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.

A Case of Refractory Posterior Scleritis with Marked Retinochoroidal Detachment Associated with Panuveitis.

An 84-year-old man presented with decreased right-eye visual acuity. Upon initial examination, the rightand left-eye visual acuities were 0.03 and 1.2, respectively; moreover, the right- and left-eye intraocular pressure was 12 mmHg and 13 mmHg, respectively. Examination revealed a shallow anterior chamber of the right eye, anterior chamber inflammation, vitreous opacity, and marked retinochoroidal detachment. Optical coherence tomography (OCT) revealed retinal detachment (RD) and choroidal folds; moreover, B-scan ultrasonography (B-scan) showed RD as well as thickened sclera with fluid in Tenon's space. Fluorescent fundus angiography revealed hyperfluorescence in the optic disc and vascular hyperpermeability in the right eye. The left eye lacked extra-ocular symptoms or abnormalities. The right ocular axis measured 23.4 mm with no apparent subretinal fluid migration due to positional changes. Accordingly, the patient was diagnosed with panuveitis associated with posterior scleritis and immediately started on 40 mg prednisolone, which improved his symptoms. However, at 3 post-treatment months, choroidal folds were observed and was restarted on 20 mg prednisolone. The choroidal folds subsequently disappeared, with a current visual acuity of 0.3 in the right eye and no recurrence. Our findings indicated the utility of accurate diagnosis of posterior scleritis by B-scan and prompt systemic steroid administration.

Case of isolated scleromalacia perforans with seropositivity for rheumatoid arthritis.

Scleromalacia perforans, or necrotising anterior scleritis, is a rare and severe form of eye disease that usually occurs in patients suffering from long-standing systemic inflammatory diseases, with rheumatoid arthritis (RA) being the most common. Here, we report the case of a patient who presented with redness of the eye and discolouration of the sclera and was diagnosed with scleromalacia perforans without any further extraophthalmic systemic involvement. Serological workup revealed highly positive cyclic citrullinated peptide (CCP) antibody (CCP-IgG/anticitrullinated protein antibodies) and positive rheumatoid factor, serologies commonly associated with RA. The patient's symptoms responded very well to rituximab therapy.

Syphilitic scleritis and episcleritis: A review.

Scleritis and episcleritis are rare, but potentially sight-threatening forms of syphilis. To provide a full description of this neglected subset of ocular syphilis, we evaluated the English literature for reports of syphilitic scleritis and episcleritis, recording the demographics, clinical characteristics, serological data, management practices, treatment responses, and visual outcomes. Previously published descriptions of 44 patients with syphilitic scleritis (50 eyes) and 9 patients with syphilitic episcleritis (14 eyes) were identified. The predominant type of scleritis was anterior scleritis, accounting for 92.9% of cases, with nodular anterior scleritis being the most frequent subtype at 58.1%. Almost one-quarter of patients were co-infected with human immunodeficiency virus (HIV). Initial misdiagnosis was common and led to delays in initiating treatment with appropriate antibiotics. Visual outcomes were often good in both scleritis and episcleritis, irrespective of HIV infection status, although complications including scleral thinning, keratitis, and uveitis, along with permanent visual loss and an association with neurosyphilis, were reported. Response to antibiotic treatment was typically rapid, often within 1 week. With the rising global incidence of syphilis, testing patients with scleritis or episcleritis for this infectious disease is important to ensure prompt diagnosis and treatment for best ocular and systemic outcomes.

Subconjunctival Dirofilaria masquerading as nodular scleritis.

A man in his late 50s presented with a gradually enlarging, painless, reddish mass on the white portion of his left eye for 2 weeks. His best-corrected visual acuity was 20/20 in both eyes. Slit-lamp examination showed a congested, nodular, elevated lesion on the temporal bulbar conjunctiva with two pustule-like elevations. Anterior segment optical coherence tomography showed a subconjunctival solid mass rather than an abscess or a cyst. Scleral deroofing was performed and a long thread-like object resembling a dead worm was identified. The worm was removed intact, and its histopathology confirmed the diagnosis of Dirofilaria Peripheral blood smear did not show any microfilariae. No recurrences or new lesions were observed during the follow-up examinations at 1 and 5 months post-surgery. This case highlights the importance of considering a parasitic aetiology in cases of nodular or infectious scleritis.

[Acute red eye]. / Een acuut rood oog, en dan?

The general physician as well as the medical specialist may be confronted with a patient with one or two red eyes. To be adequately equipped we answer a series of questions on diagnosis, treatment and referral of patients with red eyes after trauma, or with conjunctivitis, keratitis, scleritis, uveïtis, endophthalmitis or acute glaucoma. Refer to an ophthalmologist or not?

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.

Quantitative and comparative analysis of thermography parameters in posterior scleritis, choroiditis, and controls.

PURPOSE: To compare various ocular thermography parameters in posterior scleritis (PS), choroiditis (choroidal granuloma [CG], Vogt-Koyanagi-Harada [VKH] syndrome), central serous chorioretinopathy (CSCR), and healthy controls. METHODS: This retrospective, observational, comparative study evaluated cases undergoing ocular thermography between April 2017 and October 2023. The study groups included cases of PS, CG, and VKH, while the control group comprised CSCR cases and healthy individuals. Various thermography parameters were assessed, which were as follows: Ocular surface temperature (OST), central corneal temperature (CCT), average scleral temperature (ST), nasal scleral temperature (nST), temperature difference between both the eyes (∆t), and difference between scleral and corneal temperatures (ST - CCT, nST - CCT). RESULTS: It was found that ∆t was significantly higher in the PS group compared to the CG ( P = 0.005), CSCR ( P = 0.0001), and control groups (dilated control: P =0.006, undilated control: P = 0.0001). ST - CCT and nST - CCT were significantly higher in the undilated control and CSCR groups and significantly lower in the PS group. ST - CCT and nST - CCT showed less difference in the affected eyes compared to contralateral healthy eyes of PS and CG cases. OST, CCT, ST, and nST displayed statistically insignificant differences across all groups. CONCLUSION: It is advisable to focus on temperature differences between the affected and healthy eyes, or the difference between the central corneal and scleral temperature of the affected eye, utilizing parameters such as ∆t, ST - CCT, and nST - CCT. These composite parameters offer a more effective approach than individual measurements like OST, CCT, ST, and nST. Thermography can serve as a screening tool to suspect and differentiate PS.

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.

Orbital/ocular inflammatory involvement in VEXAS syndrome: Data from the international AIDA network VEXAS registry.

VEXAS syndrome is a recently described monogenic autoinflammatory disease capable of manifesting itself with a wide array of organs and tissues involvement. Orbital/ocular inflammatory manifestations are frequently described in VEXAS patients. The objective of this study is to further describe orbital/ocular conditions in VEXAS syndrome while investigating potential associations with other disease manifestations. In the present study, twenty-seven out of 59 (45.8 %) VEXAS patients showed an inflammatory orbital/ocular involvement during their clinical history. The most frequent orbital/ocular affections were represented by periorbital edema in 8 (13.6 %) cases, episcleritis in 5 (8.5 %) patients, scleritis in 5 (8.5 %) cases, uveitis in 4 (6.8 %) cases, conjunctivitis in 4 (6.8 %) cases, blepharitis in 3 (5.1 %) cases, orbital myositis in 2 (3.4 %) cases. A diagnosis of systemic immune-mediated disease was observed in 15 (55.6 %) cases, with relapsing polychondritis diagnosed in 12 patients. A significant association was observed between relapsing polychondritis and orbital/ocular involvement in VEXAS syndrome (Relative Risk: 2.37, 95 % C.I. 1.03-5.46, p = 0.048). Six deaths were observed in the whole cohort of patients after a median disease duration of 1.2 (IQR=5.35) years, 5 (83.3 %) of which showed orbital/ocular inflammatory involvement. In conclusion, this study confirms that orbital/ocular inflammatory involvement is a common finding in VEXAS patients, especially when relapsing polychondritis is diagnosed. This makes ophthalmologists a key figure in the diagnostic process of VEXAS syndrome. The high frequency of deaths observed in this study seems to suggest that patients with orbital/ocular involvement may require increased attention and more careful follow-up.

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.].

Embedded Episcleral Foreign Body Mimicking Nodular Anterior Scleritis.

A 56-year-old man was referred to our clinic for unilateral nodular scleritis unresponsive to systemic corticosteroids. A localized, nodular hyperemia on the nasal bulbar conjunctiva surrounding a central cyst-like lesion together with vascular engorgement was observed on slit-lamp examination of the left eye. No abnormal fundoscopic findings were noted. Surgical exploration revealed an embedded episcleral brown colored, soft to touch, splinter-like organic foreign body (FB) which was confirmed by the histopathological examination. Nodular hyperemia resolved during the postoperative follow-up period, and mild scar tissue accompanied by scleral thinning developed in the left nasal bulbar conjunctiva. Ocular injury associated with FBs may cause significant ocular morbidity depending on the nature and location of the FB. Severe visual disability may occur if left untreated. Subconjunctival FBs are rare and may present with a clinical picture mimicking episcleritis or scleritis. History of trauma involving a FB should always be assessed for an accurate differential diagnosis and appropriate management of patients with anterior scleritis.

Scleritis following intravitreal brolucizumab injection: a case series.

BACKGROUND: This study reports the first cases of scleritis following intravitreal brolucizumab (IVBr) injection for nAMD, emphasizing the need to be aware of the possibility of scleritis following IVBr injections. CASE PRESENTATION: Case 1. A 74-year-old Japanese man with nAMD complained of conjunctivitis and decreased vision in the right eye 8 days after his eighth IVBr injection. Examination revealed scleritis without anterior inflammation. Topical 0.1% betamethasone and 0.3% gatifloxacin eye drops were started. The scleritis worsened in the following 2 weeks and became painful. He underwent sub-Tenon's capsule triamcinolone acetonide (STTA) injection. Two days later, he returned with a complaint of severe vision loss. Fundus examination revealed retinal artery occlusion, vasculitis, and vitreous opacity in the right eye. Vitreous surgery was performed. CASE 2: An 85-year-old Japanese woman with nAMD in the right eye complained of reddening of the eye 27 days after her fifth IVBr injection. Examination showed conjunctivitis and scleritis without anterior inflammation in the right eye. She was started on 0.1% fluorometholone and 0.5% levofloxacin hydrate eye drops. The scleritis worsened in the following 3 weeks. Her treatment was switched to 0.1% betamethasone eye drops. One month later, the scleritis had improved and a sixth IVBr injection was administered. There was no worsening of the scleritis at that time. However, 1 month after a seventh IVBr injection, she complained of severe hyperemia and decreased vision. Fundus examination revealed vitreous opacification. She underwent STTA, and the vitreous opacity improved in 24 days. Case 3. A 57-year-old Japanese man with nAMD complained of pain and decreased vision in the right eye 21 days after a fourth IVBr injection. Examination revealed scleritis with high intraocular pressure but no anterior chamber or fundus inflammation. STTA and topical eye drops were performed. One month later, scleritis improved but visual acuity didn't due to progression of nAMD. CONCLUSIONS: Intraocular inflammation following IVBr injection may progress to the posterior segment. Scleritis can occur after IVBr injection, and topical eye drops alone may not be sufficient for initial treatment. Clinicians should consider the possibility of scleritis in patients with worsening inflammation after IVBr injection.

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.

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.