The enigma of sclera-specific autoimmunity in scleritis.

Scleritis is a severe and painful ophthalmic disorder, in which a pathogenic role for collagen-directed autoimmunity was repeatedly suggested. We evaluated the presence of sclera-specific antibodies in a large cohort of patients with non-infectious scleritis. Therefore, we prospectively collected serum samples from 121 patients with non-infectious scleritis in a multicenter cohort study in the Netherlands. In addition, healthy (n = 39) and uveitis controls (n = 48) were included. Serum samples were tested for anti-native human type II collagen antibodies using a validated enzyme-linked immunosorbent assay (ELISA). Further, sclera-specific antibodies were determined using indirect immunofluorescence (IIF) on primate retinal/scleral cryosections. Lastly, human leukocyte antigen (HLA) typing was performed in 111 patients with scleritis. Anti-type II collagen antibodies were found in 13% of scleritis patients, in 10% of healthy controls and in 11% of uveitis controls (p = 0.91). A specific reaction to scleral nerve tissue on IIF was observed in 33% of patients with scleritis, which was higher than in healthy controls (11%; p = 0.01), but similar to uveitis controls (25%; p = 0.36). Reactivity to the scleral nerve tissue was significantly associated with earlier onset of scleritis (48 versus 56 years; p < 0.001), bilateral involvement (65% versus 42%; p = 0.01), and less frequent development of scleral necrosis (5% versus 22%; p = 0.02). HLA-B27 was found to be twice as prevalent in patients with scleritis (15.3%) compared to a healthy population (7.2%). In conclusion, scleral nerve autoantibody reactivity was more common in scleritis and uveitis patients in contrast to healthy controls. Further research is needed to characterize these scleral-nerve directed antibodies and assess their clinical value.

ANCA-associated scleritis: impact of ANCA on presentation, response to therapy and outcome.

OBJECTIVES: To describe the characteristics, treatment and outcome of isolated ANCA-associated scleritis at diagnosis compared with idiopathic scleritis with negative ANCA tests. METHODS: This retrospective multicentre case-control study was performed within the French Vasculitis Study Group (FVSG) network and in three French tertiary ophthalmologic centres. Data from patients with scleritis without any systemic manifestation and with positive ANCA results were compared with those of a control group of patients with idiopathic scleritis with negative ANCA tests. RESULTS: A total of 120 patients, including 38 patients with ANCA-associated scleritis and 82 control patients, diagnosed between January 2007 and April 2022 were included. The median follow-up was 28 months (IQR 10-60). The median age at diagnosis was 48 years (IQR 33-60) and 75% were females. Scleromalacia was more frequent in ANCA-positive patients (P = 0.027) and 54% had associated ophthalmologic manifestations, without significant differences. ANCA-associated scleritis more frequently required systemic medications, including glucocorticoids (76% vs 34%; P < 0.001), and rituximab (P = 0.03) and had a lower remission rate after the first- and second-line treatment. Systemic ANCA-associated vasculitis (AAV) occurred in 30.7% of patients with PR3- or MPO-ANCA, after a median interval of 30 months (IQR 16.3-44). Increased CRP >5 mg/l at diagnosis was the only significant risk factor of progression to systemic AAV [adjusted hazard ratio 5.85 (95% CI 1.10, 31.01), P = 0.038]. CONCLUSION: Isolated ANCA-associated scleritis is mostly anterior scleritis with a higher risk of scleromalacia than ANCA-negative idiopathic scleritis and is more often difficult to treat. One-third of patients with PR3- or MPO-ANCA scleritis progressed to systemic AAV.

Scleritis and Development of Immune-Mediated Disease: A Retrospective Chart Review.

OBJECTIVE: Scleritis may be idiopathic or caused by trauma, infections, or an immune-mediated disease (IMD). Our study aimed to understand the relationship between scleritis and IMD, including presenting characteristics, serologies, and treatment course. Understanding these associations may allow clinicians to risk-stratify patients and predict their clinical and treatment course. METHODS: We conducted a retrospective chart review of 341 patients with scleritis seen at a tertiary care center between January 1, 2005, and December 31, 2020. Demographics, scleritis characteristics, treatment response, recurrence, and serologic data were compared among patients with idiopathic and IMD-associated scleritis. RESULTS: Among patients with scleritis seen, 145 patients (43%) had an associated IMD, most commonly rheumatoid arthritis (RA; 39%), vasculitis (21%), or inflammatory bowel disease (14%). In most cases, the IMD diagnosis predated the scleritis presentation (63%), though vasculitis cases were more likely to develop during or after scleritis episodes. There were no significant differences in demographics or treatment failures among patients with scleritis with and without associated IMDs. Patients with IMDs were more likely to have a recurrence of scleritis (62% vs 49%, P = 0.02). CONCLUSION: At our ophthalmology center, 43% of patients with scleritis had an associated IMD, and most patients with an IMD were symptomatic from this disease prior to scleritis presentation. RA was the most commonly associated condition and typically predated the scleritis, whereas vasculitis was more likely diagnosed during or after the scleritis episode. Scleritis among patients with IMD is more likely to recur compared to scleritis that is idiopathic.

Case report: Medical and surgical management of presumed canine nodular granulomatous episcleritis with progression to posterior nodular scleritis, choroiditis, and optic neuritis.

Nodular granulomatous episcleritis (NGE) typically presents as an elevated mass or elevated masses at the limbus and often infiltrates the cornea (episclerokeratitis). In the current report, a granulomatous lesion was observed subretinally in the right eye (OD) of a 5-year-old male castrated American Staffordshire Terrier dog. There was concurrent retinal hemorrhage and detachment OD; the right eye was not visual. Due to poor prognosis for vision and potential for a neoplastic etiology of the mass, staging with higher imaging was recommended but declined by the owner. Therefore, an enucleation was performed. Histopathology of the globe identified a subretinal mass, marked histiocytic and lesser lymphoplasmacytic choroiditis, posterior episcleritis, and optic neuritis with retinal detachment. The subretinal mass was composed of densely packed, large, spindle histiocytes mixed with occasional lymphocytes, plasma cells, and only rare neutrophils. Regions of the mass showed lymphocytes aggregate to form nodules. This histological presentation was a type of proliferative histiocytic disease with similarities to nodular granulomatous episcleritis or granulomatous/necrotizing scleritis. This is a novel presentation of NGE-like progression to subretinal scleral, choroidal, and retinal involvement and provides a new differential possibility for posterior segment masses observed on fundic examination.

Orbital necrobiotic xanthogranuloma with necrotizing scleritis and absent cutaneous manifestations.

Necrobiotic xanthogranuloma (NXG) is a rare systemic disease, that commonly manifests with orbital and ocular adnexal involvement, presenting with periocular yellow papules and plaques that may ulcerate. Periorbital skin lesions are a hallmark of the disease, and in their absence the diagnosis may be delayed, preventing prompt systemic evaluation and appropriate treatment of this condition. We report a unique case of a 58-year-old female patient with NXG that presented with severe bilateral necrotizing scleritis, left orbital mass and no cutaneous manifestations of NXG. This case highlights the importance of considering NXG, despite absence of skin lesions, when other ophthalmological manifestations are present. Recognition of NXG is crucial due to the high lifelong risk of developing hematological malignancy.

Association between advanced glycation end products and uveitis/scleritis activity in patients with active immune-mediated ocular inflammatory diseases.

PURPOSE: To investigate for association between skin autofluorescence (SAF) advanced glycation end products (AGEs) and uveitis/scleritis activity in systemic inflammatory disease-related active non-infectious uveitis/scleritis patients. METHODS: This cross-sectional study was conducted at Siriraj Hospital during October 2019 to March 2020. AGEs were measured by SAF method in systemic immune-related disease patients with active uveitis/scleritis, and those results were compared with those of healthy age-matched controls. RESULTS: Thirty-one active non-infectious uveitis/scleritis patients and 31 age-matched controls were enrolled. The mean age of patients was 40.0 ± 12.8 years, and most were female (55.0%). The most common associated systemic immune-related disease was Vogt-Koyanagi-Harada disease (n = 14). Mean SAF AGE level in the study group compared to the control group was 2.38 ± 0.66 arbitrary units (AU) versus 2.58 ± 0.56 AU, respectively (p = 0.20). Multivariate analysis showed decreased SAF AGE level to be significantly associated with active ocular inflammation, (odds ratio: 0.01, 95% confidence interval: 0.00004-0.81; p = 0.04). CONCLUSIONS: SAF AGE level was not so far found to be a reliable biomarker for indicating uveitis/scleritis activity in systemic immune-related disease patients with active ocular inflammation. CLINICAL TRIAL REGISTRATION: Thai Clinical Trials Registry, https://www.thaiclinicaltrials.org/ . (Reg. No. TCTR20200114004, registered date 01/01/2020, beginning date of the trial 10/01/2019).

Ocular manifestations of granulomatosis with polyangiitis: report from a tertiary eye care center.

PURPOSE: To report demographic characteristics and ophthalmological manifestations of patients with granulomatosis with polyangiitis (GPA) in a tertiary eye care center in Turkey. METHODS: Medical records of patients with GPA-related ocular manifestations evaluated between 2013 and 2023 were included. Demographic and clinical characteristics of the patients including nature of systemic involvement, ophthalmologic symptoms and signs, laboratory investigations and treatment modality were reviewed. RESULTS: Twelve eyes of 10 patients (5 female/5 male) were included. The mean age was 57.2 ± 12.2 (35-71) years. Five (50%) patients were already diagnosed with GPA. Ocular involvement was the first manifestation of GPA in 3 patients. The remaining 2 patients had simultaneous systemic and ocular symptoms at presentation. Conjunctival hyperemia (9 eyes) and pain (7 eyes) were the most frequent presenting symptoms followed by blurred vision (3 eyes). The frequencies of ocular manifestations were as follows:episcleritis (3 eyes), isolated peripheral ulcerative keratitis (PUK) (3 eyes), scleritis (3 eyes), simultaneous PUK and scleritis (2 eyes) and periorbital mass (1 eye). CONCLUSION: Ophthalmological manifestations can be the initial findings in GPA. Since GPA can affect different structures of the eye, it sometimes might be challenging for ophthalmologists. Therefore, it is crucial for ophthalmologists to be well-informed about GPA-related ocular findings and to have a high index of suspicion for GPA. Although PUK associated with scleritis is highly suggestive for GPA, isolated cases of PUK or scleritis can be seen in GPA. Therefore, it is important to adopt a multidisciplinary approach, consider GPA in differential diagnosis, and benefit from accurate diagnostic tests.

Ocular lesions in patients with ulcerative colitis.

OBJECTIVE: Aim: To analyze the data and evaluate the prevalence of ocular lesions in patients with moderate ulcerative colitis. PATIENTS AND METHODS: Materials and Methods: We observed 112 patients aged 18-75 years old with clinically, endoscopically and histologically confirmed moderate ulcerative colitis which lasted at least 6 months. An ophthalmologic exam was performed to determine the presence of ocular symptoms. RESULTS: Results: Of the 112 patients with moderate ulcerative colitis, 21 (18,75%) had the following ocular lesions: episcleritis - 7 patients (6,25%), keratopathy - 5 patients (4,46%), uveitis - 5 patients (4,46%), cataract - 2 (1,78%) and scleritis - 2 (1.78%). CONCLUSION: Conclusions: Because ocular symptoms in patients with UC are often nonspecific, it may be beneficial to perform ophthalmologic examinations as a routine follow-up component of in such patients.

[Purulent scleritis following penetrating keratoplasty for acanthamoeba keratitis]. / Gnoinyi sklerit posle skvoznoi keratoplastiki po povodu akantamebnogo keratita.

The problem of treating purulent scleral infections, rare but extremely severe complication of ophthalmic surgeries, remains unresolved. This article presents a case of successful surgical treatment of purulent scleritis - interlamellar scleral abscess - that developed in a patient after repeat penetrating keratoplasty performed due to infectious lysis of the transplant. Although the first keratoplasty was performed for acanthamoeba keratitis, there were no signs of acanthamoeba invasion in the transplant at the time of the second surgery. Scleritis manifested as an infiltrate with pus penetrating the anterior chamber and development of keratoiridocyclitis. During surgery, the abscess cavity was opened, irrigated with an antiseptic solution, and drained into the subconjunctival space; the anterior chamber was irrigated with balanced salt solution through a separate paracentesis. No infection recurrences were noted in the postoperative period and the corneal transplant remained clear.

Comprehensive profiling of extracellular vesicles in uveitis and scleritis enables biomarker discovery and mechanism exploration.

BACKGROUND: Uveitis and posterior scleritis are sight-threatening diseases with undefined pathogenesis and accurate diagnosis remains challenging. METHODS: Two plasma-derived extracellular vesicle (EV) subpopulations, small and large EVs, obtained from patients with ankylosing spondylitis-related uveitis, Behcet's disease uveitis, Vogt-Koyanagi-Harada syndrome, and posterior scleritis were subjected to proteomics analysis alongside plasma using SWATH-MS. A comprehensive bioinformatics analysis was performed on the proteomic profiles of sEVs, lEVs, and plasma. Candidate biomarkers were validated in a new cohort using ELISA. Pearson correlation analysis was performed to analyze the relationship between clinical parameters and proteomic data. Connectivity map database was used to predict therapeutic agents. RESULTS: In total, 3,668 proteins were identified and over 3000 proteins were quantified from 278 samples. When comparing diseased group to healthy control, the proteomic profiles of the two EV subgroups were more correlated with disease than plasma. Comprehensive bioinformatics analysis highlighted potential pathogenic mechanisms for these diseases. Potential biomarker panels for four diseases were identified and validated. We found a negative correlation between plasma endothelin-converting enzyme 1 level and mean retinal thickness. Potential therapeutic drugs were proposed, and their targets were identified. CONCLUSIONS: This study provides a proteomic landscape of plasma and EVs involved in ankylosing spondylitis-related uveitis, Behcet's disease uveitis, Vogt-Koyanagi-Harada syndrome, and posterior scleritis, offers insights into disease pathogenesis, identifies valuable biomarker candidates, and proposes promising therapeutic agents.

Ocular Adverse Events after Coronavirus Disease 2019 mRNA Vaccination: Matched Cohort and Self-Controlled Case Series Studies Using a Large Database.

PURPOSE: To investigate the risk of ocular adverse events after Coronavirus Disease 2019 (COVID-19) mRNA vaccination. DESIGN: Matched cohort and self-controlled case series (SCCS) studies. PARTICIPANTS: We used a population-based database of medical claims and vaccination records in a large Japanese city. In the matched cohort study, we identified individuals who received COVID-19 vaccination (BNT162b2) from February 2021 to September 2021. One control was selected from nonvaccinated individuals by matching time, date of birth, sex, Charlson comorbidity index, and the enrollment period for health insurance. In the SCCS study, we analyzed individuals who developed ocular adverse events. METHODS: In the matched cohort study, we applied the Kaplan-Meier estimator to estimate the cumulative incidence of ocular adverse events over 21 days after the first dose and 84 days after the second dose. In the SCCS method, we used conditional Poisson regression to estimate the incidence rate ratio (IRR) of ocular adverse events during the risk periods (0-21 days after the first dose and 0-84 days after the second dose) compared with the remaining periods. MAIN OUTCOME MEASURES: Composite outcome of uveitis, scleritis, retinal vein occlusion (RVO), and optic neuritis. RESULTS: There were 99 718 pairs eligible for the matched cohort study after the first dose (mean age, 69.3 years; male, 44%). The vaccinated and control groups developed 29 and 21 events, respectively, over 21 days after the first dose, and 79 and 28 events, respectively, over 84 days after the second dose. The differences in cumulative incidence (reference, the control group) were 2.9 (95% confidence interval, -14.5 to 19.1) events/100 000 persons and 51.3 (16.2-84.3) events/100 000 persons, respectively, for the first and second doses. The SCCS study showed the IRRs of 0.89 (0.62-1.28) and 0.89 (0.71-1.11) for the first and second doses, respectively. CONCLUSIONS: The matched cohort analysis found an increased risk for the composite outcome after the second dose; however, the SCCS analysis showed no increased risk. Considering that the SCCS can cancel out time-invariant confounders, the current results suggest that COVID-19 vaccination is unlikely to causally increase the risk of ocular adverse events. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

Autoimmune Inflammatory Eye Disease: Demystifying Clinical Presentations for the Internist.

PURPOSE OF REVIEW: Provide a framework for recognizing key symptoms and clinical findings in patients with autoimmune inflammatory eye disease. RECENT FINDINGS: The most common manifestations of autoimmune inflammatory eye disease are episcleritis, scleritis, uveitis (anterior, intermediate, posterior, and panuveitis), and keratoconjunctivitis sicca. Etiologies can be idiopathic or in association with a systemic autoimmune condition. Referral of patients who may have scleritis is critical for patients presenting with red eyes. Referral of patients who may have uveitis is critical for patients presenting often with floaters and vision complaints. Attention should also be directed to aspects of the history that might suggest a diagnosis of a systemic autoimmune condition, immunosuppression, drug-induced uveitis, or the possibility of a masquerade condition. Infectious etiologies should be ruled out in all cases. Patients with autoimmune inflammatory eye disease may present with ocular or systemic symptoms alone, or in combination. Collaboration with ophthalmologists and other relevant specialists is vital to optimal long-term medical care.

Updates of ocular involvement in granulomatosis with polyangiitis.

Granulomatosis with polyangiitis (GPA) is a systemic inflammatory disease characterized by necrotizing vasculitis of the small-to-medium-sized vessels. GPA results from irregular autoimmune response with elevation of anti-neutrophil cytoplasmic antibody (ANCA) and inflammatory damage of vascular endothelial cells and other tissues. Ocular involvement is common in GPA with various manifestations due to the different ocular tissues suffered, but mostly, it causes orbital mass, dacryocystitis, scleritis, conjunctivitis, and keratitis. The diagnosis of GPA is based on a comprehensive analysis of systemic manifestations of vasculitis, imaging examinations, laboratory test especially serum levels of ANCA, and histological biopsy. Immunosuppressive therapy has greatly lowered the mortality and improved the prognosis of GPA, and the emergence of biological therapy indicates a promising prospect for GPA treatment strategy. In this narrative review, we integrate the latest literature on GPA-induced ocular disorders, presenting the previous views and new understandings especially on epidemiology, etiology, molecular mechanism, clinical manifestation, diagnosis, and treatment of GPA-related ocular involvement.

Use of optical coherence tomography angiography in the uveitis clinic.

As optical coherence tomography angiography is revolutionizing the ophthalmology world, the uveitis community is learning to understand where and how this new powerful imaging tool fits into the management of the panorama of ocular inflammations and infections. A non-invasive method of studying the retinal and choroidal vasculature, OCTA allows for the assessment of vessel density changes during active and inactive uveitis making it the natural imaging application of choice in uveitis clinical trials. However, these data and results are of limited utility to the ophthalmologists who are looking to apply OCTA in their everyday uveitis clinic. If employed strategically, OCTA can be a powerful tool for the uveitis specialist to evaluate iris involvement in viral uveitis; to assess the integrity of the vascular layers in the settings of white dot syndromes; to distinguish inflammatory choroidal neovascularization from outer retinal avascular inflammatory material; and to diagnose and follow infectious choroidal granulomas and satellite foci of chorioretinal inflammation without the need to administer dyes. The present review will analyze all the recent publications that apply OCTA in uveitis to offer the reader a guide on how to maximize the utility of this imaging modality in a clinical practice.

Alterations in choroidal circulatory dynamics and choroidal thickness before and after treatment in posterior scleritis.

BACKGROUND: Posterior scleritis is an inflammatory reaction of the sclera that occurs posterior to the ora serrata. The aim of this study was to present a case of posterior scleritis and to analyze choroidal circulatory and structural changes using laser speckle flowgraphy (LSFG) and optical coherence tomography (OCT), respectively. CASE PRESENTATION: A 64-year-old man presented to our department because of hyperemia of the left eye for one week, diplopia, ocular pain, and distorted vision when looking leftward. At an initial examination, his best-corrected visual acuity was 1.0 Oculi uterque (OU), with mild conjunctival hyperemia oculus dexter (OD) and marked ciliary hyperemia oculus sinister (OS). Color fundus photographs revealed a cluster of choroidal folds extending from the macula to the inferior retinal region OS. Swept-Source OCT showed choroidal thickening OD, and bacillary layer detachment and paracentral middle maculopathy on the paracentral side of the optic nerve papilla, suggesting severe inflammation. Fluorescein angiography showed hyperfluorescence in the optic disc and window defects around the macula OU. Indocyanine green angiography showed mottled choroidal vascular hyperpermeability findings in the late stage. B-mode echography displayed thickening of the posterior wall of the left eye. Orbital magnetic resonance imaging showed the thickened posterior eyeball. The patient was diagnosed with posterior scleritis, and 30 mg of oral prednisolone was then given and tapered off over the next 4 months. The hyperemia and intraocular inflammation resolved after the treatment. The rate of change in macular blood flow assessed by the mean blur rate on LSFG was 20.5% and 20.2% decrease OD and OS, respectively, before and after treatment. The central choroidal thickness showed 8.8% and 37.8% decrease OD and OS, respectively. CONCLUSION: Posterior scleritis complicated with choroiditis was suggested to show different choroidal circulatory dynamics from those in other choroidal inflammations.

The eye is a common site of granulomatosis with polyangiitis. A collaborative study.

BACKGROUND: Ocular manifestations of granulomatosis with polyangiitis (GPA) have been reported in a limited number of studies and with largely variable frequency. Here we report on the clinical, diagnostic, and therapeutic features of a cohort of 63 GPA patients, with particular regard to 22 of them with ophthalmic involvement (35%). METHODS: Clinical manifestations, results of immunological findings, histopathological pictures, imaging data, Birmingham Vasculitis Activity Score, therapeutic regimens, and outcomes were retrospectively analyzed. At diagnosis, in addition to a structured clinical assessment, all patients underwent a comprehensive ophthalmologic examination. RESULTS: The most frequently involved organs were kidneys, lungs, ear/nose/throat, and eyes. Ocular manifestations were bilateral in 32%. The three most commonly diagnosed ophthalmologic manifestations were scleritis (36%), retro-orbital pseudotumor or orbital mass (23%), and episcleritis (13%). Ocular and systemic involvement were simultaneously present at onset in 41% of the patients; systemic involvement was followed by ocular lesions in 36%; ocular inflammation was followed by systemic manifestations in 18%; and an orbital mass in the absence of systemic disease characterized 5%. Glucocorticoids plus cyclophosphamide and glucocorticoids plus rituximab were the combined therapies most frequently employed during remission induction and remission maintenance, respectively. Persistent ophthalmologic and extra-ocular remissions were achieved in 77 and 64% of the patients, respectively. One to three systemic relapses were diagnosed in 7 patients (31.8%). At the last follow-up, a visual outcome 20/40 or better in 31 (70%) of 44 eyes was determined. CONCLUSIONS: The eye was involved in over one third of our patients with GPA. Increased awareness, early diagnosis, and multi-specialty collaboration are critical in achieving a favorable outcome of GPA.

IgG4-related ophthalmic disease masquerading as ciliary body tumors and scleritis in both eyes: a case report.

BACKGROUND: To report a rare case of IgG4-related ophthalmic disease (IgG4-ROD) manifesting as intraocular masses and scleritis in both eyes in a 61-year-old male and to investigate the changes in multimodal imaging features of the lesion sites and helper T-cell type 1 (Th 1)/Th 2/Th 17 cytokine levels in the aqueous humor. CASE PRESENTATION: A patient with IgG4-ROD seemingly manifested with an intraocular tumor in the left eye and sequentially, with an inflammatory mass in the ciliary body and scleritis in the right eye. The patient complained of vision loss of 6 months duration in the left eye at his first visit. With a preliminary diagnosis of an intraocular tumor, enucleation of the left eyeball and histopathological examination were performed. Approximately 3 months later, the patient started to experience headache, eye pain, and declining vision in the right eye. Ophthalmic imaging revealed a ciliary mass and scleritis. Th 1/Th 2/Th 17 cytokine levels and multimodal imaging findings were analyzed before and after corticosteroid treatment. Histopathological examination and immunohistochemistry (IHC) of the enucleated left eye demonstrated lymphoplasmacytic infiltration with an IgG4+/IgG+ cell ratio of approximately 40%, pointing to the diagnosis of probable IgG4-ROD. Long-term treatment with corticosteroids led to significant improvement in the signs and symptoms of the left eye. Th 1/Th 2/Th 17 cytokine profile monitoring of the aqueous humor and multimodal imaging of the right eye showed gradual regression of the mass and attenuation of ocular inflammation during treatment. CONCLUSIONS: Patients with an atypical presentation of IgG4-ROD, such as intraocular masses and scleritis, are likely to experience a significant delay in diagnosis. This case demonstrates the significance of IgG4-ROD in the differential diagnosis of intraocular tumors and ocular inflammation. IgG4-RD is a newly diagnosed disease with multi-organ involvement and little is known about its pathogenesis, particularly in the eye. The present case will open new challenges in the clinico-pathological diagnosis and research of this disease. Combined investigations of multimodal imaging and cytokine level detection of intraocular fluid provide a new and effective way to monitor disease progression.

Online support group for patients with inflammatory eye disease: Characterisation of members and assessment of benefits.

BACKGROUND: Support groups in ophthalmology may be of utmost importance for patients and caregivers. We aimed to characterise members of an online support group for patients and parents of children with inflammatory eye disease and assess the members' expectations and perceived benefits. METHODS: A survey based on a voluntary, anonymous web questionnaire was distributed to the members of the 'Lirot' association online support group. The questionnaire included demographic, ocular and systemic information, functional and psychosocial data, support group use patterns, expectations and perceived benefits. Analysis was done for patients and parents. RESULTS: Out of 67 respondents, 43 (64%) were patients, and 24 (36%) were parents. Fifty-eight (88%) were women. The mean age of respondents was 42.9 ± 1.34 years. Anterior uveitis was the most common, and most patients had good visual acuity (<0.3 logMAR). Parents reported higher anxiety levels (p = 0.044) and a more significant effect of their child's disease on their general function (p = 0.005). Most members sought several experts' opinions, psychological treatments and alternative medicine. All members used the WhatsApp group, while only approximately half used Facebook (p < 0.001). Members' expectations were fulfilled for social support, a sense of shared experience and being able to help others, but not for receiving information (p < 0.001). All members reported being willing to recommend the group to others. CONCLUSIONS: Support group participation benefited group members. We suggest that ophthalmologists encourage their inflammatory eye disease patients to use online support groups, which may significantly improve their well-being.

Nodular Posterior Scleritis With Associated Choroiditis Masquerading As A Choroidal Tumour: A Case Report.

A case of nodular posterior scleritis in a 25-year-old male who presented with a 14-day history of unilateral decline in vision, pain, and redness in his right eye. Slit lamp examination of the right eye revealed dilated episcleral vessels present nasally as well as a choroidal mass at the nasal periphery of the fundus, associated with choroidal oedema. Systemic evaluation and imaging of the choroidal mass were performed to rule out choroidal tuberculoma and choroidal metastasis. Ultrasound B-scan of the right eye showed marked thickening of the nasalsclera resulting in sympathetic choroidal oedema without the characteristic T-sign. Nodular posterior scleritis with associated choroiditis, was diagnosed without any underlying systemic illness. The patient was immediately started on systemic steroids and later on subcutaneous Methotrexate as advised by the rheumatologist, to which he responded well and his vision significantly improved from 6/60 to 6/9, gradually during his treatment course.

Diagnosis and Treatment of Central Serous Chorioretinopathy in Patients with Scleritis.

Central serous chorioretinopathy (CSCR) is characterized by central neurosensory retinal detachment from the retinal pigment epithelium. While the association between CSCR and steroid use is widely recognized, it is difficult to distinguish whether the subretinal fluid (SRF) in ocular inflammatory disease results from steroid use or an inflammation-related uveal effusion. We report the case of a 40-year-old man who presented to our department with intermittent redness and dull pain in both eyes that had persisted for three months. He was diagnosed with scleritis with SRF in both eyes and steroid therapy was started. Inflammation improved with steroid use, but SRF increased. This indicated that the fluid was not caused by the posterior scleritis-related uveal effusion but by steroid use. SRF and clinical symptoms subsided after steroids were discontinued completely and immunomodulatory therapy was initiated. Our study highlights that steroid-associated CSCR must be considered in the differential diagnosis of patients with scleritis, and prompt diagnosis with an immediate shift from steroids to immunomodulatory therapy can resolve SRF and clinical symptoms.