS AND METHODS: A retrospective study was conducted on a cohort of 125 patients with PU with a minimum follow-up of 6 months. Ocular evaluation consisted of slit-lamp examination, indirect ophthalmoscopy, tonometry, and refraction. Ancillary ophthalmic investigations such as fundus fluorescein angiography, optical coherence tomography, and B-scan ultrasonography were done. Laboratory tests were performed on blood on all patients and in aqueous humor samples obtained by anterior chamber paracentesis in cases of diagnostic dilemmas. All the data were analyzed using SPSS program. RESULTS: PU occurred in 61%. Infections were noted in 34% and autoimmune diseases in 27%. The most frequent presentation was choroiditis. The most common etiologies were tubercular uveitis in 50%, Toxoplasma retinochoroiditis in 23%, and autoimmune category of serpiginous choroiditis in 56% of patients. Complications occurred in 27% and were most commonly cystoid macular edema and macular scarring. Polymerase chain reaction (PCR), nested PCR, and real-time PCR on ocular fluids were required for diagnosis in 30%. A combination of laboratory investigations on blood and aqueous humor samples were confirmation in 88%. CONCLUSION: PU and its sequelae are known to be sight threatening and are associated with systemic diseases. They have diverse etiologies and presentations. Identification of etiology is important as management is diametrically opposite in infections and autoimmune diseases.

A rare case of lacrimal sac dirofilariasis confirmed by polymerase chain reaction.

A 69-year-old female presented with complaints of persistent watering in the left eye for the past 8 years. She underwent a left external dacryocystorhinostomy (DCR) 8 years ago. On examination, left lacrimal passage irrigation showed regurgitation of fluid from opposite punctum. She underwent a revision external DCR with bicanalicular intubation and a portion of fibrosed sac was sent for histopathological examination (HPE). HPE showed a Microfilarial worm with surrounding granulomatous inflammation. Polymerase chain reaction (PCR) on the DNA extracted from the specimen identified the species to be Dirofilaria repens. Dirofilariasis is an accidental zoonotic infection in humans. Due to its migration, the worm can affect ocular and periocular structures. There are two case reports of perilacrimal dirofilariasis. This is the first case to report a filarial worm in the lacrimal sac causing chronic inflammation which probably led to failure of DCR. Treatment of choice is the complete eradication of the worm. PCR aids in the identification of species.

Branch retinal vein occlusion in a case of recalcitrant diffuse anterior scleritis treated with tofacitinib.

A 47-year-old woman with hypertension and rheumatoid arthritis presented with non-necrotizing scleritis in both eyes. Despite a course of oral corticosteroids, she continued to experience persistent symptoms. A rheumatologist was consulted and initiated treatment with tofacitinib, a JAK/STAT inhibitor. Treatment with tofacitinib and oral corticosteroids resulted in an improvement in the scleritis in both eyes. However, a fundus examination of her left eye revealed a superior-temporal branch retinal vein occlusion. Given the growing concern regarding the increased risk of thromboembolic events with tofacitinib therapy, it is essential to consider the risk of retinal vascular occlusions when starting tofacitinib therapy, particularly in patients with underlying systemic comorbidities.