Orbital Inflammatory Disease Research Articles

Atypical presentation of sphenoid wing meningioma mimicking idiopathic orbital inflammation

Sphenoid wing meningiomas (SWMs) are slow-growing tumors that commonly present with proptosis and progressive visual deterioration. Due to the proximity of the sphenoid bone to key structures in the orbit, it may occasionally be misdiagnosed as other inflammatory and other neoplastic orbital diseases. This case report highlights an atypical presentation of SWM that mimics idiopathic inflammatory orbital disease, underscoring the importance of differential diagnosis.

Differentiation of bacterial orbital cellulitis and diffuse non-specific orbital inflammation on magnetic resonance imaging.

To examine the radiological differences between bacterial orbital cellulitis (OC) and diffuse non-specific orbital inflammation (DNSOI) on magnetic resonance imaging (MRI). Retrospective study of patients with OC and DNSOI with an MRI orbital scan. Localised orbital inflammation (e.g., idiopathic dacryoadenitis and myositis), quiescent orbital inflammation and pre-septal cellulitis were excluded. Thirty-two patients presenting between 2008 and 2023, including twenty-one OC patients (mean age: 42.5 ± 24.9 years old, male: 6), and eleven DNSOI patients (mean age: 52.3 ± 17.8 years old, male: 16). Both OC and DNSOI demonstrate orbital fat contrast-enhancement. However, whilst OC demonstrated a hyperintense T2 signal (P < 0.001), variable signal was observed in DNSOI, with a hypointense T2 signal more suggestive of DNSOI (P = 0.012). When the lacrimal glands were involved, indistinct margins were more likely in OC (P < 0.001), whilst gross enlargement and contrast-enhancement was observed in DNSOI (P = 0.032 and 0.017, respectively). Peripheral contrast-enhancement of the extraocular muscle (EOM) (P = 0.002) was more common in OC, whilst DNSOI demonstrated variable contrast-enhancement throughout the affected EOM (P < 0.001). The presence of contralateral abnormalities, such as lacrimal gland enlargement and EOM involvement, are more suggestive of DNSOI. Several MRI features, beyond overt sinogenic disease, may help to differentiate OC from DNSOI, including the orbital fat signal intensity, EOM and/or lacrimal gland involvement, and contralateral orbital abnormalities. However, these features may not be specific, and thus highlights the ongoing radiological dilemma clinicians are faced when tasked with differentiating between infectious and non-infectious orbital inflammatory disease.

Non-infectious hypertrophic pachymeningitis associated with orbital inflammatory disease: a pooled analysis.

To describe four cases of non-infectious hypertrophic pachymeningitis (HP)-associated with orbital inflammatory disease (OID). This study summarises the clinico-radiological features, outcomes, and management of HP-associated OID. Retrospective case-series of patients with radiological evidence of HP and OID. Comprehensive literature review of all published English-language non-infectious causes of HP-associated OID. Reference lists were screened for inclusion of relevant articles. Thirty-seven cases of HP-associated OID (Mean age: 49.2 ± 17.4 years old; Male: 15) were identified, including four cases from our institution. Aetiologies included ANCA-associated vasculitis (12/37), non-specific/idiopathic (11/37), IgG4/multifocal fibrosclerosis (11/37), neurosarcoidosis (1/37), inflammatory myofibroblastic tumour (1/37), and giant cell arteritis (1/37). Orbital pain, headache, visual deterioration, and cranial nerve palsies were common clinical presentations. Both "focal" and "diffuse" HP were observed, with the most common sign of orbital involvement being an inflammatory orbital mass, typically with orbital apex involvement. Orbital myositis and dacryoadenitis were less common. The cavernous sinus was the most common site of extra-orbital inflammation. There was no single differentiating specific radiological feature between non-specific and specific forms of HP-associated OID. The clinico-radiological manifestations of HP-associated OID differ from those described in isolated HP or OID. There is no single specific radiological marker differentiating non-specific/idiopathic disease from secondary causes; however, the co-existence of HP in OID should prompt suspicion of an underlying cause. The disease may be refractory or resistant to initial treatment, although guidelines surrounding its management and the long-term prognosis remain to be determined.

Treatment Outcomes of Patients with Orbital Inflammatory Diseases: Should Steroids Still Be the First Choice?

Objective: To clarify the therapy response in orbital inflammatory diseases (OID), we analyzed the treatment effects of steroid therapy, the use of disease-modifying antirheumatic drugs (DMARDS), and biologicals in our tertiary referral center cohort. Methods: We collected the clinical and demographic data of all patients treated for non-specific orbital inflammation (NSOI) (n = 111) and IgG4-ROD (n = 13), respectively at our center from 2008 to 2020 and analyzed them with descriptive statistics. NSOI were sub-grouped according to the location into either idiopathic dacryoadenitis (DAs) (n = 78) or typical idiopathic orbital myositis (n = 32). Results: Mean age at first clinical manifestation was significantly different between subgroups (IOI: 49.5 ± 18, IgG4-ROD: 63.2 ± 14, p = 0.0171). Among all examined OID, 63 patients (50%) achieved full remission (FR) with corticosteroids (NSOI 53%/IgG4-ROD 31%). In contrast, classic myositis showed a significantly higher response (76%). Disease-modifying drugs (DMARDS) for myositis accomplished only 33% FR (NSOI 57%) and 66% did not respond sufficiently (NSOI 43%). The biologic agent (Rituximab) was significantly more efficient: 19 of 23 patients (82%) achieved full remission and only 4 (17%) did not respond fully and needed orbital irradiation or orbital decompressive surgery.

The UK clinical eye research strategy: refreshing research priorities for clinical eye research in the UK

ObjectivesTo validate and update the 2013 James Lind Alliance (JLA) Sight Loss and Vision Priority Setting Partnership (PSP)’s research priorities for Ophthalmology, as part of the UK Clinical Eye Research Strategy.MethodsTwelve ophthalmology research themes were identified from the JLA report. They were allocated to five Clinical Study Groups of diverse stakeholders who reviewed the top 10 research priorities for each theme. Using an online survey (April 2021-February 2023), respondents were invited to complete one or more of nine subspecialty surveys. Respondents indicated which of the research questions they considered important and subsequently ranked them.ResultsIn total, 2240 people responded to the survey (mean age, 59.3 years), from across the UK. 68.1% were female. 68.2% were patients, 22.3% healthcare professionals or vision researchers, 7.1% carers, and 2.1% were charity support workers. Highest ranked questions by subspecialty: Cataract (prevention), Cornea (improving microbial keratitis treatment), Optometric (impact of integration of ophthalmic primary and secondary care via community optometric care pathways), Refractive (factors influencing development and/or progression of refractive error), Childhood onset (improving early detection of visual disorders), Glaucoma (effective and improved treatments), Neuro-ophthalmology (improvements in prevention, diagnosis and treatment of neurodegeneration affecting vision), Retina (improving prevention, diagnosis and treatment of dry age-related macular degeneration), Uveitis (effective treatments for ocular and orbital inflammatory diseases).ConclusionsA decade after the initial PSP, the results refocus the most important research questions for each subspecialty, and prime targeted research proposals within Ophthalmology, a chronically underfunded specialty given the substantial burden of disability caused by eye disease.

Asymmetric proptosis in thyroid eye disease

PurposePatients with thyroid eye disease (TED) can present with asymmetric disease. The purpose of this study was to evaluate the prevalence of asymmetric TED in an Australian cohort and investigate clinical, biochemical, and radiological associations of asymmetric TED.MethodsThis was a retrospective study of patients with TED who underwent Hertel exophthalmometry and orbital computed tomography (CT) scans. Asymmetry was defined as a difference in the globe protrusion of ≥ 3 mm using Hertel exophthalmometry. Data was collected on patient demographics, clinical disease presentation, thyroid function tests and antibody levels. Muscles volumes were determined by manually segmenting the extraocular muscles on CT scans using a commercially available software.Results172 orbits from 86 patients were included in the study. 28 (33%) patients had asymmetric TED. No significant differences were observed in age, gender, thyroid hormone status, anti-thyroid peroxidase levels, thyroid stimulating hormone receptor antibodies, disease activity status, or dysthyroid optic neuropathy between the asymmetric and non-asymmetric groups. The extraocular muscle volumes were significantly higher in the asymmetric orbit compared to its contralateral orbit.ConclusionAsymmetric TED is a reasonably common occurrence in the course of TED. It is associated with higher extraocular muscle volumes, suggesting muscle enlargement as one of the underlying contributors to asymmetric proptosis. Thyroid eye disease should be considered in the differential of asymmetric orbital inflammatory disease.

Ulcerated conjunctival mass as an initial presentation of idiopathic orbital inflammatory disease: a case report

Ulcerated conjunctival mass as an initial presentation of idiopathic orbital inflammatory disease: a case report

MANAGEMENT OF NON-SPECIFIC ORBITAL INFLAMMATION USING HARIDRADI BIDALAKA- A CASE REPORT.

Idiopathic orbital inflammatory disease (IOID), also called non-specific orbital inflammation (NSOI) or orbital pseudotumor, is an uncommon disorder characterized by nonneoplastic, non- infective, space-occupying orbital infiltration with inflammatory features. The process may preferentially involve any or all of the orbital soft tissues. Histopathological analysis reveals pleomorphic inflammatory cellular infiltration followed by reactive fibrosis. Unilateral disease is typical in adults, although in children bilateral involvement may occur. Intracranial extension is rare. Simultaneous orbital and sinus involvement is also rare and may be a distinct entity [1]. According to Ayurveda, this inflammatory condition on the eye lids can be correlated with Kaphaja Netra Abhishyanda and treated accordingly. Kaphaja Netra Abhishyanda is defined in Ayurveda as a disease affecting all parts of the eye characterised by Guruta (heaviness of lids), Kandu (itching), Pichila (repeated lacrimation), Alpa Vedana (Foreign body sensation) photophobia and burning sensation in eyes etc. An ocular therapeutic called Bidalaka is the application of medicated paste on the eye's outer surface expect eye lashes. Bidalaka is indicated in inflammatory conditions of the eyes. A 72-year-old female patient diagnosed with NSOI on clinical presentation was advised Haridradi Bidalaka for five days. The signs &amp; symptoms were reduced to a very mild degree. The result proved to be effective based on clinical assessment.

Exploring gut microbiota and metabolite alterations in patients with thyroid-associated ophthalmopathy using high-throughput sequencing and untargeted metabolomics.

Thyroid-associated ophthalmopathy (TAO) is an autoimmune-driven orbital inflammatory disease. Despite research efforts, its exact pathogenesis remains unclear. This study aimed to characterize the intestinal flora and metabolic changes in patients with TAO to identify the flora and metabolites associated with disease development. Thirty patients with TAO and 29 healthy controls were included in the study. The intestinal flora and metabolites were analyzed using high-throughput sequencing of the 16S rRNA gene and non-targeted metabolomics technology, respectively. Fresh fecal samples were collected from both populations for analysis. Reduced gut richness and diversity were observed in patients with TAO. Compared to healthy controls, significant differences in relative abundance were observed in patients with TAO at the order level Clostridiales, family level Staphylococcaceae, genus level Staphylococcus, Fournierella, Eubacterium siraeum, CAG-56, Ruminococcus gnavus, Intestinibacter, Actinomyces, and Erysipelotrichaceae UCG-003 (logFC>1 and P<0.05). Veillonella and Megamonas were closely associated with clinical symptoms in patients with TAO. Among the 184 significantly different metabolites, 63 were upregulated, and 121 were downregulated in patients with TAO compared to healthy controls. The biosynthesis of unsaturated fatty acids was the significantly enriched metabolic pathway. Correlation analysis revealed Actinomyces was positively correlated with NAGlySer 15:0/16:0, FAHFA 3:0/20:0, and Lignoceric Acid, while Ruminococcus gnavu was positively correlated with Cer 18:0;2O/16:0; (3OH) and ST 24:1;O4/18:2. Specific intestinal flora and metabolites are closely associated with TAO development. Further investigation into the functional associations between these flora and metabolites will enhance our understanding of TAO pathogenesis.

CASE REPORT: IDIOPATHIC SCLEROSING ORBITAL INFLAMMATION

Idiopathic sclerosing orbital inflammation (ISOI) also known as sclerosing orbital pseudotumor is a rare, idiopathic, chronic, slowly progressive orbital inflammation, characterized by scarring of the tissue that represents a mass effect. The latter leads to bulging of the eye, restriction of ocular movements and visual impairment up to potential loss of vision. It is a subtype of non-specific inflammatory orbital pseudotumor, which both radiologically and clinically mimics a malignant process or other orbital inflammatory disease, and therefore is challenging to diagnose and manage. This case highlights the challenges in diagnosis and management of ISOI.

Composition and diversity analysis of the TCR CDR3 repertoire in patients with idiopathic orbital inflammation using high-throughput sequencing

BackgroundIdiopathic orbital inflammation (IOI) is a nonspecific orbital inflammatory disease with the third highest prevalence among orbital diseases, and its pathogenesis is associated with T-cell-mediated immune responses. This study aimed to investigate the differences in T-cell receptor (TCR) expression between IOI patients and healthy subjects by high-throughput sequencing and to characterize TCR expression in patients with IOI and with respect to glucocorticoid response.MethodsA total of 19 subjects were enrolled in this study and were divided into the idiopathic orbital inflammation group (IOI group, n = 13) and the healthy control group (HC group, n = 6), and within the IOI group were further divided into the glucocorticoid therapy sensitive group (IOI(EF) group, n = 6) and the glucocorticoid therapy ineffective group (IOI(IN) group, n = 7) based on the degree of effectiveness to glucocorticoid therapy. High-throughput TCR sequencing was performed on peripheral blood mononuclear cells of IOI patients and healthy control individuals using 5’ RACE technology combined with Unique Identifier (UID) digital tag correction technology. The TCR CDR3 region diversity, sharing patterns, and differential sequences between the IOI and HC groups, and between the IOI(EF) and IOI(IN) groups were analyzed.ResultsIt was found that the diversity of TCR CDR3 in the IOI group was significantly lower than that in the HC group, and the frequency of V gene use was significantly different between groups. The diversity of TCR CDR3 in patients in the IOI(EF) group was significantly lower than that in patients in the IOI(IN) group, and the frequency of V and J gene use was significantly different between the IOI(EF) group and the IOI(IN) group. Additionally, we found 133 nucleotide sequences shared in all IOI samples and screened two sequences with higher expression from them.ConclusionsOur results suggested that abnormal clonal expansion of specific T-cells exists in IOI patients and that TCR diversity may had an impact on the prognosis of glucocorticoid-treated IOI. This study may contribute to a better understanding of the immune status of IOI and provide new insights for T-cell -associated IOI pathogenesis, diagnosis and treatment prediction.

Recurrent superior orbital fissure syndrome associated with VEXAS syndrome

PurposeTo describe a case of recurrent orbital inflammation and superior orbital fissure syndrome associated with VEXAS (vacuoles, E1 enzyme, X-linked, auto-inflammatory, somatic) syndrome.Case presentationVEXAS syndrome is a recently identified multi-system inflammatory disease of late adult onset. The authors describe the case of a 76-year-old man who presented with recurrent episodes of orbital inflammation, with superior orbital fissure syndrome, dacryoadenitis and orbital myositis. He had a constellation of systemic disorders including recurrent chest infections, congestive cardiac failure, pulmonary emboli and skin rashes. The underlying diagnosis of VEXAS syndrome was confirmed by genetic testing, which revealed the UBA1 mutation.ConclusionVEXAS syndrome should be considered in the differential diagnosis of orbital inflammatory disease associated with multi-system inflammatory disorders.

Apical idiopathic orbital inflammatory disease presenting with disc edema - A case report

Idiopathic orbital inflammatory disease (IOID) is an inflammatory pathology of the orbital contents like muscles, lacrimal glands, and periorbital fat. This report presents a case of a 24-year-old female with axial proptosis, defective vision, and lid edema in the left eye. Her best-corrected visual acuity (BCVA) was 6/36 N8 with grade 1 relative afferent pupillary defect (RAPD) and impaired color vision in the left eye. Magnetic resonance imaging (MRI) of the left orbit showed infiltration of the retro-orbital fat with thickening of the optic nerve sheath. She was diagnosed with apical IOID with optic perineuritis and was treated successfully with intravenous and oral steroids.

Inflammation and Fibrosis in Orbital Inflammatory Disease: A Histopathologic Analysis.

The purpose of this study was to compare the histopathologic inflammation and fibrosis of orbital adipose tissue in orbital inflammatory disease (OID) specimens. In this retrospective cohort study, inflammation, and fibrosis in orbital adipose tissue from patients with thyroid-associated orbitopathy (TAO), granulomatosis with polyangiitis (GPA), sarcoidosis, nonspecific orbital inflammation (NSOI), and healthy controls were scored by 2 masked ocular pathologists. Both categories were scored on a scale of 0 to 3 with scoring criteria based on the percentage of specimens containing inflammation or fibrosis, respectively. Tissue specimens were collected from oculoplastic surgeons at 8 international centers representing 4 countries. Seventy-four specimens were included: 25 with TAO, 6 with orbital GPA, 7 with orbital sarcoidosis, 24 with NSOI, and 12 healthy controls. The mean inflammation and fibrosis scores for healthy controls were 0.0 and 1.1, respectively. Orbital inflammatory disease groups' inflammation (I) and fibrosis (F) scores, formatted [I, F] with respective p -values when compared to controls, were: TAO [0.2, 1.4] ( p = 1, 1), GPA [1.9, 2.6] ( p = 0.003, 0.009), sarcoidosis [2.4, 1.9] ( p = 0.001, 0.023), and NSOI [1.3, 1.8] ( p ≤ 0.001, 0.018). Sarcoidosis had the highest mean inflammation score. The pairwise analysis demonstrated that sarcoidosis had a significantly higher mean inflammation score than NSOI ( p = 0.036) and TAO ( p < 0.0001), but no difference when compared to GPA. GPA had the highest mean fibrosis score, with pairwise analysis demonstrating a significantly higher mean fibrosis score than TAO ( p = 0.048). Mean inflammation and fibrosis scores in TAO orbital adipose tissue samples did not differ from healthy controls. In contrast, the more "intense" inflammatory diseases such as GPA, sarcoidosis, and NSOI did demonstrate higher histopathologic inflammation and fibrosis. This has implications in prognosis, therapeutic selection, and response monitoring in orbital inflammatory disease.

Unilateral Idiopathic Optic Perineuritis in a Normal Fundus

A 22-year-old woman of Malay ethnicity presented with severe blurred vision in the right eye and experienced periocular pain with eye movement. She had a visual field defect over the affected eye, while an examination showed clinically normal optic discs. Relative afferent pupillary defect was also present in the right eye, with reduced optic nerve function. Optic nerve imaging revealed the ‘doughnut sign’ and ‘tram track’ signs, which are characteristics of optic perineuritis. The patient was initiated on a steroid regime that was tapered off over four months. Her visual function recovered gradually during the treatment until reaching a satisfactory final visual outcome. This is a unique case where optic perineuritis occurred in a normal fundus and magnetic resonance imaging (MRI) served as an important diagnostic tool for the condition.&#x0D; Optic perineuritis (OPN) is an orbital inflammatory disease that involves the meningeal sheath surrounding the optic nerve.[1] It can be idiopathic or secondary to an underlying systemic autoimmune disorder.[1,2] Visual loss in OPN results from inflammation of the tissue surrounding the optic nerve, resulting in mechanical compression of the nerve and subsequent ischaemia.[1]&#x0D; OPN can be diagnosed using an optic nerve sheath biopsy, while magnetic resonance imaging (MRI) diagnoses OPN through perineural enhancement, making it a useful diagnostic tool.[2] Upon diagnosis, OPN should be treated with corticosteroids for a prolonged period to ensure visual recovery.[3]&#x0D; We report a unique case of unilateral OPN with severe visual loss in a young Malay woman with a normal fundoscopy who was successfully treated with prolonged corticosteroids.

A Rare Case of Bilateral Optic Perineuritis, Scleritis and Uveitis in a Pediatric Patient Following Influenza Vaccination (P1-9.007)

<h3>Objective:</h3> To present a rare case of bilateral optic perineuritis, scleritis and uveitis in a pediatric patient following influenza vaccination. <h3>Background:</h3> Optic perineuritis is a rare orbital inflammatory disease that typically presents with unilateral symptoms, but may be bilateral and is rare in children. Bilateral uveitis and scleritis are uncommon in children. Rarely, perineuritis, sceritis and uveitis have been reported following influenza vaccination. To our best knowledge, optic perineuritis, scleritis and uveitis occurring simultaneously following vaccination has not been reported. A seven-year-old female patient presented with two months of bilateral eye pain, vision loss, extreme photophobia and sleepiness that started two weeks following influenza vaccination. At presentation she had decreased visual acuity (VA) bilaterally (right, 20/100; left, 20/80), bilateral scleral erythema, and bilateral optic disc edema. Magnetic resonance imaging (MRI) of the brain showed bilateral scleritis and optic perineuritis (left greater than right), with classic tram-track sign on axial view and donut sign on coronal view of the optic nerves. No other brain or cervical/thoracic lesions were seen. Serum antibodies to aquaporin 4, myelin oligodendrocyte glycoprotein, rheumatological work up and infectious studies were unremarkable. C-reactive protein and erythrocyte sedimentation rate were elevated. Routine cerebrospinal fluids findings were normal. The patient was treated with 2 g/kg of intravenous immunoglobulins (IVIG) followed by 30 mg/kg methylprednisolone intravenously (given insufficient response to IVIG), and oral steroid taper that is still ongoing (current dose: 12.5 mg/day). Methotrexate was started due to uveitis (25 mg once a week). Follow-up MRI of the brain one month after treatment initiation showed a significantly improved bilateral optic per neural enhancement and scleritis. Current VA is 20/30 bilaterally. <h3>Design/Methods:</h3> NA <h3>Results:</h3> NA <h3>Conclusions:</h3> Bilateral optic perineuritis, scleritis and uveitis can occur in a single pediatric patient and can potentially represent a post-vaccinal phenomenon. Timely recognition and treatment is important for visual outcomes. <b>Disclosure:</b> Dr. Wooten has nothing to disclose. The institution of Dr. Dujmovic Basuroski has received research support from Alexion Pharmaceuticals, Inc. The institution of Dr. Dujmovic Basuroski has received research support from Biogen MA Inc. The institution of Dr. Dujmovic Basuroski has received research support from EMD Serono Research and Development Institute, Inc . The institution of Dr. Dujmovic Basuroski has received research support from Celgene Corporation/Bristol-Myers Squibb. The institution of Dr. Dujmovic Basuroski has received research support from The Bodford Family Transverse Myelitis Center Fund. Dr. Sarkissian has nothing to disclose. Dr. Kostic has nothing to disclose. Dr. Javed has nothing to disclose. Jordan Broman-Fulks has nothing to disclose.

Metagenomic Deep Sequencing for Orbital Inflammatory Disease

ABSTRACT Purpose Orbital inflammatory disease (OID) is a heterogeneous group of immunologic disorders whose etiology is often non-specific despite routine investigation. In this proof-of-concept study, metagenomic deep sequencing (MDS) is applied to examine host gene expression in two subtypes of OID. Methods Prospectively collected lacrimal gland tissue from patients with OID was processed for MDS. Differential gene expression analysis was performed to evaluate for host transcriptome signatures. Proof-of-concept comparison was made between histologically confirmed samples of idiopathic dacryoadenitis and IgG4-related disease (IgG4-RD). Results Twelve genes were identified to be differentially expressed between idiopathic dacryoadenitis and IgG4-RD. Differences in innate humoral immunity gene expression were observed. Several additional genes of interests were also found to be upregulated in idiopathic dacryoadenitis. Conclusions A unique transcriptome signature was found when comparing idiopathic dacryoadenitis to IgG4-RD. This suggests that MDS can identify differentially expressed genes in OID. Such insight could potentially provide a better understanding of host gene expression and the inflammatory pathways involved in OID.

Identification and validation of ferroptosis-related genes and immune cell infiltration in thyroid associated ophthalmopathy.

Thyroid associated ophthalmopathy (TAO) is an orbital autoimmune inflammatory disease that is commonly associated with thyroid dysfunction. Although the etiology of TAO is unclear, ROS accumulation and oxidative stress have been closely linked to the pathogenesis of TAO. Ferroptosis is an iron-dependent programmed cell death characterized by intracellular labile iron levels, excessive accumulation of reactive oxygen species (ROS) and lipid peroxidation. Currently, there are few reports regarding the role of ferroptosis in TAO. This article aimed to identify ferroptosis-related genes (FRGs) with diagnostic and therapeutic potential in TAO and explore their relationship with immune cells and lncRNAs. GSE58331 was downloaded from Gene Expression Omnibus (GEO) database. A total of 162 DEGs were identified between 27 TAO samples and 22 health samples from GSE58331, among which six FRGs (CYBB, CTSB, SLC38A1, TLR4, PEX3, and ABCC1) were obtained. The AUC of SLC38A1, TLR4, PEX3 in lacrimal gland tissues was greater than 80 which suggested high diagnostic value in TAO. The result of immune cell infiltrate analysis indicated increased infiltration of monocytes (p < 0.001), macrophages M0(p = 0.039), mast cells activated (p = 0.008), and neutrophils (p = 0.045) in orbital tissues from TAO patients. Meanwhile, mast cells resting (p = 0.043) and macrophages M2 (p = 0.02) showed reduced infiltration in TAO samples. There were no gender differences in immune cell infiltration in the TAO patients. Two differentially expressed lncRNAs, LINC01140 and ZFHX4-AS1, in TAO groups were identified as ferroptosis-related lncRNAs. CYBB-LINC01140-TLR4, CYBB- LINC01140- SLC38A1, TLR4- LINC01140- SLC38A1, and CTSB- ZFHX4-AS1- CYBB may be potential RNA regulatory pathways in TAO. Targeted drugs and transcription factors for differential expressed FRGs were also screened out in our study. In vitro, experiments revealed that CTSB, PEX3, ABCC1 and ZFHX4-AS1(lncRNA) were differentially expressed in orbital fibroblasts (OFs) between TAO groups and healthy controls at the transcriptional level.

Neuro-ophthalmic complications of immune checkpoint inhibitor therapy: Current status and future directions.

Since 2011, use of immune checkpoint inhibitors (ICI) in cancer immunotherapy dramatically expanded, both alone and in combination with either a different cancer treatment or with two different ICIs. With this increase in use have come a myriad of adverse effects from enhanced immune activation, including ophthalmic and neurologic immune related adverse events (irAE). Neuro-ophthalmic immune related adverse events (NOirAE) associated with use of ICIs are increasingly recognized and their severity may actually limit use of potentially life-saving immunotherapy. NOirAEs comprise a wide variety of presentations involving both the central and peripheral nervous system. They cause afferent or efferent visual dysfunction, including among them optic neuropathy and edema, orbital inflammatory disease, and ocular myasthenia. While treatment for irAEs typically involves immunosuppression with corticosteroids, there is no expert consensus regarding best practices for treatment of NOirAEs and whether to stop ICI immunotherapy for the cancer or not. This state-of-the-art review explores the pathophysiologic basis for NOirAEs, provides a framework for categorizing them within neuro-ophthalmology, and discusses what is needed to close the current knowledge gaps in diagnosis and management of an increasing population of cancer patients requiring neuro-ophthalmic care.

Non specific orbital inflammatory disease – A review article

Non Specific Orbital Inflammatory Disease (NSOID) is a benign, noninfective inflammatory condition of the orbit which does not have any identifiable local or systemic causes. Orbital pseudotumor was first described in 1903 by Gleason. The clinical diagnosis is one of exclusion with evaluation directed to exclude neoplasms, infections and systemic disorders. IOI is diagnosed by clinical history and evaluation to rule out other causes of orbital disease. Orbital pseudotumor is the third most common orbital disease following Graves ophthalmopathy and lymphoproliferative disease.