PURPOSE:We report a rare case of CMV-associated conjunctival atypical lymphoid hyperplasia (ALH). METHODS:A case report. RESULTS:A 34-year-old Chinese male with a 2-year history of recurrent bilateral redness, photophobia, and tearing, and no systemic or prior ocular illnesses, presented with severe bilateral conjunctival hyperemia, gelatinous limbal hyperplasia, and corneal opacities. Surgical excision was performed. Histopathology and immunohistochemistry revealed atypical lymphoid hyperplasia with a clonal B-cell population and CMV positivity within the lesional tissue. CONCLUSION:This case expands the clinical spectrum of ocular CMV disease and underscores the importance of considering CMV in the differential diagnosis of chronic, atypical conjunctival lesions. The relationship between CMV infection and ALH warrants further investigation. Long-term follow-up is needed to monitor for local recurrence or progression to overt lymphoma.
PURPOSE:To describe the demographics, clinical characteristics, treatment patterns, and outcomes of pediatric patients with noninfectious posterior and panuveitis at a single center. METHODS:Retrospective cohort study of all eyes with posterior and panuveitis between June 2015 and June 2025 that were identified using ICD codes. Charts were reviewed for demographics, clinical characteristics, disease evolution, sequelae, and treatment. Eyes with less than 3 months of follow-up were excluded. RESULTS:Between 2015 and 2025, a total of 12 004 patients (16,810 eyes) were seen for any uveitis diagnosis. Of them, 281 children (449 eyes) were referred for a diagnosis of non-infectious uveitis and 106 eyes of 69 patients met inclusion criteria. Bilateral disease occurred in 50.7% of patients. The leading diagnosis was idiopathic uveitis (51.9% of eyes), with a mean follow-up of 42 months. Nearly all eyes experienced at least one inflammatory flare (mean 1.1 per eye). Topical corticosteroids were most used (60.9% of treated eyes). Ocular sequelae were nearly universal (98.1%), and the proportion of patients with vision 20/200 or worse remained stable from baseline to follow-up (39.1% vs 40.6%). Linear mixed-effects modeling identified glaucoma (β×t = +0.0141 logMAR/month, p < 0.001) and vitreous opacities (β×t = +0.0096, p = 0.005) as the sequelae most strongly associated with visual decline. Methotrexate was independently associated with a more favorable visual acuity trajectory (β×t = -0.0071 logMAR/month, p < 0.001). CONCLUSIONS:Pediatric noninfectious posterior and panuveitis is characterized by chronic, bilateral disease with near-universal sequelae. Glaucoma and vitreous opacities were the strongest drivers of visual decline, while methotrexate was associated with visual preservation, supporting its early use in this population.
PURPOSE:To investigate the correlation between viral load, interleukin-8 (IL-8) levels and clinical outcomes during antiviral therapy in patients with acute retinal necrosis (ARN). METHODS:Before intravitreal injections of ganciclovir, aqueous humor samples were collected from 43 ARN patients (53 eyes). Viral load was quantified using polymerase chain reaction (PCR) and IL-8 concentrations were analyzed using enzyme-linked immunosorbent assay (ELISA). Relevant medical records and clinical characteristics were collected for analysis. RESULTS:Varicella zoster virus (VZV) was detected in 49 eyes (92.5%). No significant difference in Best-corrected visual acuity (BCVA) was found between baseline and final follow-up (p = 0.43). In VZV-induced ARN eyes, those with high initial viral loads (≥5.0 × 106 copies/ml) had significantly worse baseline and final BCVA than those with low viral load (p1 < 0.01, p2 < 0.01). Worse final BCVA correlated positively with both higher baseline VZV DNA loads (r = 0.14, p < 0.01) and elevated IL-8 levels (r = 0.26, p < 0.01). After treatment, viral load and IL-8 levels showed a plateau phase and a subsequent decrease. The viral load plateau lasted two weeks, while the IL-8 plateau lasted only one week. Viral load changes were positively correlated with IL-8 changes after antiviral therapy (r = 0.33, p < 0.01). CONCLUSION:Monitoring aqueous viral load and IL-8 levels may serve as a potential indicator for assessing the efficacy of antiviral treatment. Notably, IL-8 demonstrates greater sensitivity in reflecting the treatment response.
PURPOSE:To analyze the tear fluid proteome of non-infectious acute anterior uveitis to identify objective markers of inflammation and further dissect the underlying disease pathology. METHODS:Patients from the Uveitis arm of the German Spondyloarthritis Inception cohort with available tear fluid samples and consecutive patients attending the Ophthalmology Department were included. In total, 47 patients with unilateral, non-infectious acute anterior uveitis, including 23 with follow-up samples during non-inflamed state, and 23 healthy controls were enrolled. Tear fluid was collected using Schirmer strips from both eyes during unilateral inflammation and ≥5 months after uveitis resolution from the initially inflamed eye, and from the left eye of healthy individuals. Proteomic analysis was performed by mass spectrometry in data-independent acquisition mode. RESULTS:A total of 1,994 proteins were consistently identified in the tear fluid. Of these, 24 proteins were significantly differently expressed in the eye with active uveitis compared to the non-inflamed fellow eye: the most strongly upregulated proteins were protein S100-P, villin-like protein, and glutamine synthetase, while the most downregulated proteins were protein S100-A7, immunoglobulin kappa joining 3, and prostaglandin D2 synthase. The latter was also downregulated in active uveitis compared to follow-up and healthy controls. Compared to the post-inflammatory samples, active uveitis showed 201 differentially expressed proteins, including upregulation of proteins related to unfolded protein binding and downregulation of proteins involved in metabolic processes and energy-related pathways. CONCLUSION:Uveitis alters the tear fluid proteome, indicating the potential of identifying biomarkers useful for diagnostics and monitoring the course of inflammation.
Uveitis and scleritis are well-recognized extraintestinal manifestations of inflammatory bowel disease (IBD). Although ocular involvement typically follows IBD diagnosis, a meaningful subset of patients presents to ophthalmologists before intestinal disease is recognized, sometimes years earlier. Despite this, no standardized ophthalmologic screening protocols exist to guide early detection. We synthesized cohort studies, meta-analyses, and Mendelian randomization evidence on the ocular-intestinal temporal relationship in IBD. Patients with uveitis or scleritis have approximately double the risk of a subsequent IBD diagnosis (adjusted HR 1.44-2.25), with median diagnostic intervals exceeding two years and often longer in pediatric populations (>5 years). Mendelian randomization studies provide genetic evidence supporting a causal effect of IBD on uveitis. In a large pediatric cohort (n = 2,555), ocular manifestations preceded IBD in approximately 20% of cases. Uveitis occurs more frequently in Crohn's disease than in ulcerative colitis, and no ophthalmology guideline provides actionable screening criteria for IBD. We propose a tiered, risk-stratified framework to identify undiagnosed IBD in ophthalmology settings: (Tier 1) universal gastrointestinal symptom screening for non-infectious or recurrent uveitis and all scleritis; (Tier 2) targeted laboratory evaluation, including fecal calprotectin in high-risk patients; and (Tier 3) low-threshold gastroenterology referral for positive screens or persistent clinical concern. Lower referral thresholds are appropriate in pediatric populations. This framework is proposed to complement clinical judgment and requires prospective validation before adoption as standard practice. By recognizing ocular inflammation as an early systemic signal, ophthalmologists are uniquely positioned to reduce diagnostic delay and improve interdisciplinary care in IBD.
PURPOSE:To determine if ocular procedures are a risk factor for uveitis in patients without systemic autoimmune disease. METHODS:We performed a retrospective within-person matched case-control study for patients with unilateral uveitis from 2015 to 2020 on the United States Medicare-covered healthcare claims data. We determined the fellow non-uveitic eye as the control, and excluded patients with endophthalmitis, a history of autoimmune disease and uveitis within 3 months after surgery. We reviewed incisional surgeries, laser procedures, and intravitreal injections, and calculated matched odds ratios for prior ocular procedures in uveitic versus fellow control eyes using McNemar's test. RESULTS:565 patients were included. Uveitic eyes had significantly higher odds of prior ocular procedures compared with fellow control eyes (matched OR, 1.9; 95% CI, 1.3-2.9). We observed higher odds for incisional surgeries (OR 2.0; 1.3-3.1), laser procedures (OR 2.4; 1.04-5.4), and intravitreal injections (OR 4.8; 1.6-14.0). The difference was significant for complex cataract surgery (OR 3.3; 1.3-8.3), corneal surgery (OR 4.0; 1.1-14.2), glaucoma surgery (OR 3.8; 1.4-10.2), and pars plana vitrectomy (OR 4.6; 2.0-10.4), but not for routine cataract surgery (OR 1.0; 0.6-1.8). CONCLUSION:Our findings suggest that prior ocular procedures may be a risk factor for uveitis. These findings shed light on the etiology of some "idiopathic" uveitis cases in patients with prior ocular surgery.
PURPOSE:This study aims to demonstrate the efficacy of adalimumab (ADA) in reducing overall treatment burden in a cohort of patients with idiopathic multifocal choroiditis (MFC)/punctate inner choroidopathy (PIC). METHODS:This is a retrospective case series of eyes classified as having idiopathic MFC/PIC using standard multi-modal imaging, including color fundus photography, fundus autofluorescence, and spectral-domain optical coherence tomography. LogMAR best-corrected visual acuity (BCVA), doses of prednisone and other immunomodulatory therapies (IMT), injections of corticosteroids or anti-vascular endothelial growth factor (anti-VEGF), and breakthrough flares were collected at baseline (initiation of ADA), 12 months from baseline, and last follow-up. RESULTS:Twenty-five patients (21 females, 41 eyes) were included. Mean follow-up time was 51.4 ± 35.2 months (range 9.2-126.6). Mean LogMAR BCVA was 0.35 ± 0.48 at baseline and 0.34 ± 0.55 at last follow-up (p > 0.5). Mean prednisone dosage decreased significantly from 15.76 ± 18.69 mg/day at baseline to 0.23 ± 1.1 mg/day at the last follow-up (p < 0.0001). Thirty eyes received steroid or anti-VEGF injections at baseline; this decreased to one at the last follow-up (p < 0.0001). Similarly, eight patients received other IMT at baseline, but only one continued to receive supplemental IMT at the last follow-up (p < 0.05) at a decreased dosage. Six patients experienced disease flare-up within the first 12 months of treatment, and three patients flared between 12 months and last follow-up. CONCLUSIONS:Adalimumab therapy demonstrated a significant steroid-sparing effect in addition to vision preservation and reduction in both anti-VEGF injections and IMT.
PURPOSE:To evaluate the diagnostic yield, clinical utility and cost of common screening investigations in patients presenting with acute anterior uveitis (AAU). METHODS:Subjects with acute anterior uveitis were retrospectively identified from the Inflammatory Eye Disease Registry at Greenlane Clinical Centre, Auckland, New Zealand. Subjects were excluded if there was a pre-existing systemic disease known to cause AAU. Demographics, clinical presentation, diagnosis, human leukocyte antigen (HLA) B27, syphilis serology, serum angiotensin converting enzyme (ACE), chest x-ray and C-reactive protein (CRP) were assessed. RESULTS:1040 subjects were included in the study. Median age was 43.9 years with 57.1% male. Systemic disease was documented in 487 subjects (46.8%). Predictors of systemic disease on multivariate analysis included younger age (OR 0.987 p = 0.006), hypopyon (OR 4.960 p = 0.015) and high CRP (OR 1.525 p = 0.009). High intraocular pressure at presentation (≥24 mmHg) was associated with a lower risk of systemic disease (OR 0.229 p < 0.001) as was bilateral presentation (OR 0.417 p < 0.001). Cost of screening per true positive identified was $178.37 for HLA-B27, $2,144.81 for syphilis serology, $4,470.93 for serum ACE, and $5,389.17 for chest x-ray. CONCLUSIONS:The cost of screening in patients with AAU varied significantly between tests, with a high yield observed from HLA-B27 and syphilis and a low yield from sarcoid and chest x-ray. Costs can be optimized by selecting higher-risk patients via history and examination. Any AAU screening protocol must consider the diagnostic utility of an individual test and the cost of a missed systemic diagnosis.
PURPOSE:To evaluate the association between prostaglandin analogue (PGA) use and the development of uveitis in children and young adults with glaucoma. METHODS:We conducted a retrospective cohort study of patients ≤ 20 years of age with glaucoma, using the TriNetX United States Collaborative Network, a federated electronic health record database that aggregates de-identified clinical data, including diagnoses, procedures, medications, and demographics, from 67 healthcare organizations. Patients ≤ 20 years with glaucoma were identified using International Classification of Diseases, Tenth Revision (ICD-10) codes. Patients with a prior diagnosis of uveitis were excluded. We defined exposure as the new initiation of a PGA after a glaucoma diagnosis. We defined comparator groups as those who received topical β-blockers or carbonic anhydrase inhibitors (CAIs), excluding patients who used other glaucoma medications within the prior 3 months or had a history of uveitis. A minimum of 180 days of follow-up was necessary for inclusion to identify incident uveitis using International Classification of Diseases, Tenth Revision codes for anterior, posterior, or panuveitis. We used Cox proportional hazards models, adjusted for demographics, type 1 diabetes, aphakia, and ocular surgeries, to estimate adjusted hazard ratios (aHRs) and 95% confidence intervals (CIs). RESULTS:We identified 980 PGA initiators, 996 β-blocker initiators, and 477 CAI initiators. Uveitis occurred rarely within 180 days across all treatment groups, with 11 uveitis events observed among the β-blocker cohort, and fewer than 10 uveitis events observed among each of the PGA and CAI cohorts. Using β-blockers as the reference group, PGA initiation was associated with a significantly lower hazard of uveitis (aHR 0.20; 95% CI, 0.14-0.29; p < 0.01). In contrast, CAI initiation was not significantly different from β-blocker initiation (aHR 0.69; 95% CI, 0.46-1.04; p = 0.17). Increasing age increased the hazard of uveitis, whereas male sex and aphakia decreased the hazard. CONCLUSIONS/RELEVANCE:Among children and young adults with glaucoma, PGAs did not increase the hazard of uveitis during 180 days of follow-up and showed a lower hazard than β-blockers or CAIs.
Degos disease, or malignant atrophic papulosis, is an extremely rare multisystem occlusive vasculopathy characterized by pathognomonic porcelain-white cutaneous papules with frequently fatal gastrointestinal and central nervous system involvement. Ocular manifestations are varied and comprise only a small subset of the fewer than 200 total cases described in the literature. We report a rare pediatric case of systemic Degos disease with prominent ocular involvement preceding definitive diagnosis and a literature review of previously published cases of ocular Degos disease. A 13-year-old male with a history of Crohn's disease initially developed painless inferior visual field loss in the right eye with ipsilateral optic disc edema. Fundus examination revealed a chorioretinal scar in the contralateral asymptomatic eye. Over subsequent months, he developed a rash and progressive choroidal ischemia characterized by dome-shaped serous retinal detachment and extensive choroidal nonperfusion that had limited response to systemic and local corticosteroid treatment. Conjunctival telangiectatic vessels, progressive atrophic chorioretinal patches, and anisocoria followed in later progression, paralleling systemic deterioration, bowel perforation and death. Histopathology of jejunal biopsy confirmed Degos disease with C5b-9 deposition and type I interferon signaling.Recognition of unexplained choroidal nonperfusion or conjunctival vascular abnormalities with characteristic rash and GI symptoms should prompt consideration of Degos disease and multidisciplinary evaluation. Evolving understanding of Degos as interferon driven immune dysregulation leading to coagulopathy holds potential in targeted therapy that may alter the poor prognosis associated with this rare disease.
PURPOSE:Experimental autoimmune uveitis (EAU) is an animal model of non-infectious uveitis used to study immune-mediated ocular inflammation. Type I interferons and monoclonal antibodies (mAbs) represent distinct immunomodulatory strategies used in autoimmune diseases, including multiple sclerosis and associated uveitis. This study aimed to compare the therapeutic effects of these treatments in EAU. METHODS:PRISMA guidelines were followed for systematic review and meta-analysis (PROSPERO ID-1320794). PubMed, EMBASE, and Cochrane Library databases were searched through April 2025 for studies evaluating Type I interferons or monoclonal antibodies in EAU. Inclusion was rodent models of EAU treated with Type I interferons or mAbs and reporting clinical eye inflammation (EI) or histologic inflammation (H) scores pre/post treatment. Effect sizes were calculated using standardized mean differences. Risk of bias was assessed using the SYRCLE tool. RESULTS:Seven studies evaluating Type I interferons and fifty studies evaluating monoclonal antibodies were included. Type I interferon therapy significantly reduced EI scores compared with controls (SMD = -0.68, p < 0.001) but did not significantly reduce histologic inflammation (SMD ≈ -0.55, p = 0.25). Meta-regression showed no relationship between interferon dose/frequency and treatment efficacy. In contrast, monoclonal antibodies produced larger reductions in inflammation (EI: Hedges' g = -3.09 [95% CI - 3.70, -2.47]; H: Hedges' g = -1.76 [95% CI - 2.26, -1.27]), with time-stratified analysis demonstrating greater histologic improvement during later disease stages. Both therapies reduce EAU inflammation, but monoclonal antibodies demonstrate greater efficacy. CONCLUSION:These findings suggest that targeted cytokine or co-stimulatory pathway inhibition demonstrated larger anti-inflammatory effects than interferon-mediated immunomodulation in preclinical EAU models, though clinical applicability remains uncertain.
PURPOSE:To describe recurrent granulomatous uveitis associated with Bruton tyrosine kinase inhibitors (BTK inhibitors) in a patient with chronic lymphocytic leukemia (CLL), with systemic findings compatible with a sarcoidosis-like reaction. METHODS:Observational single-patient case report including clinical data, imaging and histopathological evaluation. RESULTS:A 64-year-old man with CLL developed bilateral granulomatous panuveitis eight months after initiation of ibrutinib. Inflammatory episodes recurred despite dose reduction. During treatment with ibrutinib, the patient developed cutaneous lesions at venipuncture and pressure sites; skin biopsies demonstrated non-necrotizing granulomas with suppurative inflammation, suggesting drug-induced granulomatous reaction. Ibrutinib was discontinued. The patient subsequently developed bilateral hilar enlargement on chest imaging. After disease progression, second-line therapy with pirtobrutinib was initiated. Eight days later, a new severe episode of bilateral granulomatous panuveitis occurred, fulfilling five of the seven International Workshop on Ocular Sarcoidosis (IWOS) criteria. Infectious causes were excluded. Inflammation improved with systemic and topical corticosteroids. CONCLUSIONS:The temporal relationship between BTK inhibitor exposure and recurrent granulomatous ocular inflammation, together with recurrence after exposure to a second BTK inhibitor, suggests a sarcoidosis-like reaction potentially related to this drug class and raises the possibility of a class-effect-related immune mechanism.
PURPOSE:To report the sequential occurrence of cilioretinal artery occlusion (CLRAO) and acute anterior uveitis as the initial ophthalmic and systemic manifestation of systemic lupus erythematosus (SLE). METHODS:Case report. RESULTS:A 28-year-old previously healthy man presented with sudden, painless visual loss in the right eye (BCVA 0.5). Multimodal imaging was consistent with right CLRAO accompanied by paracentral acute middle maculopathy. Work-up, prompted by a history of recurrent oral aphthous ulceration, revealed a positive antinuclear antibody and elevated anti-double-stranded DNA, with an isolated positive lupus anticoagulant (anticardiolipin and anti-β2-glycoprotein-I negative), fulfilling the 2019 EULAR/ACR criteria for SLE. Carotid imaging showed no atherosclerotic plaque but total occlusion of the right carotid system, consistent with a lupus-associated thrombotic vasculopathy. The next day, before systemic therapy, he developed unilateral acute anterior uveitis with vitreous involvement (BCVA 0.3; +3 anterior chamber cells; fine non-granulomatous keratic precipitates). After intravenous methylprednisolone, topical corticosteroid and cycloplegic therapy, the inflammation resolved and BCVA improved to 0.9 at one month. Long-term hydroxychloroquine and warfarin were maintained. CONCLUSION:A cilioretinal artery occlusion and acute anterior uveitis occurred in close succession as the initial manifestation of SLE. Rather than a causal sequence, they likely represent occlusive and inflammatory manifestations of the same active disease appearing within a narrow window. Awareness of this presentation may prompt earlier systemic evaluation.
PURPOSE:Childhood-onset sarcoidosis (COS) is a rare granulomatous autoinflammatory condition characterised by arthritis, dermatitis, and uveitis which includes early-onset forms (sporadic or Blau syndrome) and a later-onset form resembling adult sarcoidosis. Ocular involvement often occurs early and may be a prominent, sight-threatening feature. Despite this, COS is sparsely described in the literature. This case series aims to characterise the ocular manifestations, complications, and outcomes in COS. METHODS:A review of patients diagnosed with COS under a tertiary paediatric uveitis service. Data collected included age at onset, clinical findings, diagnostic methods, treatments, ocular complications, and visual acuity (VA) at presentation and last follow-up. RESULTS:Six patients were identified, all of whom presented with granulomatous uveitis. Four had posterior segment involvement including choroiditis and optic disc swelling. The mean age of ocular disease onset was 7 years. Diagnosis was supported by elevated serum angiotensin converting enzyme (ACE) followed by lymph node biopsy (n = 3), skin biopsy (n = 2), and/or NOD2 mutation (n = 3). All received systemic immunosuppression: methotrexate (n = 6), adalimumab (n = 5), mycophenolate (n = 2), oral corticosteroids (n = 3), and infliximab (n = 1). Complications included uveitic glaucoma (n = 2), cataract (n = 3), and chorioretinal scarring (n = 1). VA improved or remained stable in most, with one case of persistent visual impairment. CONCLUSION:COS-related uveitis demonstrates an aggressive, chronic course with early onset, bilateral involvement, and frequent complications with potential to cause visual loss. Careful ophthalmic screening in children with known or suspected sarcoidosis is critical.
PURPOSE:To analyze the clinical features and outcomes in patients presenting with Bilateral Acute Retinal Necrosis (BARN) at the time of initial presentation. METHODS:Retrospective, observational study of patients with bilateral Acute Retinal Necrosis (BARN) at a tertiary care center in South India from 2016 to 2025. Data collected included demographics, systemic and ocular history, BCVA (converted to logMAR for analysis), treatment modalities (medical and surgical), and complications such as retinal detachment, glaucoma, and phthisis bulbi. RESULTS:BARN was noted in 41 patients out of a total of 319 clinical records of ARN (12.8%). One-third patients (36.5%) had a history of HIV. The mean BCVA improved from 1.37 logMAR at presentation to 1.18 logMAR after a mean follow-up of 38.4 months. Rhegmatogenous retinal detachment (RRD) was the most common complication, affecting 36.5% (30/82) of eyes, with 8 eyes presenting with RRD and 22 developing it during follow-up. Exudative retinal detachment was noted in 7 eyes. Other complications included optic atrophy (10.9%), phthisis bulbi (4.8%), glaucoma (2.4%), and epiretinal membrane (1.2%). Medical treatment included antivirals and systemic corticosteroids. CONCLUSION:Bilateral acute retinal necrosis is uncommon but has more severe course and results in significant ocular complications and poor visual outcomes underscoring the aggressive nature of the disease.
PURPOSE:To report a case of delayed choroidal neovascular membrane (CNVM) following unilateral acute idiopathic maculopathy (UAIM) and to highlight the potential role of persistent choriocapillaris dysfunction and inflammation in its pathogenesis and management. METHODS:A 22-year-old woman with a previous history of steroid-responsive UAIM presented 9 months later with reduced vision (6/30) in the same eye. Multimodal imaging, including optical coherence tomography (OCT), OCT angiography (OCTA), fluorescein angiography (FFA), and indocyanine green angiography (ICGA), was performed to characterize the lesion. A subfoveal CNVM was identified and initially treated with intravitreal faricimab. Owing to persistent vascular activity and the subsequent development of adjacent inflammatory lesions, systemic corticosteroids were added in combination with further anti-VEGF therapy. RESULT:Initial anti-VEGF therapy reduced exudation but failed to achieve complete disease control. Following combined anti-VEGF and systemic corticosteroid therapy, both the inflammatory lesions and CNVM activity regressed. At final follow-up, exudation had resolved, OCTA demonstrated a compact mature vascular network without evidence of active neovascularization, and best-corrected visual acuity improved from 6/30 to 6/12. CONCLUSION:CNVM may develop as a delayed complication of UAIM despite apparent structural recovery. Persistent choriocapillaris hypoperfusion and recurrent inflammation may contribute to its development, although a causal relationship remains to be established. This case highlights the value of multimodal imaging in identifying the underlying mechanisms of delayed CNVM and suggests that combined anti-VEGF and anti-inflammatory therapy may be beneficial in selected patients. Long-term surveillance is warranted in eyes with UAIM.
PURPOSE:To report the clinical characteristics of JC virus (JCV) retinitis. METHODS:A retrospective case report. RESULTS:A 29-year-old male patient, diagnosed with HIV at AIDS stage, had brain lesions compatible with progressive multifocal leukoencephalopathy. Fundus examination showed areas of retinal atrophy and changes in the pigment epithelium. A similar worsening of the retinal and brain lesions was observed, despite several treatments. The vitreous sample analyzed using Next Generation Sequencing analysis was positive for JCV. No other pathogens were identified. The retinal lesions finally stabilized, concomitantly with the brain lesions, due to immune restoration. CONCLUSION:We report here a case of presumed JCV-related atypical retinitis associated with a progressive multifocal leukoencephalopathy in a patient with AIDS.
PURPOSE:Behçet's disease (BD) is a systemic vasculitis characterized by recurrent oral and genital ulcers, skin lesions, and ocular involvement. Uveitis is a major cause of disability and visual loss in BD. This study aimed to evaluate the safety and efficacy of adalimumab in refractory Behçet's uveitis. METHODS:This prospective longitudinal cohort study included 25 patients with Behçet's disease and uveitis refractory to conventional immunosuppressive therapy. All patients received adalimumab 40 mg subcutaneously every other week and were followed for 1 year. Outcome measures included changes in best corrected visual acuity (BCVA), anterior chamber reaction, vitreous haze, retinal vasculitis, papillitis, retinitis, cystoid macular edema, corticosteroid use, and adverse events. RESULTS:Mean BCVA improved from 0.14 ± 0.20 at baseline to 0.21 ± 0.23 at the final visit (p = 0.021). The proportion of eyes with absent anterior chamber reaction increased from 24% to 96% (p < 0.001), while vitreous haze improved in 92% of patients. Vasculitis, retinitis, and papillitis decreased from 92% at baseline to 20% at final follow-up (p < 0.001). Mean systemic corticosteroid dose decreased from 27.20 ± 11 mg/day to 15.20 ± 6 mg/day (p < 0.001). Oral and genital ulcers were controlled in 84% of patients. Two patients discontinued adalimumab because of systemic infection, and one patient was switched to infliximab due to inadequate response. CONCLUSIONS:Adalimumab demonstrated significant efficacy in controlling ocular and systemic inflammation in refractory Behçet's uveitis with a meaningful steroid-sparing effect and acceptable safety profile over 1 year of follow-up.
