[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.

Acute bilateral nodular scleritis following strabismus surgery in a patient with Down syndrome.

Scleritis is a rare complication of strabismus surgery. We present the case of an 11-year-old girl with Down syndrome who underwent strabismus surgery and developed bilateral symmetric nodular scleritis 3 weeks after surgery. Anterior segment optical coherence tomography revealed localized hyporeflective thickening of the sclera at the primary site of muscle insertion in both eyes. Treatment with topical and oral corticosteroids led to resolution of her condition.

Ocular manifestations in ANCA-associated vasculitis: a comprehensive analysis from Chinese medical centers.

INTRODUCTION: This study aimed to explore ocular manifestations in ANCA-associated vasculitis (AAV), focusing on granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA) and to examine the associations with laboratory parameters and other systemic manifestations. METHODS: This retrospective study reviewed data from 533 AAV patients across two major Chinese medical centers from January 2016 to November 2023. Data including diagnosis, cranial manifestations of disease, ocular complications, and laboratory parameters were analyzed. Univariate and multivariable logistic regression analyses assessed associations across disease manifestations. Machine learning models were also utilized to predict the risk of retinal/eye involvement in AAV patients. RESULTS: Among 533 patients (210 GPA, 217 MPA, 99 EGPA, and 7 unclassified AAV), ocular complications were observed in 20.64% of them, with a distribution of 36.67% in GPA, 7.37% in MPA, and 18.18% in EGPA. The most common ocular manifestations included scleritis and retro-orbital mass/dacryocystitis, which were notably prevalent in GPA patients. Retinal involvement was observed in 9.09% of EGPA cases. The machine learning models yielded that eosinophil percentage (EOS%), high-sensitivity C-reactive protein (hsCRP), and CD4 + T cell/CD8 + T cell ratio (T4/T8) can predict retinal involvement. Furthermore, the white blood cell, EOS%, APTT, IgA, hsCRP, PR3-ANCA, and T4/T8 can predict eye involvement. CONCLUSION: Ocular manifestations are a prevalent complication across all forms of AAV. Predictive models developed through machine learning offer promising tools for early intervention and tailored patient care. This necessitates a multidisciplinary approach, integrating rheumatology and ophthalmology expertise for optimal patient outcomes.

Unmasking an unusual presentation of Hodgkin's lymphoma masquerading as ocular inflammation: a case report.

BACKGROUND: Hodgkin's lymphoma (HL) is an extremely rare cause of ocular inflammation that is usually not considered in the typical workup of uveitis and other eye diseases. A few cases of ocular inflammation were reported previously showcasing HL with absence of typical symptoms of HL at presentation. Acknowledging the potential ocular inflammation associated with HL can prompt ophthalmologists to broaden their diagnostic approach and collaborate with internal medicine departments to investigate this rare yet significant etiology. CASE PRESENTATION: A 17-year-old Caucasian woman presenting unilateral panuveitis was later diagnosed with HL. The ocular findings were non-necrotizing scleritis, anterior uveitis, vitritis, white/yellowish chorioretinal lesions, papillitis and vasculitis. A left supra-clavicular lymph node biopsy confirmed the diagnosis of nodular sclerosing Hodgkin's lymphoma stage IIB. Other causes of uveitis were excluded. Chemotherapy led to remission of the disease and the ocular lesions became quiescent with persistent pigmented chorioretinal scars. CONCLUSIONS: Hodgkin's lymphoma should be considered in the differential diagnosis of diseases that can occasionally be revealed by unilateral ocular inflammation. A comprehensive, multidisciplinary approach is key to properly assessing such cases.

Ocular and orbital manifestations in VEXAS syndrome.

BACKGROUND: VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) is a hematoinflammatory disease that typically affects adults. It results from a somatic mutation of the E1 ubiquitin conjugating enzyme encoded by the UBA1 gene. VEXAS is frequently accompanied by myelodysplastic syndrome (MDS). The purpose of this study is to describe the ocular and orbital manifestations of VEXAS patients in a case series in our medical centre. METHODS: A retrospective chart review was performed for all patients who were diagnosed with VEXAS syndrome in a tertiary medical centre over two years. RESULTS: Eight patients were identified with VEXAS. In six patients, the diagnosis was confirmed by genomic sequencing. Two patients were identified based on their phenotype. All patients were males. The mean age at diagnosis was 78.7 years. In two patients, the ocular manifestation was the presenting symptom for VEXAS. Seven patients (87.5%) had history of MDS. Systemic inflammation manifestations include: skin rash (n = 5), recurrent fevers (n = 2), relapsing polychondritis (n = 2), pleuritis and pleural effusion (n = 2), poly arteritis nodosa- PAN (n = 1) and thrombophlebitis (n = 1). Seven (87%) patients were presented with periorbital oedema. Three patients showed orbital inflammation. Dacryoadenitis was observed in two patients, and extraocular muscle (EOM) myositis was detected in two patients. Four patients demonstrated ocular inflammation such as: episcleritis, scleritis and anterior uveitis. CONCLUSION: ocular manifestations in VEXAS include orbital inflammation, dacryoadenitis, myositis, uveitis, scleritis, episcleritis and periorbital oedema. We recommend that in old male patients, with history of haematological disorder, presenting with ocular symptom, VEXAS investigation should be taken into consideration.

Surgically Induced Scleritis Associated With Suture Hypersensitivity Following Strabismus Surgery.

A 67-year-old woman with history of mild suture hyper-sensitivity presented with localized scleritis after strabismus surgery. After infection was ruled out, the patient was prescribed topical and systemic non-steroidal anti-inflammatory drugs and systemic steroids, which led to full clinical resolution. [J Pediatr Ophthalmol Strabismus. 2024;61(1):e4-e6.].

Posterior scleritis following COVID-19 vaccination or infection simulating uveal melanoma in 8 consecutive patients.

OBJECTIVES: To determine clinical features and outcomes of posterior scleritis masquerading as uveal melanoma following vaccination against COVID-19 and/or COVID-19 infection. SUBJECTS/METHODS: All patients with posterior scleritis referred to our service to rule out intraocular tumour between February 2021 and June 2022, who previously had COVID-19 vaccination and/or infection (n = 8). A retrospective detailed review of patient charts and imaging was carried out. RESULTS: Previous COVID-19 vaccination was documented in 6 patients (75%) and previous COVID-19 infection and vaccination in 2 patients (25%). Demographic features included mean age of 59 years (median 68, range 5-86 years), white race (n = 7, 87%), and male sex (n = 5, 63%). Mean visual acuity at presentation was 0.24 LogMAR (median 0.18, range 0.0-0.70). The main presenting symptom was blurred vision with pain (n = 5, 63%). Features that suggested scleritis and not uveal melanoma included pain (n = 6, 75%), anterior scleritis (n = 3, 38%), disc oedema (n = 1, 13%), choroidal detachment (n = 3, 38%), choroidal folds (n = 3, 38%), diffusely thickened scleral wall on ultrasonography (n = 2, 25%), Tenon's oedema (n = 5, 63%), and scleral nodule with medium/high internal reflectivity on ultrasonography (n = 4, 50%). Follow-up information at mean of 2 months (range 0.25-7 months) revealed visual acuity at date last seen was mean 0.30 LogMAR (median 0.29, range 0.0-0.54). By 2 months, resolution of "tumour" was noted in 5/6 (83%) patients with follow-up. CONCLUSIONS: Posterior scleritis following COVID-19 vaccination and/or infection can masquerade as choroidal melanoma. At 2 months duration, partial or complete resolution of features with minimal visual consequence was noted.

Bilateral Scleritis with Heterogeneous Etiologies: A Diagnostic Dilemma.

PURPOSE: To report a patient with bilateral scleritis who was initially treated for infectious scleritis, and subsequently diagnosed as granulomatosis with polyangiitis (GPA). METHOD: Retrospective chart review. RESULT: A 48-year-old female with a known history of diabetes presented with pain, redness, and blurring of vision in the right eye. She was diagnosed as scleritis with retinal detachment and underwent vitrectomy, with silicone oil tamponade and intravitreal injections of antibiotics, and antifungal agents. She presented with active scleritis with additional multiple pus points. Several scleral biopsies failed to yield any microorganism and finally, the painful blind eye was enucleated. The enucleated specimen grew gram-positive bacteria which were identified as Staphylococcus arlettae by polymerase chain reaction (PCR)-based sequencing of the 16S rRNA gene. Nine months after the onset of symptoms in the right eye, the patient developed necrotizing scleritis in the left eye. Laboratory investigation revealed a positive cytoplasmic- anti-neutrophil cytoplasmic autoantibody, which was previously negative during the right eye involvement. She was diagnosed with GPA by a rheumatologist and treated accordingly. CONCLUSION: Retinal detachment may be associated with scleritis, and ANCA testing may not detect GPA in its early stages. Once infection has been excluded, clinicians should not be afraid to use high-dose immunosuppression instead of surgery to treat retinal detachment associated with scleritis.

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.

Acute-onset surgically induced necrotizing scleritis after strabismus surgery.

Surgically induced necrotizing scleritis (SINS) is an uncommon but devastating complication that may occur days to years after ocular surgery. We report the case of a 32-year-old man who underwent uncomplicated strabismus surgery for large-angle exotropia and developed SINS characterized by painless scleral inflammation, choroidal exposure, and globe ectasia within days of surgery. Work-up revealed no associated infectious process or underlying systemic inflammatory condition. Clinical resolution occurred with oral immunosuppression alone, without need for graft.

Panscleritis After Blunt Ocular Trauma in A Child with Epididymitis.

We reported an 8-year-old boy with panscleritis in left eye and right epididymitis after falling on the ground. Etiologic diagnosis played a key role in this case. Systemic examinations ruled out systemic autoimmune diseases, tumors, and infections as the cause of scleritis and suggested that the disease was caused by a local delayed-type hypersensitivity (DTH) induced by ocular trauma and was non-infectious. Still, the right epididymitis was infectious. Both conditions were treated successfully using steroids and antibiotics, respectively. Thus, early etiologic diagnosis and reasonable treatment are crucial to prevent visual loss.

Necrotizing Scleritis and Rheumatoid Arthritis: A Clinical Case Report Supported by A Brief Review with Risk of Bias Analysis.

INTRODUCTION: Necrotizing scleritis (NS) presents 30%-40% as having a systemic autoimmune condition. OBJECTIVE: To present a clinical case report and a systematic review of necrotizing scleritis with ocular manifestation as the first sign of rheumatologic disease. METHODS: The present study was elaborated according to the rules of CARE. CASE REPORT: A female patient, 63 years old, a white, administrative assistant, presented irritation, low visual acuity (LVA) in the left eye (LE), and headache. Biomicroscopy (BIO) in the right eye (RE) was normal, and the LE showed hyperemia and scleral thinning. After 1 month, the patient returns without signs of infectious diseases in the exams, and after a rheumatological evaluation with a diagnosis of rheumatoid arthritis, methotrexate and prednisone are prescribed. After 2 months, she relapsed and started treatment with anti-TNF, with remission after the 4th dose. After 1 year, she evolved with LVA in LE. RESULTS: A total of 244 articles were found, 104 articles were evaluated and 10 were included in the brief review. The symmetrical Funnel Plot does not suggest a risk of bias. CONCLUSION: Both in the present case report and the literary findings, it was evidenced that the ophthalmologic findings may precede the systemic changes of the disease for the early diagnosis of rheumatoid arthritis.

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.

Clinical profile and management of granulomatosis with polyangiitis-associated scleritis from a tertiary care hospital in South India.

Purpose: To describe the clinical features and management of patients with scleritis associated with granulomatosis with polyangiitis (GPA) at a tertiary eye care center in South India. Methods: The clinical profile and management of patients presenting to a tertiary eye care center in South India with scleritis secondary to GPA from 2003 to 2021 were analyzed retrospectively. Scleritis was classified into anterior diffuse, nodular, and necrotizing scleritis with inflammation according to Watson and Hayreh's classification. Demographic characteristics, clinical features, anti-neutrophil cytoplasmic antibody (ANCA) positivity, treatment response, ocular complications, and status at the last follow-up were analyzed. Statistical analysis of data was performed using Microsoft Excel 2019. Results: Nineteen eyes of 17 patients (15 cytoplasmic staining ANCA [c-ANCA], two p-ANCA positive) were included. Fifteen eyes had necrotizing scleritis, two had diffuse anterior scleritis, and two had nodular scleritis. Remission was induced using a combination of steroids and cyclophosphamide or rituximab. Maintenance therapy was instituted using tapering steroids and immunosuppressants like cyclophosphamide, mycophenolate mofetil, methotrexate, or rituximab. Three eyes required a scleral patch graft. Fourteen patients had good anatomical and visual outcomes, and three were lost to follow-up. Conclusion: GPA is a rare disease, while it is the most common ANCA-associated vasculitis with scleritis. As scleritis may be the presenting sign of the disease, ophthalmologists must be aware of the various features suggestive of GPA. GPA-associated scleritis can have a good prognosis when diagnosed promptly and managed aggressively in the acute stage, and remission is maintained with adequate systemic immunosuppression.

Ocular manifestations of ANCA-associated vasculitis.

OBJECTIVES: ANCA-associated vasculitis (AAV) is a group of multisystem diseases that can have several ocular manifestations. There are published data on ocular manifestations of granulomatosis with polyangiitis (GPA), but few for eosinophilic granulomatosis with polyangiitis (EGPA) or microscopic polyangiitis (MPA). There is little information concerning chronicity, complications, and association with other cranial manifestations of AAV. METHODS: This study retrospectively analysed longitudinal multicentre cohorts of individuals with AAV followed between 2006 and 2022. Data included diagnosis, demographics, cranial manifestations of disease, presence of manifestations at onset of disease and/or follow-up, and ocular complications of disease. Univariate and multivariable logistic regression analysis assessed associations across disease manifestations. RESULTS: Data from 1441 patients were analysed, including 395 with EGPA, 876 with GPA, and 170 with MPA. Ocular manifestations were seen within 23.1% of patients: 39 (9.9%) with EGPA, 287 (32.7%) with GPA, and 12 (7.1%) with MPA at any time in the disease course. There were more ocular manifestations at onset (n = 224) than during follow-up (n = 120). The most common disease-related manifestations were conjunctivitis/episcleritis and scleritis. In multivariable analysis, dacryocystitis, lacrimal duct obstruction, and retro-orbital disease were associated with sinonasal manifestations of GPA; ocular manifestations were associated with hearing loss in MPA. The most common ocular complications and/or damage seen were cataracts (n = 168) and visual impairment (n = 195). CONCLUSION: Ocular manifestations occur in all forms of AAV, especially in GPA. Clinicians should be mindful of the wide spectrum of ocular disease in AAV, caused by active vasculitis, disease-associated damage, and toxicities of therapy.

Presumed tuberculosis-related scleritis.

AIMS: To evaluate the clinical characteristics and therapeutic outcome of patients with recurrent scleritis of unknown demonstrable aetiology and positive QuantiFERON-TB Gold In-Tube test (QFT). METHODS: Retrospective chart review of the demographic, clinical, laboratory and therapeutic outcome data of 15 patients. Clinical characteristics as well as remission rate after standard antituberculous therapy (ATT) were assessed. RESULTS: There were 9 men and 6 women with a mean age of 48.9 years (range, 32-73). Scleritis was diffuse in 10 patients (66.6%) and nodular in 5 patients (33.3%), 1 of them with concomitant posterior scleritis. It was bilateral in 7 patients (46.6%) and recurrent in all of them. Scleritis appeared after prior uveitis (10 patients, 66.6%) and/or with concomitant uveitis (5 patients, 33.3%) or peripheral keratitis (5 patients, 33.3%). Previous ocular surgery was found in 7 patients (46.6%). Previous extraocular tuberculosis (TB) infection or previous TB contact was detected in 11 patients (73.3%). No radiologic findings of active extraocular TB were detected. ATT was used in 15 patients, sometimes with the addition of systemic corticosteroids (5 patients) and methotrexate (1 patient); 14 patients achieved complete remission (93.3%). CONCLUSION: Presumed TB-related scleritis may appear in recurrent scleritis of unknown origin and positive QFT. It may occur after prior uveitis and/or concomitantly with uveitis or peripheral keratitis, and it may be triggered by previous ocular surgery. No patients had evidence of concurrent active extraocular infection, although many had previous TB infection or TB contact. ATT was effective, sometimes with the addition of systemic corticosteroids and methotrexate.

Ocular surface squamous neoplasia in a patient following oral steroids for contralateral necrotising scleritis.

A male patient in his 70s presented with left eye necrotising scleritis. His ocular history was significant for pterygium excision with mitomycin C 3 months prior in the affected eye, open-angle glaucoma, nuclear sclerosis cataract and previous sixth cranial nerve palsy. Scleral culture was negative and blood work was positive for rheumatoid factor and HLA-B27. The patient was treated for necrotising scleritis with oral doxycycline, vitamin C, ranitidine and prednisone with gradual taper over 45 days. Two months after initiating treatment, his necrotising scleritis achieved complete resolution; however, a new-onset ocular surface squamous neoplasia (OSSN) was identified at the temporal limbus of the contralateral eye. He was treated with topical interferon alpha-2b 1 million IU qid and achieved complete resolution after 4 months. The case highlights both the acute precipitation of OSSN following oral steroids and a sight-threatening complication following pterygium excision.

Peripheral ulcerative keratitis, nodular episcleritis, and pulmonary nodules as the initial signs of rheumatic arthritis: A Case Report.

Background: Rheumatoid vasculitis (RV) is a rare but potentially devastating complication of rheumatoid arthritis (RA). It typically occurs in patients with extra-articular manifestations. Here we reported a case of PUK with nodular episcleritis and pulmonary nodules that occurred in the same patient without joint involvement. Case presentation: A 43-year-old Chinese woman, exhibited a partial crescent-shaped marginal corneal ulcer in the right eye at admission and the ulcer developed rapidly into nearly 360-degree ulcers in both eyes within one week. Nodular episcleritis was observed in the right eye. Conjunctival biopsy revealed vasculitis. Her rheumatoid factor (RF) and anti-cyclic citrullinated protein antibody were positive, while anti-neutrophilic cytoplasmic antibody (c-ANCA) and anti-protease 3 were negative. Pulmonary nodules were found, without joint involvement. The ocular condition did not relieve under the topical and systemic use of corticosteroids, or under other immunosuppressive agents until the infliximab therapy. PUK recurrence was observed after the discontinuation of infliximab. Conclusions: Rapidly deteriorated PUK with nodular episcleritis and pulmonary nodules occurred in the same patient is a special case of RA without joint involvement. This case reinforces the concept that RV may be the initial sign of RA. Infliximab can be used to prevent further progress of RA-related PUK in some refractory cases.